Hepatitis E
Hepatitis E is an acute, typically self-limiting viral liver infection caused by the hepatitis E virus (HEV), transmitted primarily via the fecal-oral route through contaminated water, with particular severity in pregnant women.
Hepatitis E
Hepatitis E is an acute viral hepatitis caused by the Hepatitis E virus (HEV), a non-enveloped, single-stranded RNA virus belonging to the family Hepeviridae [1][2]. The name breaks down as: "hepat-" (Greek: liver) + "-itis" (inflammation) + "E" (the fifth hepatitis virus discovered, after A–D).
Think of it this way: Hepatitis E is the "other" enteral (gut-transmitted) hepatitis alongside Hepatitis A. While HAV is classically the "shellfish" virus, HEV is the "pork liver congee" virus [3]. Both share fecal-oral transmission, but HEV has unique twists — zoonotic reservoirs, risk of chronicity in immunosuppressed patients, and devastating fulminant hepatitis in pregnant women.
Key Conceptual Distinction from HAV
Both HAV and HEV are enteric hepatitis viruses transmitted fecal-orally. However, HEV differs in several critical ways: (1) it has animal reservoirs (zoonotic), (2) it can cause chronic infection in immunosuppressed patients, (3) it carries a much higher mortality in pregnant women (up to 20–25%), and (4) natural infection does NOT confer reliable lifelong protection against reinfection [3][4].
2. Epidemiology
- Approximately 20 million new HEV infections per year worldwide, with > 55,000 deaths [4].
- Increased prevalence in developing countries — India, Burma (Myanmar), Nepal, Pakistan, China, Central Russia, Mexico [4].
- HEV is the most common cause of acute viral hepatitis globally when all genotypes are considered.
- HK seroprevalence: ~33.6% with an increasing trend (similar trend to hepatitis A seroprevalence) [4].
- HEV is a notifiable disease in Hong Kong [2].
- Demographics: usually occurs in adults in the 3rd decade of life [4].
- Important local risk: consumption of pork liver, pork blood, and undercooked pork products — a culturally relevant exposure in Hong Kong (e.g., pig liver congee / 豬潤粥) [3][4].
- Rat hepatitis E virus (rat-HEV) has been identified as a cause of human hepatitis in Hong Kong — a finding that gained international attention. Cases of chronic HEV caused by rat-HEV in immunosuppressed patients have been documented [1].
| Genotype | Geographic Distribution | Transmission | Key Features |
|---|---|---|---|
| 1 | Asia, Africa, Mexico | Waterborne (human-to-human) | Commonest in endemic outbreaks; highest risk in pregnant women |
| 2 | Mexico, West Africa | Waterborne | Less well-characterized |
| 3 | Worldwide (Western countries + Asia) | Zoonotic (pigs, shellfish) | Can cause chronic HEV in immunosuppressed; autochthonous cases in developed countries |
| 4 | China, Japan, Southeast Asia | Zoonotic (pigs) | Important in HK/China; similar to genotype 3 |
High Yield – Genotype & Clinical Correlation
Genotypes 1 and 2: waterborne, human-to-human, endemic in resource-limited countries with poor sanitation [4].
Genotypes 3 and 4: zoonotic, found in both Western countries and Asia, transmitted from animals (especially pigs and filter-feeder shellfish) to humans [4].
Consumption of pork liver or blood is an important cause in HK [4].
Fulminant hepatitis in pregnancy is usually Genotype 1 [2].
Chronicity in transplant recipients is typically Genotype 3 [4].
3. Risk Factors
- Contact with contaminated food and water [5]
- Consumption of raw or undercooked meat or meat products derived from infected animals — especially pork liver, pork intestines, wild boar, deer [5]
- Consumption of raw or undercooked shellfish (filter-feeding bivalves concentrate the virus) [5]
- Blood transfusion — HEV can be transmitted parenterally (underappreciated route) [5]
- Poor sanitation — large waterborne outbreaks in endemic regions [5]
- Occupational exposure: pig farmers, slaughterhouse workers, veterinarians
- Pregnancy (especially 3rd trimester) — 20–25% maternal mortality [1]
- Patients with major underlying illness or chronic liver disease can develop severe liver consequences [1]
- Immunosuppression (organ transplant recipients, HIV, hematological malignancies on chemotherapy) — risk of chronicity
- Pre-existing chronic liver disease (especially chronic HBV — relevant in HK where ~8% population are HBV carriers) — superinfection with HEV can precipitate decompensation and acute-on-chronic liver failure
Why does pre-existing liver disease matter? The liver already has reduced functional reserve. When HEV causes additional hepatocyte necrosis on top of an already fibrotic/cirrhotic liver, the remaining hepatocytes cannot compensate → acute-on-chronic liver failure. This is particularly important in Hong Kong where chronic HBV is prevalent [6].
4. Anatomy and Function (Relevant Hepatic Anatomy)
Understanding the clinical features of HEV requires knowledge of hepatic microanatomy:
- Hepatocytes are arranged in plates radiating from the central vein, separated by hepatic sinusoids.
- Sinusoids are lined by fenestrated endothelial cells and contain Kupffer cells (resident macrophages).
- Bile canaliculi run between adjacent hepatocytes, draining bile into the canals of Hering → interlobular bile ducts → hepatic ducts → common bile duct.
- Glisson's capsule: the fibrous capsule surrounding the liver. It is innervated by the phrenic nerve and lower intercostal nerves — stretching of this capsule produces the dull RUQ ache characteristic of hepatitis.
| Function | Relevance to HEV |
|---|---|
| Bilirubin metabolism | Hepatocyte injury → impaired conjugation and excretion of bilirubin → jaundice, dark urine, pale stools |
| Bile acid synthesis & excretion | Intrahepatic cholestasis from hepatocyte swelling → pruritus |
| Albumin synthesis | Reduced in severe/prolonged disease → edema |
| Clotting factor synthesis | Impaired synthesis of Factors II, VII, IX, X → coagulopathy (INR is the best early marker because Factor VII has the shortest half-life ~6 hours) |
| Drug/toxin metabolism | Impaired clearance → drug accumulation |
| Gluconeogenesis | Massive hepatocyte loss → hypoglycemia (important in fulminant hepatitis) |
| Kupffer cell function | Immune surveillance and endotoxin clearance — damaged Kupffer cells allow systemic endotoxemia |
HEV causes Kupffer cell damage → allows endotoxin damage to the liver. Pregnant women may be more sensitive to endotoxin effects [4]. This is one proposed mechanism for the disproportionately severe disease in pregnancy.
5. Etiology (Focus on Hong Kong)
| Feature | Detail |
|---|---|
| Family | Hepeviridae |
| Genus | Orthohepevirus |
| Genome | Single-stranded, positive-sense RNA (ssRNA) [2] |
| Envelope | Non-enveloped (in feces; quasi-enveloped in blood) |
| Incubation period | 2–10 weeks; mean 5–6 weeks [1] (or 3–8 weeks per other sources [2]) |
- The word "Hepeviridae" → "Hep-E" = hepatitis E virus family — the family was literally named after this virus.
- Non-enveloped viruses are generally more resistant to environmental degradation (bile salts, stomach acid, detergents) → this explains why it survives the fecal-oral route through the GI tract.
- The quasi-envelope in blood (membrane-wrapped virions in circulation) may help evade neutralizing antibodies during the viremic phase.
In Hong Kong, the relevant etiological considerations are:
- Genotype 4 — commonest genotype causing autochthonous (locally acquired) HEV in HK and Southern China
- Genotype 3 — also present, particularly in imported cases
- Rat HEV (Orthohepevirus C) — Hong Kong was the first place in the world to document human infection with rat hepatitis E virus (2018). This is a distinct species that was previously thought unable to infect humans. Cases have since been identified in immunosuppressed patients [1].
Important local food sources of HEV in HK:
- Pork liver congee (豬潤粥) — HEV is concentrated in pig liver
- Pork blood products
- Raw/undercooked pork
- Raw shellfish (oysters, clams)
- Pig offal / viscera
HEV = "pork liver congee" in one word, as emphasized in the GC lecture [3]. This is the exam-friendly association for HK-based questions.
- Domestic pigs — primary reservoir worldwide and in HK
- Wild boar, deer — important in Japan, Europe
- Rats — Hong Kong rat-HEV cases
- Camels — genotype 7 (Middle East)
- Rabbits — genotype 3
6. Pathophysiology
- Entry: HEV is ingested → survives gastric acid (non-enveloped, acid-resistant) → crosses intestinal mucosa → enters portal circulation.
- Hepatotropism: HEV has tropism for hepatocytes. It enters hepatocytes, replicates in the cytoplasm using its RNA-dependent RNA polymerase.
- Shedding: Progeny virions are released into bile canaliculi → excreted in feces (this is why stool HEV RNA can be detected). They also enter the bloodstream (viremia).
- Immune-mediated injury: The liver damage in HEV is primarily immune-mediated, not due to direct cytopathic effect of the virus. CD8+ cytotoxic T lymphocytes recognize viral antigens on infected hepatocytes and kill them → hepatocyte necrosis → release of intracellular enzymes (ALT, AST) and bilirubin metabolism dysfunction.
This is a classic exam question. Several mechanisms have been proposed:
- Kupffer cell damage: HEV damages Kupffer cells → impaired clearance of gut-derived endotoxins (lipopolysaccharide) from the portal blood → endotoxin-mediated hepatic injury [4]
- Pregnant women may be more sensitive to endotoxin effects [4] — possibly because:
- Pregnancy is an immunomodulated state (Th2-dominant) → reduced cell-mediated immunity → reduced viral clearance → higher viral load → more liver damage
- Higher circulating estrogen and progesterone levels may modulate immune responses
- Placental factors may amplify inflammatory cascades
- Hormonal effects on HEV replication: Some evidence suggests that steroid hormones directly enhance HEV replication in hepatocytes.
- Reduced hepatic blood flow in late pregnancy (due to IVC compression by gravid uterus) may worsen ischemic injury to already-damaged hepatocytes.
High Yield – Pregnancy and HEV
Pregnant women with hepatitis E are at increased risk of acute liver failure and fetal loss. 20–25% maternal mortality if acute HEV during the 3rd trimester [1]. This is usually Genotype 1 [2]. The mechanism involves Kupffer cell damage allowing endotoxin-mediated liver injury, with pregnant women being more sensitive to endotoxin effects [4]. This is one of the most important clinical pearls for HEV.
- In immunocompetent individuals: robust T-cell response → viral clearance → self-limited infection.
- In immunosuppressed patients (especially solid organ transplant recipients on calcineurin inhibitors like tacrolimus/cyclosporine):
- Impaired T-cell function → inability to clear the virus → persistent viral replication → chronic HEV (defined as HEV RNA detectable for > 3 months)
- NEJM (2008) reported 14 cases of acute HEV in transplant patients with 8 becoming chronic with elevated ALT and positive HEV RNA [4]
- Typically occurs with HEV genotype 3 infection [4]
- Risk of chronicity limited to transplant recipients and immunosuppressed state (e.g., HIV) [4]
- Chronic HEV can progress to cirrhosis within months to years if untreated.
This is fundamentally different from HAV, which NEVER causes chronicity. The reason is that HAV induces a very strong innate and adaptive immune response even in immunosuppressed individuals, whereas HEV's immune evasion strategies (quasi-envelope in blood, ORF3 protein that inhibits interferon signaling) can allow persistence when adaptive immunity is weakened.
- ~20% of HEV cases present with prominent cholestasis (cf < 5% in hepatitis A) [4]
- Mechanism: hepatocyte swelling from inflammation → compression of intracellular bile canaliculi → intrahepatic cholestasis → bile acid accumulation in blood → pruritus; reduced bile reaching the gut → pale stools; conjugated bilirubin refluxes into blood → filtered by kidneys → dark urine
- HEV may also directly affect bile duct epithelial cells (cholangiocytes), contributing to the cholestatic pattern
7. Classification
| Genotype | Reservoir | Transmission | Geographic Distribution | Clinical Significance |
|---|---|---|---|---|
| 1 | Humans only | Waterborne | Asia, Africa | Epidemics; pregnancy risk |
| 2 | Humans only | Waterborne | Mexico, W. Africa | Less studied |
| 3 | Pigs, deer, rabbits | Zoonotic | Worldwide | Chronic HEV in immunosuppressed |
| 4 | Pigs | Zoonotic | China, Japan, SE Asia | Important in HK |
| 7 | Camels | Zoonotic | Middle East | Rare; reported in transplant recipients |
| Rat-HEV | Rats | Zoonotic | HK (first reported) | Emerging pathogen |
| Category | Features |
|---|---|
| Acute self-limited HEV | > 95% of cases; resolves spontaneously |
| Acute HEV with cholestatic hepatitis | Prominent cholestasis (~20%); prolonged jaundice |
| Fulminant HEV | Acute liver failure; especially in pregnancy and pre-existing CLD |
| Chronic HEV | HEV RNA positive > 3 months; only in immunosuppressed; risk of rapid cirrhosis |
| Extrahepatic HEV | Neurological, renal, hematological, pancreatic manifestations |
| Feature | HAV | HEV |
|---|---|---|
| Family | Picornaviridae | Hepeviridae |
| Genome | ssRNA | ssRNA |
| Envelope | Non-enveloped | Non-enveloped |
| Incubation | 2–4 weeks | 2–10 weeks (mean 5–6 weeks) [1] |
| Transmission | Fecal-oral | Fecal-oral (genotypes 1 & 2); Zoonotic (genotypes 3 & 4) [2] |
| One-word association | Shellfish | Pork liver congee [3] |
| Chronic infection | Never | Yes — in immunosuppressed (transplant, HIV) [1][4] |
| Fulminant hepatitis | Rare | Rare except in pregnant women (25% mortality) [2] |
| Cholestatic pattern | < 5% | ~20% [4] |
| Lifelong immunity | Yes | No permanent protection against reinfection [4] |
| Higher mortality | 0.2% | 1–2% for normal patients [4] |
| Vaccine available | Yes | No (China has a vaccine but not WHO-prequalified; not available in HK) |
| Serological diagnosis | Anti-HAV IgM | Anti-HEV IgM ± Stool HEV RNA RT-PCR [2] |
| Passive immunoprophylaxis | Immunoglobulin | No [2] |
Exam Comparison: HAV vs HEV
This comparison table is extremely high-yield. The key differentiators to remember: HEV has (1) zoonotic transmission, (2) longer incubation, (3) risk of chronicity in immunosuppressed, (4) devastating in pregnancy, (5) more cholestatic, (6) higher baseline mortality, and (7) no reliable lifelong immunity. Everything else is largely similar.
8. Clinical Features
8.2 Symptoms (with Pathophysiological Basis)
The clinical course follows the classic pattern of acute viral hepatitis, divided into three phases [3]:
| Symptom | Pathophysiological Basis |
|---|---|
| Low-grade fever (usually < 39°C) [3] | Cytokine release (IL-1, IL-6, TNF-α) from immune activation against HEV-infected hepatocytes → hypothalamic thermoregulatory set-point elevation |
| Severe loss of appetite (anorexia) [1][3] | Cytokine-mediated effect on hypothalamic appetite centers; TNF-α (also called "cachexin") directly suppresses appetite |
| Severe fatigue, myalgia [3] | Systemic inflammatory response; circulating cytokines cause muscle catabolism and central fatigue |
| Nausea and vomiting [1] | Hepatic inflammation → stimulation of vagal afferents from liver capsule and porta hepatis; cytokine-mediated effects on the chemoreceptor trigger zone (area postrema) |
| Diarrhea [1][3] | More common in enteric hepatitis (HAV/HEV) than blood-borne hepatitis. Diarrhea would be more common for HEV as emphasized in the lecture [3]. Mechanism: viral replication in intestinal epithelium; bile salt malabsorption due to cholestasis |
| Right upper quadrant dull ache [3] | Liver capsule (Glisson's capsule) distension due to hepatocyte swelling and inflammatory edema. The capsule is innervated but the parenchyma is not — so pain only occurs when the capsule is stretched |
| Darkening of urine (tea-colored) [3] | Conjugated bilirubin (water-soluble) spills into blood due to hepatocyte dysfunction → filtered by kidneys → excreted in urine. Also, inefficient hepatic clearance of reabsorbed urobilinogen → increased urinary excretion of urobilinogen [5] |
| Transient pruritus [3] | Mimics obstructive jaundice, due to hepatocyte swelling [3] compressing intrahepatic bile canaliculi → intrahepatic cholestasis → bile acid accumulation in blood → bile acids deposited in skin activate itch receptors (pruritoceptors) and neuronal pathways via autotaxin-lysophosphatidic acid |
HEV has a notably higher proportion with prominent cholestasis (~20%) compared to HAV (< 5%) [4]. This means pruritus, pale stools, and dark urine are more commonly prominent features in HEV.
| Symptom | Pathophysiological Basis |
|---|---|
| Jaundice [1][3] | Hepatocyte necrosis + intrahepatic cholestasis → impaired bilirubin conjugation and excretion → accumulation of both unconjugated and conjugated bilirubin in blood → yellow discoloration of sclera (first noticeable because scleral elastin has high affinity for bilirubin) and skin |
| Pale/clay-colored stools [5] | Intrahepatic cholestasis → reduced bilirubin reaching gut → reduced conversion to stercobilinogen/stercobilin (which normally gives stool its brown color) |
| All pre-icteric symptoms begin to subside [3] | As the adaptive immune response matures and begins clearing infected hepatocytes more efficiently, the systemic cytokine storm attenuates. Paradoxically, jaundice appears as liver cell damage peaks, but the worst systemic symptoms often improve |
- Gradual resolution of jaundice and return of appetite
- Liver enzymes gradually normalize
- Full recovery in most immunocompetent patients
- Some patients may have prolonged cholestasis lasting weeks to months (especially with HEV genotype 3/4)
| Sign | Pathophysiological Basis |
|---|---|
| Jaundice (scleral icterus → skin) | As above; visible when serum bilirubin > 34 µmol/L (~2 mg/dL) |
| Hepatomegaly (tender) | Hepatocyte swelling + inflammatory infiltrate + congestion → liver enlargement. Tender because Glisson's capsule is stretched |
| Hepatic tenderness on palpation | Capsular distension — percuss for tenderness if hepatomegaly is subtle |
| Scratch marks (excoriations) | Secondary to cholestasis-induced pruritus; evidence of bile acid deposition in skin |
| Splenomegaly (mild, in ~15%) | Reticuloendothelial hyperplasia in response to viral infection; portal hypertension if underlying CLD |
| Cervical lymphadenopathy (mild) | Reactive lymphadenopathy from immune activation |
| Skin rash (uncommon) | Immune complex deposition; can occur as part of extrahepatic manifestations |
| Spider naevi, palmar erythema, etc. | NOT typical of acute HEV alone; if present, suggests underlying chronic liver disease |
Clinical Pearl – Acute HEV on Background CLD
In a Hong Kong patient with known chronic HBV who presents with acute hepatitis, always consider HEV superinfection as a cause of the flare. Superinfection with other viral agents, especially HAV and HEV, is an important cause of hepatitis flare-up in chronic HBV carriers. HEV is more common now [6]. The combination of chronic HBV + acute HEV can be devastating and may precipitate acute-on-chronic liver failure.
HEV has a wider range of extrahepatic manifestations than HAV. These are important and increasingly recognized [5]:
| System | Manifestation | Mechanism |
|---|---|---|
| Neurological | Meningoencephalitis, aseptic meningitis, transverse myelitis, cranial nerve palsy, peripheral neuropathy, Guillain-Barré syndrome [5] | Direct viral neurotropism (HEV RNA found in CSF) and/or immune-mediated (molecular mimicry, autoantibodies) |
| Renal | Glomerulonephritis (membranoproliferative or membranous) [5] | Immune complex deposition in glomerular basement membrane |
| Hematological | Thrombocytopenia, hemolysis, aplastic anemia [5] | Immune-mediated destruction; bone marrow suppression; direct viral effect on progenitor cells |
| Pancreatic | Acute pancreatitis [5] | Possibly direct viral invasion of pancreatic tissue or immune-mediated |
| Thyroid | Acute thyroiditis [5] | Immune-mediated |
| Musculoskeletal | Arthritis and arthralgia [5] | Immune complex deposition in synovium |
| Dermatological | Skin rash [5] | Immune complex–mediated vasculitis |
Neurological manifestations are the most important extrahepatic feature of HEV — particularly Guillain-Barré syndrome and neuralgic amyotrophy (Parsonage-Turner syndrome). In Europe, HEV genotype 3 has been identified as the most common infectious trigger for neuralgic amyotrophy.
9. Special Populations
- 20–25% maternal mortality if acute HEV during the 3rd trimester [1]
- Usually Genotype 1 [2]
- Mechanism: Kupffer cell damage → endotoxemia + immunomodulation of pregnancy (Th2 shift) → impaired viral clearance → massive hepatocyte necrosis
- Risk of DIC, fetal loss, premature delivery, stillbirth
- This is one of the few infections where pregnancy dramatically alters mortality
- Patients with major underlying illness or chronic liver disease can develop severe liver consequences [1]
- In HK context: chronic HBV carrier + HEV superinfection → high risk of decompensation and acute-on-chronic liver failure
- Solid organ transplant recipients (especially liver, kidney)
- HIV patients with low CD4 counts
- Patients on anti-CD20 therapy (rituximab), other immunosuppressants
- Cases of chronic HEV (caused by rat-HEV) in immunosuppressed patients [1]
- Can progress rapidly to cirrhosis if not recognized and treated
High Yield Summary
Definition: HEV is a non-enveloped ssRNA virus (family Hepeviridae) causing acute viral hepatitis, transmitted fecal-orally and zoonotically.
Epidemiology: 20M cases/year globally; HK seroprevalence ~33.6%; genotypes 3 and 4 predominate locally (zoonotic from pigs).
Key Risk Factors: Raw/undercooked pork liver (HK classic), contaminated water, shellfish; pregnancy (3rd trimester), immunosuppression, pre-existing CLD.
Pathophysiology: Immune-mediated hepatocyte injury (not direct cytopathic); Kupffer cell damage → endotoxin-mediated injury (explains pregnancy severity); impaired T-cell clearance in immunosuppressed → chronicity.
Clinical Features: Pre-icteric (fever, anorexia, fatigue, diarrhea, RUQ ache, dark urine, pruritus) → Icteric (jaundice, pale stools) → Convalescent. Notable for prominent cholestasis (~20%), extrahepatic manifestations (neuro, renal, hematological).
Critical Differentiators from HAV: Zoonotic reservoir, longer incubation, NO lifelong immunity, CAN cause chronic infection (immunosuppressed), HIGHER mortality (1–2% baseline, 20–25% in pregnancy), MORE cholestatic.
Hong Kong Pearls: "Pork liver congee virus"; rat-HEV first reported in HK; superinfection on chronic HBV is important; all viral hepatitis are notifiable.
Active Recall - Hepatitis E (Definition, Epidemiology, Etiology, Pathophysiology, Clinical Features)
[1] Lecture slides: GC 239. Viral hepatitis HAV_HBV_HCV_HEV.pdf (HEV section) [2] Senior notes: Maksim Medicine Notes.pdf (Viral hepatitis overview table, p.141) [3] Senior notes: Block A - Jaundice after raw oysters_ acute hepatitis.pdf (p.1, clinical features of enteric hepatitis) [4] Senior notes: Ryan Ho GI.pdf (p.235, HEV section) [5] Senior notes: MBBS Final MB (Medicine) (Felix PY Lai).pdf (p.766–768, HEV clinical manifestation) [6] Senior notes: Block A - I am a hepatitis B carrier.pdf (p.28, HEV superinfection on chronic HBV)
Differential Diagnosis of Hepatitis E
When a patient presents with features suggestive of acute hepatitis — fever, malaise, anorexia, jaundice, RUQ discomfort, deranged LFT with predominant ALT/AST elevation — HEV is just one possibility. The differential diagnosis must be systematically constructed because the clinical presentation of acute hepatitis is largely indistinguishable across etiologies. As the GC lecture slides emphasize: determining the final diagnosis requires history (drug history), clinical presentation, other investigations, and liver biopsy may be required [7].
The key principle: you cannot tell "acute vs acute-on-chronic hepatitis" or distinguish the causative virus by symptoms and signs alone [3]. The clinical presentation of all viral hepatitides overlaps enormously. Diagnosis rests on serological and virological testing.
The differential diagnosis framework for a patient presenting with features of acute hepatitis (which HEV may mimic or be mimicked by) can be organized by etiology:
1. Other Viral Hepatitis (Most Important DDx Category)
This is the most critical category because you cannot clinically distinguish one viral hepatitis from another — serological testing is mandatory.
| Feature | How to Differentiate from HEV |
|---|---|
| Transmission | Fecal-oral (same as HEV genotypes 1/2), classically shellfish [3] |
| Incubation | 2–4 weeks (shorter than HEV's 2–10 weeks) [2] |
| Cholestasis | Less prominent (< 5% vs ~20% in HEV) [4] |
| Pregnancy risk | Low (cf. HEV: 20–25% mortality in 3rd trimester) |
| Chronicity | Never [2] |
| Serology | Anti-HAV IgM [2] — distinct from anti-HEV IgM |
| Diarrhea | Less prominent than HEV [3] |
Why does HAV have a shorter incubation? HAV replicates more rapidly and reaches peak viral shedding faster. The immune response mounts earlier, so clinical illness appears sooner.
This is a critical distinction in Hong Kong, where chronic HBV affects ~8% of the population [6][8].
- Acute HBV: Incubation 4–20 weeks [2]. Can present identically to HEV with fever, jaundice, and markedly elevated ALT.
- Acute flare of chronic HBV: A patient with known chronic HBV carrier status may present with acute hepatitis. This can be caused by multiple triggers including superinfection with HEV [6]. The clinical picture is identical.
How to differentiate:
- HBsAg positive + anti-HBc IgM positive may indicate either acute HBV infection or acute flare of chronic HBV [8]
- HBsAg clearing in 6 months indicates acute infection with clearance [8]
- Anti-HEV IgM will be positive if HEV is the superinfecting agent
- In HK, always consider dual pathology: a chronic HBV carrier presenting with acute hepatitis may have HEV superinfection on top [6]
High Yield – HBV Flare vs HEV Superinfection in HK
In a Hong Kong patient with known HBsAg-positive status presenting with acute hepatitis: always check anti-HEV IgM alongside HBV DNA and anti-HBc IgM. Superinfection with other viral agents, especially HAV and HEV, is an important cause of hepatitis flare-up in chronic HBV carriers. HEV is more common now [6]. Getting this wrong — attributing the flare solely to HBV reactivation and missing HEV — may lead to unnecessary antiviral therapy changes.
- Acute HCV is rarely diagnosed because it is usually asymptomatic in the acute phase.
- Anti-HCV may take 12 weeks to appear [8] — so in the very early acute phase, serology may be negative. HCV RNA by PCR is needed if clinical suspicion is high (e.g., needlestick injury).
- Chronic HCV can also present with acute flares.
- In HK, HCV accounts for ~5–10% of chronic liver disease [9].
- Requires co-infection with HBV (HDV is an incomplete/defective virus that needs HBsAg as its envelope).
- Very rare in Chinese [6]. Not a major differential in HK.
- Consider only in patients with HBsAg-positive status who have unusually severe hepatitis or who come from endemic areas (Mediterranean, Middle East, South America).
- Both EBV and CMV infection can present with liver function abnormalities as well as fever, fatigue and lymphadenopathy [5][10].
- EBV classically causes infectious mononucleosis: triad of fever + tonsillar pharyngitis + lymphadenopathy [11], with hepatomegaly (15–25%), splenomegaly (50–60%), and atypical lymphocytosis [11].
- CMV can cause a mononucleosis-like syndrome without the prominent pharyngitis.
- Key differentiator: atypical lymphocytosis on blood film is characteristic of EBV/CMV but not of HEV. Also, EBV/CMV rarely cause marked jaundice (LFT derangement is usually mild).
- HSV infection can cause hepatitis as a rare complication, occurring most commonly in immunocompromised patients [5][10].
- HSV hepatitis is rare but important because it can cause fulminant hepatic failure with very high mortality if not treated with IV acyclovir.
- Look for: mucocutaneous vesicles, immunosuppressed background.
- Patients with acute HIV infection can have nausea, anorexia and diarrhea [5][10].
- Acute HIV seroconversion illness (2–4 weeks after exposure) can present with fever, lymphadenopathy, pharyngitis, rash, and mildly deranged LFT — overlapping with viral hepatitis.
- Risk factor history (unprotected sexual intercourse, IVDU) is critical.
- Adenovirus typically involves respiratory and gastrointestinal tracts. Hepatitis can be a complication in immunocompromised hosts [10].
2. Drug/Toxin-Induced Liver Injury
- Paracetamol overdose is a leading cause of acute liver failure [12].
- History of intentional overdose or accidental excess is key.
- Distinct pattern: ALT/AST can be extremely high (> 10,000 U/L), with a characteristic timeline — liver injury peaks 48–72 hours post-ingestion.
- Specific antidote: N-acetylcysteine (NAC).
- Paracetamol level and Rumack-Matthew nomogram help differentiate.
- Idiosyncratic drug reactions [12] — unpredictable, not dose-dependent.
- Common offenders: antibiotics (amoxicillin-clavulanate, isoniazid, rifampicin), NSAIDs, statins, anti-epileptics (phenytoin, carbamazepine, valproate), anti-TB drugs.
- Temporal relationship between drug initiation and hepatitis onset is the most important diagnostic clue.
- Drug history (including over-the-counter medications and supplements) is essential.
- Herbal-related hepatotoxicity [12] — very relevant in HK where traditional Chinese medicine (TCM) use is common.
- TCM and herbal teas can cause hepatocellular or cholestatic patterns of liver injury.
- Always ask about TCM intake in any patient with unexplained hepatitis in HK.
- Alcoholic hepatitis has a distinctive LFT pattern: AST > ALT (typically 2:1 ratio), and AST will usually not exceed 500 U/L [13].
- History of heavy alcohol use is essential.
- Signs: hepatomegaly (mild, soft), parotid enlargement, Dupuytren's contracture, spider naevi [13].
- Important: anyone presenting with chronic liver disease and a history of alcoholism must exclude other causes [8] — don't assume it's purely alcoholic.
- Autoimmune hepatitis — very difficult to diagnose, sometimes becomes a diagnosis of exclusion [8].
- Autoantibodies are not specific, require diagnostic criteria [8].
- May be asymptomatic or present with non-specific symptoms including malaise, nausea, abdominal pain, itching and arthralgia [10].
- Features that favour AIH over HEV: female predominance, co-existing autoimmune diseases (e.g., thyroiditis, vitiligo), elevated IgG, positive ANA/anti-SMA/anti-LKM-1, fluctuating course.
- Treatment response to steroids can be diagnostically supportive.
4. Metabolic / Storage Diseases
- Presents as acute hepatitis with jaundice, abdominal pain and deranged LFT [10].
- Can mimic Parkinson's — extrapyramidal deposition of copper [14].
- Fulminant hepatic failure due to Wilson's has a very specific feature: young patients with no reason for fulminant liver failure, exclusion of all other common causes → only finding is low hemoglobin. Coombs-negative hemolytic anemia [14].
- Kayser-Fleischer rings on slit-lamp examination, low ceruloplasmin, elevated 24-hour urinary copper.
- Important differential in any young patient (< 40 years) with unexplained acute hepatitis, especially if hemolytic anemia is present.
- Usually presents insidiously with chronic liver disease, not as acute hepatitis.
- Skin pigmentation ("bronze diabetes"), diabetes, arthropathy, cardiomyopathy.
- Elevated ferritin and transferrin saturation.
- Less relevant as an acute HEV mimic but should be on the broader DDx of chronic hepatitis.
- More relevant in Caucasian populations; rare in Chinese.
- Can present with neonatal cholestasis or adult-onset liver disease.
- Low serum α1-antitrypsin level, PiZZ phenotype.
5. Vascular Causes
- Brisk rise and rapid resolution of LDH; ALT/AST strikingly elevated, peak within 1–3 days, then fall rapidly [7].
- ALT/LDH ratio < 1.5 early in course of hepatitis [7].
- Other evidence of end-organ damage, e.g., acute renal failure [7].
- Diagnosis based on clinical features [7] — typically a patient with preceding hypotension/shock (cardiogenic, septic, hemorrhagic).
- Key differentiator from HEV: the context (ICU patient, post-cardiac arrest, post-cardiac surgery, decompensated heart failure) and the LDH pattern (massively elevated in ischaemic hepatitis but not typically in viral hepatitis).
No other conditions can give this rapid improvement (along with cholangiohepatitis and paracetamol overdose) → no imaging required [13]. If the ALT/AST drops by ~50% per day, think ischaemic hepatitis, not viral.
- Hepatic venous outflow tract obstruction [10].
- Presents with acute or subacute liver disease or acute liver failure [10].
- Classic triad: abdominal pain, hepatomegaly, ascites.
- Risk factors: hypercoagulable states (myeloproliferative neoplasms, antiphospholipid syndrome, OCP use).
- Doppler ultrasound of hepatic veins is diagnostic.
6. Other Infections (Non-Hepatitis Viruses)
- Presents with fever and jaundice due to hemolysis [10] — the jaundice is predominantly unconjugated (pre-hepatic), not hepatocellular.
- Travel history to endemic areas is critical.
- Thick and thin blood films, rapid diagnostic test for malaria antigens.
- Important DDx in HK for returned travelers from Southeast Asia or Africa.
- Weil's disease: jaundice, renal failure, hemorrhagic tendency.
- Exposure history: contact with contaminated water, animal urine (rats).
- Conjunctival suffusion is a characteristic sign.
7. Pregnancy-Related Causes
These are relevant when HEV is suspected in a pregnant patient (where HEV already carries high mortality):
- HELLP syndrome (Hemolysis, Elevated Liver enzymes and Low Platelets) [12].
- Occurs in the context of pre-eclampsia/eclampsia, typically 3rd trimester.
- Overlap with HEV: both occur in pregnancy, both cause elevated liver enzymes, both can cause fulminant liver failure.
- Differentiators: HELLP has hypertension, proteinuria, thrombocytopenia, evidence of microangiopathic hemolytic anemia (schistocytes on blood film, elevated LDH, low haptoglobin).
- Acute fatty liver of pregnancy [12] — rare but life-threatening.
- 3rd trimester, presents with nausea, vomiting, abdominal pain, jaundice, hypoglycemia.
- Differentiator from HEV: AFLP characteristically causes hypoglycemia (impaired gluconeogenesis from fatty infiltration) and DIC, with imaging showing fatty liver.
- Typically presents with chronic, mildly elevated ALT (not acute hepatitis pattern).
- Risk factors: obesity, diabetes, metabolic syndrome.
- Common in HK as dual liver disease with HBV [15].
- Not usually confused with acute HEV, but if a patient with MASLD develops acute hepatitis, HEV superinfection should be considered.
| Category | Differentials | Key Distinguishing Feature from HEV |
|---|---|---|
| Viral hepatitis | HAV, HBV (acute/flare), HCV, HDV | Serology-dependent; HAV shorter incubation, HBV check HBsAg/anti-HBc IgM |
| Other viruses | EBV, CMV, HSV, HIV, Adenovirus | Atypical lymphocytosis (EBV/CMV), vesicles (HSV), seroconversion illness (HIV) |
| Drugs/Toxins | Paracetamol, DILI, TCM, alcohol | Drug history, temporal relationship, AST:ALT ratio (alcohol), paracetamol level |
| Autoimmune | AIH | Female, autoantibodies, elevated IgG, steroid-responsive |
| Metabolic | Wilson disease, haemochromatosis, α1-AT deficiency | Young patient + Coombs-negative haemolysis (Wilson), bronze diabetes |
| Vascular | Ischaemic hepatitis, Budd-Chiari | Shock/hypotension context, LDH pattern (ischaemic); ascites + thrombophilia (Budd-Chiari) |
| Parasitic/other infection | Malaria, leptospirosis | Travel history, haemolysis (malaria), conjunctival suffusion (leptospirosis) |
| Pregnancy-related | HELLP, AFLP | Hypertension + thrombocytopenia (HELLP), hypoglycemia + DIC (AFLP) |
The approach to differentiating HEV from other causes follows a logical sequence:
-
History: This is the most powerful discriminator.
- Travel history, ingestion of seafood → HAV, HEV [16]
- Pork liver, internal organs → HEV [4]
- HBV/HCV status, family history of hepatitis and HCC, blood transfusion, IVDU, MSM → HBV, HCV [16]
- Drug history (including TCM, paracetamol, supplements) → DILI
- Alcohol history → alcoholic hepatitis
- Pregnancy → HELLP, AFLP, HEV in pregnancy
- Hemodynamic instability / shock → ischaemic hepatitis
-
LFT Pattern Recognition:
- ALT/AST > 1000 with rapid rise → viral hepatitis, ischaemic hepatitis, paracetamol, Wilson
- AST:ALT > 2:1 with AST < 500 → alcoholic hepatitis
- Prominent ALP/GGT rise → cholestatic pattern (PBC, PSC, biliary obstruction, drug-induced cholestasis, or cholestatic HEV)
- Four cases where AST rise is greater than ALT: (1) alcoholic hepatitis, (2) cirrhosis, (3) ischaemic hepatitis, (4) Wilson disease [8]
-
Serology: The definitive step.
Exam Approach – 'Acute Hepatitis' DDx
Different liver diseases have different patterns of abnormalities of the liver function test [7]. But the LFT pattern alone is insufficient — determining the final diagnosis requires history (drug history), clinical presentation, other investigations, and liver biopsy may be required [7]. For HEV specifically: the combination of appropriate exposure history (pork liver, shellfish, travel to endemic area) + anti-HEV IgM positivity + compatible clinical course (self-limiting hepatitis with prominent cholestasis) clinches the diagnosis.
| Condition | Why It Mimics HEV | How to Tell Them Apart |
|---|---|---|
| HAV | Same fecal-oral transmission, same clinical syndrome, same self-limiting course | Shorter incubation, less cholestatic, anti-HAV IgM (not anti-HEV IgM) |
| Acute HBV | Same hepatitis syndrome, can be very severe | Longer incubation, bloodborne risk factors, HBsAg + anti-HBc IgM positive |
| HBV flare with HEV superinfection | Patient already HBsAg-positive, acute hepatitis flare | Both serologies may be positive — the HEV IgM identifies the superinfection |
| Ischaemic hepatitis | Very high transaminases like severe viral hepatitis | Context of shock/hypotension, rapid fall of ALT/AST within days, elevated LDH, ALT/LDH ratio < 1.5 [7] |
| Wilson disease | Young patient with acute hepatitis ± fulminant failure | Coombs-negative hemolytic anemia, low ceruloplasmin, KF rings [14] |
| DILI | Hepatitis pattern LFT, recent drug exposure | Temporal drug-disease relationship, improvement after drug withdrawal |
High Yield Summary – DDx of Hepatitis E
-
The clinical presentation of HEV is indistinguishable from other causes of acute hepatitis — diagnosis depends on serology (anti-HEV IgM) and clinical context (exposure history).
-
In Hong Kong, always consider: (a) HEV superinfection on chronic HBV, (b) dual liver disease (HBV + MASLD + HEV), (c) rat-HEV in immunosuppressed patients.
-
Key DDx categories: viral (HAV, HBV, HCV, EBV, CMV), drugs/toxins (paracetamol, DILI, TCM, alcohol), autoimmune, metabolic (Wilson), vascular (ischaemic hepatitis, Budd-Chiari), pregnancy-related (HELLP, AFLP).
-
LFT pattern helps but is not diagnostic: viral hepatitis gives ALT-predominant rise; alcoholic hepatitis gives AST:ALT > 2:1 with AST < 500; ischaemic hepatitis gives rapid rise and fall with elevated LDH.
-
History is king: food exposure (pork liver → HEV, shellfish → HAV), drug history (paracetamol, TCM), alcohol, pregnancy, hemodynamic status (shock → ischaemic hepatitis), immunosuppression (chronic HEV risk).
Active Recall - Differential Diagnosis of Hepatitis E
References
[2] Senior notes: Maksim Medicine Notes.pdf (Viral hepatitis overview table, p.141) [3] Senior notes: Block A - Jaundice after raw oysters_ acute hepatitis.pdf (p.1) [4] Senior notes: Ryan Ho GI.pdf (p.235, HEV section) [5] Senior notes: MBBS Final MB (Medicine) (Felix PY Lai).pdf (p.767, HEV differential diagnosis) [6] Senior notes: Block A - I am a hepatitis B carrier.pdf (p.28, HEV superinfection on chronic HBV) [7] Lecture slides: Gastroenterology Hepatology Introduction to GI:Hepatology investigations from the abnormal.pdf (p.40, p.49) [8] Senior notes: Block A - Gastrointestinal Data Interpretation.pdf (p.2–5) [9] Senior notes: Block A - Abdominal distension_ ascites and cirrhosis.pdf (p.2) [10] Senior notes: MBBS Final MB (Medicine) (Felix PY Lai).pdf (p.738, HAV differential diagnosis — analogous framework) [11] Senior notes: Ryan Ho Respiratory.pdf (p.53, infectious mononucleosis) [12] Senior notes: Block A - A jaundiced and incoherent patient_ liver failure.pdf (p.3, causes of ALF) [13] Senior notes: Block A - Introduction to GI_Hepatology investigations (LFT, Endoscopy).pdf (p.15–17) [14] Senior notes: Block A - Patients with non-viral chronic liver diseases.pdf (p.2, Wilson disease) [15] Senior notes: Block A - Gastroenterology Interactive Tutorial.pdf (p.2, dual liver disease) [16] Senior notes: Ryan Ho Fundamentals.pdf (p.295, approach to jaundice)
Diagnostic Criteria, Algorithm, and Investigations for Hepatitis E
1. Diagnostic Criteria
Unlike some conditions (e.g., SLE with SLICC criteria, or rheumatic fever with Jones criteria), there is no formal "points-based" diagnostic criteria set for HEV. Instead, diagnosis is established through a combination of:
- Compatible clinical presentation — acute hepatitis syndrome (fever, jaundice, elevated transaminases) with appropriate exposure history
- Serological confirmation — detection of anti-HEV IgM antibodies
- Virological confirmation — detection of HEV RNA by RT-PCR (in serum or stool)
- Exclusion of other causes — a critical step emphasized in the GC lecture slides
The diagnosis of HEV is fundamentally a serological + virological diagnosis supported by clinical context and exclusion of alternatives. There is no single pathognomonic clinical feature.
A diagnosis of acute HEV infection can be made when:
| Component | Requirement |
|---|---|
| Clinical | Compatible acute hepatitis (elevated ALT/AST, ± jaundice, ± prodromal symptoms) |
| Serological | Anti-HEV IgM positive [2][3] |
| Virological (confirmatory) | HEV RNA positive by RT-PCR in serum or stool [2][3] |
| Exclusion | Other major causes of acute hepatitis excluded (HBV, HAV, HCV, drugs, autoimmune, ischaemic) |
High Yield – GC Lecture Slide Diagnostic Algorithm
Investigations for acute viral hepatitis: Markedly elevated ALT and AST + Specific serological test + Exclusion of other causes [17].
The GC 239 slide shows a stepwise exclusion approach:
- HBsAg → Positive (diagnoses HBV)
- Anti-HAV IgM → Negative
- Anti-HCV → Negative
- Anti-HEV IgM → Negative
- ANA, anti-smooth muscle, anti-LKM-1 → Negative (excludes autoimmune hepatitis)
- Ultrasound → No biliary obstruction / liver normal / no splenomegaly
- Toxicology screen → Paracetamol levels normal
- If all negative: HEV RNA → Positive [17]
This final step is crucial: anti-HEV IgM can be negative in a substantial proportion of acute HEV cases, so if clinical suspicion remains high despite negative IgM, HEV RNA PCR should be sent [4].
The approach to diagnosing HEV follows the general approach to any patient presenting with acute hepatitis. The algorithm below integrates the GC 239 lecture slide diagnostic flowchart [17] with HEV-specific considerations.
The algorithm emphasizes: serology first, then virological confirmation if serology is negative but suspicion remains, then exclude non-viral causes [17]. This is the exact approach shown on the GC 239 lecture slides.
3. Investigation Modalities
| Investigation | Purpose | Key Findings in HEV |
|---|---|---|
| Vital signs Q4h | Monitor for deterioration | Fever (usually < 39°C), tachycardia if dehydrated or septic |
| H'stix BD [2] | Monitor for hypoglycemia | Important in severe/fulminant hepatitis — liver failure impairs gluconeogenesis |
| Urine dipstick | Bilirubin, urobilinogen | Dark urine with positive bilirubin (conjugated); elevated urobilinogen |
| Urine color assessment | Quick bedside clue | Tea-colored = conjugated hyperbilirubinemia = hepatocellular or cholestatic jaundice |
Why H'stix BD? Because liver failure can cause hypoglycemia [2]. The liver is responsible for gluconeogenesis and glycogenolysis. In massive hepatocyte necrosis (fulminant hepatitis), these functions fail → dangerous hypoglycemia. Monitoring capillary blood glucose twice daily catches this early.
3.2 Blood Investigations
| Parameter | Expected Finding in HEV | Interpretation |
|---|---|---|
| WCC | Usually normal [2] | Viral hepatitis does NOT typically cause leukocytosis (cf. bacterial infections). Mild lymphocytosis may be seen. |
| Hemoglobin | Usually normal | If low → consider hemolytic anemia (extrahepatic manifestation of HEV, or consider Wilson disease) |
| Platelets | Usually normal | May be low in severe disease (DIC, hypersplenism if CLD) or as extrahepatic manifestation (immune-mediated thrombocytopenia) |
| Blood film | Atypical lymphocytes | If prominent → think EBV/CMV rather than HEV |
This is the cornerstone investigation. The LFT assesses three distinct hepatic functions [18]:
| LFT Component | What It Measures | Expected in Acute HEV |
|---|---|---|
| ALT (alanine aminotransferase) | Hepatocyte integrity (cytoplasmic enzyme) | Markedly elevated, usually 200–2000 U/L [2], can exceed 3000 |
| AST (aspartate aminotransferase) | Hepatocyte integrity (mitochondrial + cytoplasmic) | Elevated, but usually ALT > AST in viral hepatitis [8] |
| ALP (alkaline phosphatase) | Biliary epithelium / cholestasis | Mildly to moderately elevated (more elevated if cholestatic variant, ~20%) |
| GGT (gamma-glutamyl transferase) | Biliary / inducible enzyme | Mildly elevated |
| Total bilirubin | Bilirubin metabolism | Elevated (both conjugated and unconjugated components) |
| Albumin | Synthetic function | Usually normal in acute HEV (half-life ~21 days, so takes time to fall). Low if pre-existing CLD or severe/prolonged disease |
| Globulin | Immune response | May be mildly elevated (polyclonal immunoglobulin response) |
High Yield – LFT Interpretation Framework
Liver function tests assess three distinct aspects of hepatic function: cellular integrity through ALT and AST levels, synthetic capacity via albumin and prothrombin time, and excretory function using bilirubin, ALP and GGT [18].
In acute HEV:
- Cellular integrity → markedly deranged (ALT/AST very high)
- Synthetic function → usually preserved initially (albumin normal, PT may be mildly prolonged) — if INR significantly prolonged, worry about fulminant hepatitis
- Excretory function → deranged (elevated bilirubin, mildly elevated ALP/GGT), especially if cholestatic variant
ALT is greater than AST in the majority of primary liver pathology [8]. If AST > ALT, consider: alcoholic hepatitis, cirrhosis, ischaemic hepatitis, or Wilson disease [8].
| Parameter | Expected in HEV | Clinical Significance |
|---|---|---|
| INR / PT | May be mildly prolonged | INR is the best test for reflecting liver function because Factor VII has the shortest half-life (~6 hours) [2], making it the earliest clotting factor to decline when synthetic function fails |
| APTT | Usually normal or mildly prolonged | Less sensitive than INR for liver synthetic function |
Why is INR the best marker? The liver synthesizes clotting factors II, VII, IX, and X (the vitamin K-dependent factors). Factor VII has a half-life of only ~6 hours. So when hepatocytes are rapidly dying (as in fulminant hepatitis), Factor VII drops first → INR rises first. If INR is markedly prolonged (> 1.5) in acute viral hepatitis, this is an alarm sign for impending liver failure.
| Parameter | Finding | Significance |
|---|---|---|
| Arterial NH3 | Elevated if hepatic encephalopathy developing | The liver normally converts ammonia → urea via the urea cycle. Massive hepatocyte loss → ammonia accumulates → crosses blood-brain barrier → astrocyte swelling → cerebral edema |
| Test | Purpose | Expected in HEV |
|---|---|---|
| Amylase | Rule out pancreatitis (HEV can cause acute pancreatitis as extrahepatic manifestation) | Usually normal; elevated if pancreatic involvement |
| Renal function (RFT) | Hepatorenal syndrome in severe cases; baseline for drug dosing | Usually normal unless severe disease |
| AFP | Can be mildly elevated in acute hepatitis due to liver regeneration | High inflammation in the liver can cause marked elevation of AFP. Key difference is to see whether it comes down after symptoms disappear [8]. If persistently rising → worry about HCC |
| LDH | Distinguish from ischaemic hepatitis | In HEV: moderately elevated. In ischaemic hepatitis: massively elevated with ALT/LDH ratio < 1.5 [7] |
3.3 Specific Serological and Virological Tests for HEV
This is where the definitive diagnosis is made.
| Feature | Detail |
|---|---|
| What it detects | IgM antibodies against HEV capsid proteins |
| When it appears | Appears during early phases of clinical illness and coincides with symptoms [4] — unlike HAV IgM which may be delayed 1–2 weeks after jaundice |
| Duration | Persists for ~3 months [3][4] |
| Sensitivity | Appears in > 90% of patients [4] |
| Limitation | May be negative in a substantial proportion of immunosuppressed patients [4] (impaired antibody production) and in very early or very late presentations |
| False positives | Can occur — specificity varies by assay. In high-prevalence areas, positive predictive value may be lower |
Critical Exam Point – When Anti-HEV IgM Is Negative
Anti-HEV IgM may be negative in a substantial proportion of cases [4]. This is why the GC lecture slide algorithm shows that if anti-HEV IgM is negative but clinical suspicion remains, HEV RNA should be sent [17]. This is particularly important in:
- Immunosuppressed patients (transplant recipients) — impaired antibody production
- Very early presentation — antibodies have not yet developed
- Assay variability — commercial assays have variable sensitivity
| Feature | Detail |
|---|---|
| What it detects | IgG antibodies against HEV |
| When it appears | Follows the rise of anti-HEV IgM [3] |
| Duration | Can persist for several years [3] |
| Clinical utility | Indicates past/resolved infection, NOT useful for diagnosing acute infection (unless seroconversion documented) |
| Limitation | Does not confer permanent immunity — reinfection is possible [3] |
| Epidemiological use | Seroprevalence studies (e.g., HK ~33.6% seroprevalence) |
Why doesn't anti-HEV IgG confer lifelong immunity like anti-HAV IgG? Because HEV has multiple genotypes (1–4 plus rat-HEV) with variable cross-protection, and anti-HEV IgG wanes over time. In contrast, HAV has a single serotype, so anti-HAV IgG provides durable, lifelong protection [3].
| Feature | Detail |
|---|---|
| Specimen | Serum/plasma or stool |
| What it detects | Viral RNA — direct evidence of active viral replication |
| When it is positive | During viremia (starts before symptom onset, may persist for weeks) |
| Clinical utility | Gold standard for confirming active infection, especially when serology is equivocal or negative |
| In stool | Stool HEV RNA RT-PCR [2] — virus shed in feces; may remain positive longer than serum RNA |
| For chronic HEV | Essential — diagnosis of chronic HEV requires persistent positive HEV RNA (> 3–6 months) |
| Genotyping | Can be performed on positive samples — important for epidemiological tracking and predicting clinical course (genotype 1 → pregnancy risk; genotype 3 → chronicity risk) |
High Yield – Serological vs Virological Diagnosis
| Test | Acute Infection | Past Infection | Chronic Infection |
|---|---|---|---|
| Anti-HEV IgM | Positive | Negative | Variable (may be negative in immunosuppressed) |
| Anti-HEV IgG | Positive (late) or negative (early) | Positive | Variable |
| HEV RNA | Positive | Negative | Positive (> 3–6 months) |
For the exam: Anti-HEV IgM is the first-line serological test for acute HEV [2][17]. HEV RNA is the confirmatory/definitive test and is essential when IgM is negative or for diagnosing chronic HEV [17].
As emphasized in the GC slides, diagnosis of HEV requires exclusion of other causes [17]. The standard panel:
| Marker | What It Diagnoses | Interpretation |
|---|---|---|
| HBsAg | HBV infection (acute or chronic) | If positive → add anti-HBc IgM, HBeAg, HBV DNA [17] |
| Anti-HAV IgM | Acute HAV | If positive → acute hepatitis A |
| Anti-HCV ± HCV RNA | HCV infection | Anti-HCV may take 12 weeks to appear [8]; HCV RNA is positive earlier |
| Anti-HEV IgM | Acute HEV | If positive → acute hepatitis E |
| ANA, anti-smooth muscle, anti-LKM-1 | Autoimmune hepatitis | If positive with elevated IgG → consider AIH [17] |
Recall: IgM reflects acute reaction [8]. This is the fundamental immunological principle — IgM is the first antibody produced in a primary immune response (it's a pentamer, large, doesn't cross the placenta). IgG appears later, is smaller, crosses the placenta, and indicates past exposure or chronic infection.
Interpretation of viral hepatitis markers [19]:
| Scenario | Marker Pattern |
|---|---|
| Acute HAV | IgM anti-HAV positive |
| Acute HBV | HBsAg positive + IgM anti-HBc positive |
| Acute HEV | IgM anti-HEV positive |
| Acute HCV | No reliable acute marker — need HCV RNA |
| Past HAV | IgG anti-HAV positive |
| Recovered HBV | Anti-HBs positive + IgG anti-HBc positive |
| Prior HCV | Anti-HCV positive + HCV RNA negative |
| Modality | Purpose | Expected Findings in Acute HEV |
|---|---|---|
| Ultrasound liver | Exclude biliary obstruction / liver mass / splenomegaly [17] | Usually normal or shows mild hepatomegaly. No biliary dilatation (this helps exclude obstructive causes). May show gallbladder wall thickening (reactive). |
| Doppler ultrasound hepatic veins | If Budd-Chiari suspected | Normal in HEV (abnormal flow in Budd-Chiari) |
| FibroScan (liver elastography) | If underlying CLD suspected | Elevated liver stiffness may indicate pre-existing fibrosis/cirrhosis. CAP score assesses steatosis. Not needed in straightforward acute HEV but relevant in dual pathology. |
Urgent imaging may not be necessary [7] for conditions like acute hepatitis E where the diagnosis is serological. Imaging is primarily to exclude biliary obstruction [17].
- Liver biopsy may be required [7] — but only in specific scenarios:
- Diagnostic uncertainty after full serological workup
- Suspected autoimmune hepatitis (histological features support diagnosis)
- Suspected chronic HEV with unclear etiology of liver disease
- Assessment of fibrosis stage in chronic HEV
- NOT routinely needed for straightforward acute HEV
- Histological findings in HEV (if performed):
- Lobular hepatitis with spotty necrosis
- Portal and periportal inflammation (lymphocytic)
- Cholestatic features (bile plugs in canaliculi) — more prominent than in HAV
- In chronic HEV: progressive fibrosis
| Test | Purpose | When to Order |
|---|---|---|
| Toxicology screen / Paracetamol level | Exclude drug-induced hepatitis | If appropriate [17] — always in unexplained ALF |
| Ceruloplasmin, 24h urine copper | Exclude Wilson disease | Young patient with acute hepatitis ± hemolytic anemia |
| Ferritin, transferrin saturation | Exclude hemochromatosis | Chronic hepatitis workup |
| α1-antitrypsin level | Exclude α1-AT deficiency | Chronic hepatitis in young Caucasian |
| EBV/CMV IgM, monospot | Exclude EBV/CMV hepatitis | Atypical lymphocytosis, mononucleosis-like picture |
| Blood film for malaria parasites | Exclude malaria | Returned traveler with fever and jaundice |
Once the diagnosis of acute HEV is established, the following monitoring regimen is recommended [2]:
| Monitoring | Frequency | Rationale |
|---|---|---|
| DAT (diet as tolerated) | Ongoing | Nutritional support |
| Obs Q4h | Ongoing | Monitor for deterioration (encephalopathy, hemodynamic instability) |
| H'stix BD | Twice daily | Liver failure can cause hypoglycemia [2] |
| CBC, LRFT, INR, NH3 | Daily [2] | Track liver synthetic function (INR), cellular damage (ALT/AST), and encephalopathy risk (ammonia) |
The logic: acute HEV is usually self-limiting, but you cannot predict who will develop fulminant hepatitis. Daily monitoring of INR catches early synthetic failure. Daily ALT/AST shows the trajectory (improving or worsening). Ammonia monitoring catches early hepatic encephalopathy. H'stix catches hypoglycemia before it becomes symptomatic.
Understanding the temporal relationship between viral markers and clinical illness is essential for correct interpretation:
Key temporal points:
- HEV RNA appears first (during incubation, before symptoms) and is the earliest diagnostic marker
- Anti-HEV IgM appears during early phases of clinical illness and coincides with symptoms [4] — this is earlier than HAV IgM (which may be delayed 1–2 weeks after jaundice)
- Anti-HEV IgM persists for ~3 months [3][4]
- Anti-HEV IgG follows the rise of IgM and can persist for several years [3]
- HEV RNA in stool may persist longer than in serum
6. Special Diagnostic Considerations
In Hong Kong, a common clinical scenario is a known chronic HBV carrier presenting with acute hepatitis flare. The investigation approach:
- Check anti-HEV IgM — if positive, HEV superinfection confirmed [6]
- Simultaneously check anti-HBc IgM (to assess for HBV reactivation) and HBV DNA (to quantify viral load)
- If both anti-HEV IgM and anti-HBc IgM are positive → dual etiology possible
- Remember: AFP can be elevated in acute inflammation — key is whether it comes down after symptoms disappear [8]
- Anti-HEV IgM may be negative (impaired humoral immunity)
- HEV RNA in serum or stool is the essential diagnostic test — must be positive for > 3–6 months to define chronicity [2]
- HEV genotyping should be performed (usually genotype 3)
- Liver biopsy may show progressive fibrosis
- Standard anti-HEV IgM assays may not detect rat-HEV (different species: Orthohepevirus C)
- Specialized RT-PCR targeting rat-HEV genome is required
- Consider in immunosuppressed patients in HK with unexplained hepatitis and negative standard HEV serology [3]
High Yield Summary – Diagnosis of Hepatitis E
-
First-line diagnostic test: Anti-HEV IgM — positive in > 90% of acute cases, coincides with symptom onset, persists ~3 months.
-
Confirmatory test: HEV RNA RT-PCR (serum or stool) — essential when IgM negative but suspicion high, and mandatory for diagnosing chronic HEV.
-
GC lecture algorithm: Markedly elevated ALT and AST → Specific serological tests → Exclusion of other causes [17]. HEV RNA is the final step when serology is negative.
-
Exclusion panel: HBsAg, anti-HAV IgM, anti-HCV, autoantibodies (ANA, anti-SMA, anti-LKM-1), ultrasound, toxicology screen/paracetamol level.
-
LFT pattern: ALT-predominant rise (ALT > AST), total bilirubin elevated, ± prominent cholestasis (~20%). INR is the critical synthetic function marker.
-
Monitoring: DAT, Obs Q4h, H'stix BD, daily CBC/LRFT/INR/NH3.
-
Anti-HEV IgG indicates past infection, NOT acute — does not confer permanent immunity, reinfection possible.
Active Recall - Diagnosis of Hepatitis E
References
[2] Senior notes: Maksim Medicine Notes.pdf (Viral hepatitis overview table and approach, p.141) [3] Senior notes: Block A - Jaundice after raw oysters_ acute hepatitis.pdf (p.20, HEV diagnosis) [4] Senior notes: Ryan Ho GI.pdf (p.235, HEV diagnosis section) [6] Senior notes: Block A - I am a hepatitis B carrier.pdf (p.28, HEV superinfection) [7] Lecture slides: Gastroenterology Hepatology Introduction to GI:Hepatology investigations from the abnormal.pdf (p.40, p.49) [8] Senior notes: Block A - Gastrointestinal Data Interpretation.pdf (p.2–5, LFT interpretation and viral markers) [17] Lecture slides: GC 239. Viral hepatitis HAV_HBV_HCV_HEV.pdf (p.11, p.20, diagnostic algorithm) [18] Senior notes: Learning_Points_All_Lectures.txt (GI/Hepatology data interpretation learning points) [19] Lecture slides: 1213_DI_GI_Prof_WK_Leung.ppt.pdf (p.14, interpretation of viral hepatitis markers)
Management of Hepatitis E
The management of Hepatitis E is defined by one overarching principle: in the vast majority of immunocompetent patients, HEV is self-limiting and requires no specific treatment [1][2][3][5]. The clinical approach therefore stratifies patients by immune status and disease severity, and management ranges from simple supportive care through to liver transplantation in extreme cases.
As Prof. CL Lai emphasizes: for acute hepatitis, there is no known drug or herb that hastens recovery. The disease must essentially "run its course" — "definitely do not try TCM" [3]. This is in stark contrast to chronic HBV or HCV where specific antivirals exist.
2. Management of Acute HEV in Immunocompetent Patients
This is the commonest clinical scenario and the management is straightforward.
The GC 239 lecture slide states clearly: No specific treatment. Supportive measures: maintaining hydration, electrolyte balance, nutritional balance [1].
| Intervention | Detail | Rationale |
|---|---|---|
| IV fluids if dehydrated | Normal saline or Hartmann's | Vomiting, poor oral intake, and fever cause dehydration. Adequate hydration maintains renal perfusion (hepatorenal physiology) |
| Nutritional support | Eat anything — no solid diet modifications needed [3] | There is no evidence that any dietary restriction accelerates recovery. Fatty diet is harmless. Glucose drip does not help and may actually induce fatty liver [3] |
| Rest | If extremely tired, rest — but rest does not shorten the course of hepatitis [3] | Historical evidence from US soldiers in Vietnam showed identical outcomes between those who rested and those who continued activity [3] |
| Antiemetics | For nausea/vomiting (e.g., metoclopramide, ondansetron) | Symptomatic relief only — avoid hepatotoxic antiemetics |
| Cholestyramine | For severe pruritus (cholestatic variant) | Bile acid sequestrant — binds bile acids in the gut, reducing enterohepatic recirculation and thus reducing circulating bile acid levels that cause itch |
No drugs to be administered — no known drugs or herbs that hasten recovery of acute hepatitis [3]. This is a critical exam point: students often feel they should "do something" for acute viral hepatitis. The answer is: supportive care only.
Why 6 months? The liver needs time to regenerate fully after acute hepatitis. During this recovery phase, the cytochrome P450 enzyme system (especially CYP2E1, which metabolizes ethanol) is already stressed. Adding alcohol during recovery imposes additional oxidative stress via acetaldehyde production and reactive oxygen species, potentially prolonging inflammation and risking progression to more severe liver injury.
- Avoid hepatotoxic medications during the acute phase (paracetamol in high doses, NSAIDs, statins, azole antifungals, etc.)
- Review all current medications for hepatotoxic potential
- Definitely do not try TCM [3] — herbal preparations are a recognized cause of drug-induced liver injury and may worsen hepatitis
As established in the diagnosis section [2]:
| Parameter | Frequency | Rationale |
|---|---|---|
| DAT | Ongoing | Diet as tolerated |
| Obs Q4h | Ongoing | Detect early signs of deterioration (encephalopathy, hemodynamic instability) |
| H'stix BD | Twice daily | Liver failure can cause hypoglycemia — early detection prevents neuroglycopenic damage |
| CBC, LRFT, INR, NH3 | Daily | Track hepatocyte integrity (ALT/AST), synthetic function (INR, albumin), and encephalopathy risk (ammonia) |
| Red Flag | Significance |
|---|---|
| INR ≥ 1.5 | Impaired synthetic function → risk of fulminant hepatic failure [5] |
| Falling ALT in context of worsening clinical status | Paradoxical ALT drop = massive hepatocyte death with few remaining cells to release enzymes ("burnt-out liver") |
| Hepatic encephalopathy | Confusion, asterixis, fetor hepaticus → acute liver failure |
| Hypoglycemia | Failing gluconeogenesis |
| Progressive jaundice beyond 4 weeks | Consider cholestatic variant or alternative diagnosis |
| Pregnancy (3rd trimester) | 20–25% maternal mortality [1] — low threshold for ICU admission |
3. Management of Fulminant HEV (Acute Liver Failure)
Fulminant HEV is rare in the general population but carries high mortality, especially in pregnant women [1] and patients with pre-existing chronic liver disease [1]. The management follows the general principles of acute liver failure (ALF) management [20]:
Five principles of management of acute liver failure [20]:
- Supportive care → standard ICU care
- Identifying and removing/treating the insult
- Manage complications
- High-volume plasma exchange
- Liver transplantation
| Principle | Application in Fulminant HEV |
|---|---|
| Supportive ICU care | Airway protection (intubation if GCS < 8), hemodynamic support (vasopressors if needed), mechanical ventilation |
| Identify/treat the insult | For HEV, no specific antiviral has proven efficacy in fulminant disease. The insult is immune-mediated hepatocyte destruction — removing the cause is not directly possible as with paracetamol (NAC) or HBV (antivirals) [20] |
| Manage complications | See below |
| High-volume plasma exchange | Washes away cytokines causing liver failure [20]. Very expensive but useful as adjunctive therapy. Evidence from the FULMAR trial showed improved transplant-free survival |
| Liver transplantation | The final line → after exhausting all other treatments [20] |
| Complication | Management | Why It Occurs |
|---|---|---|
| Cerebral edema / intracranial hypertension | Head elevation 30°, mannitol/hypertonic saline, ICP monitoring in severe cases | Ammonia crosses BBB → astrocyte swelling (glutamine theory) → cerebral edema |
| Coagulopathy | FFP/cryoprecipitate only if active bleeding or pre-procedure (NOT to "correct" INR) | Loss of clotting factor synthesis. But also loss of anticoagulant proteins (protein C, S) — so the patient may paradoxically have balanced hemostasis despite high INR |
| Hypoglycemia | H'stix monitoring + IV dextrose infusion | Loss of glycogen stores + impaired gluconeogenesis |
| Sepsis | Empirical broad-spectrum antibiotics if suspected; surveillance cultures | Impaired Kupffer cell function + reduced complement synthesis → immunodeficiency |
| Hepatorenal syndrome | Volume resuscitation, terlipressin + albumin | Splanchnic vasodilation → reduced effective arterial blood volume → renal vasoconstriction |
| Metabolic acidosis | Sodium bicarbonate, renal replacement therapy if severe | Impaired lactate metabolism by liver + tissue hypoperfusion |
Role of antiviral therapy in fulminant HEV is not well established [5]. There are case reports of ribavirin use in severe acute HEV, but no randomized trial evidence. The management remains primarily supportive.
- Liver transplantation may be indicated in patients with acute HEV infection who develop fulminant liver failure [5]
- Decision guided by King's College criteria (for non-paracetamol ALF): INR > 6.5, OR any 3 of: age < 10 or > 40, etiology non-A non-B hepatitis, jaundice to encephalopathy interval > 7 days, INR > 3.5, bilirubin > 300 µmol/L
- Practical considerations: organ availability, patient fitness for surgery, contraindications
4. Management of Chronic HEV in Immunosuppressed Patients
This is the second major management scenario and is highly exam-relevant because it is one of the few situations where specific treatment for HEV exists.
- Reduction of immunosuppression → facilitates clearance of chronic HEV infection [5]
- This is the first-line intervention
- In transplant recipients: reduce tacrolimus/cyclosporine dose, reduce or stop mycophenolate mofetil (MMF), reduce corticosteroid dose
- Rationale: the reason HEV becomes chronic is T-cell suppression from immunosuppressive drugs. By reducing immunosuppression, you restore T-cell-mediated viral clearance
Why reduce immunosuppression first before trying antivirals? Because approximately 30% of transplant patients with chronic HEV will achieve viral clearance with immunosuppression reduction alone — no antiviral needed. This avoids unnecessary drug side effects and simplifies management. However, this must be balanced against rejection risk.
| Feature | Detail |
|---|---|
| Drug | Ribavirin (riba- = related to ribose, a nucleoside analogue) |
| Mechanism | Nucleoside analogue with multiple mechanisms: (1) inhibits inosine monophosphate dehydrogenase (IMPDH) → depletes GTP pools → impairs viral RNA synthesis; (2) direct inhibition of viral RNA-dependent RNA polymerase; (3) enhancement of T-cell-mediated immunity via Th1 shift |
| Regimen | Ribavirin monotherapy × 12 weeks [2][5] |
| Dose | Typically 600–800 mg/day (weight-based); adjust for renal function |
| Indication | Immunosuppressive therapy cannot be reduced, OR persistent HEV RNA despite reduction in immunosuppressive therapy [5] |
| SVR assessment | Check HEV RNA 12 weeks after completion of ribavirin course |
| Relapse | If HEV RNA recurs after stopping ribavirin → extended course (up to 6 months or longer) |
High Yield – Chronic HEV Management
Chronic hepatitis E (detection of HEV RNA in serum/stool > 6 months) in transplant recipients → Management: Reduce immunosuppressants, ribavirin monotherapy × 12 weeks [2].
The stepwise approach:
- Reduce immunosuppression → check HEV RNA at 12 weeks
- If still positive → Ribavirin monotherapy × 12 weeks → check HEV RNA 12 weeks post-treatment (SVR)
- If relapse → Extended ribavirin course
This is directly from lecture material and is high-yield for exams.
| Side Effect | Mechanism | Management |
|---|---|---|
| Hemolytic anemia (most important and dose-limiting) | Ribavirin accumulates in erythrocytes → oxidative damage to RBC membranes → extravascular hemolysis | Monitor Hb closely; dose reduction or EPO supplementation; transfusion if severe |
| Teratogenicity (ABSOLUTE contraindication in pregnancy) | Ribavirin is a category X drug — causes fetal malformation in animal studies | Pregnancy must be excluded before starting; effective contraception required during treatment AND for 6 months after stopping |
| Fatigue, headache | Non-specific | Symptomatic management |
| Rash, pruritus | Hypersensitivity | Dose reduction or discontinuation |
| Hyperuricemia | Hemolysis → increased purine breakdown → uric acid | Monitor uric acid |
| Contraindication | Rationale |
|---|---|
| Pregnancy / breastfeeding | Teratogenic |
| Severe renal impairment (CrCl < 50 mL/min) | Ribavirin is renally cleared → accumulation → severe hemolysis |
| Hemoglobinopathies (thalassemia, sickle cell) | Already at risk of hemolysis; ribavirin will worsen |
| Unstable cardiac disease | Acute anemia from hemolysis may precipitate cardiac ischemia |
| Autoimmune hepatitis | May exacerbate immune-mediated disease |
The critical irony: HEV is most dangerous in pregnancy (20–25% mortality in 3rd trimester), yet the only specific antiviral (ribavirin) is absolutely contraindicated in pregnancy due to teratogenicity. This means management of severe HEV in pregnancy is entirely supportive ± liver transplantation. There is no antiviral option.
| Agent | Evidence | Status |
|---|---|---|
| PEG-interferon alpha | Case reports of efficacy in chronic HEV (transplant patients) | Generally avoided in transplant recipients due to risk of precipitating graft rejection |
| Sofosbuvir | In vitro activity against HEV; some case reports | Investigational; not recommended as standard |
| Ribavirin + PEG-IFN combination | More effective than ribavirin alone in some reports | Limited use due to interferon toxicity in immunosuppressed patients |
5. Prevention
Prevention is a key component of HEV management at the population and individual level.
Prevention measures [5]:
- Avoidance of water of unknown purity
- Avoidance of food from street vendors
- Avoidance of raw or undercooked seafood, meat or pork products and raw vegetables
- Avoid internal organs (especially pig liver) [2] — highly relevant in HK
In Hong Kong specifically: the most actionable advice is to avoid raw or undercooked pork liver (豬潤), pork blood products, and raw shellfish. Cooking to an internal temperature of ≥ 71°C for at least 20 minutes inactivates HEV.
- A recombinant vaccine to prevent hepatitis E virus infection called Hecolin® was developed in China [1]
- Licensed in China since 2011 [1]
- Licensed in Pakistan in 2020 [1]
- Has been used as an outbreak response measure (e.g., vaccination campaign in South Sudan) via MSF [1]
- Clinical trials in USA being conducted [1]
- NOT currently available in Hong Kong [3]
- NOT WHO-prequalified (though under review)
- Based on recombinant HEV genotype 1 capsid protein (p239)
- 3-dose schedule: 0, 1, and 6 months
- Efficacy: ~97% in preventing symptomatic HEV genotype 4 infection in clinical trials
- Pre- or post-exposure immunoglobulin prophylaxis for prevention of HEV has not been established [5]
- No passive prevention available for HEV [2]
- This is in contrast to HAV, where immunoglobulin can be given for post-exposure prophylaxis
Why no immunoglobulin prophylaxis for HEV? Because (1) anti-HEV antibodies in pooled human immunoglobulin preparations are present at variable and often low titers, (2) the protective titer of anti-HEV has not been well-defined, and (3) anti-HEV antibodies do not confer 100% immunity — reinfection is still possible [3].
- All viral hepatitis are notifiable diseases [2] in Hong Kong
- Report to the Centre for Health Protection (CHP) upon diagnosis
- Contact tracing is not as critical as for HAV (since most HEV transmission is zoonotic in HK, not person-to-person)
6. Special Population Management
| Aspect | Management |
|---|---|
| Severity | 20–25% maternal mortality in 3rd trimester [1] — aggressive supportive care |
| Admission threshold | Low — consider ICU admission early |
| Antiviral therapy | Ribavirin is absolutely contraindicated (teratogenic) |
| Delivery | May need urgent delivery if maternal condition deteriorating (balance fetal maturity vs. maternal survival) |
| Liver transplantation | May be considered as last resort |
| Aspect | Management |
|---|---|
| Risk | Acute-on-chronic liver failure — very high morbidity and mortality |
| Monitoring | More intensive — consider early ICU involvement |
| HBV carriers | Ensure HBV antiviral therapy is optimized; HEV superinfection management is supportive |
| Alcohol | Absolute abstinence |
As detailed in Section 4 above: reduce immunosuppression → ribavirin if needed → monitor for SVR.
| Scenario | Treatment | Key Points |
|---|---|---|
| Acute HEV, immunocompetent, mild | Supportive only | No antiviral. Alcohol abstinence × 6 months. Monitor daily. |
| Acute HEV, immunocompetent, severe/fulminant | ICU supportive care, high-volume plasma exchange, liver transplantation if refractory | Role of antiviral therapy is not well-established [5] |
| Acute HEV in pregnancy | Aggressive supportive care, delivery if needed, liver transplantation as last resort | Ribavirin CONTRAINDICATED |
| Chronic HEV in transplant recipient | Reduce immunosuppression → Ribavirin × 12 weeks if persistent [2][5] | Check SVR (HEV RNA) 12 weeks post-treatment |
| HEV superinfection on chronic HBV | Supportive for HEV + optimize HBV antivirals | Watch for acute-on-chronic liver failure |
High Yield Summary – Management of Hepatitis E
-
Acute HEV in immunocompetent patients: No specific treatment. Supportive measures: hydration, electrolyte balance, nutritional balance [1]. No alcohol for 6 months [2][3]. No drugs or herbs hasten recovery [3].
-
Fulminant HEV: Standard ALF management — ICU care, manage complications, high-volume plasma exchange (washes away cytokines) [20], liver transplantation as final line [20].
-
Chronic HEV in immunosuppressed: Reduce immunosuppression first → Ribavirin monotherapy × 12 weeks if persistent [2][5]. SVR = absence of HEV RNA 12 weeks after stopping treatment [5].
-
Ribavirin: Nucleoside analogue; main side effect = hemolytic anemia; absolutely contraindicated in pregnancy (teratogenic).
-
Prevention: Avoid internal organs (especially pig liver), raw/undercooked meat, shellfish [2]. Hecolin® vaccine developed in China, licensed since 2011, NOT available in HK [1]. No passive immunoprophylaxis [2][5].
-
Pregnancy paradox: HEV most dangerous in pregnant women (20–25% mortality) but the only antiviral (ribavirin) is contraindicated → management entirely supportive.
Active Recall - Management of Hepatitis E
References
[1] Lecture slides: GC 239. Viral hepatitis HAV_HBV_HCV_HEV.pdf (p.17, p.21, treatment and prevention) [2] Senior notes: Maksim Medicine Notes.pdf (Viral hepatitis overview and management, p.141) [3] Senior notes: Block A - Jaundice after raw oysters_ acute hepatitis.pdf (p.1, p.3, management of symptomatic hepatitis) [5] Senior notes: MBBS Final MB (Medicine) (Felix PY Lai).pdf (p.769–771, treatment, complications, prevention) [20] Senior notes: Block A - A jaundiced and incoherent patient_ liver failure.pdf (p.24, ALF management principles)
Complications of Hepatitis E
The complications of HEV span a wide spectrum — from benign, self-resolving cholestasis through to fulminant hepatic failure and death. The likelihood and type of complication depend heavily on the host (immunocompetent vs. immunosuppressed, pregnant vs. non-pregnant, pre-existing liver disease vs. healthy liver). Understanding why each complication occurs requires linking back to the pathophysiology covered earlier.
1. Hepatic Complications
Cholestatic hepatitis [5]:
- Characterized by marked jaundice, pruritus, fever, weight loss, diarrhea and malaise [5]
- Resolves spontaneously within weeks to months with no sequelae [5]
- Recovery is marked by viral clearance with an increase in IgG and decrease in IgM levels [5]
Pathophysiology: Hepatocyte swelling from inflammation compresses intrahepatic bile canaliculi → intrahepatic cholestasis → bile acids cannot be excreted into the gut → accumulate in blood → deposit in skin (pruritus), spill into urine (dark urine), fail to reach the colon (pale stools). Conjugated bilirubin refluxes into the bloodstream → deep jaundice.
HEV has a notably higher proportion with prominent cholestasis (~20%) compared to HAV (< 5%) [4]. This means a patient with HEV may have prolonged jaundice lasting weeks to months even after the transaminases have normalized — this is the "cholestatic phase" [3]. Bilirubin may be elevated for a long time even after clinical and essential histological recovery [3]. This can be alarming to patients and trainees but is benign and self-resolving — you do NOT need to investigate further if the clinical trajectory is otherwise improving.
Clinical Pearl – Prolonged Jaundice After HEV
If a patient with confirmed HEV has persistent jaundice for weeks after ALT has normalized, do not panic. This is the cholestatic phase — bilirubin clears more slowly than transaminases. The key reassurance is that synthetic function markers (INR, albumin) are stable or improving. If they are deteriorating, then reconsider the diagnosis or worry about a second pathology.
Acute liver failure [5]:
- Characterized by hepatic encephalopathy, elevated AST, ALT, ALP and bilirubin, impaired synthetic function with INR ≥ 1.5 [5]
- Fulminant Hepatitis E is actually very rare [13], but when it occurs, it is devastating
Definition of ALF (from the HK internal medicine handbook) [21]:
- A severe liver injury (coagulopathy with INR ≥ 1.5)
- With onset of hepatic encephalopathy within 26 weeks of the first symptoms
- In the absence of pre-existing liver disease
Who is at risk of fulminant HEV?
| Population | Risk | Mechanism |
|---|---|---|
| Pregnant women (3rd trimester) | 20–25% maternal mortality [1][5] | Kupffer cell damage → endotoxin-mediated injury; pregnant women more sensitive to endotoxin effects [4]; Th2-dominant immune state impairs viral clearance |
| Patients with pre-existing chronic liver disease | Can develop severe liver consequences [1] | Reduced hepatic reserve — acute injury on already-compromised liver → acute-on-chronic liver failure |
| General population | 1–2% mortality [5] | Overwhelmingly self-limiting; fulminant failure in immunocompetent non-pregnant patients is rare |
Complications within ALF (these are the organ-specific complications that develop once fulminant hepatitis occurs) [20]:
| Complication | Mechanism | Clinical Manifestation |
|---|---|---|
| Hepatic encephalopathy | Hepatocyte mass loss → impaired ammonia clearance → ammonia crosses BBB → astrocyte swelling (glutamine synthesis from ammonia + glutamate) → cerebral edema | Confusion, asterixis (flapping tremor), fetor hepaticus → coma |
| Coagulopathy | Loss of synthetic function → reduced Factors II, VII, IX, X, fibrinogen | Bleeding (GI hemorrhage, mucosal bleeding, hematomas). INR ≥ 1.5 is the defining threshold [5][21] |
| Infections / Sepsis | Reticuloendothelial dysfunction and reduced opsonization [20] → impaired Kupffer cell function → reduced complement and immunoglobulin production | Bacteria especially from respiratory and urinary tract — Staph, Strep, gram-negative rods. Bacteremia in up to 25% of fulminant hepatic failure patients. Fungal infection especially Candida [20] |
| Hypoglycemia | Loss of glycogen stores + impaired gluconeogenesis | Neuroglycopenia → seizures, coma (why H'stix BD is essential) |
| Hepatorenal syndrome | Splanchnic vasodilation → reduced effective arterial blood volume → renal vasoconstriction → functional renal failure | Oliguria, rising creatinine, low urine sodium |
| Cerebral edema | Ammonia-induced astrocyte swelling → increased intracranial pressure | Papilledema, posturing, brainstem herniation |
| Metabolic acidosis | Impaired hepatic lactate clearance + tissue hypoperfusion | Low pH, high lactate, compensatory hyperventilation |
| Multi-organ failure | End-stage of systemic inflammatory cascade | Cardiovascular collapse, ARDS, DIC |
The LFT in fulminant hepatitis shows a paradoxical finding: reductions in AST/ALT as disease progresses [3] — not because the liver is recovering, but because there are so few remaining viable hepatocytes to release enzymes. The "burnt-out liver" phenomenon. You differentiate this from true recovery by looking at synthetic function: if AST/ALT is falling along with a fall in PT/INR, the patient is probably improving. If AST/ALT is falling with worsening and prolongation of PT, keep fulminant hepatitis in mind [3].
High Yield – The Paradox of Falling ALT in Severe Hepatitis
A falling ALT does NOT always mean improvement. In fulminant hepatitis, ALT falls because hepatocytes have been destroyed to the point where there are insufficient cells left to release enzymes. The critical discriminator: check INR alongside ALT. Falling ALT + improving INR = recovery. Falling ALT + worsening INR = catastrophe [3]. This is one of the most important clinical correlations in hepatology.
- The patient's disease course was complicated with GI bleeding → fulminant hepatic failure [13]
- GI bleeding in acute HEV occurs due to:
- Coagulopathy — impaired clotting factor synthesis → cannot form adequate clots
- Portal hypertension (if underlying cirrhosis) → variceal bleeding
- Stress ulceration — critically ill patients in ICU develop stress-related mucosal disease
- GI bleeding accelerates liver failure by increasing ammonia load (blood protein in the gut is broken down by bacteria → ammonia absorbed → worsens encephalopathy)
Chronic hepatitis E [5]:
- Defined empirically as detection of HEV RNA in serum or stool > 6 months [5]
- Exclusively occurs in settings of immunosuppression, especially transplant recipients [5]
- Immunosuppressed patients can develop chronic hepatitis E [1]
Pathophysiology: In immunocompetent individuals, CD8+ cytotoxic T cells clear HEV-infected hepatocytes within weeks. In immunosuppressed patients (especially those on calcineurin inhibitors like tacrolimus for organ transplant), T-cell function is impaired → virus persists → chronic inflammation → progressive fibrosis → cirrhosis.
Key data points:
- First report of 14 cases of acute HEV in transplant patients; 8 became chronic with raised ALT and positive HEV RNA [3][4]
- Recent report of 5 cases of post-transplant chronic HEV in HK (2018) identified as rat HEV [3]
- Typically occurs with HEV genotype 3 infection [4]
Consequences of chronic HEV:
| Consequence | Timeline | Mechanism |
|---|---|---|
| Persistent hepatitis | Months | Ongoing immune-mediated hepatocyte damage (though attenuated compared to acute) |
| Progressive fibrosis | Months to years | Chronic inflammation activates hepatic stellate cells → collagen deposition → fibrosis |
| Cirrhosis | Can develop rapidly (within 1–2 years) | Sustained fibrosis → architectural distortion → regenerative nodules → cirrhosis |
| Decompensated cirrhosis | Late | Ascites, variceal bleeding, hepatic encephalopathy, hepatorenal syndrome |
The speed of progression to cirrhosis in chronic HEV is notably faster than in chronic HBV or HCV. This is because the immunosuppressed host cannot mount any effective antiviral response, so viral replication continues unchecked at high levels, causing relentless hepatocyte injury and stellate cell activation.
This is a critically important complication in the Hong Kong context, where chronic HBV prevalence is ~8%.
Scenario: A patient with compensated chronic HBV cirrhosis acquires acute HEV infection → the acute insult (HEV) on a chronically damaged liver causes decompensation → ACLF.
Superinfection with other viral agents, especially HAV and HEV, is an important cause of hepatitis flare-up in chronic HBV carriers. HEV is more common now [6].
Pathophysiology: The liver in compensated cirrhosis operates near its functional limit. Portal hypertension is present but compensated. When HEV causes additional hepatocyte necrosis:
- The remaining functional hepatocyte mass drops below the critical threshold
- Portal pressure rises further
- Synthetic function collapses (INR rises, albumin drops)
- Ammonia clearance fails (encephalopathy)
- Splanchnic vasodilation worsens (renal failure)
Prognostic factors for ACLF depend on 6 organ-specific factors: cerebral (HE), respiration (SaO2/FiO2), circulation (need for vasopressor), liver (bilirubin level), coagulation (INR level), kidney (creatinine level) [20].
4. Extrahepatic Complications
These are increasingly recognized and are more common in HEV than in HAV. They are thought to result from either direct viral tissue tropism (HEV RNA found in non-hepatic tissues) or immune-mediated mechanisms (molecular mimicry, immune complex deposition).
| Complication | Mechanism | Notes |
|---|---|---|
| Guillain-Barré syndrome (GBS) [5] | Molecular mimicry — anti-HEV antibodies cross-react with gangliosides on peripheral nerve myelin → autoimmune demyelination | HEV genotype 3 is the most common infectious trigger for neuralgic amyotrophy in Europe |
| Meningoencephalitis [5] | Direct viral neurotropism (HEV RNA detected in CSF) and/or immune-mediated | Presents with headache, confusion, seizures, focal neurological deficits |
| Transverse myelitis [5] | Immune-mediated demyelination of the spinal cord | Acute back pain, bilateral lower limb weakness, sensory level, bladder dysfunction |
| Cranial nerve palsy [5] | Direct viral involvement or immune complex deposition affecting cranial nerves | Various presentations depending on which nerve is affected |
| Peripheral neuropathy [5] | Axonal damage from immune-mediated or direct viral mechanisms | Distal sensory loss, weakness |
| Aseptic meningitis [5] | Meningeal inflammation without bacterial infection — viral or immune-mediated | Headache, photophobia, neck stiffness; CSF shows lymphocytic pleocytosis |
Neurological complications are the most clinically significant extrahepatic manifestations of HEV. The key teaching point: if a patient develops GBS or neuralgic amyotrophy, check HEV serology — it may be the unrecognized trigger. This is frequently tested.
| Complication | Mechanism |
|---|---|
| Glomerulonephritis [5] | Immune complex deposition (HEV antigen–antibody complexes) in glomerular basement membrane → complement activation → inflammatory damage. Can present as membranoproliferative or membranous GN |
- Presents with proteinuria, hematuria, rising creatinine
- Usually resolves with viral clearance but may become chronic in immunosuppressed patients with persistent HEV viremia
| Complication | Mechanism |
|---|---|
| Thrombocytopenia [5] | Immune-mediated platelet destruction (anti-platelet antibodies); sequestration if splenomegaly; DIC in severe disease |
| Hemolysis [5] | Autoimmune hemolytic anemia (immune complex–mediated) or direct viral effect on erythrocyte membrane |
| Aplastic anemia [5] | T-cell-mediated destruction of hematopoietic stem cells triggered by HEV infection; similar mechanism to post-hepatitis aplastic anemia seen with other hepatitis viruses |
Hepatitis is well known to precede aplastic anemia [22]. The mechanism: viral hepatitis (including HEV) triggers an aberrant T-cell response that cross-reacts with bone marrow hematopoietic stem cells → pancytopenia. This is not a direct cytopathic effect of the virus on the marrow but an immune-mediated phenomenon.
| Complication | Mechanism |
|---|---|
| Acute pancreatitis [5] | Possibly direct viral invasion of pancreatic acinar cells (HEV RNA has been detected in pancreatic tissue) or immune-mediated inflammation |
- Presents with epigastric pain radiating to back, elevated amylase/lipase
- Usually mild and self-limiting
| Complication | Mechanism |
|---|---|
| Acute thyroiditis [5] | Immune-mediated inflammation of thyroid gland triggered by HEV infection |
| Complication | Mechanism |
|---|---|
| Fetal loss / Stillbirth | Maternal systemic inflammation → placental insufficiency; direct transplacental viral transmission to fetus |
| Premature delivery | Maternal illness severity triggers premature labor |
| DIC (Disseminated Intravascular Coagulation) | Massive hepatocyte necrosis → release of tissue factor → activation of coagulation cascade → consumptive coagulopathy |
| Maternal death | Fulminant liver failure, DIC, multi-organ failure. 20% maternal mortality [5] |
- Anti-HEV antibodies do not confer 100% immunity — reinfection is still possible (given the multiple genotypes + antibodies don't last that long) [3]
- This is a complication in the broader epidemiological sense: unlike HAV (where one infection = lifelong immunity), patients who recover from HEV can be reinfected with a different genotype or even the same genotype as antibody titers wane
- Long-term immunity after recovery from acute hepatitis E [1] is mentioned in the GC slide, but this is not absolute — it is partial and wanes over time, and cross-genotype protection is incomplete
| Population | Mortality | Key Determinant |
|---|---|---|
| General (immunocompetent, non-pregnant) | 1–2% [5] | Self-limiting in > 95% |
| Pregnant women | 20% [5] (up to 25% in 3rd trimester [1]) | Fulminant liver failure |
| Pre-existing CLD | Higher than general (exact figure varies) | Acute-on-chronic liver failure |
| Immunosuppressed (chronic HEV) | Variable; dependent on recognition and treatment | Progression to cirrhosis if untreated |
High Yield Summary – Complications of Hepatitis E
Hepatic Complications:
- Cholestatic hepatitis (~20%) — marked jaundice, pruritus; resolves spontaneously; bilirubin may remain elevated long after ALT normalizes (cholestatic phase).
- Acute liver failure — rare overall but devastating; defined by INR ≥ 1.5 + encephalopathy. Risk: pregnant women (20–25% mortality), pre-existing CLD.
- Chronic HEV — HEV RNA > 6 months; only in immunosuppressed (transplant, HIV); genotype 3; can progress rapidly to cirrhosis.
Key ALF Complication: Falling ALT + worsening INR = "burnt-out liver" = worse prognosis (distinguish from falling ALT + improving INR = recovery).
Extrahepatic: Neurological (GBS, meningoencephalitis, transverse myelitis), renal (glomerulonephritis), hematological (thrombocytopenia, hemolysis, aplastic anemia), pancreatic, thyroid.
ACLF: HEV superinfection on chronic HBV (common in HK) → acute-on-chronic liver failure. Always check anti-HEV IgM in HBV carriers with acute flares.
Obstetric: Fetal loss, premature delivery, DIC, maternal death.
Reinfection: Possible — antibodies do not confer permanent immunity.
Active Recall - Complications of Hepatitis E
References
[1] Lecture slides: GC 239. Viral hepatitis HAV_HBV_HCV_HEV.pdf (p.17, p.21) [3] Senior notes: Block A - Jaundice after raw oysters_ acute hepatitis.pdf (p.2, p.3, p.20, p.21) [4] Senior notes: Ryan Ho GI.pdf (p.235, HEV section) [5] Senior notes: MBBS Final MB (Medicine) (Felix PY Lai).pdf (p.769–771, complications and prognosis) [6] Senior notes: Block A - I am a hepatitis B carrier.pdf (p.28, HEV superinfection) [13] Senior notes: Block A - Introduction to GI_Hepatology investigations (LFT, Endoscopy).pdf (p.15, fulminant HEV case) [20] Senior notes: Block A - A jaundiced and incoherent patient_ liver failure.pdf (p.12, p.24, ALF complications and ACLF prognostic factors) [21] Lecture slides: Handbook of Internal Medicine 2024.pdf (p.88, ALF definition) [22] Senior notes: Block A - Hematology Data Interpretation.pdf (p.1, hepatitis preceding aplastic anemia)
High Yield Summary
Definition: HEV is a non-enveloped ssRNA virus (family Hepeviridae) causing acute viral hepatitis, transmitted fecal-orally and zoonotically.
Epidemiology: 20M cases/year globally; HK seroprevalence ~33.6%; genotypes 3 and 4 predominate locally (zoonotic from pigs).
Key Risk Factors: Raw/undercooked pork liver (HK classic), contaminated water, shellfish; pregnancy (3rd trimester), immunosuppression, pre-existing CLD.
Pathophysiology: Immune-mediated hepatocyte injury (not direct cytopathic); Kupffer cell damage → endotoxin-mediated injury (explains pregnancy severity); impaired T-cell clearance in immunosuppressed → chronicity.
Clinical Features: Pre-icteric (fever, anorexia, fatigue, diarrhea, RUQ ache, dark urine, pruritus) → Icteric (jaundice, pale stools) → Convalescent. Notable for prominent cholestasis (~20%), extrahepatic manifestations (neuro, renal, hematological).
Critical Differentiators from HAV: Zoonotic reservoir, longer incubation, NO lifelong immunity, CAN cause chronic infection (immunosuppressed), HIGHER mortality (1–2% baseline, 20–25% in pregnancy), MORE cholestatic.
Hong Kong Pearls: "Pork liver congee virus"; rat-HEV first reported in HK; superinfection on chronic HBV is important; all viral hepatitis are notifiable.
High Yield Summary – DDx of Hepatitis E
-
The clinical presentation of HEV is indistinguishable from other causes of acute hepatitis — diagnosis depends on serology (anti-HEV IgM) and clinical context (exposure history).
-
In Hong Kong, always consider: (a) HEV superinfection on chronic HBV, (b) dual liver disease (HBV + MASLD + HEV), (c) rat-HEV in immunosuppressed patients.
-
Key DDx categories: viral (HAV, HBV, HCV, EBV, CMV), drugs/toxins (paracetamol, DILI, TCM, alcohol), autoimmune, metabolic (Wilson), vascular (ischaemic hepatitis, Budd-Chiari), pregnancy-related (HELLP, AFLP).
-
LFT pattern helps but is not diagnostic: viral hepatitis gives ALT-predominant rise; alcoholic hepatitis gives AST:ALT > 2:1 with AST < 500; ischaemic hepatitis gives rapid rise and fall with elevated LDH.
-
History is king: food exposure (pork liver → HEV, shellfish → HAV), drug history (paracetamol, TCM), alcohol, pregnancy, hemodynamic status (shock → ischaemic hepatitis), immunosuppression (chronic HEV risk).
High Yield Summary – Diagnosis of Hepatitis E
-
First-line diagnostic test: Anti-HEV IgM — positive in > 90% of acute cases, coincides with symptom onset, persists ~3 months.
-
Confirmatory test: HEV RNA RT-PCR (serum or stool) — essential when IgM negative but suspicion high, and mandatory for diagnosing chronic HEV.
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GC lecture algorithm: Markedly elevated ALT and AST → Specific serological tests → Exclusion of other causes [17]. HEV RNA is the final step when serology is negative.
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Exclusion panel: HBsAg, anti-HAV IgM, anti-HCV, autoantibodies (ANA, anti-SMA, anti-LKM-1), ultrasound, toxicology screen/paracetamol level.
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LFT pattern: ALT-predominant rise (ALT > AST), total bilirubin elevated, ± prominent cholestasis (~20%). INR is the critical synthetic function marker.
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Monitoring: DAT, Obs Q4h, H'stix BD, daily CBC/LRFT/INR/NH3.
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Anti-HEV IgG indicates past infection, NOT acute — does not confer permanent immunity, reinfection possible.
High Yield Summary – Management of Hepatitis E
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Acute HEV in immunocompetent patients: No specific treatment. Supportive measures: hydration, electrolyte balance, nutritional balance [1]. No alcohol for 6 months [2][3]. No drugs or herbs hasten recovery [3].
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Fulminant HEV: Standard ALF management — ICU care, manage complications, high-volume plasma exchange (washes away cytokines) [20], liver transplantation as final line [20].
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Chronic HEV in immunosuppressed: Reduce immunosuppression first → Ribavirin monotherapy × 12 weeks if persistent [2][5]. SVR = absence of HEV RNA 12 weeks after stopping treatment [5].
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Ribavirin: Nucleoside analogue; main side effect = hemolytic anemia; absolutely contraindicated in pregnancy (teratogenic).
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Prevention: Avoid internal organs (especially pig liver), raw/undercooked meat, shellfish [2]. Hecolin® vaccine developed in China, licensed since 2011, NOT available in HK [1]. No passive immunoprophylaxis [2][5].
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Pregnancy paradox: HEV most dangerous in pregnant women (20–25% mortality) but the only antiviral (ribavirin) is contraindicated → management entirely supportive.
High Yield Summary – Complications of Hepatitis E
Hepatic Complications:
- Cholestatic hepatitis (~20%) — marked jaundice, pruritus; resolves spontaneously; bilirubin may remain elevated long after ALT normalizes (cholestatic phase).
- Acute liver failure — rare overall but devastating; defined by INR ≥ 1.5 + encephalopathy. Risk: pregnant women (20–25% mortality), pre-existing CLD.
- Chronic HEV — HEV RNA > 6 months; only in immunosuppressed (transplant, HIV); genotype 3; can progress rapidly to cirrhosis.
Key ALF Complication: Falling ALT + worsening INR = "burnt-out liver" = worse prognosis (distinguish from falling ALT + improving INR = recovery).
Extrahepatic: Neurological (GBS, meningoencephalitis, transverse myelitis), renal (glomerulonephritis), hematological (thrombocytopenia, hemolysis, aplastic anemia), pancreatic, thyroid.
ACLF: HEV superinfection on chronic HBV (common in HK) → acute-on-chronic liver failure. Always check anti-HEV IgM in HBV carriers with acute flares.
Obstetric: Fetal loss, premature delivery, DIC, maternal death.
Reinfection: Possible — antibodies do not confer permanent immunity.