Related Subjects:
|Chronic liver disease
|Cirrhosis
|Alkaline phosphatase (ALP)
|Liver Function Tests
|Ascites Assessment and Management
|Budd-Chiari syndrome
|Autoimmune Hepatitis
|Primary Biliary Cirrhosis
|Primary Sclerosing Cholangitis
|Wilson disease
|Hereditary Haemochromatosis
|Alpha-1 Antitrypsin (AAT) deficiency
|Non alcoholic steatohepatitis (NASH)
|Spontaneous Bacterial Peritonitis
|Alcoholism and Alcoholic Liver Disease
|Liver Transplantation
⚠️ Autoimmune Hepatitis (AIH) is a chronic inflammatory liver disease caused by immune-mediated attack on hepatocytes.
Frequently associated with other autoimmune conditions such as Coombs-positive haemolytic anaemia, thyroiditis, and ITP.
Untreated, AIH progresses to cirrhosis and hepatocellular carcinoma.
ℹ️ About
- Suspect in hepatitis with ⛔ negative viral serology, ⛔ no toxin history, ✅ positive autoantibodies.
- Immune dysregulation leads to interface hepatitis (piecemeal necrosis) on biopsy.
🩺 Clinical Features
- 👩 Predominantly affects young/middle-aged women.
- Fatigue, anorexia, jaundice.
- Signs of chronic liver disease: hepatomegaly, splenomegaly, spider naevi, ascites.
Types of AIH
| Features | Type 1 (Classical) | Type 2 |
| Prevalence | Most common | Less common, often aggressive |
| Age | Adults + children | Children/young adults |
| Autoantibodies |
- ANA
- Anti-smooth muscle (SMA)
- Anti-dsDNA
- ± p-ANCA
|
- Anti-LKM1 (liver kidney microsomal type 1)
|
| Presentation |
Insidious onset, chronic hepatitis |
Often acute hepatitis, esp. in girls |
| Associations |
Other autoimmune disorders (thyroid, RA, DM1) |
Target antigen = CYP2D6 |
Clinical Manifestations
- Non-specific: malaise, fatigue, jaundice, RUQ pain.
- “Serum sickness” picture: arthralgia, rash, polyarthritis, dry eyes.
- Advanced: ascites, varices, encephalopathy, dark urine & pale stools.
Associated Autoimmune Conditions
- Hashimoto’s thyroiditis
- Type 1 diabetes
- Rheumatoid arthritis
- Sjögren’s syndrome
- Vitiligo
- Autoimmune haemolytic anaemia
🔎 Investigations
- 🧪 Bloods:
- Cytopenias (anaemia, leukopenia, thrombocytopenia)
- ↑ ALT/AST, ↑ bilirubin, ± ↑ ALP
- Prolonged PT/INR
- Hypergammaglobulinaemia (↑ polyclonal IgG)
- Autoantibodies:
– Type 1 = ANA, SMA, ± anti-dsDNA, p-ANCA
– Type 2 = anti-LKM1
- 🧫 Liver Biopsy: Interface hepatitis, bridging necrosis, cirrhosis in late stages.
- 🖥️ Imaging: USS/CT/MRI – exclude other causes.
⚠️ Complications
- Progression to cirrhosis and end-stage liver disease.
- Portal hypertension & variceal bleeding.
- Hepatic encephalopathy.
- Hepatocellular carcinoma (HCC).
💊 Management
- 💊 Corticosteroids: Prednisolone 30 mg/day → taper. Effective at inducing remission.
- 💊 Immunosuppressants: Azathioprine as steroid-sparing (check TPMT activity).
Alternatives: mycophenolate, ciclosporin, tacrolimus.
- 📈 Monitoring: LFTs, IgG, antibody titres, bone density, screen for HCC with USS + AFP.
- 🩺 Liver Transplant: End-stage disease or steroid-refractory ALF. Outcomes excellent (>80% 5-yr survival).
- 💉 Vaccinate: Against hepatitis A & B to avoid co-infection.
