• Chronic inflammatory liver disease
• Immune-mediated damage to liver cells
• Primarily affects young females

• Increased serum transaminases
• Elevated immunoglobulin G
• Inflammatory liver histology
• Presence of circulating autoantibodies

• Complement allele C4AQO
• HLA haplotypes B8, B14, DR3, DR4, Dw3
• Antinuclear antibody (ANA)
• Anti-smooth muscle antibody (ASMA)
• Anti-liver-kidney microsomal-1 (anti-LKM-1) antibody
• Antibodies against soluble liver antigen (anti-SLA)
• Antimitochondrial antibody (AMA)
• Antiphospholipid antibodies

• Type 1: Presence of ASMA or ANA (75% of patients)
• Type 2: Presence of anti-LKM-1 or anti-LC-1 antibodies

• Affects young girls from infancy to late adolescence
• Active liver damage with high aminotransferase activity
• High immunoglobulin G levels
• Interface hepatitis on liver biopsy

• Type I: Associated with Anti-nuclear antibodies (ANA) and/or anti-smooth muscle antibodies (SMA); affects adults and children.
• Type II: Associated with Anti-liver/kidney microsomal type 1 antibodies (LKM1); affects children only.
• Type III: Associated with Soluble liver-kidney antigen; affects middle-aged adults.

• Genetic predisposition: Certain genetic factors may increase susceptibility to autoimmune diseases including AIH.
• Immunological dysregulation: Leads to the immune system attacking healthy liver cells.
• Environmental triggers: Viral infections, exposure to medications, chemicals, or toxins.

• Family history of autoimmune diseases
• More common in women
• Genetic predisposition (HLA B8, DR3)
• Exposure to medications, chemicals, or toxins

• Autoimmune thyroid disorders: Graves’ disease, thyroiditis
• Haematological disorders: Anaemias, thrombocytopenia
• Gastrointestinal disorders: Crohn’s, ulcerative colitis, coeliac
• Kidney disorders: Proliferative glomerulonephritis
• Respiratory conditions: Fibrosing alveolitis
• Heart conditions: Pericarditis, myocarditis
• Endocrine disorders: Type 1 Diabetes, thyroiditis
• Rheumatological conditions: Rheumatoid arthritis, Sjögren’s, systemic sclerosis
• Skin conditions: Erythema nodosum, lichen planus
• Eye inflammation: Uveitis

• Immune system attacks the liver causing inflammation and destruction
• Produces autoantibodies against liver cells
• Untreated AIH can lead to liver fibrosis and cirrhosis

• Genetic predisposition
• Environmental influences
• Immune dysregulation
• Exact mechanisms are not fully known but involve immune dysregulation

• Viral hepatitis: Hepatitis B and C
• Alcoholic liver disease
• Drug-induced liver injury
• Primary biliary cholangitis
• Primary sclerosing cholangitis
• Metabolic liver diseases

• Prevalence: Approximately 1 in 100,000 individuals
• Higher prevalence in women
• Can manifest at any age but more common in young to middle-aged adults

• Global Prevalence: 10-17 cases per 100,000 individuals in Europe
• Occurs worldwide with likely underestimated prevalence
• Commonly affects young to middle-aged women but can occur in both genders and all age groups

• Fatigue
• Jaundice (in around 50% of patients)
• Upper abdominal discomfort
• Hepatomegaly (enlarged liver)
• Elevated liver enzymes
• Symptoms of systemic inflammation (e.g., joint pain, skin rashes, fever)

• Acute hepatitis presentation: fever, jaundice (in 25% of cases)
• Amenorrhoea
• Laboratory findings: ANA/SMA/LKM1 antibodies, raised IgG levels

• Inflammation extending beyond the limiting plate
• Piecemeal necrosis
• Bridging necrosis

• Can present as acute, severe, asymptomatic, or chronic
• Subclinical disease often precedes symptoms
• Additional symptoms may include myalgia, pruritus, nausea, arthralgias, skin rashes, hirsutism, edema, chest pain, weight loss, and intense pruritus

• Hepatomegaly
• Jaundice
• Splenomegaly
• Spider angiomata
• Ascites
• Encephalopathy

• Liver function tests: Elevated AST and ALT, normal or mildly raised alkaline phosphatase
• Autoantibodies: ANA, ASMA, LKM-1
• Serum protein electrophoresis and immunoglobulins: Elevated IgG
• FBC and blood film: May show mild leukopenia, normochromic anemia, thrombocytopenia, eosinophilia
• Hypoalbuminemia and prothrombin time: Indicate severe hepatic dysfunction
• Blood markers: Elevated CRP and ESR

• Used to rule out hepatocellular carcinoma
• May suggest inflammation, necrosis, or cirrhosis
• Nodular liver on imaging may indicate cirrhosis

• Most important diagnostic tool
• Provides prognostic information
• Enables early treatment initiation
• Detects cirrhosis or bridging necrosis which indicate a poorer prognosis

• Combination of biochemical, immunological, and histological features
• Autoantibodies, liver enzyme levels, and liver biopsy findings are key
• Additional tests to rule out other diagnoses and assess disease severity

• Immunosuppressive therapy.

• Corticosteroids (e.g., prednisolone) for induction.
• Azathioprine or mycophenolate mofetil for maintenance.

• Severe liver damage.
• Treatment-resistant cases.
• Note: Recurrence of AIH can occur post-transplantation.

• Regular liver function tests and autoantibody monitoring.
• Assess hepatitis A and B immunity and administer vaccines if necessary.
• Monitor calcium and vitamin D levels with DXA scans for bone density.
• Glaucoma and cataract screening after prolonged corticosteroid use.

• Achieve biochemical remission: Normalized transaminases and IgG levels.
• Maintain remission with adjusted therapy based on clinical response.

• Generally favorable with proper treatment and adherence.
• Long-term management is necessary to maintain positive outcomes.

• 50% mortality within approximately five years.
• Progressive liver damage leading to cirrhosis, liver failure, and possibly requiring transplantation.

• Cirrhosis develops in up to 50% of AIH patients.

• Recurrent disease occurs in 10-50% of cases.

• Positive outlook with a maintenance dose of azathioprine or azathioprine with prednisolone to reduce relapse risk.
• Outcomes vary among individuals.

• Cirrhosis development
• Liver failure
• Portal hypertension
• Increased risk of hepatocellular carcinoma (liver cancer)
• Side effects of immunosuppressive medications

• Ascites
• Hepatic encephalopathy
• Variceal bleeding

• Hyperviscosity syndrome due to elevated IgG levels
• Increased risk of hepatocellular carcinoma (10-20% in cirrhosis)
• Monitoring: 6-monthly ultrasound and alpha-fetoprotein testing for cirrhosis patients