What are the symptoms of autoimmune hepatitis (AIH)?

Symptoms of Autoimmune Hepatitis

Autoimmune hepatitis (AIH) typically presents with nonspecific fatigue as the most common symptom, though approximately 25-37% of patients are asymptomatic at diagnosis despite having elevated liver enzymes and potentially advanced disease. 1, 2

Common Symptoms

• Fatigue is the predominant complaint in approximately 85% of patients with AIH 3, 2
• General ill health and malaise are frequently reported 1, 2
• Anorexia (loss of appetite) and weight loss are common symptoms 1
• Nausea is often a prominent symptom 1, 2
• Right upper quadrant or epigastric abdominal pain may occur 3, 2
• Joint pains (polyarthralgia) affecting small joints without arthritis occur in 30-60% of patients 1, 2
• Amenorrhea is common in women 1
• Jaundice may be present, especially in acute presentations 1, 2

Clinical Presentation Patterns

Asymptomatic Presentation

• 25-37% of patients are asymptomatic at diagnosis, discovered incidentally through routine blood tests 1
• Asymptomatic patients have significantly lower levels of liver enzyme elevation and immunoglobulin G (IgG) 1
• Symptoms may develop in approximately 25.8% of initially asymptomatic patients within an average of 2 years 1
• Despite being asymptomatic, these patients may already have advanced fibrosis or cirrhosis 1, 2

Insidious Chronic Presentation

• Most common presentation pattern with gradual onset of symptoms 1
• Characterized by persistent fatigue, malaise, and nonspecific symptoms 1, 2
• May have been present for months or years before diagnosis 1
• Laboratory findings include hypergammaglobulinemia and autoantibodies 1

Acute Presentation

• Approximately 25% of AIH patients present with acute hepatitis symptoms 1, 2
• May mimic viral or toxic hepatitis with jaundice 1, 2
• Often preceded by anorexia, nausea, and influenza-like symptoms 1
• Serum AST/ALT levels may be markedly elevated (several thousands) 1

Acute Severe/Fulminant Presentation

• 3-6% of patients present with acute liver failure 1, 2
• Defined as jaundice with prothrombin time INR ≥2 or development of hepatic encephalopathy 1
• In acute severe AIH, 29-39% of patients may have negative or weakly positive autoantibodies 1
• Normal serum IgG levels are found in 25-39% of acute severe cases 1, 2

Advanced Disease Manifestations

• Approximately 25-33% of patients have cirrhosis at the time of diagnosis regardless of symptoms 1
• Cirrhosis may present with:
  • Ascites (fluid accumulation in abdomen) 3
  • Spider nevi and palmar erythema (skin manifestations) 3
  • Hepatomegaly (enlarged liver) and splenomegaly (enlarged spleen) 3, 2
  • Esophageal varices which may bleed 3
  • Hepatic encephalopathy (confusion, altered mental status) 1, 3

Associated Conditions

• AIH is frequently associated with other autoimmune diseases in 14-44% of patients 2
• Common associated conditions include:
  • Autoimmune thyroiditis 2
  • Graves' disease 2
  • Synovitis 2
  • Ulcerative colitis 2
  • Type 1 diabetes mellitus 2
  • Vitiligo 2

Clinical Pitfalls

• The absence of symptoms does not exclude significant liver disease - 25-37% of patients are asymptomatic despite having elevated liver enzymes 1
• In acute severe presentations, autoantibodies may be negative or weakly positive in 29-39% of cases 1
• Normal IgG levels can be found in 25-39% of acute severe cases 1
• Low-grade fever and rash are less common symptoms 1
• AIH should be considered in the differential diagnosis of most liver diseases regardless of the degree of activity or fibrosis 1