What are the symptoms of Autoimmune Hepatitis (AIH)?

Symptoms of Autoimmune Hepatitis (AIH)

The most common symptoms of autoimmune hepatitis include fatigue, jaundice, abdominal pain, and joint pain, though approximately 25-37% of patients may be asymptomatic at diagnosis. 1

Common Presentations

Insidious Onset (Most Common)

• Fatigue is the predominant complaint in approximately 85% of patients 1
• General ill health and malaise 1
• Right upper quadrant abdominal pain 1
• Lethargy 1
• Anorexia and weight loss 1
• Nausea 1
• Fluctuating jaundice 1
• Polyarthralgia involving small joints without arthritis 1
• Amenorrhea in women 1

Asymptomatic Presentation

• Occurs in 25-37% of patients 1
• Often discovered incidentally through abnormal liver enzymes 1
• May develop symptoms within an average of 2 years after diagnosis 1
• Lower levels of liver enzyme elevation and IgG compared to symptomatic patients 1

Acute Presentation

• Occurs in approximately 25% of patients 1
• Can present as:
  • Acute exacerbation of previously undiagnosed chronic AIH 1
  • True acute AIH without chronic histological changes 1
• May mimic viral or toxic hepatitis 1
• In acute severe cases, 29-39% may have negative or weakly positive autoantibodies 1
• Normal serum IgG levels in 25-39% of acute severe cases 1

Fulminant Presentation

• Occurs in 3-6% of North American and European patients 1
• Characterized by jaundice with prothrombin time INR ≥2 or development of hepatic encephalopathy 1
• Heterogeneous hypo-attenuated regions of the liver on non-contrast CT in 65% of cases 1

Physical Examination Findings

• Physical signs are often absent in early disease 1
• Hepatomegaly, occasionally painful 1
• Splenomegaly 1
• In advanced disease/cirrhosis:
  • Spider nevi 1
  • Palmar erythema 1
  • Caput medusa (dilated periumbilical veins) 1
  • Ascites 1

Associated Conditions

• Concurrent autoimmune diseases occur in 14-44% of patients 1
• Common associated conditions:
  • Autoimmune thyroiditis 1
  • Graves' disease 1
  • Synovitis 1
  • Ulcerative colitis 1
  • Type 1 diabetes mellitus (especially in European anti-LKM1+ AIH patients) 1
  • Vitiligo 1
  • Inflammatory bowel disease 1

Special Considerations

Age-Related Differences

• Patients aged ≥60 years have more autoimmune thyroid and rheumatic diseases than adults ≤30 years (42% versus 13%) 1
• Young adults more often have inflammatory bowel disease and autoimmune hemolytic anemia (13% versus 0%) 1

Autoantibody-Negative AIH

• ANA, SMA, and anti-LKM1 are absent in 19-34% of patients initially diagnosed as cryptogenic hepatitis 1
• In acute severe AIH, autoantibodies may be absent or weakly positive in 29-39% of cases 1

Cirrhosis at Diagnosis

• Approximately 25-33% of AIH patients have liver cirrhosis at the time of diagnosis regardless of symptoms 1
• Risk factors for cirrhosis at diagnosis include male gender, black or Hispanic race, older age (≥60 years), and lower education level 1

Atypical Symptoms (Less Common)

• Pruritus (inconsistent with the diagnosis) 1
• Hyperpigmentation (inconsistent with the diagnosis) 1
• Low-grade fever (rare) 1
• Maculopapular skin rash (rare) 1

Clinical Pitfalls

• Significant weight loss may suggest a serious complication such as malignancy 1
• Presence of pruritus or hyperpigmentation is inconsistent with AIH diagnosis and should prompt consideration of other liver diseases 1
• Asymptomatic patients may still develop symptoms within months to years (range 2-120 months) 1
• Absence of symptoms should not discourage treatment, as untreated asymptomatic patients have worse 10-year survival than treated symptomatic patients (67% versus 98%) 1