What are the symptoms of autoimmune hepatitis?

Symptoms of Autoimmune Hepatitis

Autoimmune hepatitis presents with a broad clinical spectrum ranging from asymptomatic disease (25-37% of patients) to nonspecific symptoms like fatigue and jaundice, to acute severe hepatitis or fulminant liver failure, with approximately one-third of patients already having cirrhosis at diagnosis. 1, 2

Most Common Symptoms

The typical presentation includes nonspecific symptoms that may have been present for months to years:

• Fatigue is the predominant complaint, occurring in approximately 85% of symptomatic patients 1, 2
• General malaise and ill health are frequently reported 1, 2
• Right upper quadrant abdominal pain is a common complaint 1, 2
• Fluctuating jaundice is characteristic of the disease 1, 2
• Anorexia and weight loss occur frequently 1, 2
• Nausea is a common symptom 1
• Polyarthralgia involving small joints without arthritis may be present 1, 2
• Amenorrhea occurs in women with AIH 1, 2
• Pruritus may be present, though its presence is inconsistent with the diagnosis according to some sources 1

Asymptomatic Presentation (Critical Pitfall)

A substantial proportion of patients (25-37%) are completely asymptomatic at diagnosis, which can lead to delayed recognition and treatment. 1, 2 This is a critical diagnostic pitfall because:

• Asymptomatic patients have histological findings similar to symptomatic patients 1
• They may develop symptoms within an average of 2 years after diagnosis 1, 2
• They have lower levels of liver enzyme elevation and IgG compared to symptomatic patients 1, 2
• Their 10-year survival may be less than that of treated symptomatic patients (67% versus 98%) 1
• The absence of symptoms should not discourage treatment 1

Acute Presentation (25% of Cases)

Approximately 25% of patients present with acute onset symptoms mimicking viral or toxic hepatitis: 1, 2

• Duration of symptoms less than 30 days 1
• Acute hepatitis-like presentation that can be confused with other causes 1
• Critical diagnostic challenge: In acute severe AIH, autoantibodies (ANA) are absent or weakly positive in 29-39% of patients 1, 2
• Serum IgG levels are normal in 25-39% of acute severe cases 1, 2
• This represents either acute exacerbation of chronic AIH or genuine acute AIH without chronic histological changes 1

Fulminant Presentation (3-6% of Cases)

The most severe presentation includes: 1, 2

• Jaundice with prothrombin time INR ≥2 or hepatic encephalopathy 1
• Acute liver failure requiring urgent consideration for liver transplantation 1
• Heterogeneous hypo-attenuated regions on unenhanced CT scan in 65% of cases 1, 2
• Delay in diagnosis and treatment initiation results in poorer prognosis 1

Physical Examination Findings

Physical signs vary depending on disease stage:

Early disease:

• Physical findings may be completely normal 1
• Hepatomegaly, occasionally painful 1, 2
• Splenomegaly 1, 2

Advanced disease with cirrhosis:

• Spider nevi 1, 2
• Palmar erythema 1, 2
• Caput medusa 1, 2
• Ascites 1, 2
• Signs of portal hypertension 1

Associated Autoimmune Conditions (14-44% of Patients)

Concurrent autoimmune diseases are present in 14-44% of AIH patients and should be actively sought: 1, 2

• Autoimmune thyroid disease (thyroiditis, Graves' disease) 2
• Inflammatory bowel disease (ulcerative colitis) 1, 2
• Type 1 diabetes mellitus 2
• Vitiligo 1, 2
• Synovitis 2
• Celiac disease (2.8-3.5% frequency, higher than general population) 1

Age-related patterns of associated conditions:

• Patients ≥60 years have more autoimmune thyroid and rheumatic diseases than adults ≤30 years (42% versus 13%) 1, 2
• Young adults more often have inflammatory bowel disease and autoimmune hemolytic anemia (13% versus 0%) 1, 2

Critical Clinical Pitfalls to Avoid

1. Do not exclude AIH based on absence of symptoms - 25-37% are asymptomatic 1, 2

2. Do not exclude AIH based on negative autoantibodies in acute presentation - 29-39% of acute severe cases have negative or weakly positive ANA/SMA 1, 2

3. Do not exclude AIH based on normal IgG levels in acute cases - 25-39% of acute severe cases have normal IgG 1, 2

4. Do not dismiss the diagnosis if cirrhosis is already present - 25-33% of patients have cirrhosis at diagnosis regardless of symptoms 1, 2

5. Presence of pruritus or hyperpigmentation is inconsistent with AIH diagnosis and should prompt consideration of other conditions 1

6. Weight loss suggests a serious complication such as malignancy rather than uncomplicated AIH 1

Rare Features

• Maculopapular skin rash is rare 1
• Unexplained low-grade fever is rare 1