Autoimmune hepatitis history and symptoms

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]

History and Symptoms

• Patients with AH can present anywhere in the spectrum from totally asymptomatic disease (only to be diagnosed when routine liver function tests (LFTs) are abnormal), to an acute, fulminant hepatitis.
• Patients can also present with a variety of constitutional symptoms such as malaise, fatiguability, anorexia, abdominal pain and pruritis.

• Small joint arthralgia is also a common complaint.

• Extrahepatic manifestations are generally due to the company that AH keeps:

• Chronic autoimmune thyroiditis
• Hyperthyroidism (Graves’ disease)
• Ulcerative colitis
• Hemolytic anemia
• Idiopathic thrombocytopenia
• Diabetes mellitus
• Diabetes insipidus
• Celiac disease
• Polymyositis
• Myasthenia gravis
• Pulmonary fibrosis
• Pericarditis
• Glomerulonephritis
• Acute lichenoid pityriasis
• Febrile panniculitis
• Hypereosinophilic syndrome
• Sjogren’s syndrome
• Mixed connective tissue disease

• Although PSC is generally thought to be the liver disease most commonly associated with uterine contractions (UC), AH and UC often coexist, with the liver disease predating the colonic disease.
• AH is also associated with hepatocellular carcinoma, though many patients who eventually develop hepatocellular carcinoma have also been found to have hepatitis C.
• The LFT abnormalities are generally that of a marked transaminitis with a milder increase in Total bilirubin, alkalen phosphatase and PT.

• Some patients, however, have more of a cholestatic picture.

• Unfortunately, the symptoms and degree of enzyme abnormalities do not necessarily correlate with the pathology.

• As above, however, most patients have at least some degree of cirrhosis at the time of initial biopsy.

• Hyperglobulinemia (usually gamma-globulin) is a characteristic feature of AH, though is not present in 100% of cases.
• Anti-actin antibodies are generally not tested for in most labs, despite the fact that they are more specific than the other tests for AH.

• An anti-smooth muscle Ab titer > 1:320, however, is generally thought to reflect the presence of anti-actin antibodies.

• In the spectrum of autoimmune disease ranging from organ-specific to non-organ-specific, AH falls somewhere in the middle, as the disease is often confined to the liver, but the antibodies are mostly nonspecific.
• A scoring system has been developed by the International Autoimmune Hepatitis Group in order to facilitate the diagnosis.