Autoimmune Hepatitis Clinical Presentation

Updated: Jun 11, 2019
  • Author: David C Wolf, MD, FACP, FACG, AGAF, FAASLD; Chief Editor: BS Anand, MD  more...
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Presentation

History

Clinical features of autoimmune hepatitis widely vary. Most cases have an insidious onset. Patients may be asymptomatic or have nonspecific symptoms (eg, fatigue, anorexia, weight loss, behavioral changes, amenorrhea). Systemic or cutaneous abnormalities occur in 25% of patients. Epistaxis, bleeding gums, and bruises with minimal trauma are frequent complaints.

Autoimmune hepatitis may present as acute hepatitis, chronic hepatitis, or well-established cirrhosis. Autoimmune hepatitis rarely presents as fulminant hepatic failure.

Approximately one third of patients present with symptoms of acute hepatitis marked by fever, hepatic tenderness, and jaundice. In some patients, the acute illness may appear to resolve spontaneously; however, patients invariably develop signs and symptoms of chronic liver disease. Other patients experience rapid progression of the disease to acute liver failure, as marked by coagulopathy and jaundice. Ascites and hepatic encephalopathy also may ensue.

The chronic hepatitis associated with autoimmune hepatitis may range in severity from a subclinical illness without symptoms and with abnormal results on liver chemistries to a disabling chronic liver disease. Symptoms and physical examination findings may stem from the various extrahepatic diseases associated with autoimmune hepatitis. Common symptoms include the following:

  • Fatigue

  • Upper abdominal discomfort

  • Mild pruritus

  • Anorexia

  • Myalgia

  • Diarrhea

  • Cushingoid features

  • Arthralgias

  • Skin rashes (including acne)

  • Edema

  • Hirsutism

  • Amenorrhea

  • Chest pain from pleuritis

  • Weight loss and intense pruritus (unusual)

Many patients have histologic evidence of cirrhosis at the onset of symptoms. This is true both for patients with an initial presentation of acute hepatitis and for patients with chronic hepatitis. Thus, subclinical disease often precedes the onset of symptoms.

As many as 20% of patients present initially with signs of decompensated cirrhosis. In other patients, chronic hepatitis progresses to cirrhosis after several years of unsuccessful immunosuppressant therapy marked by multiple disease relapses. This occurs in 20%-40% of patients. Patients with cirrhosis may experience classic symptoms of portal hypertension, namely variceal bleeding, ascites, and hepatic encephalopathy. Patients with complications of cirrhosis should be referred for consideration of liver transplantation.

Associated disease

Autoimmune hepatitis, especially type 2, is associated with a wide variety of other disorders. Involvement of other systems may present at disease onset or may develop during the course of the active liver disease. Most of these conditions are immunologic in origin.

Patients may present with manifestations of the following hematologic disorders:

  • Hypersplenism

  • Autoimmune hemolytic anemia

  • Coombs-positive hemolytic anemia

  • Pernicious anemia

  • Idiopathic thrombocytopenic purpura

  • Eosinophilia

Gastrointestinal disease associated with autoimmune hepatitis includes inflammatory bowel disease, which is seen in 6% of cases. The presence of ulcerative colitis in patients with autoimmune hepatitis should prompt performance of cholangiography to exclude primary sclerosing cholangitis (PSC). A study of 140 pediatric patients with autoimmune hepatitis, autoimmune cholangitis, and overlap syndrome identified 23 patients with celiac disease. [38]

Associated endocrinologic conditions include Graves disease (6%) and autoimmune thyroiditis (12%). Associated rheumatologic complications include the following:

  • Rheumatoid arthritis and Felty syndrome

  • Sjögren syndrome

  • Systemic sclerosis

  • Mixed connective-tissue disease

  • Erythema nodosum

  • Leukocytoclastic vasculitis (patients may present with leg ulcers)

Other associated conditions are as follows:

  • Proliferative glomerulonephritis

  • Fibrosing alveolitis

  • Pericarditis and myocarditis

  • Febrile panniculitis

  • Lichen planus

  • Uveitis

Pediatric presentation

In 1997, Gregorio et al published a series of 52 cases of autoimmune hepatitis in children (32 children with autoimmune hepatitis type 1 [AIH-1] and 20 children with autoimmune hepatitis type 2 [AIH-2]). [37] The following summary of clinical features of AIH was based on 20 years of treating these children at King's College Hospital.

The median patient ages were 10 years for AIH-1 and 7.4 years for AIH-2. Other autoimmune disorders occurred in 20% of patients and 40% of their relatives; these included autoimmune thyroiditis, celiac disease, inflammatory bowel disease, diabetes mellitus, and other disorders. AIH-2 can be part of autoimmune polyendocrinopathy-candidiasis-ectodermal dystrophy (APECED), an autosomal recessive genetic disorder in which liver disease is reportedly present in about 20% of cases. [39]

In 50% of the children, an acute presentation mimicked acute viral hepatitis (ie, abdominal discomfort, vomiting, nausea, jaundice). Fulminant hepatic failure occurred in 11% of the children and was more common in patients with AIH-2. Insidious presentation was characterized by intermittent jaundice or nonspecific symptoms. Routine blood analysis revealed incidental findings of abnormal liver enzymes. Patients with autoimmune hepatitis developed cirrhosis and portal hypertension.

In 2005, Oettinger et al published a series of 142 children with autoimmune hepatitis. [1] Clinical findings included the following:

  • Jaundice (58%)

  • Nonspecific weakness (57%)

  • Anorexia (47%)

  • Abdominal pain (38%)

  • Pallor (26%)

AIH-1 was found in 73% of the children, AIH-2 was found in 25% of the children, and 4 children could not be classified. Liver biopsy showed active hepatitis (52%), cirrhosis (38%), and mild inflammatory activity (10%).

Additional autoimmune disorders often occur in children with autoimmune hepatitis. In children with AIH-1, associated autoimmune disorders include the following:

  • Ulcerative colitis

  • Sclerosing cholangitis

  • Arthritis

  • Vasculitis

  • Glomerulonephritis

  • Diabetes mellitus

In children with AIH-2, associated autoimmune disorders include the following:

  • Polyendocrinopathy

  • Alopecia areata

  • Diabetes mellitus

  • Thyroiditis

Acute liver failure occurs primarily between the ages of 13 months and 4 years in children with AIH-2. It typically occurs after puberty in patients with AIH-1.

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Physical Examination

Common findings on physical examination are as follows:

  • Hepatomegaly (83%)

  • Jaundice (69%)

  • Splenomegaly (32%)

  • Spider angiomata (58%)

  • Ascites (20%)

  • Encephalopathy (14%)

All of these findings may be observed in patients with disease that has progressed to cirrhosis with ensuing portal hypertension. However, hepatomegaly, jaundice, splenomegaly, and spider angiomata also may be observed in patients who do not have cirrhosis.

Complications may include the following:

  • Cirrhosis and complications of cirrhosis (eg, ascites, coagulopathy, hepatic coma)

  • Portal hypertension

  • Esophageal varices

  • Malnutrition (with poor growth in children)

GI tract bleeding as a complication of portal hypertension is uncommon.

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