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Autoimmune Hepatitis Clinical Presentation

  • Author: David C Wolf, MD, FACP, FACG, AGAF, FAASLD; Chief Editor: BS Anand, MD  more...
 
Updated: Feb 18, 2016
 

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 is said to occur 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 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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Contributor Information and Disclosures
Author

David C Wolf, MD, FACP, FACG, AGAF, FAASLD Medical Director of Liver Transplantation, Westchester Medical Center; Professor of Clinical Medicine, Division of Gastroenterology and Hepatobiliary Diseases, Department of Medicine, New York Medical College

David C Wolf, MD, FACP, FACG, AGAF, FAASLD is a member of the following medical societies: American Association for the Study of Liver Diseases, American College of Gastroenterology, American College of Physicians, American Gastroenterological Association

Disclosure: Serve(d) as a speaker or a member of a speakers bureau for: Salix; Gilead; Abbvie<br/>Received research grant from: Vital Therapies.

Coauthor(s)

Unnithan V Raghuraman, MD, FACG, FACP, FRCP Consulting Staff, Department of Gastroenterology, St John Medical Center

Unnithan V Raghuraman, MD, FACG, FACP, FRCP is a member of the following medical societies: American Association for the Study of Liver Diseases, American College of Gastroenterology, American College of Physicians, American Gastroenterological Association, American Society for Gastrointestinal Endoscopy

Disclosure: Nothing to disclose.

Chief Editor

BS Anand, MD Professor, Department of Internal Medicine, Division of Gastroenterology, Baylor College of Medicine

BS Anand, MD is a member of the following medical societies: American Association for the Study of Liver Diseases, American College of Gastroenterology, American Gastroenterological Association, American Society for Gastrointestinal Endoscopy

Disclosure: Nothing to disclose.

Acknowledgements

BS Anand, MD Professor, Department of Internal Medicine, Division of Gastroenterology, Baylor College of Medicine

BS Anand, MD is a member of the following medical societies: American Association for the Study of Liver Diseases, American College of Gastroenterology, American Gastroenterological Association, and American Society for Gastrointestinal Endoscopy

Disclosure: Nothing to disclose.

Robert Baldassano, MD Director, Center for Pediatric Inflammatory Bowel Disease, Children's Hospital of Philadelphia; Professor, Department of Pediatrics, Division of Gastroenterology and Nutrition, University of Pennsylvania School of Medicine

Robert Baldassano, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Pediatrics, American Gastroenterological Association, and North American Society for Pediatric Gastroenterology and Nutrition

Disclosure: Nothing to disclose.

Carmen Cuffari, MD Associate Professor, Department of Pediatrics, Division of Gastroenterology/Nutrition, Johns Hopkins University School of Medicine

Carmen Cuffari, MD is a member of the following medical societies: American College of Gastroenterology, American Gastroenterological Association, North American Society for Pediatric Gastroenterology, Hepatology and Nutrition, and Royal College of Physicians and Surgeons of Canada

Disclosure: Nothing to disclose.

Mohammad F El-Baba, MD Associate Professor of Pediatrics, Division of Pediatric Gastroenterology, Wayne State University School of Medicine; Divison Chief of Pediatric Gastroenterology, Children's Hospital of Michigan

Mohammad F El-Baba, MD is a member of the following medical societies: American Gastroenterological Association and North American Society for Pediatric Gastroenterology and Nutrition

Disclosure: Nothing to disclose.

Husam H Sukerek, MD Consulting Staff, Department of Gastroenterology, Sabine Medical Center

Husam H Sukerek, MD is a member of the following medical societies: American Academy of Pediatrics and American Medical Association

Disclosure: Nothing to disclose.

Francisco Talavera, PharmD, PhD Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Medscape Salary Employment

Mary L Windle, PharmD, Adjunct Associate Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Nothing to disclose.

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Table 1. Clinical Characteristics of Autoimmune Hepatitis [19]
Clinical Features Type 1 Type 2 Type 3
Diagnostic autoantibodies ASMA



ANA



Antiactin



Anti-LKM



P-450 IID6



Synthetic core motif peptides 254-271



Soluble liver-kidney antigen



Cytokeratins 8 and 18



Age 10 y-elderly Pediatric (2-14 y)



Rare in adults



Adults (30-50 y)
Women (%) 78 89 90
Concurrent immune disease (%) 41 34 58
Gamma globulin elevation +++ + ++
Low IgA* No Occasional No
HLA association B8, DR3, DR4 B14, Dr3, C4AQO Uncertain
Steroid response +++ ++ +++
Progression to cirrhosis (%) 45 82 75
*Immunoglobulin A
Table 2. Indications for Treatment of Autoimmune Hepatitis in Adults
Absolute Relative
Serum AST 10-fold or more greater than the upper limit of normal Symptoms (eg, fatigue, arthralgia, jaundice)
Serum AST 5-fold or more greater than the upper limit of normal and gamma-globulin level 2-fold or more greater than normal Serum AST and/or gamma-globulin less than absolute criteria
Bridging necrosis or multiacinar necrosis on



histologic examination



Interface hepatitis
AST = aspartate aminotransferase.
Table 3. Treatment Regimens for Adults
  Prednisone only (mg/d) Combination
Prednisone (mg/d) Azathioprine (mg/d)
Week 1 60 30 50
Week 2 40 20 50
Week 3 30 15 50
Week 4 30 15 50
Maintenance until



end point



20 10 50
Reasons for Preference Cytopenia



Thiopurine methyltransferase deficiency



Pregnancy



Malignancy



Short course (6 mo or less)



Postmenopausal state



Osteoporosis



Brittle diabetes



Obesity



Acne



Emotional lability



Hypertension



Table 4. Treatment Regimens for Children
Initial Regimen Maintenance Regimen End Point
Prednisone, 1-2 mg/kg/d (up to 60 mg/d), for 2 weeks, either alone or in combination with azathioprine, 1-2 mg/kg/d a. Prednisone taper over 6-8 weeks to 0.1-0.2 mg/kg daily or 5 mg daily



b. Azathioprine at constant dose if added initially



c. Continue daily prednisone dose with or without azathioprine or switch to alternate day prednisone dose adjusted to response with or without azathioprine



a. Normal liver tests for 1-2 years during treatment



b. No flare during entire interval



c. Liver biopsy examination discloses no inflammation



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