Autoimmune hepatitis

Background

• Hepatocellular inflammation caused by autoimmune pathology, ultimately leading to necrosis and cirrhosis
• Presentation may be acute or chronic, rarely presents as fulminant hepatic failure
• Usually affects women (~70% between age 15-40 years
• Often occurs comorbidly with other autoimmune disorders (e.g. type 1 diabetes, thyroid disease, inflammatory bowel disease, Sjögren's)
• 3 subtypes of autoimmune hepatitis: types 1-3, with type 1 being the most common

Clinical Features

Jaundice of the skin

Pediatric jaundice with icterus of sclera.

Findings of liver disease and systemic signs/symptoms of autoimmune disorders

• Nausea/vomiting, diarrhea
• Epigastric pain, RUQ pain
• Anorexia
• Jaundice
• Ascites, SBP, edema
• Pruritus
• Fatigue, malaise, weakness
• Arthralgia, myalgia
• Spider angiomata, hirsutism, amenorrhea
• Chest pain due to pleuritis
• Weight loss
• Hepatosplenomegaly
• Hepatic encephalopathy
• Coagulopathy
• GI bleed

Differential Diagnosis

• Viral hepatitis
• Acute alcoholic hepatitis, alcoholic liver disease
• Acetaminophen toxicity and other drug-induced hepatotoxicity (
• Mushroom toxicity
• Ischemic hepatitis
• Non-alcoholic steatohepatitis
• Cardiac cirrhosis
• Primary biliary cirrhosis
• primary sclerosing cholangitis
• Budd-Chiari
• Alpha1 anti-trypsin Deficiency
• Cystic Fibrosis
• Wilson's disease
• Sarcoidosis

Causes of acute hepatitis

• Acetaminophen toxicity (most common cause of acute liver failure in the US)
• Viral hepatitis
  • Hepatitis A
  • Hepatitis B
  • Hepatitis C
  • Hepatitis D
  • Hepatitis E
  • HSV
  • Epstein-Barr virus
  • Varicella zoster virus
• Toxoplasmosis
• Acute alcoholic hepatitis
• Toxins
  • Mushroom toxicity
  • Crotinarius mushrooms
    • Gyromitra mushrooms
    • Amanita mushrooms
• Ischemic hepatitis
• Autoimmune hepatitis
• Wilson's disease

Hepatic Dysfunction

Infectious

• Hepatitis
  • Viral hepatitis
• Malaria
• HIV (present in 50% of AIDS patients)
• EBV
• Babesiosis, leptospirosis
• Typhoid
• Hepatic abscess, amebiasis

Neoplastic

• Metastases
• Hepatocellular carcinoma
• Multiple myeloma
• Leukemia
• Lymphoma
• Myeloproliferative disorders

Metabolic

• Hemochromatosis
• Wilson's disease
• Non-alcoholic steatohepatitis
• Inborn errors of metabolism

Biliary

• Biliary cirrhosis

Drugs

• Alcoholic cirrhosis
• Alcoholic hepatitis
• Hepatotoxic drugs

Miscellaneous

• Other causes of cirrhosis
• Autoimmune hepatitis
• Veno-occlusive disease
• CHF (right heart failure)

Evaluation

Workup

• LFTs
  • ALT/AST 1.5-50 times normal
  • Mild to moderate elevations in bilirubin and alk phos
• CBC
  • +/- mild leukopenia,
  • +/- normocytic or hemolytic anemia
  • +/- thrombocytopenia
• BMP: evaluate for electrolyte derangements, hypoglycemia, hepatorenal syndrome
• PT/INR
• Ammonia if suspect hepatic encephalopathy
• Paracentesis if suspect SBP
• Consider RUQ ultrasound and/or MRCP

Diagnosis

• Evaluate for other causes of liver disease, if not previously diagnosed with autoimmune hepatitis:
  • Viral hepatitis serologies
  • Acetaminophen level
• Autoantibodies: may have positive ANA, SMA, LKM-1, and/or anti-LC1 ; ANA & SMA usually positive in type 1 autoimmune hepatitis, LJM-1 and atypical p-ANCA positive in type 2 and liver soluble antigen usually in type 3
• Definitive diagnosis usually by liver biopsy

Management

• Corticosteroids +/- Azathioprine
• Manage complications, see:
  • Ascites, SBP
  • Upper gastrointestinal bleed
  • Hepatorenal syndrome
  • Hepatic encephalopathy
• Ultimately, may require transplant
• Hepatology/Gastroenterology consult should be made

See Also

• Acute hepatitis
• Cirrhosis