Autoimmune hepatitis: Difference between revisions
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*Usually affects women (~70%<ref>http://www.liverfoundation.org/abouttheliver/info/aihep/</ref> between age 15-40 years | *Usually affects women (~70%<ref>http://www.liverfoundation.org/abouttheliver/info/aihep/</ref> between age 15-40 years | ||
*Often occurs comorbidly with other autoimmune disorders (e.g. type 1 [[diabetes]], [[thyroid disease]], [[inflammatory bowel disease]], [[Sjögren]]'s) | *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== | ==Clinical Features== | ||
Revision as of 07:02, 18 August 2022
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%[1] 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
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
Evaluation
- 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
- Autoantibodies: may have positive ANA, SMA, LKM-1, and/or anti-LC1[2]
- Evaluate for other causes of liver disease, if not previously diagnosed with autoimmune hepatitis:
- Viral hepatitis serologies
- Acetaminophen level
- Consider RUQ ultrasound
- Definitive diagnosis usually by liver biopsy
Management
- Corticosteroids +/- Azathioprine
- Manage complications, see:
- Ultimately, may require transplant