Autoimmune hepatitis: Difference between revisions
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==Background== | ==Background== | ||
*Hepatocellular inflammation caused by autoimmune pathology, ultimately leading to necrosis and cirrhosis | *Hepatocellular inflammation caused by autoimmune pathology, ultimately leading to necrosis and cirrhosis | ||
*Presentation may be acute or chronic, rarely presents as fulminant hepatic failure | *Presentation may be acute or chronic, rarely presents as fulminant [[hepatic failure]] | ||
*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]], | *Often occurs comorbidly with other autoimmune disorders (e.g. type 1 [[diabetes]], [[thyroid disease]], [[inflammatory bowel disease]], [[Sjögren]]'s) | ||
==Clinical Features== | ==Clinical Features== | ||
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*[[Chest pain]] due to pleuritis | *[[Chest pain]] due to pleuritis | ||
*Weight loss | *Weight loss | ||
*Hepatosplenomegaly | *[[hepatomegaly|Hepatosplenomegaly]] | ||
*[[Hepatic encephalopathy]] | *[[Hepatic encephalopathy]] | ||
*Coagulopathy | *[[liver disease induced coagulopathy|Coagulopathy]] | ||
*[[GI bleed]] | *[[GI bleed]] | ||
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*[[Mushroom toxicity]] | *[[Mushroom toxicity]] | ||
*[[Ischemic hepatitis]] | *[[Ischemic hepatitis]] | ||
*Non-alcoholic steatohepatitis | *[[Non-alcoholic steatohepatitis]] | ||
*Cardiac cirrhosis | *Cardiac cirrhosis | ||
*Primary biliary cirrhosis, [[primary sclerosing cholangitis]] | *Primary biliary cirrhosis, [[primary sclerosing cholangitis]] | ||
*Budd-Chiari | *[[Budd-Chiari]] | ||
*Alpha1 anti-trypsin Deficiency | *Alpha1 anti-trypsin Deficiency | ||
*[[Cystic Fibrosis]] | *[[Cystic Fibrosis]] | ||
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*PT/INR | *PT/INR | ||
*Ammonia if suspect [[hepatic encephalopathy]] | *Ammonia if suspect [[hepatic encephalopathy]] | ||
*Paracentesis if suspect [[SBP]] | *[[Paracentesis]] if suspect [[SBP]] | ||
*Autoantibodies: may have positive ANA, SMA, LKM-1, and/or anti-LC1<ref>http://emedicine.medscape.com/article/172356-overview</ref> | *Autoantibodies: may have positive ANA, SMA, LKM-1, and/or anti-LC1<ref>http://emedicine.medscape.com/article/172356-overview</ref> | ||
*Evaluate for other causes of liver disease, if not previously diagnosed with autoimmune hepatitis: | *Evaluate for other causes of liver disease, if not previously diagnosed with autoimmune hepatitis: | ||
**Viral hepatitis serologies | **[[Viral hepatitis]] serologies | ||
**[[Acetaminophen]] level | **[[Acetaminophen]] level | ||
*Consider RUQ ultrasound | *Consider [[RUQ ultrasound]] | ||
*Definitive diagnosis usually by liver biopsy | *Definitive diagnosis usually by liver biopsy | ||
Revision as of 19:30, 29 September 2019
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)
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