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) | ||
*3 subtypes of autoimmune hepatitis: types 1-3, with type 1 being the most common | |||
==Clinical Features== | ==Clinical Features== | ||
[[File:Jaundice08.jpg|thumb|Jaundice of the skin]] | |||
[[File:Jaundice.jpg|thumb|Pediatric jaundice with icterus of sclera.]] | |||
''Findings of liver disease and systemic signs/symptoms of autoimmune disorders'' | ''Findings of liver disease and systemic signs/symptoms of autoimmune disorders'' | ||
*[[Nausea/vomiting]], diarrhea | *[[Nausea/vomiting]], diarrhea | ||
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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 biliary cirrhosis | ||
*Budd-Chiari | *[[primary sclerosing cholangitis]] | ||
*[[Budd-Chiari]] | |||
*Alpha1 anti-trypsin Deficiency | *Alpha1 anti-trypsin Deficiency | ||
*[[Cystic Fibrosis]] | *[[Cystic Fibrosis]] | ||
*[[Wilson's disease]] | |||
*[[Sarcoidosis]] | |||
{{Acute hepatitis causes}} | |||
{{Hepatomegaly DDX}} | |||
==Evaluation== | ==Evaluation== | ||
===Workup=== | |||
*[[LFTs]] | *[[LFTs]] | ||
**ALT/AST 1.5-50 times normal | **ALT/AST 1.5-50 times normal | ||
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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]] | ||
* | *Consider [[RUQ ultrasound]] and/or MRCP | ||
===Diagnosis=== | |||
*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 | ||
* | *Autoantibodies: may have positive ANA, SMA, LKM-1, and/or anti-LC1<ref>http://emedicine.medscape.com/article/172356-overview</ref> ; 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 | *Definitive diagnosis usually by liver biopsy | ||
| Line 63: | Line 75: | ||
**[[Hepatic encephalopathy]] | **[[Hepatic encephalopathy]] | ||
*Ultimately, may require transplant | *Ultimately, may require transplant | ||
*Hepatology/Gastroenterology consult should be made | |||
==Disposition== | ==Disposition== | ||
Latest revision as of 20:22, 28 February 2024
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
- Wilson's disease
- Sarcoidosis
Causes of acute hepatitis
- Acetaminophen toxicity (most common cause of acute liver failure in the US[2])
- Viral hepatitis
- Toxoplasmosis
- Acute alcoholic hepatitis
- Toxins
- Ischemic hepatitis
- Autoimmune hepatitis
- Wilson's disease
Hepatic Dysfunction
Infectious
- Hepatitis
- Malaria
- HIV (present in 50% of AIDS patients)[3]
- EBV
- Babesiosis, leptospirosis
- Typhoid
- Hepatic abscess, amebiasis
Neoplastic
Metabolic
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[4] ; 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:
- Ultimately, may require transplant
- Hepatology/Gastroenterology consult should be made
Disposition
See Also
External Links
References
- ↑ http://www.liverfoundation.org/abouttheliver/info/aihep/
- ↑ Ostapowicz G, Fontana RJ, Schiodt FV, et al. Results of a prospective study of acute liver failure at 17 tertiary care centers in the United States. Ann Intern Med. 2002 Dec 17; 137(12): 947-54.
- ↑ Tintanelli's
- ↑ http://emedicine.medscape.com/article/172356-overview