Fulminant hepatic failure in autoimmune polyendocrine syndrome type-1.

Abstract

Fulminant hepatic failure is liver disease that causes encephalopathy within 8 weeks of onset of symptoms or within 2 weeks of onset of jaundice in a patient without prior evidence of liver disease. Autoimmune polyendocrine syndrome type-1 is an autoimmune autosomal-recessive condition causing parathyroid and adrenal insufficiency, alopecia, chronic mucocutaneous candidiasis, ectodermal dystrophy and, rarely, hepatitis. Although the liver can be affected as a consequence of the autoimmune process, the spectrum of disease activity is varied. Autoimmune hepatitis develops in 10-20% of patients and successful liver transplantation has been reported in pediatric patients who failed immunosuppressive treatment. We report fulminant hepatic failure in an adult patient with autoimmune polyendocrine syndrome type-1 who responded to medical treatment and did not require liver transplantation. We highlight the diagnostic scoring system for autoimmune hepatitis and the referral criteria for liver transplantation in fulminant hepatic failure.

• Authors: R Sinha, A R Chapman, G T Reid, P C Hayes
• Journal: The journal of the Royal College of Physicians of Edinburgh (J R Coll Physicians Edinb) Vol. 45 Issue 2 Pg. 136-40 ( 2015) ISSN: 2042-8189 [Electronic] England
• PMID: 26181530 (Publication Type: Case Reports, Journal Article)

Chemical References

• Immunosuppressive Agents

Topics

• Female
• Hepatitis, Autoimmune (complications, diagnosis, drug therapy)
• Humans
• Immunosuppressive Agents (therapeutic use)
• Liver (pathology)
• Liver Failure, Acute (diagnosis, etiology, therapy)
• Liver Transplantation
• Polyendocrinopathies, Autoimmune (complications, drug therapy)
• Young Adult