Document Detail

Recurrent liver failure caused by IgG4 associated cholangitis.
MedLine Citation:
PMID:  21911900     Owner:  NLM     Status:  MEDLINE    
Immunoglobulin G4 associated cholangitis (IAC) is an autoimmune disease associated with autoimmune pancreatitis (AIP). It presents with clinical and radiographic findings similar to primary sclerosing cholangitis (PSC). IAC commonly has a faster, more progressive onset of symptoms and it is more common to see obstructive jaundice in IAC patients compared to those with PSC. One of the hallmarks of IAC is its responsiveness to steroid therapy. Current recommendations for treatment of AIP demonstrate excellent remission of the disease and associated symptoms with initiation of steroid therapy followed by steroid tapering. If untreated, it can progress to irreversible liver failure. This report describes a 59 year-old female with undiagnosed IAC who previously had undergone a pancreaticoduodenectomy for a suspected pancreatic cancer and later developed liver failure from presumed PSC. The patient underwent an uncomplicated liver transplantation at our institution, but experienced allograft failure within five years due to progressive and irreversible bile duct injury. Radiology and histology suggested recurrence of PSC, but the diagnosis of IAC was suspected based on her past history and confirmed when IgG4 positive cells were found within the intrahepatic bile duct walls on a liver biopsy. A successful liver retransplantation was performed and the patient is currently on triple immunosuppressive therapy. Our experience in this case and review of the current literature regarding IAC management suggest that patients with suspected or recurrent PSC with atypical features including history of pancreatitis should undergo testing for IAC as this entity is highly responsive to steroid therapy.
Jacob N Clendenon; Jaime Aranda-Michel; Murli Krishna; C Burcin Taner; Darrin L Willingham
Publication Detail:
Type:  Case Reports; Journal Article    
Journal Detail:
Title:  Annals of hepatology     Volume:  10     ISSN:  1665-2681     ISO Abbreviation:  Ann Hepatol     Publication Date:    2011 Oct-Dec
Date Detail:
Created Date:  2011-09-13     Completed Date:  2011-11-23     Revised Date:  2013-05-16    
Medline Journal Info:
Nlm Unique ID:  101155885     Medline TA:  Ann Hepatol     Country:  Mexico    
Other Details:
Languages:  eng     Pagination:  562-4     Citation Subset:  IM    
Department of Transplantation, Mayo Clinic Florida, Jacksonville, Florida 32224, USA.
Export Citation:
APA/MLA Format     Download EndNote     Download BibTex
MeSH Terms
Autoimmune Diseases / diagnosis,  drug therapy,  immunology*
Cholangitis, Sclerosing / diagnosis,  drug therapy,  immunology*
Drug Therapy, Combination
Immunoglobulin G / blood*
Immunosuppressive Agents / therapeutic use
Liver Failure / immunology*,  surgery
Liver Transplantation
Middle Aged
Pancreatitis, Chronic / diagnosis,  drug therapy,  immunology*,  surgery
Steroids / therapeutic use
Treatment Failure
Reg. No./Substance:
0/Immunoglobulin G; 0/Immunosuppressive Agents; 0/Steroids
Comment In:
Ann Hepatol. 2011 Oct-Dec;10(4):552-5   [PMID:  21911897 ]

From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine

Previous Document:  Takayasu arteritis associated with hepatic sinusoidal dilatation.
Next Document:  Cholestasis as the initial presentation of relapsing polychondritis.