Document Detail

Autoimmune pancreatocholangitis: a series of ten patients.
MedLine Citation:
PMID:  16782625     Owner:  NLM     Status:  MEDLINE    
BACKGROUND: During a 10-year period we observed 10 patients who suffered from an inflammatory-fibrosing disease mimicking pancreatic carcinoma and primary sclerosing cholangitis (PSC). METHODS: A review of the presenting features, the clinical course and the relevant literature. RESULTS: Ten male patients (mean age 55 years) presented with weight loss, jaundice and pruritus. Pancreatic cancer was suggested by imaging studies, which showed focal or generalized pancreatic enlargement and compression of the distal common bile duct. Cholangiography also demonstrated intrahepatic biliary stenoses consistent with sclerosing cholangitis. None had evidence of IBD. Exocrine pancreatic insufficiency was found in six cases and diabetes in four. Pancreatic histology (n=3) showed fibrosis and extensive inflammatory infiltrates. Immunosuppressive treatment was instituted in five patients. Clinical and biochemical remission occurred in three; in one other patient, previously documented intrahepatic biliary strictures had disappeared after 3 months. One patient had concomitant Sjögren's disease. The clinical features, pancreatic involvement, age at presentation, absence of IBD and response to steroids all plead against a diagnosis of "classical" PSC. The natural course of the disease was highly variable. Thirty-five comparable cases, with a largest series of three, have been reported in the literature. The disease has been associated with Sjögren's disease, retroperitoneal fibrosis and other fibrosing conditions, and may be a manifestation of a systemic fibro-inflammatory disorder. CONCLUSION: Autoimmune pancreatocholangitis is a distinct inflammatory disorder involving the pancreas and biliary tree. The disease may mimick pancreatic carcinoma and PSC and responds to immunosuppressives.
Henk R van Buuren; Frank P Vleggaar; G Willemien Erkelens; Pieter E Zondervan; Wilco Lesterhuis; Casper H J Van Eijck; Julien B C M Puylaert; Sjoerd D J Van Der Werf
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Publication Detail:
Type:  Case Reports; Journal Article    
Journal Detail:
Title:  Scandinavian journal of gastroenterology. Supplement     Volume:  -     ISSN:  0085-5928     ISO Abbreviation:  Scand. J. Gastroenterol. Suppl.     Publication Date:  2006  
Date Detail:
Created Date:  2006-06-19     Completed Date:  2007-03-15     Revised Date:  2010-10-08    
Medline Journal Info:
Nlm Unique ID:  0437034     Medline TA:  Scand J Gastroenterol Suppl     Country:  Norway    
Other Details:
Languages:  eng     Pagination:  70-8     Citation Subset:  IM    
Department of Gastroenterology and Hepatology, Erasmus Medical Centre, Rotterdam, The Netherlands.
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MeSH Terms
Aged, 80 and over
Autoimmune Diseases / diagnosis*,  drug therapy
Cholagogues and Choleretics / therapeutic use
Cholangiopancreatography, Endoscopic Retrograde
Cholangitis, Sclerosing / diagnosis*,  drug therapy
Common Bile Duct / pathology,  radiography,  ultrasonography
Diagnosis, Differential
Fibrosis / diagnosis,  drug therapy
Immunosuppressive Agents / therapeutic use
Liver / pathology,  radiography,  ultrasonography
Middle Aged
Pancreatic Ducts / pathology,  radiography,  ultrasonography
Pancreatic Neoplasms / diagnosis,  drug therapy
Pancreatitis / diagnosis*,  drug therapy
Tomography, X-Ray Computed
Ultrasonography, Interventional
Ursodeoxycholic Acid / therapeutic use
Reg. No./Substance:
0/Cholagogues and Choleretics; 0/Immunosuppressive Agents; 128-13-2/Ursodeoxycholic Acid

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