Autoimmune pancreatocholangitis: a series of ten patients.
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.


CONCLUSIONS
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.
van Buuren
Henk R
HR
Department of Gastroenterology and Hepatology, Erasmus Medical Centre, Rotterdam, The Netherlands. h.vanbuuren@erasmusmc.nl
Vleggaar
Frank P
FP
Willemien Erkelens
G
G
Zondervan
Pieter E
PE
Lesterhuis
Wilco
W
Van Eijck
Casper H J
CH
Puylaert
Julien B C M
JB
Van Der Werf
Sjoerd D J
SD
D000328:Adult; D000369:Aged, 80 and over; D001327:Autoimmune Diseases / Q000175:diagnosis* / Q000188:drug therapy; D002756:Cholagogues and Choleretics / Q000627:therapeutic use; D002760:Cholangiopancreatography, Endoscopic Retrograde; D015209:Cholangitis, Sclerosing / Q000175:diagnosis* / Q000188:drug therapy; D003113:Colonoscopy; D003135:Common Bile Duct / Q000000981:diagnostic imaging / Q000473:pathology; D003937:Diagnosis, Differential; D005355:Fibrosis / Q000175:diagnosis / Q000188:drug therapy; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D008099:Liver / Q000000981:diagnostic imaging / Q000473:pathology; D008297:Male; D008875:Middle Aged; D010183:Pancreatic Ducts / Q000000981:diagnostic imaging / Q000473:pathology; D010190:Pancreatic Neoplasms / Q000175:diagnosis / Q000188:drug therapy; D010195:Pancreatitis / Q000175:diagnosis* / Q000188:drug therapy; D014057:Tomography, X-Ray Computed; D018084:Ultrasonography, Interventional; D014580:Ursodeoxycholic Acid / Q000627:therapeutic use
D002756:Cholagogues and Choleretics; D007166:Immunosuppressive Agents; D014580:Ursodeoxycholic Acid