Clinical–Liver, Pancreas, and Biliary TractImmunoglobulin G4–Associated Cholangitis: Clinical Profile and Response to Therapy
Section snippets
Materials and Methods
The protocol was approved by the Mayo Clinic institutional review board. From a database of patients with AIP, prospectively maintained by one of the authors (S.T.C.), we identified 49 patients with biliary strictures and proven AIP; 14 of these patients had undergone a pancreaticoduodenectomy for suspected pancreatic cancer owing to presentation with obstructive jaundice and a pancreatic head mass. AIP was diagnosed by the recently published HISORt criteria (Histology, Imaging, Serology, Other
Demographics and Presenting Clinical Features
The mean patient age was 62 ± 2 years (range, 14–85 y) and 83% were older than age 50. The majority of patients were men (85%). Clinical features on presentation included obstructive jaundice in 41 (77%), weight loss in 27 (51%), steatorrhea in 8 (15%), new-onset diabetes mellitus in 4 (8%), and abdominal pain in 14 of the 53 patients (26%). Abdominal pain did not require narcotics in any patient.
Histology and IgG4 Immunostaining
Four patients had resection (surgical) specimens that revealed a lymphoplasmacytic infiltrate in
Discussion
AIP now is viewed as the pancreatic manifestation of a systemic disease referred to as ISD.6 Several small case series have described biliary strictures in association with AIP13, 14, 15, 16, 17, 18, 19, 20, 21, 22 (Table 3) that characterize the biliary manifestation of ISD. We have proposed the term IAC to describe this entity.12 Similar to AIP, IAC patients have a striking male preponderance and typically present in the sixth and seventh decades of life with obstructive jaundice, weight
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