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Open Access Case report

Concurrent autoimmune pancreatitis and primary Biliary cirrhosis: a rare case report and literature review

Aiqing Li, Yongjie Wang* and Zheng Deng

Author Affiliations

Department of Gastroenterology, Sir Run Run Shaw Hospital Affiliated to Medical School, Zhejiang University, Hangzhou, Zhejiang Province 310016, China

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BMC Gastroenterology 2014, 14:10  doi:10.1186/1471-230X-14-10

Published: 10 January 2014

Abstract

Background

Both autoimmune pancreatitis (AIP) and primary biliary cirrhosis (PBC) are related to various diseases. But the concurrence of AIP and PBC is extremely rare, with only 2 cases reported. Here we report the concurrence of AIP and PBC in a Chinese patient for the first time.

Case presentation

A 65-year-old male was admitted to our hospital with jaundice, pruritus, mild abdominal pain and darkening urine. Serum alkaline phosphatase, γ-glutamyltransferase, bilirubin and IgG4 were prominently elevated. The antimitochondrial antibody was positive. Radiological examination revealed diffusive enlargement of the pancreas. Pancreatic biopsy showed lymphoplasmacytic infiltration, fibrosis and abundant IgG4+ plasma cells. The patient was diagnosed with AIP and PBC. Nasobiliary tube was placed to facilitate biliary drainage. A combination therapy of steroid and UDCA was administered and the patient was gradually recovered, during which the patient was complicated with biliary infecion, herpes zoster and pulmonary abscess.

Conclusion

We present this case together with literature evidence to support the concurrence of AIP and PBC, share our experience of using combination therapy with steroid and UDCA, and raise the awareness of infectious complications in immunosuppressed patients.

Keywords:
Autoimmune pancreatitis; Primary biliary cirrhosis; Steroid; Ursodeoxycholic acid; Infection