A 59-year-old man presented with upper abdominal pain, cholestasis and radiological evidence of common hepatic duct hilar stricture which was suggestive of cholangiocarcinoma. The patient initially underwent percutaneous drainage and a laparotomy. No evidence of malignancy was identified. He was noted to have retroperitoneal fibrosis, which was confirmed on histology. The combination of cholangiopathy and retroperitoneal fibrosis suggested an underlying autoimmune process. Although the investigations did not show any evidence of IgG4 related disease, the combination of a cholangiopathy and retroperitoneal fibrosis is in keeping with autoimmune cholangiopathy and a steroid regimen was commenced. Our patient is now symptom-free with no further episodes of cholangitis. He has commenced azathioprine to maintain long term remission.
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Competing interests: none.
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