Rapidly progressive sclerosing cholangitis following surgical treatment of pancreatic pseudotumor

Two patients with sclerosing cholangitis presented with a distal stricture of the common bile duct in association with pancreatic pseudotumors. Jaundice resolved following surgery to correct biliary obstruction, but diffuse cholangio-graphic abnormalities and clinical evidence of sclerosing cholangitis became evident 2 and 4 months later. Rapid progression of symptomatic disease necessitated liver transplantation in one patient, but the other had a complete response to methotrexate therapy. The rapid disease progression in these two patients may have been triggered by surgery that resulted in a generalized fibroproliferative response of the biliary tree, already affected with localized sclerosing cholangitis contiguous to a pancreatic pseudotumor. We suggest that localized sclerosing cholangitis associated with pancreatic pseudo-tumors may be a unique variant that can progress rapidly but respond dramatically to antiinflammatory therapy.