Three patients with primary sclerosing cholangitis and Hodgkin's disease, a previously unrecognized association, are reported. All three patients were men, and one patient had Crohn's disease of the colon. Primary sclerosing cholangitis was diagnosed 2 , l l and 17 yr before diagnosis of Hodgkin's disease in the three patients, and all three had advanced biliary cirrhosis prompting referral for liver transplantation. The symptoms of Hodgkin's disease were often masked by similar manifestations of primary sclerosing cholangitis, particularly symptoms of recurrent biliary sepsis. Hodgkin's disease is another disorder that may occur in patients with primary sclerosing cholangitis, particularly in the setting of advanced disease, and may be masked by the underlying hepatobiliary disease. (HEPA-TOLOGY 1993;18: 1127-1131.)
Primary sclerosing cholangitis (PSC) is a chronic cholestatic liver disease characterized by progressive inflammation of the biliary tree (1-3). Diagnosis depends on the cholangiographic demonstration of irregular stricturing and dilation of the intrahepatic and extrahepatic bile ducts. Secondary sclerosing cholangitis, with similar cholangiographic findings, has been described in patients with metastatic adenocarcinoma (41, floxuridine administration (51, AIDS (6) and cholangiocarcinoma (7, 8). Approximately two thirds of patients with PSC have inflammatory bowel disease, particularly ulcerative colitis (91, and as many as 10% may contract cholangiocarcinoma (7,8). PSC has been associated with other diseases, which are often cited as single case reports, but established PSC has not been associated with lymphomas. Hodgkin's disease is a distinct variety of malignant lymphoma, which is occasionally associated with liver dysfunction and jaundice; however, the biliary tree is typically not involved in the disease process (10).
We report three patients with PSC and Hodgkin's disease, a relationship that has not previously been noted.