Primary sclerosing cholangitis. A report of nine cases and clinical review.

Nine cases of primary sclerosing cholangitis were reviewed as to methods of diagnosis, association with other disease states and clinical course of the patients. There were four cases of primary sclerosing cholangitis occurring alone, four cases associated with inflammatory bowel disease (three with chronic ulcerative colitis and one case with proctosigmoiditis) and one case associated with porphyria cutanea tarda. All cases of primary sclerosing cholangitis occurring alone, progressed to secondary biliary cirrhosis, however, none of the cases associated with chronic ulcerative colitis progressed to secondary biliary cirrhosis. In all cases, the diagnosis was established by operative findings and biopsy results. The mode of clinical presentation was similar in all cases and was characterized by slowly progressive jaundice. Intravenous and oral cholangiography were not useful in establishing a diagnosis but endoscopic retrograde cholangiography offers preoperative diagnostic hope and use for follow-up evaluation. One case with ulcerative colitis had a Strongyloides infection and the organism was found in the fibrotic duct and pericholedochal lymph nodes. The etiological considerations are reviewed and the classification of sclerosing cholangitis associated with ulcerative colitis, as primary, is discussed. Therapeutic modalities are discussed, though therapy is mainly empirical at present.