Primary sclerosing cholangitis is an uncommon diffuse inflammation of the extrahepatic bile duct eventuating in sufficient widespread occlusion of these ducts to cause jaundice and other manifestations of post-hepatic biliary obstruction. Sclerosing cholangitis has also been referred to as "obliterative cholangitis" and "stenosing cholangitis," but, since the obliteration and stenosis of the ducts are the result of a thickened duct wall, the term "sclerosing cholangitis" more accurately connotes the disease process. A review of the literature revealed only 13 cases which were acceptable for classification as primary sclerosing cholangitis. The surgical management of six cases in Rochester, N. Y., during the past 10 years suggests that the disease is commoner than previously considered, and therefore its recognition and therapy assume greater significance. While many questions regarding the etiology, course, and final prognosis remain speculative, review of the literature coupled with a study of these recent experiences suggests certain conclusions.
SCHWARTZ SI, DALE WA. Primary Sclerosing CholangitisReview and Report of Six Cases. AMA Arch Surg. 1958;77(3):439–451. doi:10.1001/archsurg.1958.01290030139016