ERCPからみた原発性硬化性胆管炎と原発性胆汁性肝硬変症

ERCP Findings of Primary Sclerosing Cholangitis and Primary Biliary Cirrhosis

　わが国では原発性硬化性胆管炎（primary sclerosing cholangitis，以下PSCと略す）の報告例は欧米に比べて極端に少ないようである．われわれは，ERCP が診断の手掛かりとなった3例のPSC近縁疾患を経験している¹⁾このなかで，診断から現在まで6年間経過をみている潰瘍性大腸炎を伴った典型的なPSCの症例を報告する．また，本疾患と鑑別が必要な原発性胆汁性肝硬変症（primary biliary cirrhosis，以下PBCと略す）を呈示し，両疾患のERCP像の違いに言及する．

　Primary sclerosing cholangitis (PSC) and primary biliary cirrhosis (PBC) seemed to be rare in Japan. They may be confused because of the similarity of the clinical and histological features. Recently some clinical views with a lot of cases of PSC have been reported by Chapman et al., Wiesner and LaRussor and so on. We have experienced three cases of PSC, which were first diagnosed by using ERCP. Therefore we present the clinical features, especially some findings of the cholangiogram of the typical case associated with ulcerative colitis.

　On the cholangiogram by ERCP and PTC, long stricture of the common bile duct, multiple segmental strictures and dilatations of the intrahepatic bile ducts, and normal distal branches were demonstrated clearly. On the other hand, one case of PBC showed no characteristic findings except slight rigidity of the distal branches of the intrahepatic bile duct on the cholangiogram. Of course, this patient was easily diagnosed by positive mitochondrial antibody and some characteristic features of liver biopsy.

　We conclude that ERCP is the most useful procelure to make clinically an accurate diagnosis of PSC and to distinguish between PSC and PBC with negative mitochondrial antibody.