원발성 경화성 담관염 28예의 임상상
28 cases of primary sclerosing cholangitis
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초록
목적 : 원발성 경화성 담관염은 간내 또는 간외 담관의 다발성 협착을 특징으로 하는 원인 미상의 만성 염증성 질환이다. 이 질환은 담관벽의 점진적인 섬유화를 초래하여 담즙성 간경변을 거쳐 결국 간부전으로 진행한다. 저자들은 본원에서 경험한 28예의 원발성 경화성 담관염의 임상상을 분석하였다. 대상 및 방법 : 1991년부터 2003년 8월 사이에 서울 아산 병원에서 원발성 경화성 담관염으로 진단된 28명의 임상적, 혈청학적, 방사선학적, 조직학적 특징을 후향적으로 분석하였다. 결과 : 진단시 평균 나이는 39.3세였고, 남자 환자가 더 많았다(남:여=2.1:1). 주증상으로는 복통과 우연히 발견된 간기능 이상이 가장 흔하였다. 담관조영술상 82.1%에서 간내외 담관을 모두 침범하였고, 10.7%에서는 간내 담관에 국한되어 있었고, 간문부 침범만 보이는 경우도 7.1%에서 있었다. 주췌관 침범은 10.5% (2/19)에서 관찰되었다. 자가 항체는 26예 중 8예(30.8%)에서 양성이었다. 염증성 장질환은 50% (6/12)에서 동반되었고 궤양성 대장염이 5예, 크론병이 1예였다. 담도 협착 확장 목적의 내시경적 또는 중재적 방사선학적 시술은 각각 14.3%, 71.4%에서 시행되었고, 간이식을 받은 경우는 2예(7.1%)였다. 결론 : 원발성 경화성 담관염은 국내에서는 드문 질환이지만 이 질환에 대한 지식의 축적과 관심이 증가함에 따라 점점 진단되는 증례수가 증가할 것으로 예상된다.
Abstract
Backgroud : Primary sclerosing cholangitis (PSC) is a chronic progressive cholestatic hepatobiliary disease of unknown etiology, a characterized by inflammation, scarring and obliteration of bile ducts, eventually leading to biliary cirrhosis and liver failure. In Korea, reports on PSC have only been done anecdotally and there are hardly any large scale studies. Methods : We retrospectively analyzed the clinical, laboratory, radiologic and histologic feature of 28 patients, who were diagnosed in Asan Medical Center during the last decade. A diagnosis of PSC was made by typical direct cholangiographic findings. Results : The median age of the enrolled patients at diagnosis was 39.3 years, and there was a preponderance of men (M:F = 2.1:1). The major clinical features were abdominal pain and abnormal liver function tests of cholestatic pattern. The rate of positivity for autoantibody was 30.8% (8/26). On cholangiogram, 82.1% had evidence of both extra- and intrahepatic bile duct involvement, 10.7% of intrahepatic involvement only, and 7.1% of hilar involvement only. The pancreatic duct involvement was seen in 10.5% (2/19). The incidence of associated inflammatory bowel disease was 50% (6/12), UC in 5 cases and Crohn's disease in 1 case, respectively. Endoscopic or radiologic intervention for the dilatation of narrowed bile ducts were performed in 14.3% and 71.4%, respectively. Two cases (7.1%) underwent liver transplantation. Conclusion : Although PSC is a rare disease in Korea, we prospect that more cases will be reported as concern and knowledge of this entity increase.
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