PRIMARY SCLEROSING CHOLANGITIS - A REVIEW OF ITS CLINICAL-FEATURES, CHOLANGIOGRAPHY, AND HEPATIC HISTOLOGY
PRIMARY SCLEROSING CHOLANGITIS - A REVIEW OF ITS CLINICAL-FEATURES, CHOLANGIOGRAPHY, AND HEPATIC HISTOLOGY
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DOI：
10.1136/gut.21.10.870
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发表时间：
1980-01-01
期刊：
影响因子：
24.5
通讯作者：
SHERLOCK, S
中科院分区：
文献类型：
--
作者：
CHAPMAN, RWG;ARBORGH, BAM;SHERLOCK, S
本文回顾了29例原发性硬化性胆管炎患者。男性占多数（2：1）。76%的患者表现为胆汁淤积和胆管炎，17%的患者表现为肝硬化和门静脉高压，7%的患者无症状，表现为血清碱性磷酸酶升高。45%的患者血清IgM浓度升高，但无患者血清线粒体抗体存在。72%患有溃疡性直肠结肠炎。溃疡性直肠结肠炎的持续时间或严重程度与原发性硬化性胆管炎的发展之间没有关系。4例患者未从结肠切除术中获益。所有患者均无克罗恩病。预后是可变的。皮质类固醇和硫唑嘌呤无效。11例患者（38%）死亡，平均生存时间为7年。3例溃疡性直肠结肠炎患者发生胆管癌。本文报告了41例其他胆道疾病患者的胆管造影和肝活检，未参考临床资料。22例患者中18例（82%）行胆道造影诊断。8/22例（36%）肝组织学诊断。10例表现为大胆管病变，3例误诊为原发性胆汁性肝硬化。胆管数量减少、胆管增生、门静脉炎症和大量铜沉积，结合碎片状坏死，在原发性硬化性胆管炎中常见，提示需要进行胆管造影。
Twenty-nine patients with primary sclerosing cholangitis were reviewed. Males predominated (2:1). Seventy-six percent presented with cholestasis and cholangitis, 17% with cirrhosis and portal hypertension and 7% were asymptomatic, presenting with a raised serum alkaline phosphatase. The serum IgM concentration was raised in 45% of the patients, but no patient had serum mitochondrial antibody present. Seventy-two percent had ulcerative proctocolitis. There was no relationship between duration or severity of ulcerative proctocolitis and the development of primary sclerosing cholangitis. Four patients were not benefited by colectomy. None of the patients had Crohn''s disease. The prognosis was variable. Corticosteroids and azathioprine were ineffective. Eleven patients (38%) died with a mean survival time of 7 yr from diagnosis. Three patients with ulcerative proctocolitis developed bile duct carcinoma. The cholangiograms and liver biopsies were reported without reference to clinical information together with 41 patients with other biliary diseases. Cholangiography was diagnostic in 18/22 (82%). Hepatic histology was diagnostic in 8/22 (36%). Ten showed features of large bile duct disease and 3 were misdiagnosed as primary biliary cirrhosis. Reduced numbers of bile ducts, ductular proliferation, portal inflammation and substantial Cu deposition, in combination with piecemeal necrosis, are commonly seen in primary sclerosing cholangitis and indicate the need for cholangiography.