Clinicopathologic features of the syndrome of primary sclerosing cholangitis
Clinicopathologic features of the syndrome of primary sclerosing cholangitis
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DOI：
10.1016/0016-5085(80)90131-6
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发表时间：
1980-08
期刊：
影响因子：
29.4
通讯作者：
R. Wiesner;N. LaRusso
中科院分区：
文献类型：
--
作者：
R. Wiesner;N. LaRusso
我们回顾了 1970 年至 1977 年间通过手术和/或放射学诊断为原发性硬化性胆管炎的 50 名患者的病史。患者通常是年轻男性，表现为黄疸、瘙痒和肝肿大。炎症性肠病（通常是慢性溃疡性结肠炎）很常见（54%）。通常存在胆汁淤积的生化特征，并且明显缺乏血清学标志物。 1974 年内窥镜逆行胆管造影的启动与每年诊断频率的翻倍相关。通常会发现肝外和肝内导管受累的放射学证据（81%）。 34/36 名患者的异常肝活检显示胆汁淤积、门静脉或门静脉周围肝炎和/或肝硬化。肝铜水平在 12/12 时升高，平均水平（244 ± 71 μg/g 干重肝脏，平均值 ± SEM）与原发性胆汁性肝硬化相当。对 39 名患者的充分随访数据显示，13 名患者在诊断后 5 至 108 个月内死亡，通常死于肝功能衰竭。许多患者（42%）接受了类固醇治疗，但通常没有明显的益处。总之，在我们机构，特别是自从内镜逆行胆管造影出现以来，原发性硬化性胆管炎的诊断并不罕见。该疾病的特征是男性占主导地位、进行性胆汁淤积、肝硬化、肝铜超载、明显的类固醇衰竭以及因肝衰竭而过早死亡。
We reviewed the histories of 50 patients diagnosed surgically and/or radiographically as having primary sclerosing cholangitis seen from 1970 through 1977. Patients were generally young males who presented with jaundice, pruritus, and hepatomegaly. Inflammatory bowel disease, usually chronic ulcerative colitis, was common (54%). A cholestatic biochemical profile was ordinarily present, and serologic markers were conspicuously absent. Initiation of endoscopic retrograde cholangiography in 1974 was associated with a doubling of the annual frequency of diagnosis. Radiologic evidence of both extra- and intrahepatic duct involvement was usually found (81%). Abnormal liver biopsies from 34/36 patients showed cholestasis, portal or periportal hepatitis, and/or cirrhosis. Hepatic copper levels were elevated in 12/12, and the mean level (244 ± 71 μg/g dry weight liver, mean ± SEM) was comparable to that in primary biliary cirrhosis. Adequate follow-up data in 39 patients showed that 13 died from 5 to 108 mo after diagnosis, usually from liver failure. Many patients (42%) received steroid therapy, generally without apparent benefit. In summary, the diagnosis of primary sclerosing cholangitis at our institution, particularly since the advent of endoscopic retrograde cholangiography, is not rare. The disease is characterized by male predominance, progressive cholestasis, cirrhosis, hepatic copper overload, apparent steroid failure, and premature death from liver failure.