Raj V, Lichtenstein D R
Department of Internal Medicine, University of Arkansas for Medical Sciences College of Medicine, McClellan VA Hospital (VR), Little Rock, USA.
Gastroenterol Clin North Am. 1999 Jun;28(2):491-513. doi: 10.1016/s0889-8553(05)70067-4.
PSC is the most common of the clinically significant hepatobiliary diseases seen in association with IBD, with an incidence that varies from 2.5% to 7.5%. Conversely, 50% to 75% of patients with PSC have IBD. This high degree of association suggests a common pathogenetic mechanism; however, no causal relationship has been established. The etiopathogenesis of PSC remains poorly understood, despite a large number of studies looking at differing hypotheses. The diagnosis is usually established by cholangiography. Liver biopsy can sometimes be helpful in diagnosing pericholangitis. There is a significant overlap of the histology with chronic hepatitis. Serum markers have been studied for diagnosing PSC, particularly for early diagnosis of cholangiocarcinoma, but none have shown the high sensitivity and specificity needed to use them clinically. PSC usually progresses insidiously and eventually leads to cirrhosis. Despite progress in early recognition, optimal management of patients with PSC remains a challenge requiring a multidisciplinary approach among hepatologists, endoscopists, surgeons, and interventional radiologists. Colectomy for ulcerative colitis does not alter the natural history of PSC. There is a high (10% to 15%) incidence of cholangiocarcinoma in patients with PSC. This incidence along with the risk of colon cancer in patients with ulcerative colitis makes it necessary to follow these patients closely. A number of pharmacologic therapies have been evaluated, but none has proven successful in slowing the progression of PSC or prolonging survival. Endoscopic therapy has a proven utility in treating complications of recurrent cholangitis or worsening jaundice in the setting of a dominant stricture, but endoscopy has not been shown to improve survival or decrease the need for liver transplantation. Liver transplantation is life-saving for patients with advanced PSC. Pericholangitis, gallstones, and chronic hepatitis are additional disorders noted in association with IBD, but they are much less common and easier to manage than PSC.
原发性硬化性胆管炎（PSC）是与炎症性肠病（IBD）相关的临床上最常见的重要肝胆疾病，发病率在2.5%至7.5%之间。相反，50%至75%的PSC患者患有IBD。这种高度关联提示存在共同的发病机制；然而，尚未确立因果关系。尽管有大量研究探讨了不同的假说，但PSC的病因发病机制仍了解甚少。诊断通常通过胆管造影术确立。肝活检有时有助于诊断胆管周围炎。其组织学表现与慢性肝炎有显著重叠。人们已对血清标志物进行研究以诊断PSC，尤其是用于胆管癌的早期诊断，但尚无一种标志物显示出临床应用所需的高敏感性和特异性。PSC通常隐匿进展，最终导致肝硬化。尽管在早期识别方面取得了进展，但对PSC患者的最佳管理仍然是一项挑战，需要肝病学家、内镜医师、外科医生和介入放射科医生采取多学科方法。溃疡性结肠炎患者行结肠切除术并不会改变PSC的自然病程。PSC患者胆管癌的发病率很高（10%至15%）。这一发病率以及溃疡性结肠炎患者患结肠癌的风险使得有必要密切随访这些患者。已评估了多种药物治疗方法，但尚无一种被证明能成功减缓PSC的进展或延长生存期。内镜治疗在治疗复发性胆管炎的并发症或在存在主要狭窄的情况下改善黄疸方面已被证明有效，但内镜检查尚未显示能提高生存率或减少肝移植需求。肝移植对晚期PSC患者是挽救生命的。胆管周围炎、胆结石和慢性肝炎是与IBD相关的其他疾病，但它们比PSC少见得多且更易于处理。
