针对经选择患者组全部患者的治疗推荐
胆总管狭窄（肝外胆管分支的狭窄）快速恶化的患者（迅速恶化的黄疸和皮肤瘙痒，急性细菌性胆管炎，肝功能恶化）需要内镜逆行胰胆管造影（ERCP），和狭窄球囊扩张术。这种治疗方法可以改善胆汁淤积，瘙痒，甚至可能提高存活率。[56]Gluck M, Cantone NR, Brandaburr JJ, et al. A twenty-year experience with endoscopic therapy for symptomatic primary sclerosing cholangitis. J Clin Gastroenterol. 2008;42:1032-1039.http://www.ncbi.nlm.nih.gov/pubmed/18580600?tool=bestpractice.com[57]Gotthardt DN, Rudolph G, Klöters-Plachky P, et al. Endoscopic dilation of dominant stenoses in primary sclerosing cholangitis: outcome after long-term treatment. Gastrointest Endosc. 2010;71:527-534.http://www.ncbi.nlm.nih.gov/pubmed/20189511?tool=bestpractice.com[58]Aljiffry M, Renfrew PD, Walsh MJ, et al. Analytical review of diagnosis and treatment strategies for dominant bile duct strictures in patients with primary sclerosing cholangitis. HPB (Oxford). 2011;13:79-90.http://www.ncbi.nlm.nih.gov/pubmed/21241424?tool=bestpractice.com疗效：有中等级别的证据表明，治疗性ERCP可改善胆汁淤积，瘙痒，以及生存率。[56]Gluck M, Cantone NR, Brandaburr JJ, et al. A twenty-year experience with endoscopic therapy for symptomatic primary sclerosing cholangitis. J Clin Gastroenterol. 2008;42:1032-1039.http://www.ncbi.nlm.nih.gov/pubmed/18580600?tool=bestpractice.com受试者＜200名的随机对照临床试验（RCT）、受试者＞200名且方法学存在缺陷的随机对照临床试验（RCT）、方法学存在缺陷的系统评价或者高质量的观察性（队列）研究。对于复发性胆管狭窄患者，应考虑在狭窄部位放置可移除的塑料支架。[25]Chapman R, Fevery J, Kalloo A, et al. Diagnosis and management of primary sclerosing cholangitis. Hepatology. 2010;51:660-678.http://onlinelibrary.wiley.com/doi/10.1002/hep.23294/fullhttp://www.ncbi.nlm.nih.gov/pubmed/20101749?tool=bestpractice.com[58]Aljiffry M, Renfrew PD, Walsh MJ, et al. Analytical review of diagnosis and treatment strategies for dominant bile duct strictures in patients with primary sclerosing cholangitis. HPB (Oxford). 2011;13:79-90.http://www.ncbi.nlm.nih.gov/pubmed/21241424?tool=bestpractice.com内镜下取出原发性胆管结石（由狭窄处以上的胆汁淤积造成）也可能会改善临床症状。[56]Gluck M, Cantone NR, Brandaburr JJ, et al. A twenty-year experience with endoscopic therapy for symptomatic primary sclerosing cholangitis. J Clin Gastroenterol. 2008;42:1032-1039.http://www.ncbi.nlm.nih.gov/pubmed/18580600?tool=bestpractice.com弥漫性肝内胆管狭窄但无局灶性肝外胆管狭窄的患者，从内镜治疗中获益的可能性较小。PSC患者ERCP治疗发生并发症的可能性约为10%，因此推荐ERCP前预防性应用抗生素。[46]Etzel JP, Eng SC, Ko CW, et al. Complications after ERCP in patients with primary sclerosing cholangitis. Gastrointest Endosc. 2008;67:643-648.http://www.ncbi.nlm.nih.gov/pubmed/18061595?tool=bestpractice.com[56]Gluck M, Cantone NR, Brandaburr JJ, et al. A twenty-year experience with endoscopic therapy for symptomatic primary sclerosing cholangitis. J Clin Gastroenterol. 2008;42:1032-1039.http://www.ncbi.nlm.nih.gov/pubmed/18580600?tool=bestpractice.com[44]Stiehl A, Rudolph G, Kloters-Plachky P, et al. Development of dominant bile duct stenoses in patients with primary sclerosing cholangitis treated with ursodeoxycholic acid: outcome after endoscopic treatment. J Hepatol. 2002;36:151-156.http://www.ncbi.nlm.nih.gov/pubmed/11830325?tool=bestpractice.com[59]Hirota WK, Petersen K, Baron TH, et al. Guidelines for antibiotic prophylaxis for GI endoscopy. Gastrointest Endosc. 2003;58:475-482.http://www.ncbi.nlm.nih.gov/pubmed/14520276?tool=bestpractice.com
胆道内镜治疗也有不成功的案例。在这些情况下，可进行经皮肝穿刺胆道造影（PTC）引流胆汁，放置支架，和/或狭窄扩张。尽管刷片细胞学检查只有约18%的阳性率，但仍应进行以排除胆管癌。[43]Baron TH, Harewood GC, Rumalla A, et al. A prospective comparison of digital image analysis and routine cytology for the identification of malignancy in biliary tract strictures. Clin Gastroenterol Hepatol. 2004;2:214-219.http://www.ncbi.nlm.nih.gov/pubmed/15017605?tool=bestpractice.com当某些非肝硬化患者胆总管狭窄内镜治疗无效时，可采取外科手术治疗。[61]Pawlik TM, Olbrecht VA, Pitt HA, et al. Primary sclerosing cholangitis: role of extrahepatic biliary resection. J Am Coll Surg. 2008;206:822-830.http://www.ncbi.nlm.nih.gov/pubmed/18471705?tool=bestpractice.com
