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Sclerosing cholangitis

机译：硬化性胆管炎

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Primary sclerosing cholangitis is a chronic cholestatic disease that may have an autoimmune basis. Most patients have a circulating antineutrophil cytoplasmic antibody that appears to be targeted against a 50-kD nuclear envelope protein. The clinical applications of this antibody have not yet been defined. Other autoantibodies directed against antigens, such as cathepsin G, elastase, and anticardiolipin, may also be detected in some patients. It is suggested that primary sclerosing cholangitis may have a bacterial cause. Helicobacter gene sequences have been detected in liver tissues in primary sclerosing cholangitis. The role of Helicobacter spp and other bacteria in the etiopathogenesis of primary sclerosing cholangitis remains to be determined. Primary sclerosing cholangitis may overlap with autoimmune hepatitis in some cases, although the real prevalence of this association remains to be determined. Many prognostic models have been created, but they lack cross-validation, and their clinical usefulness remains limited. Endoscopic retrograde cholangiography remains the gold standard for diagnosis, but magnetic resonance imaging may be a viable alternative in many cases. Clinical trials with cladibrine, pentoxifylline, and budesonide have failed to demonstrate benefits. Orthotopic liver transplantation remains the only effective treatment.
机译：原发性硬化性胆管炎是一种慢性胆汁淤积性疾病，可能具有自身免疫性基础。大多数患者具有循环抗中性粒细胞胞浆抗体，似乎针对50 kD核包膜蛋白。该抗体的临床应用尚未确定。在某些患者中也可能检测到其他针对抗原的自身抗体，例如组织蛋白酶G，弹性蛋白酶和抗心磷脂。建议原发性硬化性胆管炎可能是细菌引起的。在原发性硬化性胆管炎的肝组织中已检测到幽门螺杆菌基因序列。幽门螺杆菌和其他细菌在原发性硬化性胆管炎的病因中的作用仍有待确定。原发性硬化性胆管炎在某些情况下可能与自身免疫性肝炎重叠，尽管这种关联的真正患病率尚待确定。已经建立了许多预后模型，但它们缺乏交叉验证，其临床实用性仍然有限。内镜逆行胆管造影术仍然是诊断的金标准，但在许多情况下磁共振成像可能是可行的选择。克拉屈滨，己酮可可碱和布地奈德的临床试验未能证明其益处。原位肝移植仍然是唯一有效的治疗方法。

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