系统性红斑狼疮合并原发性硬化性胆管炎三例并文献复习

Systemic lupus erythematosus with primary sclerosing cholangitis

目的探讨SLE合并原发性硬化性胆管炎(PSC)的临床表现、实验室和影像学特征、诊治及预后。方法分析3例SLE合并PSC患者的临床、实验室、影像学表现及治疗和预后,对相关文献进行复习并与本研究结果对比。结果搜索到文献报道7例,结合本文病例3例,共10例纳入研究。男性5例,女性5例,平均年龄(43±17)岁,临表表现多见黄疸、肾脏受累、关节痛;实验室检查见抗dsDNA抗体阳性,多见血液系统损害、ESR增快、低补体血症以及肝酶和胆酶升高,肝功能异常以ALP升高为主。磁共振胰胆管造影(MRCP)的特异表现为胆管狭窄、扩张、串珠样改变,可同时累及肝内外胆管。治疗多采用激素和熊去氧胆酸(UDCA),部分采用胆道引流。多数患者预后良好。结论PSC虽然少见,但在SLE合并肝功能异常,特别以ALP升高为主的患者中,要注意鉴别PSC。

Objective To investigate the clinical manifestations,laboratory and imaging features,treatment and prognosis of systemic lupus erythematosus(SLE)with primary sclerosing cholangitis(PSC).Methods This is a retrospective case series study describing the clinical,laboratory and imaging manife-stations,treatment and prognosis of 3 SLE patients with PSC.The related literatures were reviewed.Results In total,10 patients were included.SLE with PSC,with an average age of(43±17)years old,was more common with hematological and renal involvement,jaundice and arthralgia,positive anti-double-stranded DNA(anti-dsDNA)antibody,hypocomplementemia,elevated erythrocyte sedimentation rate(ESR)and abnormal liver function with predominately elevated alkaline phosphatase(ALP).The classic magnetic resonance cholangio-pancreatography(MRCP)features of PSC include multifocal strictures,beading and areas of dilatation involving the intra-and/or extrahepatic bile ducts.The treatment included glucocorticosteroids,immunosup-pressants and ursodeoxycholic acid(UDCA),and some patients required biliary drainage.Most patients had a good prognosis.Conclusion Although PSC is rare,attention should be paid to SLE patients with abnormal liver function,especially with elevated ALP,in order to differentiate from PSC.