原发干燥综合征肾脏损害临床病理特点及治疗进展

Clinicopathological Features and Treatment of Renal Impair in Primary Sj?gren Syndrome

原发干燥综合征是常见的累及多系统的自身免疫性疾病,以自身免疫性上皮炎为病理基础,肾脏是重要的受累靶器官。肾小管间质病变常见,可分为远端、近端小管和集合管受累。部分患者起病隐匿,逐渐出现肾功能损伤甚至终末期肾病,伴有小分子蛋白尿、肾小管酸中毒和电解质紊乱,病理表现为间质淋巴细胞浸润、肾小管萎缩和间质纤维化。肾小球损伤相对少见,主要病理类型为膜增性肾小球肾炎。治疗方案以糖皮质激素联合免疫抑制剂为主,也有使用B细胞清除靶向治疗的尝试。多数患者对治疗反应较好。本文主要综述近年原发干燥综合征肾脏损害的发病概况、临床病理特点及治疗进展。

Primary Sj gren syndrome,characterized by autoimmune epithelitis,is a prevalent systemic autoimmune disease involving multiple organs,among which kidney is a major target organ.Tubulointerstitial lesion is the most frequent form,involving proximal tubule,distal tubule,or collec ting duct.The disease has an occult onset and may progressively develop into renal function impairment and end-stage renal disease,which can be accompanied with low-molecular-weight proteinuria,renal tubule acidosis and electrolyte disturbance.Pathologically,it is featured by lymphocyte infiltration,renal tubule atrophy,and interstitial fibrosis.Glomerular lesion is less common and usually takes the form of membranoproliferative glomerulitis.Glucocorticoid combined with immunosuppresant is the main treatment option,and B cell-targeted therapy has been reported.Most patients respond well to these treatments.In this article we review the prevalence,clinicopathological features,and treatment of renal disease in primary Sj gren syndrome.