McCurdy D K, Lehman T J, Bernstein B, Hanson V, King K K, Nadorra R, Landing B H
Division of Rheumatology, Childrens Hospital of Los Angeles, CA.
Pediatrics. 1992 Feb;89(2):240-6.
As newer treatment modalities become available for patients with severe lupus nephritis, it becomes increasingly important to identify patients at risk for renal failure. In this study, the records of 90 children presenting with systemic lupus erythematosus over a 13-year period were reviewed. Nineteen were lost to follow-up prior to completion of the study. Of the 71 remaining children, 16 (22%) progressed to chronic renal failure. Persistent hypertension lasting greater than 4 months, anemia, abnormalities of the urinalysis, and elevated serum creatinine level were significantly associated with progression to renal failure. Sex, race, age, abnormalities of creatinine clearance, and 24-hour urine protein collection were not associated with progression to renal failure. Renal biopsies were obtained in 45 children. Biopsies were initially classified according to World Health Organization criteria. Diffuse proliferative glomerulonephritis was significantly associated with progression to renal failure. The 45 biopsies available were reviewed by one of the authors and categorized by activity and chronicity indices. Both the active lesions of fibrinoid necrosis, synechiae, tubular casts, and vasculitic lesions and the chronic lesion of glomerular sclerosis correlated with progression to renal failure. Of the 16 children who progressed to renal failure, 2 had cadaver kidney transplants and are well 5 years posttransplant; 4 had fulminant lupus and died within 1 month of commencing dialysis; 10 began chronic dialysis. Five of the 10 children on chronic dialysis died from sepsis. These data suggest that children with systemic lupus erythematosus who undergo dialysis do poorly.(ABSTRACT TRUNCATED AT 250 WORDS)
随着针对重症狼疮性肾炎患者的更新治疗方式不断涌现,识别有肾衰竭风险的患者变得越发重要。在本研究中,回顾了90名在13年期间出现系统性红斑狼疮的儿童的记录。19名儿童在研究完成前失访。在其余71名儿童中,16名(22%)进展为慢性肾衰竭。持续高血压超过4个月、贫血、尿液分析异常以及血清肌酐水平升高与进展至肾衰竭显著相关。性别、种族、年龄、肌酐清除率异常以及24小时尿蛋白收集量与进展至肾衰竭无关。45名儿童进行了肾活检。活检最初根据世界卫生组织标准进行分类。弥漫性增殖性肾小球肾炎与进展至肾衰竭显著相关。现有的45份活检标本由一位作者进行复查,并根据活动度和慢性度指数进行分类。纤维蛋白样坏死、粘连、肾小管管型和血管病变的活动性病变以及肾小球硬化的慢性病变均与进展至肾衰竭相关。在进展至肾衰竭的16名儿童中,2名接受了尸体肾移植,移植后5年情况良好;4名患有暴发性狼疮,在开始透析后1个月内死亡;10名开始进行慢性透析。10名接受慢性透析的儿童中有5名死于败血症。这些数据表明,接受透析的系统性红斑狼疮儿童预后较差。(摘要截短至250字)