【前沿进展】男性狼疮肾炎多为IV型病理类型的肾病综合征
摘要:介绍:因为系统性红斑狼疮和狼疮性肾炎在男性中的少见性,对其的理解也较缺乏。我们的目的在于分析既往史明确诊断的男性系统性红斑狼疮和狼疮性肾炎的临床表现及病因,并分析疾病进展至肾病终末期的危险因素。方法学:对55位来自西班牙和乌拉圭的历史队列研究中的患者进行回顾性分析,并与一组年龄及疾病特点匹配的女性患者队列进行对比分析。结果:肾脏活检的中位年龄是27岁(区间:8-79岁)。主要临床表现形式是肾病综合征(50位患者中有26位,占52%)和Ⅳ型狼疮性肾炎(50位患者中有34位,占68%)。经过治疗,21位患者(45.6%)获得完全肾脏缓解。在随访期间,12位患者需要肾脏替代治疗,3位患者死于感染相关疾病。当对需要肾脏替代治疗治疗患者及不需要替代的患者的肾脏活检时期指标进行对比分析时,以下几项参数有统计学差异(P<0.05):估算的肾小球滤过率<60ml/min,高血压,低白蛋白血症,相伴随的其他脏器受累(神经系统,心血管和或肺脏)。在多变量分析中,仅估算的肾小球滤过率<60ml/min这一项仍为疾病进展至肾病终末期的危险因素。当与女性狼疮性肾炎患者的队列进行对比分析时,缓解或肾脏保存的指标均无显著性差异。讨论:男性狼疮性肾炎常表现为肾病综合征,Ⅳ型狼疮性肾炎是最常见病理表型。进行肾脏活检时的估算的肾小球滤过率<60ml/min与肾脏不良预后相关。在对比男性和女性狼疮性肾炎患者队列时,狼疮性肾炎的缓解或进展并无显著性差异。
附原文:
Abstract INTRODUCTION:Becauseof their rarity in men, systemiclupuserythematous andlupusnephritis(LN) are poorly understood in men. Our aim was to analyze theclinical presentation and course of histology-provenystemiclupuserythematous and LN inmalesand to determinethe risk factors for progression to end-stage renal disease. METHODS:Fiftypatients from 2 historical cohorts in Spain (Hospital 12 de Octubre) andUruguay were retrospectively analyzed and compared with a female cohort matchedfor age and disease characteristics.RESULTS:The median age at the time of renalbiopsy was 27 years (range, 8-79 years). The main forms of presentation werenephrotic syndrome in 26 of 50 patients (52%), and class IV LN in 34 of 50(68%). After treatment, 21 patients (45.6%) achieved complete renal remission.During follow-up, 12 patients required renal replacement therapy, and 3patients died of infectious causes. When patients who required renalreplacement therapy were compared with those who did not require it, severalparameters showed significant differences (P< 0.05) at the timeof renal biopsy: estimated glomerular filtration rate< 60 ml/min,hypertension, hypoalbuminemia, and concomitant visceral involvement(neurologic, cardiovascular, and/or pulmonary). In the multivariate analysis,only estimated glomerular filtration rate< 60 ml/min persisted as arisk factor for progression to end-stage renal disease. When compared with acohort of female patients with LN, there were no significant differences inremission or renal survival.DISCUSSION:LN inmalesusually presents as nephroticsyndrome, and type IV LN is the most frequent form. An estimated glomerularfiltration rate< 60 ml/min at the time of renal biopsy is associatedwith poor renal outcomes. There were no differences in remission or progressionof LN inmaleswhen compared with a cohort of female patients withLN.
引自:Urrestarazú A,Otatti G,SilvariñoR,et al. LupusNephritisinMales: Clinical Features, Course, and Prognostic Factorsfor End-Stage Renal Disease.Kidney Int Rep.2017 Jun16;2(5):905-912. doi: 10.1016/j.ekir.2017.05.011. eCollection 2017 Sep.
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