【SLE/AAV重叠综合征患者…

SLE/AAV重叠综合征患者的肾损害较重

作者：Jarrot PA , et al. 译者：北医三院于若寒<1834379778@qq.com>
发布者：于若寒，校正：刘佩玲
摘要: 本文研究目的是总结继发于系统性红斑狼疮（SLE）/ANCA相关性血管炎（AAV）重叠综合征的肾小球肾炎患者的临床、生物学和病理学特征。这是一个关于SLE/AAV重叠综合征患者的全国性调查。数据来源于SLE和AAV法国研究组。入选标准是在1995到2014年间，根据国际分类标准确诊的SLE和AAV和肾活检证实的肾小球肾炎。通过系统的文献复习确定了另外一些患者。一个连续的经肾活检证实的肾小球肾炎队列用来研究重叠抗体或重叠综合征的患病率。这个全国范围的调查确诊了8例SLE/AAV重叠综合征患者。所有患者均为女性，中位年龄为40岁。3例患者AAV发生在SLE之前，3例患者AAV发生在SLE之后，2例患者二者同时发生。6例患者有急性进展性肾小球肾炎，3例患者有肺泡出血。所有患者ANA均阳性，7例患者p-ANCA （MPO）阳性。肾活检提示狼疮肾炎（LN）或寡免疫复合物肾小球肾炎。4例患者达到了缓解。通过文献复习确定了31例有相似严重表现的患者。在肾小球肾炎队列中，30% 的LN患者ANCA阳性，52%的寡免疫复合物肾小球肾炎ANA阳性，与病理表现无相关性。SLE/AAV重叠综合征的估计患病率是2/101（2%）。在肾小球肾炎患者中，可能出现SLE/AAV重叠综合征，但发生率较低。多数患者有严重的肾脏表现， ANA和MPO抗体常同时阳性。需要进一步的研究以确定他们的共同的病理机制和治疗选择。
附原文：
Abstract The aim of the study was to report the clinical, biological, and pathological characteristics of patients with glomerulonephritis (GN) secondary to systemic lupus erythematosus (SLE)/antineutrophil cytoplasmic antibodies (ANCA)-associated vasculitis (AAV) overlap syndrome.A nationwide survey was conducted to identify cases of SLE/AAV overlap syndrome. Data were collected from SLE and AAV French research groups. Inclusion criteria were diagnosis of both SLE and AAV according to international classification criteria and biopsy-proven GN between 1995 and 2014. Additional cases were identified through a systematic literature review. A cohort of consecutive biopsy-proven GN was used to study the prevalence of overlapping antibodies and/or overlap syndrome.The national survey identified 8 cases of SLE/AAV overlap syndrome. All patients were female; median age was 40 years. AAV occurred before SLE (n = 3), after (n = 3), or concomitantly (n = 2). Six patients had rapidly progressive GN and 3/8 had alveolar hemorrhage. All patients had antinuclear antibodies (ANA); 7/8 had p-ANCA antimyeloperoxidase (MPO) antibodies. Renal biopsies showed lupus nephritis (LN) or pauci-immune GN. Remission was obtained in 4/8 patients. A literature review identified 31 additional cases with a similarly severe presentation. In the GN cohort, ANCA positivity was found in 30% of LN, ANA positivity in 52% of pauci-immune GN, with no correlation with pathological findings. The estimated prevalence for SLE/AAV overlap syndrome was 2/101 (2%).In patients with GN, SLE/AAV overlap syndrome may occur but with a low prevalence. Most patients have an aggressive renal presentation, with usually both ANA and anti-MPO antibodies. Further studies are needed to assess shared pathogenesis and therapeutic options.
引自：JARROT P A, CHICHE L, HERVIER B, et al. Systemic Lupus Erythematosus and Antineutrophil Cytoplasmic Antibody-Associated Vasculitis Overlap Syndrome in Patients With Biopsy-Proven Glomerulonephritis [J]. Medicine, 2016, 95(22): e3748.