Jarrot Pierre-Andre, Chiche Laurent, Hervier Baptiste, Daniel Laurent, Vuiblet Vincent, Bardin Nathalie, Bertin Daniel, Terrier Benjamin, Amoura Zahir, Andrés Emmanuel, Rondeau Eric, Hamidou Mohamed, Pennaforte Jean-Loup, Halfon Philippe, Daugas Eric, Dussol Bertrand, Puéchal Xavier, Kaplanski Gilles, Jourde-Chiche Noemie
From the Department of Internal Medicine and Clinical Immunology (PAJ, GK), AP-HM Hôpital de La Conception; Inserm UMR-S 1076 Vascular Research Center of Marseille (PAJ, GK, NJ-C), Aix-Marseille Université; Department of Internal Medicine (LC, PH), Hôpital Européen de Marseille, Marseille; Reference Center for Systemic Lupus Erythematosus (BH, ZA), Department of Internal Medicine, AP-HP Hôpital Pitie-Salpêtrière, Paris; Department of Pathology (LD), AP-HM Hôpital de La Timone, Aix-Marseille Université; Department of Pathology (VV), Paul Bouin Laboratory, Reims, France; Laboratory of Immunology (NB, DB), AP-HM Hôpital de la Conception, Aix-Marseille Université, Marseille; French Vasculitis Study Group (BT, XP), Department of Internal Medicine, National Reference Center for Necrotizing Vasculitis, AP-HP Hôpital Cochin, University Paris-Descartes; Department of Internal Medicine (EA), Hôpital Civil, Strasbourg; Department of Nephrology (ER), AP-HP Hôpital Tenon, Paris; Department of Internal Medicine (MH), Hôpital Hôtel-Dieu, Nantes; Department of Internal Medicine (J-LP), Hôpital Robert Debré, Reims Université, Reims; Groupe Coopératif sur le Lupus Rénal (ED), Department of Nephrology, AP-HP Hôpital Bichat, Paris; and Department of Nephrology (BD, NJ-C), AP-HM Hôpital Conception, Aix-Marseille Université, Marseille, France.
Medicine (Baltimore). 2016 May;95(22):e3748. doi: 10.1097/MD.0000000000003748.
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.
本研究旨在报告继发于系统性红斑狼疮（SLE）/抗中性粒细胞胞浆抗体（ANCA）相关血管炎（AAV）重叠综合征的肾小球肾炎（GN）患者的临床、生物学和病理学特征。开展了一项全国性调查以确定SLE/AAV重叠综合征病例。数据收集自法国的SLE和AAV研究小组。纳入标准为根据国际分类标准诊断为SLE和AAV，且在1995年至2014年间经活检证实为GN。通过系统的文献综述确定了其他病例。使用一组经连续活检证实的GN病例来研究重叠抗体和/或重叠综合征的患病率。全国性调查确定了8例SLE/AAV重叠综合征病例。所有患者均为女性；中位年龄为40岁。AAV发生在SLE之前（n = 3）、之后（n = 3）或同时发生（n = 2）。6例患者患有快速进展性GN，8例中有3例发生肺泡出血。所有患者均有抗核抗体（ANA）；8例中有7例有抗髓过氧化物酶（MPO）的p-ANCA抗体。肾活检显示为狼疮性肾炎（LN）或寡免疫性GN。8例患者中有4例获得缓解。文献综述确定了另外31例表现同样严重的病例。在GN队列中，30%的LN患者ANCA阳性，52%的寡免疫性GN患者ANA阳性，与病理结果无相关性。SLE/AAV重叠综合征的估计患病率为2/101（2%）。在GN患者中，SLE/AAV重叠综合征可能发生，但患病率较低。大多数患者有侵袭性肾脏表现，通常同时有ANA和抗MPO抗体。需要进一步研究以评估共同的发病机制和治疗选择。
