Guibert Fanny, Garnier Anne-Sophie, Wacrenier Samuel, Piccoli Giorgina, Djema Assia, Gansey Renaud, Demiselle Julien, Brilland Benoit, Cousin Maud, Besson Virginie, Duveau Agnès, El Nasser Khuzama, Coindre Jean-Philippe, Croue Anne, Saint-André Jean-Paul, Chevailler Alain, Subra Jean-François, Augusto Jean-François
CHU Angers, Service de Néphrologie-Dialyse-Transplantation, Université d'Angers, 49100 Angers, France.
Service de Néphrologie, Centre Hospitalier du Mans, 72037 Le Mans, France.
J Clin Med. 2019 Aug 14;8(8):1218. doi: 10.3390/jcm8081218.
The overlap between antineutrophil cytoplasmic antibody (ANCA) associated glomerulonephritis (ANCA-GN) and connective tissue diseases (CTD) has been reported mainly as case series in the literature. Frequency of this association, as well as presentation and outcomes are unknown.
Patients from the Maine-Anjou ANCA-associated vasculitides (AAV) registry with ANCA-GN diagnosed between 01/01/2000 and 01/01/2018, ANCA positivity, and at least six months of follow-up, were included.
106 out of 142 patients fulfilled the inclusion criteria and were analyzed. CTD was present at ANCA-GN diagnosis in 16 (15.1%) patients. The most common CTD were rheumatoid arthritis, Sjogren syndrome and systemic sclerosis. Compared to the control group, females were more represented in the CTD group (75%, = 0.001). Renal presentation was comparable between groups, including the pathological analysis of renal biopsies. Patients of CTD group presented a higher rate of non-renal relapse (25% versus 7.7%, = 0.037), and experienced more frequently a venous thrombotic event (31.2% versus 10%, = 0.021). No difference between groups was observed according to major outcomes.
Association between CTD and ANCA-GN is not a rare condition and predominantly affects females. While AAV presentation is not significantly different, CTD patients experience more frequently non-renal relapse and venous thrombotic events.
抗中性粒细胞胞浆抗体（ANCA）相关肾小球肾炎（ANCA-GN）与结缔组织病（CTD）之间的重叠在文献中主要作为病例系列报道。这种关联的频率以及表现和结局尚不清楚。
纳入缅因-安茹ANCA相关血管炎（AAV）登记处2000年1月1日至2018年1月1日期间诊断为ANCA-GN、ANCA阳性且至少随访6个月的患者。
142例患者中有106例符合纳入标准并进行了分析。16例（15.1%）患者在ANCA-GN诊断时存在CTD。最常见的CTD是类风湿关节炎、干燥综合征和系统性硬化症。与对照组相比，CTD组女性比例更高（75%，P = 0.001）。两组间肾脏表现相当，包括肾活检的病理分析。CTD组患者非肾脏复发率更高（25%对7.7%，P = 0.037），且静脉血栓形成事件更频繁（31.2%对10%，P = 0.021）。根据主要结局，两组间未观察到差异。
CTD与ANCA-GN之间的关联并非罕见情况，且主要影响女性。虽然AAV的表现无显著差异，但CTD患者非肾脏复发和静脉血栓形成事件更频繁。
