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Open AccessArticle

Patients with ANCA-Associated Glomerulonephritis and Connective Tissue Diseases: A Comparative Study from the Maine-Anjou AAV Registry

1
CHU Angers, Service de Néphrologie-Dialyse-Transplantation, Université d’Angers, 49100 Angers, France
2
Service de Néphrologie, Centre Hospitalier du Mans, 72037 Le Mans, France
3
Service de Néphrologie, Centre Hospitalier de Cholet, 49300 Cholet, France
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Service de Néphrologie, Centre Hospitalier de Laval, 53000 Laval, France
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CRCINA, INSERM, Université de Nantes, Université d’Angers, 49100 Angers, France
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CHU Angers, Département de Pathologie Cellulaire et Tissulaire, Université d’Angers, 49100 Angers, France
7
CHU Angers, Laboratoire d’Immunologie, Université d’Angers, 49100 Angers, France
*
Author to whom correspondence should be addressed.
These authors contributed equally to this work.
J. Clin. Med. 2019, 8(8), 1218; https://doi.org/10.3390/jcm8081218
Received: 15 July 2019 / Revised: 5 August 2019 / Accepted: 12 August 2019 / Published: 14 August 2019
(This article belongs to the Section Nephrology & Urology)
Background and objectives: 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. Materials and Methods: 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. Results: 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%, p = 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%, p = 0.037), and experienced more frequently a venous thrombotic event (31.2% versus 10%, p = 0.021). No difference between groups was observed according to major outcomes. Conclusion: 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. View Full-Text
Keywords: ANCA; vasculitis; connective tissue disease; glomerulonephritis ANCA; vasculitis; connective tissue disease; glomerulonephritis
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Guibert, F.; Garnier, A.-S.; Wacrenier, S.; Piccoli, G.; Djema, A.; Gansey, R.; Demiselle, J.; Brilland, B.; Cousin, M.; Besson, V.; Duveau, A.; El Nasser, K.; Coindre, J.-P.; Croue, A.; Saint-André, J.-P.; Chevailler, A.; Subra, J.-F.; Augusto, J.-F. Patients with ANCA-Associated Glomerulonephritis and Connective Tissue Diseases: A Comparative Study from the Maine-Anjou AAV Registry. J. Clin. Med. 2019, 8, 1218.

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