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

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 Ma...

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Published in:Journal of clinical medicine 2019-08, Vol.8 (8), p.1218
Main Authors: 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
Format: Article
Language:English
Subjects:
Cancer
Life Sciences
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Summary: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.
ISSN:2077-0383
2077-0383
DOI:10.3390/jcm8081218