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. 2019 Aug 14;8(8):1218.
doi: 10.3390/jcm8081218.

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

Fanny Guibert  1 , Anne-Sophie Garnier  1 , Samuel Wacrenier  1 , Giorgina Piccoli  2 , Assia Djema  3 , Renaud Gansey  4 , Julien Demiselle  1 , Benoit Brilland  1   5 , Maud Cousin  1 , Virginie Besson  1 , Agnès Duveau  1 , Khuzama El Nasser  3 , Jean-Philippe Coindre  2 , Anne Croue  6 , Jean-Paul Saint-André  6 , Alain Chevailler  5   7 , Jean-François Subra  1   5 , Jean-François Augusto  8   9
Affiliations

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

Fanny Guibert et al. J Clin Med. .

Abstract

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.

Keywords: ANCA; connective tissue disease; glomerulonephritis; vasculitis.

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Conflict of interest statement

The authors declare no conflict of interest.

Figures

Figure 1
Figure 1
Flowchart of the study. AAV, ANCA-associated vasculitis; ANCA, antineutrophil cytoplasmic antibody; MPO, myeloperoxidase; PR3, proteinase-3.
Figure 2
Figure 2
Incidence of ANCA-associated vasculitides (AAV) and AAV+CTD in the cohort study according to the year of diagnosis. AAV, ANCA-associated vasculitis; CTD, connective tissue disease.
Figure 3
Figure 3
Analysis of light microscopy of kidney biopsy and comparison according to groups. (A) percentage of normal, crescentic and fibrotic glomeruli according to the presence or absence of CTD. (B) histopathological classes of ANCA-GN according to groups.
See this image and copyright information in PMC

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