1. Renal involvement in eosinophilic granulomatosis with polyangiitis (EGPA): a multicentric retrospective study of 63 biopsy-proven cases
- Author
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Durel, C, Sinico, R, Teixeira, V, Jayne, D, Belenfant, X, Marchand-Adam, S, Pugnet, G, Gaultier, J, Le Gallou, T, Titeca-Beauport, D, Agard, C, Barbet, C, Bardy, A, Blockmans, D, Boffa, J, Bouet, J, Cottin, V, Crabol, Y, Deligny, C, Essig, M, Godmer, P, Guilpain, P, Hirschi-Santelmo, S, Rafat, C, Puéchal, X, Taillé, C, Karras, A, Durel, Cécile-Audrey, Sinico, Renato A, Teixeira, Vitor, Jayne, David, Belenfant, Xavier, Marchand-Adam, Sylvain, Pugnet, Gregory, Gaultier, Jacques, Le Gallou, Thomas, Titeca-Beauport, Dimitri, Agard, Christian, Barbet, Christelle, Bardy, Antoine, Blockmans, Daniel, Boffa, Jean-Jacques, Bouet, Julien, Cottin, Vincent, Crabol, Yoann, Deligny, Christophe, Essig, Marie, Godmer, Pascal, Guilpain, Philippe, Hirschi-Santelmo, Sandrine, Rafat, Cédric, Puéchal, Xavier, Taillé, Camille, Karras, Alexandre, Durel, C, Sinico, R, Teixeira, V, Jayne, D, Belenfant, X, Marchand-Adam, S, Pugnet, G, Gaultier, J, Le Gallou, T, Titeca-Beauport, D, Agard, C, Barbet, C, Bardy, A, Blockmans, D, Boffa, J, Bouet, J, Cottin, V, Crabol, Y, Deligny, C, Essig, M, Godmer, P, Guilpain, P, Hirschi-Santelmo, S, Rafat, C, Puéchal, X, Taillé, C, Karras, A, Durel, Cécile-Audrey, Sinico, Renato A, Teixeira, Vitor, Jayne, David, Belenfant, Xavier, Marchand-Adam, Sylvain, Pugnet, Gregory, Gaultier, Jacques, Le Gallou, Thomas, Titeca-Beauport, Dimitri, Agard, Christian, Barbet, Christelle, Bardy, Antoine, Blockmans, Daniel, Boffa, Jean-Jacques, Bouet, Julien, Cottin, Vincent, Crabol, Yoann, Deligny, Christophe, Essig, Marie, Godmer, Pascal, Guilpain, Philippe, Hirschi-Santelmo, Sandrine, Rafat, Cédric, Puéchal, Xavier, Taillé, Camille, and Karras, Alexandre
- Abstract
Objective: Eosinophilic granulomatosis with polyangiitis (EGPA) is a systemic small-vessel vasculitis characterized by asthma, hypereosinophilia and ANCA positivity in 40% of patients. Renal involvement is rare and poorly described, leading to this renal biopsy-proven based study in a large EGPA cohort. Methods: We conducted a retrospective multicentre study including patients fulfilling the 1990 ACR criteria and/or the 2012 revised Chapel Hill Consensus Conference criteria for EGPA and/or the modified criteria of the MIRRA trial, with biopsy-proven nephropathy. Results: Sixty-three patients [27 women, median age 60 years (18-83)] were included. Renal disease was present at vasculitis diagnosis in 54 patients (86%). ANCA were positive in 53 cases (84%) with anti-MPO specificity in 44 (83%). All patients had late-onset asthma. Peripheral neuropathy was present in 29 cases (46%), alveolar haemorrhage in 10 (16%). The most common renal presentation was acute renal failure (75%). Renal biopsy revealed pauci-immune necrotizing GN in 49 cases (78%). Membranous nephropathy (10%) and membranoproliferative GN (3%) were mostly observed in ANCA-negative patients. Pure acute interstitial nephritis was found in six cases (10%); important interstitial inflammation was observed in 28 (44%). All patients received steroids with adjunctive immunosuppression in 54 cases (86%). After a median follow-up of 51 months (1-296), 58 patients (92%) were alive, nine (14%) were on chronic dialysis and two (3%) had undergone kidney transplantation. Conclusion: Necrotizing pauci-immune GN is the most common renal presentation in ANCA-positive EGPA. ANCA-negative patients had frequent atypical renal presentation with other glomerulopathies such as membranous nephropathy. An important eosinophilic interstitial infiltration was observed in almost 50% of cases.
- Published
- 2021