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.
Durel, C., Sinico, R., Teixeira, V., Jayne, D., Belenfant, X., Marchand-Adam, S., et al. (2021). Renal involvement in eosinophilic granulomatosis with polyangiitis (EGPA): a multicentric retrospective study of 63 biopsy-proven cases. RHEUMATOLOGY, 60(Issue 1, January 2021), 359-365.
|Citazione:||Durel, C., Sinico, R., Teixeira, V., Jayne, D., Belenfant, X., Marchand-Adam, S., et al. (2021). Renal involvement in eosinophilic granulomatosis with polyangiitis (EGPA): a multicentric retrospective study of 63 biopsy-proven cases. RHEUMATOLOGY, 60(Issue 1, January 2021), 359-365.|
|Tipo:||Articolo in rivista - Articolo scientifico|
|Carattere della pubblicazione:||Scientifica|
|Presenza di un coautore afferente ad Istituzioni straniere:||Si|
|Titolo:||Renal involvement in eosinophilic granulomatosis with polyangiitis (EGPA): a multicentric retrospective study of 63 biopsy-proven cases|
|Autori:||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|
SINICO, RENATO ALBERTO (Secondo)
|Data di pubblicazione:||2021|
|Digital Object Identifier (DOI):||http://dx.doi.org/10.1093/rheumatology/keaa416|
|Appare nelle tipologie:||01 - Articolo su rivista|