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Multicenter Study
. 2021 Jan 5;60(1):359-365.
doi: 10.1093/rheumatology/keaa416.

Renal involvement in eosinophilic granulomatosis with polyangiitis (EGPA): a multicentric retrospective study of 63 biopsy-proven cases

Cécile-Audrey Durel  1 , Renato A Sinico  2 , Vitor Teixeira  3 , David Jayne  4 , Xavier Belenfant  5 , Sylvain Marchand-Adam  6 , Gregory Pugnet  7 , Jacques Gaultier  8 , Thomas Le Gallou  9 , Dimitri Titeca-Beauport  10 , Christian Agard  11 , Christelle Barbet  12 , Antoine Bardy  13 , Daniel Blockmans  14 , Jean-Jacques Boffa  15 , Julien Bouet  16 , Vincent Cottin  17 , Yoann Crabol  18 , Christophe Deligny  19 , Marie Essig  20 , Pascal Godmer  18 , Philippe Guilpain  21 , Sandrine Hirschi-Santelmo  22 , Cédric Rafat  15 , Xavier Puéchal  23 , Camille Taillé  24 , Alexandre Karras  25 ; French Vasculitis Study Group (FVSG)
Affiliations
Multicenter Study

Renal involvement in eosinophilic granulomatosis with polyangiitis (EGPA): a multicentric retrospective study of 63 biopsy-proven cases

Cécile-Audrey Durel et al. Rheumatology (Oxford). .

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.

Keywords: EGPA; glomerulonephritis; renal biopsy; renal involvement; vasculitis.

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