JOURNAL ARTICLE

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

Cécile-Audrey Durel, Renato A Sinico, Vitor Teixeira, David Jayne, Xavier Belenfant, Sylvain Marchand-Adam, Gregory Pugnet, Jacques Gaultier, Thomas Le Gallou, Dimitri Titeca-Beauport, Christian Agard, Christelle Barbet, Antoine Bardy, Daniel Blockmans, Jean-Jacques Boffa, Julien Bouet, Vincent Cottin, Yoann Crabol, Christophe Deligny, Marie Essig, Pascal Godmer, Philippe Guilpain, Sandrine Hirschi-Santelmo, Cédric Rafat, Xavier Puéchal, Camille Taillé, Alexandre Karras
Rheumatology 2020 August 28
32856066

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.

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Christoph Haller

V. A. ANCA+ machen GN

0

Christoph Haller

TN retrospektiv. Nierenbiopsate. Vor Paper renal in EGPA 10%.
(TN Aussage 30 bis 40%Anca positiv).
Hier viele mpo
V.a. Nekrotisierend, interstitielle Nephritis (wichtiges Thema).

35% halbmond (meist also rasch progressive GN)
35 fokal
15
Sklerotisch
15 gemischt
(Berden)

0

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