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

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.

• Authors: 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, French Vasculitis Study Group (FVSG)
• Journal: Rheumatology (Oxford, England) (Rheumatology (Oxford)) Vol. 60 Issue 1 Pg. 359-365 (01 05 2021) ISSN: 1462-0332 [Electronic] England
• PMID: 32856066 (Publication Type: Journal Article, Multicenter Study, Research Support, Non-U.S. Gov't)
• Copyright: © The Author(s) 2020. Published by Oxford University Press on behalf of the British Society for Rheumatology. All rights reserved. For permissions, please email: [email protected].

Topics

• Acute Kidney Injury (etiology, pathology)
• Adolescent
• Adult
• Aged
• Aged, 80 and over
• Churg-Strauss Syndrome (complications, pathology)
• Female
• Humans
• Kidney (pathology)
• Male
• Middle Aged
• Retrospective Studies
• Young Adult