Renal involvement in eosinophilic granulomatosis with polyangiitis (EGPA): a multicentric retrospective study of 63 biopsy-proven cases
| Autor: | Marie Essig, Cécile-Audrey Durel, David Jayne, Christelle Barbet, Sandrine Hirschi-Santelmo, Thomas Le Gallou, Antoine Bardy, Jean-Jacques Boffa, Sylvain Marchand-Adam, Dimitri Titeca-Beauport, Grégory Pugnet, Xavier Belenfant, Camille Taillé, Xavier Puéchal, Cédric Rafat, Pascal Godmer, Vincent Cottin, Vítor Teixeira, Alexandre Karras, Julien Bouet, Renato Alberto Sinico, Jacques Gaultier, Philippe Guilpain, Daniel Engelbert Blockmans, Yoann Crabol, Christian Agard, Christophe Deligny |
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| Přispěvatelé: | 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 |
| Rok vydání: | 2020 |
| Předmět: |
Adult
Male 0301 basic medicine medicine.medical_specialty Adolescent 030232 urology & nephrology Churg-Strauss Syndrome Kidney Gastroenterology Nephropathy Young Adult 03 medical and health sciences 0302 clinical medicine renal biopsy Rheumatology Membranous nephropathy Internal medicine Eosinophilic medicine Humans Pharmacology (medical) Kidney transplantation Aged Retrospective Studies Aged 80 and over medicine.diagnostic_test business.industry Acute Kidney Injury Middle Aged medicine.disease renal involvement EGPA 030104 developmental biology medicine.anatomical_structure vasculiti glomerulonephriti Female Renal biopsy Granulomatosis with polyangiitis business Vasculitis |
| Zdroj: | Rheumatology. 60:359-365 |
| ISSN: | 1462-0332 1462-0324 |
| Popis: | 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. |
| Databáze: | OpenAIRE |
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