Cécile-Audrey Durel1, Renato A Sinico2, Vitor Teixeira3, David Jayne4, Xavier Belenfant5, Sylvain Marchand-Adam6, Gregory Pugnet7, Jacques Gaultier8, Thomas Le Gallou9, Dimitri Titeca-Beauport10, Christian Agard11, Christelle Barbet12, Antoine Bardy13, Daniel Blockmans14, Jean-Jacques Boffa15, Julien Bouet16, Vincent Cottin17, Yoann Crabol18, Christophe Deligny19, Marie Essig20, Pascal Godmer18, Philippe Guilpain21, Sandrine Hirschi-Santelmo22, Cédric Rafat15, Xavier Puéchal23, Camille Taillé24, Alexandre Karras25. 1. Department of Internal Medicine, Hôpital Edouard Herriot, Hospices Civils De Lyon, Lyon, France. 2. Department of Medicine and Surgery, Universita di Milano-Biococca, Milano, Italy. 3. Department of Rheumatology, Centro Hospitalar Universitário do Algarve, Faro, Portugal. 4. Department of Medicine, University of Cambridge, Addenbrooke's Hospital, Cambridge, UK. 5. Department of Nephrology, Centre Hospitalier Intercommunal André Grégoire, Montreuil. 6. Department of Pneumology, Hôpital Bretonneau, Tours. 7. Department of Internal Medicine, Hôpital Purpan, Toulouse. 8. Department of Internal Medicine, CH Gap, Gap. 9. Department of Internal Medicine, CHRI Rennes Site Hôpital Sud, Rennes. 10. Department of Nephrology, CHU Amiens-Picardie, Amiens. 11. Department of Internal Medicine, CHU de Nantes Site Hôtel Dieu-HME, Nantes. 12. Department of Nephrology, CHRU Bretonnneau-Tours, Tours. 13. Department of Internal Medicine, Centre Hospitalier Moulins-Yzeure, Moulins, France. 14. Department of General Internal Medicine, KU Leuven, Leuven, Belgium. 15. Department of Nephrology, Hôpital Tenon AP-HP, Paris. 16. Department of Nephrology, CHPC Site Cherbourg, Cherbourg Octeville. 17. National Coordinating Reference Centre for Rare Pulmonary Diseases, Hôpital Louis Pradel, Hospices Civils De Lyon, University Claude Bernard Lyon 1, Lyon. 18. Department of Internal Medicine, CHBA Site de Vannes, Vannes. 19. Department of Rheumatology and Internal Medicine, CHU Martinique, Hôpital P. Zobda-Quitman, Fort-de-France. 20. Department of Nephrology, Hopital Ambroise Paré, Boulogne-Billancourt. 21. Department of Internal Medicine-Multi-Organ Diseases, Montpellier University-Saint Eloi Hospital, Montpellier. 22. Department of Pneumology, Nouvel Hôpital Civil, HUS, Strasbourg. 23. Department of Internal Medicine, Hôpital Cochin. 24. Department of Respiratory Diseases, Hôpital Bichat. 25. Department of Nephrology, Hôpital Européen Georges Pompidou, AP-HP, Paris, France.
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.
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: David Faz-Muñoz; Andrea Hinojosa-Azaola; Juan M Mejía-Vilet; Norma O Uribe-Uribe; Marina Rull-Gabayet; Wallace Rafael Muñoz-Castañeda; Nancy Janeth Vargas-Parra; Eduardo Martín-Nares Journal: Immunol Res Date: 2022-04-21 Impact factor: 4.505