Raphael Lhote1, Marie Chilles1, Matthieu Groh1, Xavier Puéchal1, Philippe Guilpain1, Félix Ackermann1, Zahir Amoura1, Isabella Annesi-Maesano1, Thomas Barba1, Emilie Catherinot1, Fleur Cohen-Aubart1, Pascal Cohen1, Vincent Cottin1, Louis-Jean Couderc1, Hubert De Boysson1, Xavier Delbrel1, Stéphane Dominique1, Pierre Duhaut1, Olivier Fain1, Eric Hachulla1, Mohamed Hamidou1, Jean-Emmanuel Kahn1, Christophe Legendre1, Alain Le Quellec1, François Lhote1, François Lifermann1, Alexis Mathian1, Antoine Néel1, Hilario Nunes1, Jean-François Subra1, Benjamin Terrier1, Luc Mouthon1, Elisabeth Diot1, Loïc Guillevin1, Pierre-Yves Brillet1, Colas Tcherakian. 1. From the Department of Pulmonology, Hôpital Foch, Suresnes, Faculté des Sciences de la Vie, Simone Veil, Université de Versailles, UPRES EA 220, France; Sorbonne Université, UPMC, Pierre Louis Institute of Epidemiology and Public Health (IPLESP UMRS 1136), Epidemiology of Allergic and Respiratory Diseases Department (EPAR), Saint-Antoine Medical School, Paris; Department of Internal Medicine, Orléans, France; Department of Internal Medicine, CHU, Tours, France; Department of Internal Medicine, CEREO (National Referral Center for Hypereosinophilic Syndromes), Hôpital Foch, Suresnes, France; Department of Internal Medicine, National Referral Center for Rare Systemic Autoimmune Diseases, Hôpital Cochin, AP-HP, Université Paris Descartes, Paris; Department of Internal Medicine, Saint Eloi hospital, Montpellier, France; 8Sorbonne Université, INSERM UMRS 1135, Department of Internal Medicine 2, Centre National de Référence Maladies Auto-Immunes et Systémiques Rares Lupus et Syndrome des Anticorps Antiphospholipides Centre de Référence des Histiocytoses, Institut E3M, AP-HP, Hôpital Pitié-Salpêtrière, Paris, France; Department of Pulmonology , Louis Pradel hospital, Lyon, France; Departments of Internal Medicine, University Hospital, Caen, France; Department of Internal Medicine, François Mitterand hospital, Pau, France; Department of Pulmonology department, Charles Nicolle University hospital, Rouen, France; Department of Internal Medicine, University Hospital, Amiens, France; Department of Internal Medicine, Saint Antoine hospital, Paris, France; Department of Internal Medicine, Centre de Reference des Maladies Auto-immunes Systémique Rares du Nord et du Nord-Ouest de France (CeRAINO), CHRU de Lille, Université de Lille, Lille, France; Department of internal medicine, CHU, Nantes, France; Department of Nephrology, Necker Enfants Malades hospital, Paris, France, Department of Internal Medicine, Delafontaine hospital, Saint Denis, France; Department of Internal medicine, Dax hospital, Dax, France; Department of Pulmonology , Avicenne hospital, Bobigny, France; Department of Nephrology-Dialysis-Transplantation, Angers University Hospital, Angers, France; Department of Radiology, Avicenne hospital, Bobigny, France. Corresponding author: Dr Colas Tcherakian, Service de Pneumologie, hôpital Foch, 40 rue Worth, 92150, Suresnes, France; E-mail: c.tcherakian@hopital-foch.org.
Abstract
OBJECTIVE: To report on a large series of patients with antineutrophil cytoplasmic antibodies (ANCA)-associated vasculitis (AAV) and bronchiectasis, with a specific focus on the timeline of occurrence of both features. METHODS: Retrospective nationwide multicentric study of patients diagnosed with both AAV and bronchiectasis. RESULTS: Sixty-one patients were included among which 27 (44.25 %) had microscopic polyangiitis (MPA), 27 (44.25%) had granulomatosis with polyangiitis and 7 (11.5%) had eosinophilic granulomatosis with polyangiitis. Thirty-nine (64%) had myeloperoxidase (MPO)-ANCA and 13 (21%) had proteinase 3-ANCA. The diagnosis of bronchiectasis either preceded (n=25; median time between both diagnoses: 16 IQR [4-54] years), was concomitant to (n=13) or followed (n=36; median time between both diagnoses: 1 IQR [0-6] year) that of AAV. Patients in whom bronchiectasis precedes the onset of AAV (B-AAV group) have more frequent mononeuritis multiplex, MPA, MPO-ANCA and a five-fold increase of death. The occurrence of an AAV relapse tended to be protective against bronchiectasis worsening (HR = 0.6; 95% CI [0.4-0.99], p=0.049), while a diagnosis of bronchiectasis before AAV (HR = 5.8; 95% CI [1.2-28.7]; p=0.03) or MPA (HR = 18.1; 95% CI [2.2-146.3]; p=0.01) were associated with shorter survival during AAV follow-up. CONCLUSION: The association of bronchiectasis with AAV is likely not fortuitous and is mostly associated with MPO-ANCA. Patients in whom bronchiectasis precedes the onset of AAV tend to have distinct clinical and biological features and could carry a worse prognosis.
OBJECTIVE: To report on a large series of patients with antineutrophil cytoplasmic antibodies (ANCA)-associated vasculitis (AAV) and bronchiectasis, with a specific focus on the timeline of occurrence of both features. METHODS: Retrospective nationwide multicentric study of patients diagnosed with both AAV and bronchiectasis. RESULTS: Sixty-one patients were included among which 27 (44.25 %) had microscopic polyangiitis (MPA), 27 (44.25%) had granulomatosis with polyangiitis and 7 (11.5%) had eosinophilic granulomatosis with polyangiitis. Thirty-nine (64%) had myeloperoxidase (MPO)-ANCA and 13 (21%) had proteinase 3-ANCA. The diagnosis of bronchiectasis either preceded (n=25; median time between both diagnoses: 16 IQR [4-54] years), was concomitant to (n=13) or followed (n=36; median time between both diagnoses: 1 IQR [0-6] year) that of AAV. Patients in whom bronchiectasis precedes the onset of AAV (B-AAV group) have more frequent mononeuritis multiplex, MPA, MPO-ANCA and a five-fold increase of death. The occurrence of an AAV relapse tended to be protective against bronchiectasis worsening (HR = 0.6; 95% CI [0.4-0.99], p=0.049), while a diagnosis of bronchiectasis before AAV (HR = 5.8; 95% CI [1.2-28.7]; p=0.03) or MPA (HR = 18.1; 95% CI [2.2-146.3]; p=0.01) were associated with shorter survival during AAV follow-up. CONCLUSION: The association of bronchiectasis with AAV is likely not fortuitous and is mostly associated with MPO-ANCA. Patients in whom bronchiectasis precedes the onset of AAV tend to have distinct clinical and biological features and could carry a worse prognosis.