TY - JOUR T1 - Spectrum and Prognosis of Antineutrophil Cytoplasmic Antibody–associated Vasculitis-related Bronchiectasis: Data from 61 Patients JF - The Journal of Rheumatology JO - J Rheumatol SP - 1522 LP - 1531 DO - 10.3899/jrheum.190313 VL - 47 IS - 10 AU - Raphael Lhote AU - Marie Chilles AU - Matthieu Groh AU - Xavier Puéchal AU - Philippe Guilpain AU - Félix Ackermann AU - Zahir Amoura AU - Isabella Annesi-Maesano AU - Thomas Barba AU - Emilie Catherinot AU - Fleur Cohen-Aubart AU - Pascal Cohen AU - Vincent Cottin AU - Louis-Jean Couderc AU - Hubert De Boysson AU - Xavier Delbrel AU - Stéphane Dominique AU - Pierre Duhaut AU - Olivier Fain AU - Eric Hachulla AU - Mohamed Hamidou AU - Jean-Emmanuel Kahn AU - Christophe Legendre AU - Alain Le Quellec AU - François Lhote AU - François Lifermann AU - Alexis Mathian AU - Antoine Néel AU - Hilario Nunes AU - Jean-François Subra AU - Benjamin Terrier AU - Luc Mouthon AU - Elisabeth Diot AU - Loïc Guillevin AU - Pierre-Yves Brillet AU - Colas Tcherakian Y1 - 2020/10/01 UR - http://www.jrheum.org/content/47/10/1522.abstract N2 - Objective To report on a large series of patients with antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) and bronchiectasis, with a specific focus on the timeline of occurrence of both features.Methods Retrospective nationwide multicenter study of patients diagnosed with both AAV and bronchiectasis.Results Sixty-one patients were included, among whom 27 (44.25%) had microscopic polyangiitis (MPA), 27 (44.25%) had granulomatosis with polyangiitis (GPA), and 7 (11.5%) had eosinophilic GPA. 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 yrs, IQR 4–54 yrs), was concomitant to (n = 12), or followed (n = 24; median time between both diagnoses: 1, IQR 0–6 yrs) 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 5-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 accidental 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. ER -