PT  - JOURNAL ARTICLE
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
TI  - Spectrum and prognosis of ANCA-associated vasculitis-related bronchiectasis: data from 61 patients
AID  - 10.3899/jrheum.190313
DP  - 2019 Dec 01
TA  - The Journal of Rheumatology
PG  - jrheum.190313
4099  - http://www.jrheum.org/content/early/2019/11/25/jrheum.190313.short
4100  - http://www.jrheum.org/content/early/2019/11/25/jrheum.190313.full
AB  - 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.