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.

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We found that the composite score was strongly correlated to the risk score (r s = 0.88, p < 10 −5 ) and not to the frequency (r s = 0.06, p = 0.75) or uselessness score (r s = 0.17, p = 0.38). CONCLUSIONS: This Top-5 list provides an opportunity to discuss appropriate use of health care practices in internal medicine.

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