Spectrum and Prognosis of Antineutrophil Cytoplasmic Antibody-associated Vasculitis-related Bronchiectasis: Data from 61 Patients.
J Rheumatol
; 47(10): 1522-1531, 2020 10 01.
Article
in En
| MEDLINE
| ID: mdl-31787599
ABSTRACT
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.Key words
Full text:
1
Collection:
01-internacional
Database:
MEDLINE
Main subject:
Bronchiectasis
/
Granulomatosis with Polyangiitis
/
Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis
Type of study:
Diagnostic_studies
/
Etiology_studies
/
Observational_studies
/
Prognostic_studies
/
Risk_factors_studies
Limits:
Humans
Language:
En
Journal:
J Rheumatol
Year:
2020
Document type:
Article