Bronchiectasis preceding ANCA-related vasculitis onset tied to shorter survival

The association between bronchiectasis and antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) does not appear to be incidental and is mostly correlated with myeloperoxidase (MPO)-ANCA, suggests a recent study.

In addition, patients in whom bronchiectasis precedes the onset of AAV “tend to have distinct clinical and biological features and could carry a worse prognosis,” the authors said.

This retrospective nationwide multicentre study reported on a large series of patients diagnosed with AAV and bronchiectasis, with a specific focus on the timeline of occurrence of both features.

Sixty-one patients participated in this study, of whom 27 (44.25 percent) had microscopic polyangiitis (MPA), 27 (44.25 percent) had granulomatosis with polyangiitis (GPA), and seven (11.5 percent) had eosinophilic GPA. In addition, 39 patients (64 percent) had MPO-ANCA and 13 (21 percent) had proteinase 3–ANCA.

The diagnosis of bronchiectasis in these patients either preceded (n=25; median time between both diagnoses, 16 years; interquartile range [IQR], 4–54 years), was concomitant to (n=12), or followed that of AAV (n=24; median time between both diagnoses, 1 years; IQR, 0–6 years). Patients with a bronchiectasis diagnosis that preceded AAV onset had more frequent mononeuritis multiplex, MPA, MPO-ANCA, and a fivefold increase of death.

Of note, AAV relapse appeared to have a protective effect against bronchiectasis worsening (hazard ratio [HR], 0.6, 95 percent confidence interval [CI], 0.4–0.99; p=0.049) in this population. On the other hand, a bronchiectasis diagnosis prior to AAV (HR, 5.8, 95 percent CI, 1.2–28.7; p=0.03) or MPA (HR, 18.1, 95 percent CI, 2.2–146.3; p=0.01) correlated with shorter survival during AAV follow-up.