Spectrum and Prognosis of Antineutrophil Cytoplasmic Antibody-associated Vasculitis-related Bronchiectasis: Data from 61 Patients.

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.

• Authors: Raphael Lhote, Marie Chilles, Matthieu Groh, Xavier Puéchal, Philippe Guilpain, Félix Ackermann, Zahir Amoura, Isabella Annesi-Maesano, Thomas Barba, Emilie Catherinot, Fleur Cohen-Aubart, Pascal Cohen, Vincent Cottin, Louis-Jean Couderc, Hubert De Boysson, Xavier Delbrel, Stéphane Dominique, Pierre Duhaut, Olivier Fain, Eric Hachulla, Mohamed Hamidou, Jean-Emmanuel Kahn, Christophe Legendre, Alain Le Quellec, François Lhote, François Lifermann, Alexis Mathian, Antoine Néel, Hilario Nunes, Jean-François Subra, Benjamin Terrier, Luc Mouthon, Elisabeth Diot, Loïc Guillevin, Pierre-Yves Brillet, Colas Tcherakian, French Vasculitis Study Group (FVSG) and the Groupe d’Etudes et de Recherche sur les Maladies Orphelines Pulmonaires (GERM“O”P)
• Journal: The Journal of rheumatology (J Rheumatol) Vol. 47 Issue 10 Pg. 1522-1531 (10 01 2020) ISSN: 0315-162X [Print] Canada
• PMID: 31787599 (Publication Type: Journal Article, Multicenter Study)

Chemical References

• Antibodies, Antineutrophil Cytoplasmic
• Peroxidase

Topics

• Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis (complications, diagnosis)
• Antibodies, Antineutrophil Cytoplasmic
• Bronchiectasis (etiology)
• Granulomatosis with Polyangiitis
• Humans
• Peroxidase
• Prognosis
• Retrospective Studies