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

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)

doi:10.3899/jrheum.190313

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

J Rheumatol. 2020 Oct 1;47(10):1522-1531. doi: 10.3899/jrheum.190313. Epub 2019 Dec 1.

Authors

Raphael Lhote¹, Marie Chilles², Matthieu Groh³, Xavier Puéchal⁴, Philippe Guilpain⁵, Félix Ackermann³, Zahir Amoura⁶, Isabella Annesi-Maesano⁷, Thomas Barba⁸, Emilie Catherinot⁹, Fleur Cohen-Aubart⁶, Pascal Cohen⁴, Vincent Cottin⁸, Louis-Jean Couderc⁹, Hubert De Boysson¹⁰, Xavier Delbrel¹¹, Stéphane Dominique¹², Pierre Duhaut¹³, Olivier Fain¹⁴, Eric Hachulla¹⁵, Mohamed Hamidou¹⁶, Jean-Emmanuel Kahn³, Christophe Legendre¹⁷, Alain Le Quellec⁵, François Lhote¹⁸, François Lifermann¹⁹, Alexis Mathian⁶, Antoine Néel¹⁶, Hilario Nunes²⁰, Jean-François Subra²¹, Benjamin Terrier⁴, Luc Mouthon⁴, Elisabeth Diot²², Loïc Guillevin⁴, Pierre-Yves Brillet²³, Colas Tcherakian; French Vasculitis Study Group (FVSG) and the Groupe d’Etudes et de Recherche sur les Maladies Orphelines Pulmonaires (GERM“O”P)

Affiliations

¹ R. Lhote, MD, Department of Pulmonology, Hôpital Foch, Suresnes, Faculté des Sciences de la Vie, Simone Veil, Université de Versailles, UPRES EA 220, and Sorbonne Université, University of Pierre and Marie Curie (UPMC), Pierre Louis Institute of Epidemiology and Public Health (IPLESP UMRS 1136), Epidemiology of Allergic and Respiratory Diseases Department (EPAR), Saint-Antoine Medical School, Paris.
² M. Chilles, MD, Department of Internal Medicine, Orléans, and Department of Internal Medicine, CHU, Tours.
³ M. Groh, MD, F. Ackermann, MD, J.E. Kahn, MD, PhD, Department of Internal Medicine, CEREO (National Referral Center for Hypereosinophilic Syndromes), Hôpital Foch, Suresnes.
⁴ X. Puéchal, MD, PhD, P. Cohen, MD, B. Terrier, MD, PhD, L. Mouthon, MD, PhD, L. Guillevin, MD, Department of Internal Medicine, National Referral Center for Rare Systemic Autoimmune Diseases, Hôpital Cochin, AP-HP, Université Paris Descartes, Paris.
⁵ Philippe Guilpain, MD, PhD, A. Le Quellec, MD, PhD, Department of Internal Medicine, Saint Eloi Hospital, Montpellier.
⁶ Z. Amoura, MD, MSc, F. Cohen-Aubart, MD, PhD, A. Mathian, MD, PhD, Sorbonne Université, INSERM UMRS 1135, Department of Internal Medicine 2, Centre National de Référence Maladies Auto-Immunes et Systémiques Rares Lupus et Syndrome des Anticorps Antiphospholipides Centre de Référence des Histiocytoses, Institut E3M, AP-HP, Hôpital Pitié-Salpêtrière, Paris.
⁷ I. Annesi-Maesano, MD, PhD, Sorbonne Université, UPMC, Pierre Louis Institute of Epidemiology and Public Health (IPLESP UMRS 1136), Epidemiology of Allergic and Respiratory Diseases Department (EPAR), Saint-Antoine Medical School, Paris.
⁸ T. Barba, MD, V. Cottin, MD, PhD, Department of Pulmonology, Louis Pradel Hospital, Lyon.
⁹ E. Catherinot, MD, PhD, L.J. Couderc, MD, C. Tcherakian, MD, PhD, 1Department of Pulmonology, Hôpital Foch, Suresnes, Faculté des Sciences de la Vie, Simone Veil, Université de Versailles, UPRES EA 220.
¹⁰ H. De Boysson, MD, Departments of Internal Medicine, University Hospital, Caen.
¹¹ X. Delbrel, MD, Department of Internal Medicine, François Mitterand Hospital, Pau.
¹² S. Dominique, MD, Department of Pulmonology Department, Charles Nicolle University Hospital, Rouen.
¹³ P. Duhaut, MD, PhD, Department of Internal Medicine, University Hospital, Amiens.
¹⁴ Olivier Fain, MD, PhD, Department of Internal Medicine, Saint Antoine Hospital, Paris.
¹⁵ E. Hachulla, MD, PhD, Department of Internal Medicine, Centre de Reference des Maladies Auto-immunes Systémique Rares du Nord et du Nord-Ouest de France (CeRAINO), CHRU de Lille, Université de Lille, Lille.
¹⁶ M. Hamidou, MD, PhD, A. Néel, MD, PhD, Department of Internal Medicine, CHU, Nantes.
¹⁷ Christophe Legendre, MD, PhD, Department of Nephrology, Necker Enfants Malades Hospital, Paris.
¹⁸ F. Lhote, MD, Department of Internal Medicine, Delafontaine Hospital, Saint Denis.
¹⁹ F. Lifermann, MD, Department of Internal Medicine, Dax Hospital, Dax.
²⁰ H. Nunes, MD, PhD, Department of Pulmonology, Avicenne Hospital, Bobigny.
²¹ J.F. Subra, MD, PhD, Department of Nephrology-Dialysis-Transplantation, Angers University Hospital, Angers.
²² E. Diot, MD, PhD, Department of Internal Medicine, CHU, Tours.
²³ P.Y. Brillet, MD, PhD, Department of Radiology, Avicenne Hospital, Bobigny, France.

PMID: 31787599
DOI: 10.3899/jrheum.190313

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.

Keywords: age; antineutrophil cytoplasmic antibody-associated vasculitis; bronchiectasis; immunosuppressive agents.

Publication types

Multicenter Study

MeSH terms

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

Substances

Antibodies, Antineutrophil Cytoplasmic
Peroxidase