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Medicine (Baltimore). 2014 Nov;93(24):340-9. doi: 10.1097/MD.0000000000000217.

Pulmonary fibrosis in antineutrophil cytoplasmic antibodies (ANCA)-associated vasculitis: a series of 49 patients and review of the literature.

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From the Departement Hospitalo-Universitaire I2B (CC, B. Hervier, PC, DS), UPMC Univ Paris 06, UMR 7211, F-75005, Paris; INSERM, UMR_S 959 (CC, PC, DS), F-75013, Paris; CNRS, UMR 7211 (CC, PC, DS), F-75005, Paris; AP-HP, Groupe Hospitalier Pitié-Salpêtrière, Department of Internal Medicine and Clinical Immunology (CC, B. Hervier, FCA, PC, DS), F-75013, Paris; AP-HP, Hôpital Bichat, Service de Pneumologie A (BC), Université Paris Diderot-Paris VII, Paris; AP-HP, Hôpital Saint-Louis, Service de Pneumologie (AT), Université Paris Diderot-Paris VII, Sorbonne Paris Cité, INSERM UMR 717, Paris; Centre Hospitalier Régional et Universitaire de Tours, Service de Pneumologie (SAM), Tours; AP-HP, Centre Hospitalier et Universitaire d'Avicenne, Service de Pneumologie (HN), Bobigny; AP-HP, Hôpital Tenon, Service de Pneumologie (MW, J. Cadranel), UPMC-Paris VI, Paris; Centre Hospitalier Intercommunal de Créteil, Service de Pneumologie (B. Housset), Créteil; AP-HP, Hôpital Lariboisière, Service de Médecine Interne A (CLL), Paris; Hospices Civils de Lyon, Hôpital de la Croix-Rousse, Service de Médecine Interne (PS), Lyon; AP-HP, Centre hospitalier et universitaire d'Avicenne, Service de Médecine Interne (SA), Bobigny; Centre Hospitalier Victor Dupouy, Service de Pneumologie (J. Camuset), Argenteuil; Centre Hospitalier et Universitaire de Caen, Service de Médecine Interne (BB), Caen; Centre Hospitalier Universitaire de Grenoble, Service de Pneumologie, Pôle Thorax et Vaisseaux (MD), Grenoble; Hôpital Claude Huriez, Service de Médecine Interne (EH), Université Lille Nord-de-France, Lille; AP-HP, Hôpital Européen Georges Pompidou, Service de Médecine Interne (JBA), Paris; Centre Hospitalier Universitaire de Nantes, Hôtel-Dieu, Service de Médecine Interne (MH), Nantes; AP-HP, Hôpital Saint-Louis, Service de Médecine Interne (AM), Université Paris Diderot-Paris VII, Paris; AP-HP, Hôpital Saint-Louis, Service de Biostatistiques (MRR), Université Paris Did


Pulmonary fibrosis (PF) is an uncommon manifestation observed in patients with antineutrophil cytoplasmic antibodies (ANCA)-associated vasculitis (AAV), particularly microscopic polyangiitis (MPA). While patients with PF associated with AAV seem to have a worse prognosis, these patients have been described only in case reports or small retrospective case series. In this retrospective multicenter study, we report the main features and long-term outcomes of patients with PF associated with AAV, fulfilling the American College of Rheumatology criteria and/or Chapel Hill definitions. Forty-nine patients (30 men [61%]; median age at diagnosis of AAV, 68 [interquartile range, 58-73] years) with PF associated with AAV were identified. Forty (81.6%) patients had MPA and 9 (18.4%) had granulomatosis with polyangiitis. The diagnosis of PF preceded the onset of vasculitis in 22 (45%) patients. Usual interstitial pneumonia was the main radiologic pattern (n = 18, 43%). ANCA were mostly of antimyeloperoxidase specificity (88%). All patients were treated with glucocorticoids as induction therapy, combined with cyclophosphamide (CYC) (n = 36, 73.5%) or rituximab (RTX) (n = 1, 2%). Factors associated with mortality included occurrence of chronic respiratory insufficiency (hazard ratio [HR], 7.44; 95% confidence interval [CI], 1.6-34.5; p = 0.003), induction therapy with glucocorticoids alone (HR, 2.94; CI, 1.05-8.33; p = 0.04), and initial weigh loss (HR, 2.83; CI, 1.05-7.65; p = 0.041). The 3-year survival rate in patients treated with glucocorticoids alone or combined with an immunosuppressant (CYC or RTX) as induction therapy was 64% (95% CI, 41-99) and 94% (95% CI, 86-100), respectively (p = 0.03). After a median follow-up of 48 months [interquartile range, 14-88 mo], 18 (37%) patients died, including 11 related to respiratory insufficiency. PF is a rare manifestation of AAV with a very poor prognosis. Induction therapy with CYC might improve the outcome.

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