Thibault Maillet1, Tiphaine Goletto2, Guillaume Beltramo3, Henry Dupuy4, Stéphane Jouneau5, Raphael Borie6, Bruno Crestani6, Vincent Cottin7, Daniel Blockmans8, Estibaliz Lazaro4, Jean-Marc Naccache9, Grégory Pugnet10, Hilario Nunes11, Mathilde de Menthon12, Hervé Devilliers13, Philippe Bonniaud3, Xavier Puéchal14, Luc Mouthon14, Bernard Bonnotte1, Loïc Guillevin14, Benjamin Terrier14, Maxime Samson1. 1. Department of Internal Medicine and Clinical Immunology, CHU Dijon Bourgogne, Dijon, France. 2. Department of Pulmonology, Hôpital Saint-Louis, APHP, Paris, France. 3. Respiratory and ICU Department, Referral Center for Adults Rare Pulmonary Diseases, Inserm 1231, CHU Dijon-Bourgogne, Dijon, France. 4. Department of Internal Medicine and Infectious Diseases, Hôpital Haut-Lévêque, Bordeaux, France. 5. Department of Pulmonology, Univ Rennes, CHU Rennes, Inserm, EHESP, Irset (Institut de Recherche en Santé, Environnement et Travail), UMR S, 1085, Rennes, France. 6. Department of Pulmonology, Hôpital Bichat, APHP, Paris, France. 7. Department of Pulmonology, Hôpital Louis-Pradel, Bron, France. 8. Department of Internal Medicine, UZ Leuven Hospital, Leuven, Belgium. 9. Assistance Publique-Hôpitaux de Paris (APHP), Hôpital Tenon, Service de Pneumologie, Site Constitutif Du Centre de Référence des Maladies Pulmonaires Rares OrphaLung, Paris, France. 10. Department of Internal Medicine, CHU de Toulouse, Toulouse, France. 11. Department of Pulmonology, Hôpital Avicenne, APHP, Bobigny, France. 12. Department of Internal Medicine, Hôpital Bicêtre, APHP, Le Kremlin-Bicêtre, France. 13. Department of Internal Medicine and Systemic Diseases, CHU Dijon-Bourgogne, Dijon, France. 14. Department of Internal Medicine, Referral Center for Rare Autoimmune and Systemic Diseases, Hôpital Cochin, APHP, Inserm U1016, Université Paris Descartes, Paris, France.
Abstract
BACKGROUND: Progressive fibrosing interstitial lung disease (ILD) is rarely associated with antineutrophil cytoplasm antibody (ANCA)-associated vasculitis (AAV). This study focused on the outcomes of ILD patients with associated AAV (AAV-ILD). METHODS: AAV-ILD (cases: microscopic polyangiitis (MPA) or granulomatosis with polyangiitis (GPA) with ILD) were compared to AAV patients without ILD (controls). ILD was defined as a usual interstitial pneumonia (UIP) or non-specific interstitial pneumonia (NSIP) pattern. Two controls were matched to each case for age (>or ≤65 years), ANCA status (PR3-or MPO-positive) and creatininemia (≥or <150 μmol/L). RESULTS: Sixty-two cases (89% MPO-ANCA+) were included. Median age at AAV diagnosis was 66 years. ILD (63% UIP), was diagnosed before (52%) or simultaneously (39%) with AAV. Cases versus 124 controls less frequently had systemic vasculitis symptoms. One-, 3- and 5-year overall survival rates, respectively, were: 96.7%, 80% and 66% for cases versus 93.5%, 89.6% and 83.8% for controls (p = 0.008). Multivariate analyses retained age >65 years (hazard ratio (HR) 4.54; p < 0.001), alveolar haemorrhage (HR 2.25; p = 0.019) and UIP (HR 2.73; p = 0.002), but not immunosuppressant use, as factors independently associated with shorter survival. CONCLUSION: For AAV-ILD patients, only UIP was associated with poorer prognosis. Immunosuppressants did not improve the AAV-ILD prognosis. But in analogy to idiopathic pulmonary fibrosis, anti-fibrosing agents might be useful and should be assessed in AAV-ILD patients with a UIP pattern.
BACKGROUND: Progressive fibrosing interstitial lung disease (ILD) is rarely associated with antineutrophil cytoplasm antibody (ANCA)-associated vasculitis (AAV). This study focused on the outcomes of ILDpatients with associated AAV (AAV-ILD). METHODS: AAV-ILD (cases: microscopic polyangiitis (MPA) or granulomatosis with polyangiitis (GPA) with ILD) were compared to AAV patients without ILD (controls). ILD was defined as a usual interstitial pneumonia (UIP) or non-specific interstitial pneumonia (NSIP) pattern. Two controls were matched to each case for age (>or ≤65 years), ANCA status (PR3-or MPO-positive) and creatininemia (≥or <150 μmol/L). RESULTS: Sixty-two cases (89% MPO-ANCA+) were included. Median age at AAV diagnosis was 66 years. ILD (63% UIP), was diagnosed before (52%) or simultaneously (39%) with AAV. Cases versus 124 controls less frequently had systemic vasculitis symptoms. One-, 3- and 5-year overall survival rates, respectively, were: 96.7%, 80% and 66% for cases versus 93.5%, 89.6% and 83.8% for controls (p = 0.008). Multivariate analyses retained age >65 years (hazard ratio (HR) 4.54; p < 0.001), alveolar haemorrhage (HR 2.25; p = 0.019) and UIP (HR 2.73; p = 0.002), but not immunosuppressant use, as factors independently associated with shorter survival. CONCLUSION: For AAV-ILDpatients, only UIP was associated with poorer prognosis. Immunosuppressants did not improve the AAV-ILD prognosis. But in analogy to idiopathic pulmonary fibrosis, anti-fibrosing agents might be useful and should be assessed in AAV-ILDpatients with a UIP pattern.
Authors: Domenico Sambataro; Gianluca Sambataro; Francesca Pignataro; Giovanni Zanframundo; Veronica Codullo; Evelina Fagone; Emanuele Martorana; Francesco Ferro; Martina Orlandi; Nicoletta Del Papa; Lorenzo Cavagna; Lorenzo Malatino; Michele Colaci; Carlo Vancheri Journal: Diagnostics (Basel) Date: 2020-04-09