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Abstract Number: 1751

Interstitial Lung Disease during ANCA-Associated Vasculitis: A Poor-Prognosis Factor

Thibault Maillet1, Tiphaine Goletto2, Guillaume Beltramo3, Henry Dupuy4, Stéphane Jouneau5, Raphaël 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 Guillevin15, Benjamin Terrier14 and Maxime Samson1, 1Service de Médecine Interne et Immunologie Clinique, CHU de Dijon, Dijon, France, 2Service de Pneumologie, Hôpital Saint-Louis, APHP, Paris, France, 3Service de Pneumologie, CHU de Dijon, Dijon, France, 4Service de Médecine Interne et Maladies Infectieuses, Hôpital Haut-Lévêque, Bordeaux, France, 5Service de Pneumologie, CHU de Rennes; IRSET UMR 1085, Université de Rennes 1, Rennes, France, 6Service de Pneumologie, Hôpital Bichat, APHP, Paris, France, 7Service de Pneumologie, Hôpital Louis-Pradel, Bron, France, 8Rheumatology, UZ Leuven, Leuven, Belgium, 9Service de Pneumologie, Hôpital Tenon, APHP, Paris, France, 10Service de Médecine Interne, CHU de Toulouse, Toulouse, France, 11Pulmonary diseases department, Avicenne Hospital (AP-HP), Bobigny, France, 12Service de Médecine Interne, Hôpital Bicêtre, APHP, Le Kremlin-Bicêtre, France, 13Service de Médecine Interne et Maladies Systémiques, CHU de Dijon, Dijon, France, 14Centre de Référence des Maladies Auto-immunes et Systémiques Rares, Vascularites Nécrosantes et Sclérodermie Systémique, Service de Médecine Interne, Hôpital Cochin, APHP, INSERM U1016, Université Paris Descartes, Paris, France, 15Department of Internal Medicine, INSERM Unité 1016, Centre de Référence pour les Maladies Auto-immunes Rares, National Referral Center for Rare Systemic Autoimmune Diseases, Paris Cochin, France, Paris, France

Meeting: 2018 ACR/ARHP Annual Meeting

Keywords: ANCA, pulmonary fibrosis and vasculitis

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Session Information

Date: Monday, October 22, 2018

Title: Vasculitis – ANCA-Associated Poster I

Session Type: ACR Poster Session B

Session Time: 9:00AM-11:00AM

Background/Purpose: Interstitial lung disease (ILD), rarely described in ANCA-associated vasculitis (AAV) patients, was mainly associated with anti-MPO ANCA. ILD’s prognostic value remains unclear. This study focused on the outcomes of patients with ILD associated with AAV (ILD–AAV).

Methods: This case–control study compared ILD–AAV cases and to AAV patients without ILD (controls). Case AAVs were microscopic polyangiitis (MPA) or granulomatosis with polyangiitis (GPA) (meeting Chapel Hill definitions) and ILD was either usual interstitial pneumonia (UIP) or nonspecific interstitial pneumonia (NSIP) (satisfying the 2001 American Thoracic Society/European Respiratory Society criteria). Eosinophilic granulomatosis with polyangiitis, non-fibrosing pulmonary involvement, ILD without AAV criteria and late ILD (>2 yr after AAV diagnosis) were excluded. Two controls with MPA or GPA without ILD were matched to each case for age (> or ≤65 years), ANCA status (PR3- or MPO-positive, or negative) and creatininemia (≥ or <150 µmol/L).

Results: Sixty-five cases were included: 88% MPO-ANCA–positive and 83% with MPA. Median (IQR) age at AAV diagnosis was 65.7 (56.6–74.0) yr. ILD was mainly diagnosed before (53%) or simultaneously (39%) to AAV. CT-scan pattern was mostly UIP (65%). Median case follow-up was 40 (21–62) months vs. 60 (27–128) months for controls (P=0.001). Cases, compared to 130 controls, had less frequent fever (P=0.002), peripheral neuropathy (P=0.03), or ear nose & throat (P=0.03) or gastrointestinal involvement (P=0.002). Five Factor Scores (1996 version) at AAV diagnosis were similar for the 2 groups (P=0.08). Cases more frequently received immunosuppressants for induction (91% vs. 76%; P=0.01) and maintenance (84% vs. 58%; P<0.001). Notably, 17/65 (26%) cases suffered relapses vs. 56/130 (43.1%) controls (P=0.03). Relapses were mainly minor for cases (12/17; 70.6%) vs. controls (19/56; 33.9%) (P=0.01). Only 2 cases experienced ILD exacerbation at relapse. Major-relapse–free survival was comparable for cases and controls (P=0.90). During follow-up, 19 cases died: 6 of acute respiratory failure related to pulmonary fibrosis exacerbation, 2 end-stage respiratory failure, 2 infections (1 pneumonia, 1 endocarditis), 1 lung cancer, 1 digestive hemorrhage and 7 unknown causes. For cases and controls, respective 1-, 3- and 5-year overall survival rates were: 97%, 83% and 77% vs. 93%, 91% and 87% (P=0.008). Compared to controls, case survival was shorter for those with UIP (P<0.001) and unchanged for NSIP (P=0.50). Multivariate analyses retained age >65 yr (hazard ratio (HR) 4.366; P<0.001), alveolar hemorrhage (HR 2.38; P=0.01) and UIP (HR 2.63; P=0.003) as independently associated with shorter survival.

Conclusion: For ILD–AAV patients, UIP, but not NSIP, was associated with poorer prognosis. Although the greater majority of ILD–AAV patients included in this study received immunosuppressants for induction and maintenance, their survival was shorter than that of controls, mainly due to pulmonary fibrosis exacerbation, suggesting that anti-fibrosing agents should be evaluated in these patients.


Disclosure: T. Maillet, None; T. Goletto, None; G. Beltramo, None; H. Dupuy, None; S. Jouneau, None; R. Borie, None; B. Crestani, None; V. Cottin, None; D. Blockmans, None; E. Lazaro, None; J. M. Naccache, None; G. Pugnet, None; H. Nunes, None; M. De Menthon, None; H. Devilliers, None; P. Bonniaud, None; X. Puéchal, None; L. Mouthon, None; B. Bonnotte, None; L. Guillevin, None; B. Terrier, None; M. Samson, None.

To cite this abstract in AMA style:

Maillet T, Goletto T, Beltramo G, Dupuy H, Jouneau S, Borie R, Crestani B, Cottin V, Blockmans D, Lazaro E, Naccache JM, Pugnet G, Nunes H, De Menthon M, Devilliers H, Bonniaud P, Puéchal X, Mouthon L, Bonnotte B, Guillevin L, Terrier B, Samson M. Interstitial Lung Disease during ANCA-Associated Vasculitis: A Poor-Prognosis Factor [abstract]. Arthritis Rheumatol. 2018; 70 (suppl 9). https://acrabstracts.org/abstract/interstitial-lung-disease-during-anca-associated-vasculitis-a-poor-prognosis-factor/. Accessed .
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