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

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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