Characterisation of airway disease associated with Sjögren disease
Eric Hachulla,
Xavier Mariette,
Jacques-Eric Gottenberg,
Marie-Pierre Debray,
Philippe Dieude,
Alain Saraux,
Olivier Vittecoq,
Emanuelle Dernis,
Raphaele Seror,
Gaetane Nocturne,
Cindy Marques,
Veronique Le Guern,
Claire Larroche,
Robin Dhote,
Pierre-Antoine Juge,
Anne Laure Fauchais,
Antoine Beurnier,
Loïc Meudec
Affiliations
Eric Hachulla
CHU Lille, Département de Médecine Interne et Immunologie Clinique, Centre de Référence des Maladies Auto-Immunes et Auto-Inflammatoires Rares du Nord, Nord-Ouest, Méditerranée et Guadeloupe (CeRAINOM), Lille, France
Xavier Mariette
Department of Rheumatology, Université Paris-Saclay, AP-HP-Hôpital Bicêtre, Paris, France
Jacques-Eric Gottenberg
Immunologie, Immunopathologie et Chimie Thérapeutique, CNRS UPR 3572, IBMC, Strasbourg, France
Marie-Pierre Debray
Department of Radiology, Hôpital Bichat Claude-Bernard, Assistance Publique-Hôpitaux de Paris, Paris, France
Philippe Dieude
Department of Rheumatology, Hôpital Bichat Claude-Bernard, Assistance Publique-Hôpitaux de Paris, Paris, France
Alain Saraux
Department of Rheumatology, Hopital Cavale Blanche, Brest, France
Olivier Vittecoq
Department of Rheumatology, CHU Rouen Normandie, Rouen, France
Emanuelle Dernis
Rheumatology, Le Mans Hospital, Le Mans, France
Raphaele Seror
22 Center for Immunology of Viral Infections and Autoimmune Diseases, INSERM UMR 1184, Université Paris Sud, Université Paris-Saclay, Paris, France
Gaetane Nocturne
Department of Rheumatology, Hôpital Bicêtre, Le Kremlin-Bicetre, France
Cindy Marques
Department of Rheumatology, Université Paris-Saclay, Hôpital Bicêtre, Assistance Publique-Hôpitaux de Paris, Le Kremlin Bicêtre, France
Veronique Le Guern
Department of Internal Medicine, APHP, Paris, France
Claire Larroche
Department of Internal Medicine, Hôpital Avicenne, Assistance Publique-Hôpitaux de Paris, Bobigny, France
Robin Dhote
Department of Internal Medicine, Hopital Avicenne, Bobigny, France
Pierre-Antoine Juge
Service de Rhumatologie, Hôpital Bichat-Claude Bernard, APHP, Université Paris Cité, Paris, France
Anne Laure Fauchais
Department of Rheumatology, Centre Hospitalier Universitaire de Limoges, Limoges, France
Antoine Beurnier
Department of Functional Explorations, Hôpital Bicetre, Assistance Publique-Hôpitaux de Paris, Le Kremlin-Bicetre, France
Loïc Meudec
Department of Rheumatology, Université Paris-Saclay, Hôpital Bicêtre, Assistance Publique-Hôpitaux de Paris, Le Kremlin Bicêtre, France
Objective Although airway disease associated with Sjögren’s disease (Sjo-AD) is common, it is poorly studied compared with interstitial lung disease (ILD). In this study, we aimed to assess factors associated with Sjo-AD, the characteristics and prognosis of this manifestation.Methods We performed a retrospective multicentric study involving nine centres. We included Sjo-AD patients confirmed by at least one clinician and one CT scan report. Clinical and biological data, pulmonary function test (PFT), and CT scans were collected. A single radiologist specialist in thoracic diseases reviewed CT scans. Sjo-AD patients were compared with Sjo controls without pulmonary involvement, randomly selected after matching for age and disease duration.Results We included 31 Sjo-AD and 62 Sjo controls without pulmonary history. Sjo-AD had a higher disease activity (ESSDAI) compared with controls, even when excluding the pulmonary domain of the score (7 vs 3.8, p<0.05), mainly due to the biological activity. Sjo-AD was multilobar (72%) and associated with signs of both bronchiectasis and bronchiolitis (60%). Obstructive lung disease occurred in 32% at the time of Sjo-AD diagnosis. Overall, PFT was stable after 8.7±7 years follow-up but repeated CT scans showed extended lesions in 41% of cases within 6±3.2 years. No patient developed Sjo-ILD. Sjo-AD progression was independent of the global disease activity.Conclusions Sjo-AD preferentially affects Sjo patients with higher biological activity. It is often characterised as a diffuse disease, affecting both proximal and distal airways, with a slow evolution over time and no progression to Sjo-ILD.
