Characterisation of airway disease associated with Sjögren disease

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...

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Main Authors: 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
Format: Article
Language:English
Published: BMJ Publishing Group 2024-02-01
Series:RMD Open
Online Access:https://rmdopen.bmj.com/content/10/1/e003866.full
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author 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
author_facet 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
author_sort Eric Hachulla
collection DOAJ
description 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.
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spelling doaj-art-59e35ec4f8a84b42a7749e1492c2070e2025-08-20T03:48:31ZengBMJ Publishing GroupRMD Open2056-59332024-02-0110110.1136/rmdopen-2023-003866Characterisation of airway disease associated with Sjögren diseaseEric Hachulla0Xavier Mariette1Jacques-Eric Gottenberg2Marie-Pierre Debray3Philippe Dieude4Alain Saraux5Olivier Vittecoq6Emanuelle Dernis7Raphaele Seror8Gaetane Nocturne9Cindy Marques10Veronique Le Guern11Claire Larroche12Robin Dhote13Pierre-Antoine Juge14Anne Laure Fauchais15Antoine Beurnier16Loïc Meudec17CHU 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, FranceDepartment of Rheumatology, Université Paris-Saclay, AP-HP-Hôpital Bicêtre, Paris, FranceImmunologie, Immunopathologie et Chimie Thérapeutique, CNRS UPR 3572, IBMC, Strasbourg, FranceDepartment of Radiology, Hôpital Bichat Claude-Bernard, Assistance Publique-Hôpitaux de Paris, Paris, FranceDepartment of Rheumatology, Hôpital Bichat Claude-Bernard, Assistance Publique-Hôpitaux de Paris, Paris, FranceDepartment of Rheumatology, Hopital Cavale Blanche, Brest, FranceDepartment of Rheumatology, CHU Rouen Normandie, Rouen, FranceRheumatology, Le Mans Hospital, Le Mans, France22 Center for Immunology of Viral Infections and Autoimmune Diseases, INSERM UMR 1184, Université Paris Sud, Université Paris-Saclay, Paris, FranceDepartment of Rheumatology, Hôpital Bicêtre, Le Kremlin-Bicetre, FranceDepartment of Rheumatology, Université Paris-Saclay, Hôpital Bicêtre, Assistance Publique-Hôpitaux de Paris, Le Kremlin Bicêtre, FranceDepartment of Internal Medicine, APHP, Paris, FranceDepartment of Internal Medicine, Hôpital Avicenne, Assistance Publique-Hôpitaux de Paris, Bobigny, FranceDepartment of Internal Medicine, Hopital Avicenne, Bobigny, FranceService de Rhumatologie, Hôpital Bichat-Claude Bernard, APHP, Université Paris Cité, Paris, FranceDepartment of Rheumatology, Centre Hospitalier Universitaire de Limoges, Limoges, FranceDepartment of Functional Explorations, Hôpital Bicetre, Assistance Publique-Hôpitaux de Paris, Le Kremlin-Bicetre, FranceDepartment of Rheumatology, Université Paris-Saclay, Hôpital Bicêtre, Assistance Publique-Hôpitaux de Paris, Le Kremlin Bicêtre, FranceObjective 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.https://rmdopen.bmj.com/content/10/1/e003866.full
spellingShingle 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
Characterisation of airway disease associated with Sjögren disease
RMD Open
title Characterisation of airway disease associated with Sjögren disease
title_full Characterisation of airway disease associated with Sjögren disease
title_fullStr Characterisation of airway disease associated with Sjögren disease
title_full_unstemmed Characterisation of airway disease associated with Sjögren disease
title_short Characterisation of airway disease associated with Sjögren disease
title_sort characterisation of airway disease associated with sjogren disease
url https://rmdopen.bmj.com/content/10/1/e003866.full
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