Title :

Systemic sclerosis associated interstitial lung disease: a survey of current practices in France.

Author(s) :

Nicolas, Amelie [Auteur]
Institute for Translational Research in Inflammation - U 1286 [INFINITE]
Leroy, Sylvie [Auteur]
Centre Hospitalier Universitaire de Nice [CHU Nice]
Mouthon, Luc [Auteur]
Hôpital Cochin [AP-HP]
Uzunhan, Yurdagul [Auteur]
Hôpital Avicenne [AP-HP]
Cottin, Vincent [Auteur]
Hôpital Louis Pradel [CHU - HCL]
Mekinian, Arsene [Auteur]
CHU Saint-Antoine [AP-HP]
Queyrel, Viviane [Auteur]
Centre Hospitalier Universitaire de Nice [CHU Nice]
Hachulla, Eric [Auteur]
Institut de Recherche Translationnelle sur l'Inflammation (INFINITE) - U1286
Gachet, Benoit [Auteur]
Centre Hospitalier Gustave Dron [Tourcoing]
Launay, David [Auteur]
Institut de Recherche Translationnelle sur l'Inflammation (INFINITE) - U1286
Martis, Nihal [Auteur]
Centre Hospitalier Universitaire de Nice [CHU Nice]

Journal title :

Therapeutic advances in musculoskeletal disease

Abbreviated title :

Ther Adv Musculoskelet Dis

Volume number :

15

Pages :

1759720X231159712

Publication date :

2023-05-17

ISSN :

1759-720X

English keyword(s) :

diagnostic management
interstitial lung disease
systemic sclerosis
therapeutic management

HAL domain(s) :

Sciences du Vivant [q-bio]

English abstract : [en]

Background: Interstitial lung disease (ILD) is the leading cause of mortality in systemic sclerosis (SSc). Objective: We performed an overview of the diagnostic approaches, follow-up and treatment strategies used in ...
Show more >Background: Interstitial lung disease (ILD) is the leading cause of mortality in systemic sclerosis (SSc). Objective: We performed an overview of the diagnostic approaches, follow-up and treatment strategies used in France for the management of SSc-associated ILD (SSc-ILD). Design: Structured nationwide online survey.Methods: A structured nationwide online survey was submitted to participants via the French Medical Societies for Internal Medicine and Pneumology, and research groups on SSc-ILD from May 2018 to June 2020. The 79 multiple-choice and 9 open-ended questions covered the screening of ILD at baseline, monitoring of patients with established SSc-ILD and its management. Fourteen optional vignettes exploring different clinical phenotypes of SSc-ILD were submitted to evaluate therapeutic decisions. Results: All of the 93 participants screened SSc patients for ILD at baseline with 83 (89%) participants relying on a systematic chest computed tomography (CT) scan. Pulmonary function tests (PFT) were prescribed by 87 (94%) participants at baseline and during follow-up. Treatment was started based on abnormal PFT (95%), chest CT scan characteristics (89%), worsening dyspnoea (72%) and drop in SpO2 during 6-min walk tests (66%). First-line therapy was cyclophosphamide (CYC) (89%), mycophenolate mofetil (MMF) (83%) and prednisone (73%). Rituximab as second-line immunosuppressive therapy (41%) was preferred to antifibrotic agents (18%), and a median daily prednisone dose of 10 mg (interquartile range, 10–15) was prescribed by 73% participants. Extensive SSc-ILD with worsening PFT (95%), regardless of diffusing capacity for carbon monoxide values and skin extension, were more likely to be treated, and CYC was favoured over MMF (p < 0.01). Extensive SSc-ILD with disease duration of less than 5 years was also a criterium for treatment initiation. Conclusion: This overview of practices in diagnosis, follow-up and treatment of SSc-ILD in France describes real-life management of patients. It highlights heterogeneity in this management and gaps in current strategies that should be addressed to improve and harmonize clinical practices in SSc-ILD.Show less >

Language :

Anglais

Audience :

Internationale

Popular science :

Non

Administrative institution(s) :

Université de Lille
Inserm
CHU Lille

Collections :

• Institut de Recherche Translationnelle sur l'Inflammation (INFINITE) - U1286

Submission date :

2024-01-11T22:42:48Z
2024-03-04T11:31:05Z