Benjamin Chaigne1, Alexandre Bense2, Christian AGARD3, Yannick ALLANORE4, Gregory Pugnet5, Eric Hachulla6, Jérôme Avouac7, Boris Bienvenu8, Sylvain Palat9, Claire Grange10, Sabine Berthier11, Emmanuel Chatelus12, sebastien riviere11, Marie-Elise Truchetet13, Jean-Emmanuel Kahn14, Francois Maurier15, elisabeth diot16, Alice Berezne11 and Luc Mouthon17, 1Paris Cité University, Paris, France, 2APHP, Paris, France, 3Internal medicine, Nantes University Hospital, Nantes, France, 4Université Paris Cité, Paris, France, 5CHU Toulouse Rangueil Service de Medecine Interne et Immunologie Clinique, Toulouse, France, 6University of Lille, Lille, France, 7Service de Rhumatologie, Hôpital Cochin, AP-HP.Centre – Université Paris Cité, Paris, France, 8Saint Joseph Hospital, Marseille, France, 9...., Paris, France, 10..., Paris, France, 11Internal Medicine - Cochin University Hospital, Paris, France, 12Internal Medicine - Cochin University Hospital, Strasbourg, France, 13Bordeaux University Hospital, Bordeaux, France, 14AP-HP, Suresnes Cedex, France, 15Hôpitaux privés de Metz, Vaux / Frankreich, France, 16Internal Medicine - Cochin University Hospital, Tours, France, 17Hopital Cochin - Paris University, Paris, France
Background/Purpose: Few studies have evaluated mouth opening (MO) in systemic sclerosis (SSc). None have studied MO trajectories.
Methods: We performed a multicentre study patients enrolled in the French national SSc cohort with at least one MO assessment, described patients based on MO baseline measure, modelized MO trajectories, and associated MO measures with SSc prognosis.
Results: 1101 patients were included in the study. Baseline MO was associated with disease severity. Kaplan Meier analysis showed that MO < 30mm was associated with a worse 30-year-survival (p< 0.01) and higher risk of pulmonary arterial hypertension occurrence (p< 0.05). Individual MO trajectories were heterogenous between patients. The best model of MO trajectories using latent process mixed models showed that 88.8% patients had a stable MO trajectory, and clustered patients into 3 groups which were predictive of SSc survival (p< 0.05). and interstitial lung disease (ILD) (p< 0.05). It highlighted a cluster of 9.5% patients with diffuse SSc (dcSSc) (p< 0.05) and a high but decreasing MO over one year (p< 0.0001) at higher risk of a poor survival and ILD occurrence.
Conclusion: MO, which is a simple and reliable measure, is predictive of disease severity and survival in SSc. Although it remains stable in most SSc patients, dcSSc patients with high but decreasing MO are at risk of poor survival and ILD occurrence.
B. Chaigne: None; A. Bense: None; C. AGARD: None; Y. ALLANORE: AbbVie/Abbott, 2, Alpine Immunoscience, 5, AstraZeneca, 2, Bayer, 2, Boehringer-Ingelheim, 2, Janssen, 2, Medsenic, 2, 5, Mylan, 2, OSE Immunotherapeutics, 5, Prometeus, 2, Roche, 2, Sanofi, 2; G. Pugnet: None; E. Hachulla: Bayer, 2, CSL Behring, 5, GlaxoSmithKlein(GSK), 2, 5, 6, johnson&Johnson, 2, 5, 6, Novartis, 2, 5, Otsuka, 6, Roche-Chugai, 2, 5, 6, sanofi-genzyme, 2, 5, Sobi, 5; J. Avouac: AbbVie, 6, AstraZeneca, 6, Biogen, 6, BMS, 5, 6, Fresenius Kabi, 5, 6, Galapagos, 5, 6, Janssen, 6, Lilly, 6, Medac, 6, MSD, 6, Nordic Pharma, 6, Novartis, 6, Novartis (Dreamer), 5, Pfizer, 6, Pfizer (Passerelle), 5, Roche-Chugai, 6, Sandoz, 6, Sanofi, 6; B. Bienvenu: None; S. Palat: None; C. Grange: None; S. Berthier: None; E. Chatelus: None; s. riviere: None; M. Truchetet: AbbVie/Abbott, 2, 6, Boehringer-Ingelheim, 2, 6, Gilead, 5, 6, Merck/MSD, 6, UCB, 6, 12, support for conferences; J. Kahn: None; F. Maurier: None; e. diot: None; A. Berezne: None; L. Mouthon: None.