• Benjamin Chaigne, Alexandre Bense, Christian Agard, Yannick Allanore, Grégory Pugnet, Eric Hachulla, Jérôme Avouac, Boris Bienvenu, Sylvain Palat, Claire Grange, Sabine Berthier, Emmanuel Chatelus, Sébastien Rivière, Marie-Elise Truchetet, Jean-Emmanuel Kahn, Francois Maurier, Elisabeth Diot, Alice Berezne, Luc Mouthon, and French Systemic Sclerosis Network Collaborators.
• Service de Médecine Interne, Centre de Référence Maladies Autoimmunes Systémiques Rares d'Ile de France, Hôpital Cochin, Assistance Publique-Hôpitaux de Paris (AP-HP), Ile de France, France.
• J. Intern. Med. 2023 Jun 27.

BackgroundFew studies have evaluated mouth opening (MO) in systemic sclerosis (SSc). None have studied MO trajectories.ObjectiveTo study MO trajectories in SSc.MethodsThis multicentre study included patients enrolled in the French national SSc cohort with at least one MO assessment, described patients based on MO baseline measure, modeled MO trajectories, and associated MO measures with SSc prognosis.ResultsWe included 1101 patients. Baseline MO was associated with disease severity. On Kaplan-Meier analysis, MO < 30 mm was associated with worse 30-year-survival (p<0.01) and risk of pulmonary arterial hypertension (p<0.05). Individual MO trajectories were heterogenous among patients. The best model of MO trajectories according to latent-process mixed modeling showed that 88.8% patients had a stable MO trajectory and clustered patients into 3 groups that predicted SSc survival (p<0.05) and interstitial lung disease (ILD) occurrence (p<0.05). The model highlighted a cluster of 9.5% patients with diffuse cutaneous SSc (dcSSc) (p<0.05) and high but decreasing MO over 1 year (p<0.0001) who were at increased risk of poor survival and ILD.ConclusionMO, which is a simple and reliable measure, could be used to predict disease severity and survival in SSc. Although MO remained stable in most SSc patients, dcSSc patients with high but decreasing MO were at risk of poor survival and ILD. This article is protected by copyright. All rights reserved.This article is protected by copyright. All rights reserved.

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