Camelia Frantz1, Dorte Huscher2, Jérôme Avouac1, Eric Hachulla3, Alexandra Balbir-Gurman4, Gabriela Riemekasten5, Elise Siegert6, Maria-Grazia Lazzaroni7, Patricia E Carreira8, Serena Vettori9, Elisabetta Zanatta10, Susanne Ullman11, Laszlo Czirjàk12, Otylia Kowal-Bielecka13, Oliver Distler14, Marco Matucci-Cerinic15, Yannick Allanore16. 1. Rheumatology A Department, Cochin Hospital, Paris Descartes University, Paris, France. 2. Institute for Biometry and Clinical Epidemiology, Berlin Institute of Health, Charité-Universitaetsmedizin Berlin, Berlin, Germany. 3. Department of Internal Medicine and Clinical Immunology, Hôpital Claude Huriez, Lille University, Lille, France. 4. B Shine Rheumatology Unit, Rambam Health Care Campus, Haifa, Israel. 5. Department of Rheumatology, University of Lübeck, Lübeck, Germany. 6. Department of Rheumatology and Clinical Immunology, Charité University Hospital, Berlin, Germany. 7. Rheumatology and Clinical Immunology, Department of Molecular and Tanslational Medicine, Spedali Civili and University of Brescia, Brescia, Italy. 8. Servicio de Reumatologia, Hospital Universitario 12 de Octubre, Madrid, Spain. 9. Department of Precision Medicine, University of Campania "Luigi Vanvitelli", Napoli, Italy. 10. Rheumatology Unit, Department of Medicine, University of Padova, Padova, Italy. 11. Department of Dermatology, University Hospital of Copenhagen, Hospital Bispebjerg, Copenhagen, Denmark. 12. Department of Immunology and Rheumatology, University of Pécs, Pécs, Hungary. 13. Department of Rheumatology and Internal Medicine, Medical University of Bialystok, Bialystok, Poland. 14. Department of Rheumatology, University Hospital Zurich, Zurich, Switzerland. 15. Department of Experimental and Clinical Medicine, University of Florence and Division of Rheumatology AOUC, Florence, Italy. 16. Rheumatology A Department, Cochin Hospital, Paris Descartes University, Paris, France. Electronic address: yannick.allanore@cch.aphp.fr.
Abstract
OBJECTIVES: Limited cutaneous systemic sclerosis (LcSSc) is the most common subset of SSc but it has been overlooked in the past years. At a time at which clinical trials focus on diffuse cutaneous SSc (DcSSc) we aimed at clarifying the outcomes of LcSSc and at evaluating whether potential drug positioned in DcSSc may also be used in LcSSc. METHODS: The EUSTAR database was used to investigate skin, lung and peripheral vasculopathy outcomes in LcSSc. Worsening of skin fibrosis, ILD and peripheral vasculopathy were defined by an increase in modified Rodnan skin score (mRSS) > 3.5 points, a decrease of FVC > 10% in patients with ILD at baseline, and by the development of new digital ulcers (DU) in patients without DU at baseline. RESULTS: 8013 LcSSc and 4786 DcSSc patients were included. In contrast to DcSSc, skin disease was remarkably stable in the majority of LcSSc patients with >80% having a change lower than ±4 units of mRSS at 12, 24 and 36 months follow-up. Conversely, FVC changes over time were very similar between LcSSc and DcSSc. Regarding DU, numbers of patients with new DU over time seemed to be almost similar between the two subsets. CONCLUSIONS: LcSSc patients have a low mRSS at baseline with marginal changes with time. Conversely, SSc-ILD can be as progressive as in DcSSc supporting the inclusion of LcSSc patients in SSc-ILD trials and suggesting potential benefit of any anti-ILD drugs. Similarly, although slightly less common, DU should receive the same attention in the two subsets.
OBJECTIVES: Limited cutaneous systemic sclerosis (LcSSc) is the most common subset of SSc but it has been overlooked in the past years. At a time at which clinical trials focus on diffuse cutaneous SSc (DcSSc) we aimed at clarifying the outcomes of LcSSc and at evaluating whether potential drug positioned in DcSSc may also be used in LcSSc. METHODS: The EUSTAR database was used to investigate skin, lung and peripheral vasculopathy outcomes in LcSSc. Worsening of skin fibrosis, ILD and peripheral vasculopathy were defined by an increase in modified Rodnan skin score (mRSS) > 3.5 points, a decrease of FVC > 10% in patients with ILD at baseline, and by the development of new digital ulcers (DU) in patients without DU at baseline. RESULTS: 8013 LcSSc and 4786 DcSScpatients were included. In contrast to DcSSc, skin disease was remarkably stable in the majority of LcSSc patients with >80% having a change lower than ±4 units of mRSS at 12, 24 and 36 months follow-up. Conversely, FVC changes over time were very similar between LcSSc and DcSSc. Regarding DU, numbers of patients with new DU over time seemed to be almost similar between the two subsets. CONCLUSIONS: LcSSc patients have a low mRSS at baseline with marginal changes with time. Conversely, SSc-ILD can be as progressive as in DcSSc supporting the inclusion of LcSSc patients in SSc-ILD trials and suggesting potential benefit of any anti-ILD drugs. Similarly, although slightly less common, DU should receive the same attention in the two subsets.
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