Alain Lescoat1, Suiyuan Huang2, PATRICIA E CARREIRA3, Elise Siegert4, Jeska de Vries-Bouwstra5, Jörg Distler6, Vanessa Smith7, Branimir Anic8, Francesco Del Galdo9, Nemanja Damjanov10, Simona Rednic11, camillo Ribi12, Dominique Farge Bancel13, Anna-Maria Hoffmann-vold14, Armando Gabrielli15, Oliver Distler16, Dinesh Khanna17 and Yannick Allanore18, 1CHU Rennes - University Rennes 1, Rennes, France, 2University of Michigan, Ann Arbor, MI, 3HOSPITAL 12 DE OCTUBRE, Madrid, Spain, 4Charité Hospital, Berlin, Germany, 5Leiden University Medical Center, Leiden, Netherlands, 6Friedrich-Alexander-University Erlangen-Nürnberg (FAU) and University Hospital Erlangen, Erlangen, Germany, 7Department of Rheumatology, Ghent University Hospital – Department of Internal Medicine, Ghent University, Belgium – Unit for Molecular Immunology and Inflammation, VIB Inflammation Research Center (IRC), Gent, Belgium, 8Clinical Hospital Centre Zagreb, School of Medicine, University of Zagreb, Division of Clinical Immunology and Rheumatology, Department of Internal Medicine, Zagreb, Croatia, 9Leeds Institute of Rheumatic and Musculoskeletal Medicine, Faculty of Medicine and Health and NIHR Biomedical Research Centre, University of Leeds, Leeds, United Kingdom, 10Institute of Rheumatology, Belgrade, Serbia, 11Iuliu Hatieganu University of Medicine and Pharmacy, Cluj-Napoca, Romania, 12Service of Immunology and Allergy / CHUV, Lausanne, Switzerland, 13Saint Louis Hospital, Paris, France, 14Oslo University Hospital, Oslo, Norway, 15Università Politecnica delle Marche, Ancona, Italy, 16Department of Rheumatology, University Hospital Zurich, University of Zurich, Zürich, Switzerland, 17Division of Rheumatology, Department of Internal Medicine, Scleroderma Program, University of Michigan, Ann Arbor, MI, 18Department of Rheumatology A, Descartes University, APHP, Cochin Hospital, Paris, France, Paris, France
Background/Purpose: This study aimed to characterise the main clinical features of patients with systemic sclerosis (SSc) sine scleroderma (ssSSc) in comparison with the limited cutaneous (lcSSc) and diffuse cutaneous (dcSSc) subsets within the international EUSTAR database.
Methods: All patients from the EUSTAR database fulfilling the ACR2013 or 1980 classification criteria for SSc assessed by the modified Rodnan Skin score (mRSS) at inclusion and with at least one follow-up visit were eligible. Sine scleroderma (ssSSc) was defined by the absence of skin thickening (mRSS=0 and no sclerodactyly) at all available visits. The clinical characteristics of these ssSSc patients were compared to those of patients with lcSSc and dcSSc with similar disease duration at last follow-up visit. Descriptive statistics were applied.
Results: Among the 4263 patients fulfilling the inclusion criteria, 376 (8.8%) were classified as ssSSc. Among them, 40.3% had puffy fingers, 39.4% had interstitial lung disease (ILD), 1.6% had a history of scleroderma renal crisis at inclusion visit. At last available visit, in comparison with 708 lcSSc and 708 dcSSc with the same disease duration, ssSSc patients had a lower prevalence of previous or current digital ulcers (28.2% versus 53.1% in lcSSc (P< 0.001) and 68.3% in dcSSc (P< 0.001)), of joint synovitis (16.9% versus 24.3% in lcSSc (P< 0.01) and 30.8% in dcSSc (P< 0.0001)), and of elevated sPAP on echocardiogram (15.2% versus 23.9% in lcSSc (P< 0.01) and 28.7% in dcSSc (P< 0.0001)). Despite similar disease duration, disease activity at follow up visit (assessed by the EScSG disease activity index 2001 and 2016) was lower in ssSSc in comparison with lcSSc and dcSSc. By contrast, the prevalence of ILD was almost similar in ssSSc and lcSSc (49.8% and 57.1% (P=0.03)) but significantly higher in dcSSc (75.0%, P< 0.0001). Based on forced vital capacity, ILD was less severe in ssSSc in comparison with the other subsets (mean FVC 100%( pred) versus 93% in lcSSc and 82% in dcSSc (P< 0.0001 for both)). Anti-centromere antibodies were most represented in ssSSc (61.7% versus 41.9% in lcSSc (P< 0.0001) and 16.3% in dcSSc (P< 0.0001), whereas the opposite distribution was observed for anti-Scl70 antibodies. Survival was higher in ssSSc patients compared to lcSSc (P= 0.05) and dcSSc (P< 0.0001).
Conclusion: This study highlights that ssSSc patients account for almost 10% of SSc patients with milder disease severity compared to both lcSSc and dcSSc.
Disclosures: A. Lescoat, None; S. Huang, None; P. CARREIRA, None; E. Siegert, None; J. de Vries-Bouwstra, None; J. Distler, Fibrocure, 4D Science; V. Smith, Boehringer-Ingelheim, Janssen; B. Anic, None; F. Del Galdo, AbbVie/Abbott, AstraZeneca, Boehringer-Ingelheim, Mitsubishi-Tanabe, Capella biosciences, Chemomab LTD, Kymab; N. Damjanov, None; S. Rednic, None; c. Ribi, None; D. Farge Bancel, None; A. Hoffmann-vold, Boehringer-Ingelheim, Actelion, Janssen, Roche, Merck/MSD, ARXX Therapeutics, Eli Lilly, Medscape, EUSTAR, EULAR, ACR, ERS; A. Gabrielli, None; O. Distler, AbbVie/Abbott, Amgen, GlaxoSmithKlein(GSK), Novartis, Roche, UCB, Kymera, Mitsubishi Tanabe, Boehringer Ingelheim, 4P-Pharma, Acceleron, Alcimed, Altavant Sciences, AnaMar, Arxx, AstraZeneca, Blade Therapeutics, Bayer, Corbus Pharmaceuticals, CSL Behring, Galapagos, Glenmark, Horizon, Inventiva, Lupin, Miltenyi Biotec, Merck/MSD, Prometheus Biosciences, Redx Pharma, Roivant, Sanofi, Topadur, Pfizer, Janssen, Medscape, Patent issued “mir-29 for the treatment of systemic sclerosis” (US8247389, EP2331143), FOREUM Foundation, ERS/EULAR Guidelines, EUSTAR, SCQM (Swiss Clinical Quality Management in Rheumatic Diseases), Swiss Academy of Medical Sciences (SAMW), Hartmann Müller Foundation; D. Khanna, Boehringer Ingelheim, Genentech, Prometheus, Horizon, Chemomab, Talaris, Gesynta, Amgen, Acceleron, Actelion, Bayer, CSL Behring, Paracrine Cell Therapy, Mitsubishi Tanabe, Theraly, Eicos Sciences; Y. Allanore, Boehringer Ingelheim, Sanofi, Janssen, AbbVie, Menarini, Curzion, Medsenic, Prometheus, AstraZeneca.
