Late skin fibrosis in systemic sclerosis: a study from the EUSTAR cohort
Author:
Hughes Michael12ORCID, Huang Suiyuan34, Alegre-Sancho Juan Jose5, Carreira Patricia E6, Engelhart Merete7, Hachulla Eric8, Henes Joerg9ORCID, Kerzberg Eduardo10, Pozzi Maria Rosa11, Riemekasten Gabriela12, Smith Vanessa131415ORCID, Szücs Gabriella16, Vanthuyne Marie17, Zanatta Elisabetta18, Distler Oliver19ORCID, Gabrielli Armando G20, Hoffmann-Vold Anna-Maria21ORCID, Steen Virginia D22, Khanna Dinesh323, Airò P, Allanore A, Ananieva L P, Anic B, Balbir-Gurman A, Becvar R, Benvenuti F, Cantatore F P, Chung L S, Cuomo G, Cutolo M, Czirják L, Damjanov N, de Vries-Bouwstra J, Del Galdo F, Distler J, Eyerich K, Farge D, Foti R, Gheorghiu A M, Giollo A, Heitmann S, Herrick A, Hesselstrand R, Hsu I M, Hunzelmann N, Iannone F, Iudici M, Ionescuc M R, Ingegnoli F, Jose J, Joven B E, Kerzberg E, Kucharz E J, Kuwana M, Langhe E D, Launay D, Lefebvre P, Litinsky I, García de la Peña Lefebvre P, González-Martín J J, Li M, Loyo E, Martin T, Matucci-Cerinic M, Maurer B, Moroncini G, Mouthon L, Müller C S, Müller-Ladner U, Novak S, Pastor P, Pecher A-C, Pellerito R, Pozzi M R, Oksel F, Rednic S, Rezus E, Riccieri V, Rosato E, Saketkoo L A, Salvador M J, Schmeiser T, Selmi C F, Sibilia J, Siegert E, Solanki K, Sommerlatte S, Spertini F, Stamenkovic B, Stamp L, Tanaseanu C-M, Tikly M, Tineo C, Ullman S, Üprus M, Vanthuyne M, Veale D, Walker U, Wiland P, Yargucu F, Yavuz S,
Affiliation:
1. Tameside Hospital, Tameside and Glossop Integrated Care NHS Foundation Trust , Ashton-under-Lyne 2. Division of Musculoskeletal and Dermatological Sciences, The University of Manchester , Manchester, UK 3. Department of Internal Medicine, Division of Rheumatology, Scleroderma Program 4. Department of Biostatistics, School of Public Health, University of Michigan , Ann Arbor, MI, USA 5. Department of Rheumatology, Hospital Universitario Dr Peset , Valencia 6. Rheumatology Department, University Hospital 12 de Octubre , Madrid, Spain 7. Center for Rheumatology and Spine Diseases, Gentofte Hospital , Hellerup, Denmark 8. Department of Internal Medicine and Clinical Immunology, Referral Centre for Centre for Rare Systemic Autoimmune Diseases North and North-West of France (CeRAINO, CHU Lille, Univ. Lille, Inserm, U1286 – INFINITE—Institute for Translational Research in Inflammation) , Lille, France 9. Centre for Interdisciplinary Clinical Immunology, Rheumatology and Autoinflammatory Diseases and Department of Internal Medicine II (Hematology, Oncology, Immunology and Rheumatology), University Hospital Tuebingen , Tuebingen, Germany 10. Rheumatology Department, J. M. Ramos Mejía Hospital , Buenos Aires, Argentina 11. Rheumatology Unit, S. Gerardo Hospital , Monza, Italy 12. Department of Rheumatology and Clinical Immunology, University Clinic Schleswig-Holstein, University of Lübeck , Lübeck, Germany 13. Department of Internal Medicine, Ghent University 14. Department of Rheumatology, Ghent University Hospital 15. Unit for Molecular Immunology and Inflammation, VIB Inflammation Research Center (IRC) , Ghent, Belgium 16. Division of Rheumatology, Department of Internal Medicine, University of Debrecen , Debrecen, Hungary 17. Rheumatology Department, Cliniques Universitaires Saint-Luc , Brussels, Belgium 18. Rheumatology Unit, Department of Medicine, University of Padova , Padova, Italy 19. Department of Rheumatology, University Hospital Zurich, University of Zurich , Zurich, Switzerland 20. Dipartimento di Scienze Cliniche e Molecolari, Università Politecnica delle Marche , Ancona, Italy 21. Department of Rheumatology, Oslo University Hospital , Oslo, Norway 22. Division of Rheumatology, Department of Medicine, School of Medicine, Georgetown University , Washington, DC 23. University of Michigan Scleroderma Program , Ann Arbor, MI, USA
Abstract
Abstract
Objectives
The early trajectory of skin fibrosis provides insights into the disease course of systemic sclerosis (SSc) including mortality; however, little is known about late skin fibrosis. The aims of our study were to ascertain the prevalence and characteristics of late skin fibrosis in SSc.
Methods
We developed and tested three conceptual scenarios of late (>5 years after first non-RP feature) skin fibrosis including new worsening of skin disease, and failure to improve after worsening within 5-year window. We defined skin worsening as change in modified Rodnan skin score (mRSS) ≥5 units or ≥25%. Using strict inclusion criteria including complete mRSS, we identified 1,043 (out of 19 115) patients within the EUSTAR database for our analysis. We further restricted analysis within 887 (out of 1043) patients who had lcSSc or dcSSc at baseline.
Results
One-fifth of patients among the whole cohort (n = 208/1043, 19.9%) experienced mRSS worsening, including in patients with lcSSc or dcSSc at baseline (n = 193/887, 21.8%). This was largely due to new skin worsening after the 5-year window or failure to improve with worsening within the 5-year window. Patients with lower baseline mRSS and lcSSc were more likely to develop late skin fibrosis. Anti-Scl-70 was associated with progression from baseline lcSSc to dcSSc, and anticentromere was protective.
Conclusions
Late skin fibrosis is not uncommon in SSc. We have identified different patterns relevant to clinical practice and trial design. Late skin fibrosis is a neglected manifestation of SSc and warrants further investigation including to determine clinical outcomes and optimal therapeutic strategy.
Funder
Johnson & Johnson Boehringer Ingelheim Bayer
Publisher
Oxford University Press (OUP)
Subject
Pharmacology (medical),Rheumatology
Reference27 articles.
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