Cutaneous Manifestations, Clinical Characteristics, and Prognosis of Patients With Systemic Sclerosis Sine Scleroderma
-
Published:2023-08-01
Issue:8
Volume:159
Page:837
-
ISSN:2168-6068
-
Container-title:JAMA Dermatology
-
language:en
-
Short-container-title:JAMA Dermatol
Author:
Lescoat Alain1234, Huang Suiyuan34, Carreira Patricia E.5, Siegert Elise6, de Vries-Bouwstra Jeska7, Distler Jörg H. W.8, Smith Vanessa9, Del Galdo Francesco10, Anic Branimir11, Damjanov Nemanja12, Rednic Simona13, Ribi Camillo14, Bancel Dominique Farge151617, Hoffmann-Vold Anna-Maria18, Gabrielli Armando19, Distler Oliver20, Khanna Dinesh34, Allanore Yannick2122, Matucci Cerinic Marco23, Walker Ulrich23, Iannone Florenzo23, Becvar Radim23, Kowal Bielecka Otylia23, Pizzorni Carmen23, Ciccia Francesco23, Siegert Elise23, Rednic Simona23, Vlachoyiannopoulos Panayiotis G.23, Stork Jiri23, Inanc Murat23, Carreira Patricia E.23, Novak Srdan23, Czirják László23, Iudici Michele23, Kucharz Eugene J.23, Perdan-Pirkmajer Katja23, Coleiro Bernard23, Moroncini Gianluca23, Farge Bancel Dominique23, Hesselstrand Roger23, Radic Mislav23, Balbir-Gurman Alexandra23, Lo Monaco Andrea23, Pellerito Raffaele23, Giollo Alessandro23, Morovic-Vergles Jadranka23, Denton Christopher23, Vonk Madelon23, Damjanov Nemanja23, Henes Jörg23, Ortiz Santamaria Vera23, Heitmann Stefan23, Krasowska Dorota23, Hasler Paul23, Kohm Michaela23, Foeldvari Ivan23, Bajocchi Gianluigi23, João Salvador Maria23, Stamenkovic Bojana23, Selmi Carlo F.23, Tikly Mohammed23, Ananieva Lidia P.23, Herrick Ariane23, Müller-Ladner Ulf23, Søndergaard Klaus23, Puppo Francesco23, Engelhart Merete23, Szücs Gabriela23, de la Puente Carlos23, Riccieri Valeria23, Ionescu Ruxandra Maria23, Sha Ami23, Gheorghiu Ana Maria23, Sunderkötter Cord23, Distler Jörg23, Ingegnoli Francesca23, Mouthon Luc23, Smith Vanessa23, Paolo Cantatore Francesco23, Ullman Susanne23, von Mühlen Carlos Alberto23, Pozzi Maria Rosa23, Eyerich Kilian23, Wiland Piotr23, Vanthuyne Marie23, Alegre-Sancho Juan Jose23, Herrmann Kristine23, De Langhe Ellen23, Anic Branimir23, Baresic Marko23, Mayer Miroslav23, Üprus Maria23, Otsa Kati23, Yavuz Sule23, Granel Brigitte23, de Souza Müller Carolina23, Agachi Svetlana23, Stebbings Simon23, Mathieu Alessandro23, Vacca Alessandra23, Sampaio-Barros Percival D.23, Stamp Lisa23, Solanki Kamal23, Veale Douglas23, Loyo Esthela23, Tineo Carmen23, Toloza Sergio23, Li Mengtao23, Mohamed Walid Ahmed Abdel Atty23, Olas Jacek23, Oksel Fahrettin23, Yargucu Figen23, Tanaseanu Cristina-Mihaela23, Foti Rosario23, Ancuta Codrina23, Furst Daniel E.23, Maurer Britta23, van Laar Jacob23, Olesinska Marzena23, Kayser Cristiane23, Fathi Nihal23, de la Peña Lefebvre Paloma García23, Martin Jorge Juan Gonzalez23, Carpentier Patrick23, Imbert Bernard23, Francès Camille23, Senet Patricia23, Sibilia Jean23, Litinsky Ira23, Senécal Jean Luc23, Koenig Martial23, Joval France23, Tamara Grodzicky23, Del Galdo Francesco23, Seskute Goda23, Saketkoo Lesley Ann23, Kerzberg Eduardo23, Bianchi Washington23, Valdetaro Bianchi Breno23, Castellví Ivan23, Milas-Ahic Jasminka23, Visevic Roberta23, Limonta Massimiliano23, Rimar Doron23, Couto Maura23, Spertini François23, Marcoccia Antonella23, Kahl Sarah23, Hsu Vivien M.23, Martin Thierry23, Moiseev Sergey23, Novikov Pavel23, Chung Lorinda S.23, Schmeiser Tim23, Majewski Dominik23, Zdrojewski Zbigniew23, Martínez-Barrio Julia23, Khanna Dinesh23, Bernardino Vera23, Santo Lelita23, Levy Yair23, Rezus Elena23, Nuri Pamuk Omer23, Brito de Araujo Daniel23, Sarzi Puttini Piercarlo23, Brzosko Marek23, Poormoghim Hadi23, Maman Marta23, Kötter Ina23, Cuomo Giovanna23, Gaches Francis23, Belloli Laura23, Sfikakis Petros23, Markus Juliana23, Furst Daniel23, Ramazan Ana-Maria23, Truchetet Marie-Elise23, Jego Patrick23, Dagna Lorenzo23, van Laar Jacob M.23, Voigt Lidia23, Oliveira Susana23, Atzeni Fabiola23, Kuwana Masataka23, Mekinian Arsene23, Martin Mickaël23, Tanaka Yoshiya23, Yasuoka Hidekata23, Simeón-Aznar Carmen-Pilar23, Atsumi Tatsuya23, Parvu Magda23, Cordeiro Ines23, Del Papa Nicoletta23, Karonitsch Thomas23, Bazela-Ostromecka Anna23, Selvi Enrico23, Kawaguchi Yasushi23, Soukup Tomas23, Rodriguez-Pinto Ignasi23, Geroldinger-Simic Marija23, Espinosa Gerard23, Voigt Karen23, Kubacki Torsten23, Garmish Olena23, Mosca Marta23, Gerth Ulrich23, Antonenko Ludmila23, Mendoza Fabian A.23, Airò Paolo23, de Vries-Bouwstra Jeska23, Riemekasten Gabriela23, Hachulla Eric23, Doria Andrea23, Rosato Edoardo23, Hunzelmann Nicolas23, Montecucco Carlomaurizio23, Gabrielli Armando23, Hoffmann-Vold Anna-Maria23, Distler Oliver23, Ben Shimol Jennifer23, Cutolo Maurizio23, Allanore Yannick23,
Affiliation:
1. University of Rennes, CHU Rennes, Inserm, EHESP, Irset (Institut de Recherche en Santé, Environnement et Travail) - UMR_S 1085, Rennes, France 2. Department of Internal Medicine and Clinical Immunology, Rennes University Hospital, Rennes, France 3. Division of Rheumatology, Department of Internal Medicine, University of Michigan, Ann Arbor 4. Scleroderma Program, University of Michigan, Ann Arbor 5. Rheumatology Department, University Hospital 12th of October, Madrid, Spain 6. Charité University Hospital, Berlin, Germany 7. Department of Rheumatology, Leiden University Medical Center, Leiden, the Netherlands 8. University of Erlangen-Nuremberg, Erlangen, Germany 9. Department of Rheumatology, Ghent University Hospital, Ghent, Belgium 10. Institute of Rheumatic and Musculoskeletal Medicine and NIHR Biomedical Research Centre, University of Leeds, Leeds, United Kingdom 11. Division of Clinical Immunology and Rheumatology, University of Zagreb School of Medicine and University Hospital Centre, Zagreb, Croatia 12. Institute of Rheumatology, Faculty of Medicine University of Belgrade, Belgrade, Serbia 13. Department of Rheumatology, Emergency County Teaching Hospital, Cluj-Napoca, Romania 14. Department of Clinical Immunology and Allergy, Lausanne, Switzerland 15. Department of Internal Medicine: CRMR MATHEC, Maladies Auto-Immunes et Thérapie Cellulaire, Centre de Référence des Maladies Auto-Immunes Systémiques Rares d’Ile-de-France, FAI2R, Hôpital Saint-Louis, Assistance Publique des Hôpitaux de Paris, Paris, France 16. Department of Medicine, McGill University, Montreal, Quebec, Canada 17. Université de Paris-Cité, Paris, France 18. Department of Rheumatology, Rikshospitalet University Hospital, Oslo, Norway 19. Arche Polytechnic University, Institute of Clinical Medicine, University of Ancona, Ancona, Italy 20. Department of Rheumatology, University Hospital Zurich, University of Zurich, Zurich, Switzerland 21. Service de Rhumatologie, Hôpital Cochin, AP-HP.CUP, Paris, France 22. INSERM U1016 and CNRS UMR8104, Institut Cochin, Paris, France 23. for the EUSTAR collaborators
Abstract
ImportanceSystemic sclerosis (SSc) sine scleroderma (ssSSc) is a subset of SSc defined by the absence of skin fibrosis. Little is known about the natural history and skin manifestations among patients with ssSSc.ObjectiveTo characterize the clinical phenotype of patients with ssSSc compared with patients with limited cutaneous SSc (lcSSc) and diffuse cutaneous SSc (dcSSc) within the EUSTAR database.Design, Setting, and ParticipantsThis longitudinal observational cohort study based on the international EUSTAR database included all patients fulfilling the classification criteria for SSc assessed by the modified Rodnan Skin score (mRSS) at inclusion and with at least 1 follow-up visit; ssSSc was defined by the absence of skin fibrosis (mRSS = 0 and no sclerodactyly) at all available visits. Data extraction was performed in November 2020, and data analysis was performed from April 2021 to April 2023.Main Outcomes and MeasuresMain outcomes were survival and skin manifestations (onset of skin fibrosis, digital ulcers, telangiectasias, puffy fingers).ResultsAmong the 4263 patients fulfilling the inclusion criteria, 376 (8.8%) were classified as having ssSSc (mean [SD] age, 55.3 [13.9] years; 345 [91.8%] were female). At last available visit, in comparison with 708 patients with lcSSc and 708 patients with dcSSc with the same disease duration, patients with ssSSc had a lower prevalence of previous or current digital ulcers (28.2% vs 53.1% in lcSSc; P < .001; and 68.3% in dcSSc; P < .001) and puffy fingers (63.8% vs 82.4% in lcSSc; P < .001; and 87.6% in dcSSc; P < .001). By contrast, the prevalence of interstitial lung disease was similar in ssSSc and lcSSc (49.8% and 57.1%; P = .03) but significantly higher in dcSSc (75.0%; P < .001). Skin telangiectasias were associated with diastolic dysfunction in patients with ssSSc (odds ratio, 4.778; 95% CI, 2.060-11.081; P < .001). The only independent factor for the onset of skin fibrosis in ssSSc was the positivity for anti–Scl-70 antibodies (odds ratio, 3.078; 95% CI, 1.227-7.725; P = .02). Survival rate was higher in patients with ssSSc (92.4%) compared with lcSSc (69.4%; P = .06) and dcSSc (55.5%; P < .001) after up to 15 years of follow-up.Conclusions and RelevanceSystemic sclerosis sine scleroderma should not be neglected considering the high prevalence of interstitial lung disease (>40%) and SSc renal crisis (almost 3%). Patients with ssSSc had a higher survival than other subsets. Dermatologists should be aware that cutaneous findings in this subgroup may be associated with internal organ dysfunction. In particular, skin telangiectasias in ssSSc were associated with diastolic heart dysfunction.
Publisher
American Medical Association (AMA)
Cited by
3 articles.
订阅此论文施引文献
订阅此论文施引文献，注册后可以免费订阅5篇论文的施引文献，订阅后可以查看论文全部施引文献
|
|