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Kennzeichnend ist eine Kombination aus Vaskulopathie und entz\u00fcndlicher Fibrose von Haut und Organen. Die Erkrankung kann sich sehr heterogen pr\u00e4sentieren, was die Diagnosestellung erschwert und in der \u00dcberwachung und Therapie ber\u00fccksichtigt werden muss. Dank Forschungsresultaten der letzten Jahrzehnte stehen mittlerweile deutlich mehr Medikamente f\u00fcr die Behandlung der SSc zur Verf\u00fcgung. Die Wahl der Therapie ist bei jedem Erkrankungsfall an die individuelle Auspr\u00e4gung anzupassen. Der vorliegende Beitrag bietet einen \u00dcberblick \u00fcber die verschiedenen Krankheitsmanifestationen und Behandlungsm\u00f6glichkeiten bei SSc. F\u00fcr eine bestm\u00f6gliche Betreuung betroffener Patienten ist eine gute haus\u00e4rztliche und rheumatologische Zusammenarbeit essenziell. Weitere internistische Fach\u00e4rzte (Pneumologen, Gastroenterologen, Kardiologen, Nephrologen) m\u00fcssen je nach Organbefall hinzugezogen werden.<\/jats:p>","DOI":"10.1007\/s00108-025-02024-x","type":"journal-article","created":{"date-parts":[[2025,12,1]],"date-time":"2025-12-01T11:05:51Z","timestamp":1764587151000},"page":"63-71","update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":0,"title":["Systemische Sklerose","Systemic sclerosis"],"prefix":"10.1007","volume":"67","author":[{"given":"Caroline","family":"Evers","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"J\u00f6rg","family":"Distler","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Oliver","family":"Distler","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"297","published-online":{"date-parts":[[2025,12,1]]},"reference":[{"issue":"7","key":"2024_CR1","doi-asserted-by":"publisher","first-page":"3121","DOI":"10.1093\/rheumatology\/keab190","volume":"60","author":"M Bairkdar","year":"2021","unstructured":"Bairkdar\u00a0M et\u00a0al (2021) Incidence and prevalence of systemic sclerosis globally: a\u00a0comprehensive systematic review and meta-analysis. 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Finanzielle Interessen: Diverse nicht kommerzielle Stiftungen: im Stiftungsrat oder chair executive committee; nationale und internationale Wissenschaftsorganisationen: diverse Funktionen, z.B. grant reviewer, abstract reviewer, Projektmitarbeiter, Mitglied wissenschaftliches Komitee; Boehringer Ingelheim, Kymera, Mitsubishi: Unterst\u00fctzung f\u00fcr wissenschaftliche Projekte.\u00a0\u2013 Referentenhonorar: Boehringer, Medscape, Novartis, Sanofi, Bayer, Janssen.\u00a0\u2013 Im Bereich der Sklerodermie und assoziierter Erkrankungen: 4P-Pharma, AbbVie, Acepodia, Aera, AnaMar, Anaveon, Argenx, AstraZeneca, Boehringer Ingelheim, BMS, Calluna, Cantargia, CSL Behring, EMD Serono, Galderma, Galapagos, Gossamer, Hemetron, Innovaderm, Janssen, Kali, Lilly, MSD Merck, Nkarta, Novartis, Oorja Bio, Orion, Pliant, Prometheus, Quell, Redx Pharma, Scleroderma Research Foundation, Sumitomo, Tandem, Topadur, UCB und Umlaut.bio.\u00a0\u2013 Patent issued \u201emir-29 for the treatment of systemic sclerosis\u201c (US8247389, EP2331143).\u00a0\u2013 B.\u00a0Nichtfinanzielle Interessen: Professor, Klinikdirektor, Klinik f\u00fcr Rheumatologie, Universit\u00e4tsspital Z\u00fcrich; Direktor Forschung, Zentrum f\u00fcr Experimentelle Rheumatologie, Schlieren; Fakult\u00e4tsmitglied, Medizinische Fakult\u00e4t, Universit\u00e4t Z\u00fcrich | Mitgliedschaften: Schweizerische Gesellschaft f\u00fcr Rheumatologie, Chairman European Scleroderma Trials and Research Group (EUSTAR), Walter-Siegenthaler-Gesellschaft, Chef\u00e4rztegesellschaft der Universit\u00e4tskliniken des Kantons Z\u00fcrich (CUK), Stiftungsrat Swiss Clinical Quality Management in Rheumatic Diseases, Z\u00fcrich, Stiftungsrat Hartmann M\u00fcller-Stiftung, Schweizerische Akademie der Medizinischen Wissenschaften (SAMW), Vorsitzender beim Ausschuss der FOREUM Foundation.\n                      C.\u00a0Evers\n                      : A.\u00a0Finanzielle Interessen: C.\u00a0Evers gibt an, dass kein finanzieller Interessenkonflikt besteht.\u00a0\u2013 B.\u00a0Nichtfinanzielle Interessen: Angestellte Ober\u00e4rztin, Klinik f\u00fcr Rheumatologie, Universit\u00e4tsspital Z\u00fcrich, Z\u00fcrich, Schweiz | Mitgliedschaften: Foederatio Medicorum Helveticorum (FMH), Verband Schweizerischer Assistenz- und Ober\u00e4rztinnen und -\u00e4rzte (VSAO).\n                      J.\u00a0Distler\n                      : A.\u00a0Finanzielle Interessen: Forschungsf\u00f6rderung von Anamar, AstraZeneca, ARXX, BMS, Boehringer Ingelheim, Cantargia, Celgene, CSL Behring, Exo Therapeutics, Galapagos, GSK, Incyte, Inventiva, Kiniksa, Kyverna, Lassen Therapeutics, Mestag, Sanofi-Aventis, Spica Therapeutics, RedX, UCB und Zenas BioPharma.\u00a0\u2013 Unterst\u00fctzung bei wissenschaftlichen Publikationen von Boehringer Ingelheim und Endeavor BioMedicines.\u00a0\u2013 Beratungst\u00e4tigkeit f\u00fcr Active Biotech, Anamar, ARXX, AstraZeneca, Bayer Pharma, Boehringer Ingelheim, Calliditas Therapeutics, Calluna, Galapagos, GSK, Janssen, Kyverna, Ono Pharmaceutical, Merck, Novartis, Quell Therapeutics, Tyra und UCB.\u00a0\u2013 J.\u00a0Distler ist CEO von 4D Science und wissenschaftlicher Leiter bei FibroCure.\u00a0\u2013 B.\u00a0Nichtfinanzielle Interessen: Klinikdirektor Universit\u00e4tsklinikum.","order":3,"name":"Ethics","group":{"name":"EthicsHeading","label":"Autoren"}},{"value":"Die vollst\u00e4ndige Erkl\u00e4rung zum Interessenkonflikt der Wissenschaftlichen Leitung finden Sie am Kurs der zertifizierten Fortbildung auf\n                      \n                      .","order":4,"name":"Ethics","group":{"name":"EthicsHeading","label":"Wissenschaftliche Leitung"}},{"value":"erkl\u00e4rt, dass f\u00fcr die Publikation dieser CME-Fortbildung keine Sponsorengelder an den Verlag flie\u00dfen.","order":5,"name":"Ethics","group":{"name":"EthicsHeading","label":"Der Verlag"}},{"value":"F\u00fcr diesen Beitrag wurden von den Autor\/-innen keine Studien an Menschen oder Tieren durchgef\u00fchrt. 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