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Der Internist

, Volume 60, Issue 12, pp 1251–1269 | Cite as

Systemische Sklerose

  • Susanne SchinkeEmail author
  • Gabriela Riemekasten
CME

Zusammenfassung

Die systemische Sklerose (SSc) ist eine seltene fibrosierende rheumatische Multisystemerkrankung, sodass viele nicht nur internistische Fachdisziplinen mit der SSc in Kontakt kommen. Das Sterblichkeitsrisiko bei der SSc wird führend durch die Lungenfibrose, pulmonal arterielle Hypertonie und kardiale Beteiligung bedingt. Bei rechtzeitiger und intensiver Behandlung kann die Erkrankung aufgehalten oder sogar verbessert werden. Dieser Übersichtsbeitrag fasst Erkenntnisse zu Pathophysiologie, Klassifikationskriterien, dem klinischen Bild und erfolgreichen Therapieoptionen zusammen. Dabei wird auch auf aktuelle Studien zu Immunsuppressiva und Biologika eingegangen.

Schlüsselwörter

Lungenfibrose Pulmonal arterielle Hypertonie CREST-Syndrom Akrale Nekrosen Digitale Ulzera 

Systemic sclerosis

Abstract

Systemic sclerosis (SSc) is a rare fibrosing rheumatic multi-systemic disease involving many medical specialties. The mortality of SSc is determined by lung fibrosis, pulmonary arterial hypertension and cardiac involvement. With early and intensive treatment, the disease can be stabilized and symptoms relieved. This review summarizes insights into pathophysiology, disease classification, clinical manifestations and successful therapies, as well as recent studies on new immunosuppressant, biological and vasoactive therapies.

Keywords

Pulmonary fibrosis Pulmonary arterial hypertension CREST syndrome Acral necrosis Digital ulcers 

Notes

Einhaltung ethischer Richtlinien

Interessenkonflikt

Gemäß den Richtlinien des Springer Medizin Verlags werden Autoren und Wissenschaftliche Leitung im Rahmen der Manuskripterstellung und Manuskriptfreigabe aufgefordert, eine vollständige Erklärung zu ihren finanziellen und nichtfinanziellen Interessen abzugeben.

Autoren

S. Schinke: Finanzielle Interessen: Jansen (Kostenerstattung European League Against Rheumatism [EULAR] 2018, 2019) | MSD (Fortbildung TNF‑α Forum 2019) | Pfizer (Referentenhonorar Lübecker Rheuma Visite 2018, 2019) | Pfizer (Fortbildung i-FemMe 2018, Reise‑/Übernachtungskosten) | Celgene (Fortbildung ASV 2017, Reisekosten) | AbbVie (Fortbildung 2017, Reise und Hotel) | UCB (Fortbildung Deutsche Gesellschaft für Rheumatologie [DGRh] 2017, Reise‑/Übernachtungskosten) | ChemoCentryx (Studie, Reise/Unterkunft 2017) (kein Bezug zu diesem Artikel). Nichtfinanzielle Interessen: Angestellte Rheumatologin, Universitätsklinikum Schleswig-Holstein (UKSH), Campus Lübeck | Mitgliedschaften: Deutsche Gesellschaft für Innere Medizin (DGIM), DGRh, British Society for Immunology (BSI). G. Riemekasten: Finanzielle Interessen: Referentenhonorar oder Kostenerstattung als passiver Teilnehmer: Roche, Amgen, Novartis, Actelion, GSK, Lilly (kein Bezug zu diesem Artikel). Nichtfinanzielle Interessen: Klinikdirektion, UKSH Lübeck | Mitgliedschaften: Deutsches Netzwerk für Systemische Sklerodermie (DNSS), European Scleroderma Trials and Research Group (EUSTAR).

Wissenschaftliche Leitung

Die vollständige Erklärung zum Interessenkonflikt der Wissenschaftlichen Leitung finden Sie am Kurs der zertifizierten Fortbildung auf www.springermedizin.de/cme.

Der Verlag

erklärt, dass für die Publikation dieser CME-Fortbildung keine Sponsorengelder an den Verlag fließen.

Für diesen Beitrag wurden von den Autoren keine Studien an Menschen oder Tieren durchgeführt. Für die aufgeführten Studien gelten die jeweils dort angegebenen ethischen Richtlinien.

Supplementary material

108_2019_699_MOESM1_ESM.pdf (79 kb)
Zusätzliche ESM-Tab. 1: Interventionelle Studienansätze bei SSc

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Copyright information

© Springer Medizin Verlag GmbH, ein Teil von Springer Nature 2019

Authors and Affiliations

  1. 1.Klinik für Rheumatologie und ImmunologieUniversitätsklinikum Schleswig-Holstein, Campus LübeckLübeckDeutschland

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