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Der Hautarzt

, Volume 70, Issue 9, pp 723–741 | Cite as

Systemische Sklerose – klinisches Bild, Diagnostik und Therapie

  • M. O. BeckerEmail author
  • O. Distler
  • B. Maurer
CME
  • 127 Downloads

Zusammenfassung

Die systemische Sklerose ist eine seltene rheumatische Erkrankung, die durch Haut- und Organfibrose, vaskuläre Veränderungen und das Auftreten spezifischer Autoantikörper charakterisiert ist. Sie zeigt eine hohe Morbidität und Mortalität, weist dabei aber in ihrem Verlauf und den Manifestationen eine beträchtliche Heterogenität auf. Dies macht eine umfassende Diagnostik zur Diagnosestellung und bei den Verlaufsuntersuchungen notwendig, da auch die Therapie den jeweiligen Krankheitsmanifestationen angepasst werden muss. Obwohl spezifische Therapien für gastrointestinale, pulmonale oder auch vaskuläre Komplikationen existieren, sprechen die Patienten meistens nur teilweise an und neue Therapieansätze sind notwendig.

Schlüsselwörter

Limitierte systemische Sklerose Diffuse systemische Sklerose CREST-Syndrom Lungenfibrose Pulmonalarterielle Hypertonie 

Systemic sclerosis—clinical picture, diagnosis, and treatment

Abstract

Systemic sclerosis is a rare rheumatologic disease that is characterised by skin and organ fibrosis as well as vascular changes and the occurrence of specific autoantibodies. It has a high morbidity and mortality while its manifestations show significant heterogeneity in patients. Thus, diagnosis and follow-up of patients with systemic sclerosis has to be extensive, the more so because treatment must be adapted to organ manifestations. Although specific therapies for gastrointestinal, pulmonary or vascular complications exist, patients respond only partly to these and new therapeutic approaches are still needed.

Keywords

Limited systemic sclerosis Diffuse systemic sclerosis CREST syndrome Pulmonary fibrosis Pulmonary hypertension 

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

M. Becker: finanzielle Interessen: Bayer – Reisekosten | MSD – Referentenhonorar/Reisekosten, | IBSA Institut Biochimique SA – Reisekosten, Roche – Reisekosten – Patente, Geschäftsanteile, Aktien an einer im Medizinbereich aktiven Firma (ausgenommen fondgebundene Beteiligungen): Heidelberg Pharma | Esperion Therapeutics. Nichtfinanzielle Interessen: Angestellter Oberarzt (Rheumatologe), Klinik für Rheumatologie, Universitätsspital Zürich, Schweiz | Mitglied der Deutschen Gesellschaft für Rheumatologie und der Deutschen Gesellschaft für Innere Medizin | Mitglied im Verband Schweizerischer Assistenz- und Oberärztinnen und -ärzte. O. Distler: finanzielle Interessen: Forschungsförderung zur persönlichen Verfügung (finanziell oder als geldwerte Leistungen): Diverse nicht kommerzielle Stiftungen und nationale Wissenschaftsorganisationen (Schweizer Nationalfond (Forschungsförderung) [SNF] etc.) | Actelion, Bayer, Boehringer Ingelheim, Mitsubishi Tanabe – Referentenhonorar: Actelion, Mepha, Pfizer, MSD SHARP & DOHME, Roche, Novartis | Kongress Kostenerstattung: Pfizer (2016, 2015) – bezahlter Berater, interner Schulungsreferent oder Bezug eines Gehalts o. ä.: im Bereich der Sklerodermie und assoziierter Erkrankung: A. Menarini, Amgen, AnaMar, Bayer, Boehringer Ingelheim, Catenion, CSL Behring, ChemomAb, Ergonex, Roche, GSK, Inventiva, Italfarmaco, iQone, iQvia, Lilly, Medac, Medscape, Mitsubishi Tanabe Pharma, MSD SHARP & DOHME, Novartis, Pfizer, Roche, Sanofi, Target Bio Science und UCB. Nichtfinanzielle Interessen: Professor, Klinikdirektor, Klinik für Rheumatologie, UniversitätsSpital Zürich | Direktor Forschung, Zentrum für Experimentelle Rheumatologie, Schlieren | Bereichsleiter Bereich Traumatologie/Dermatologie/Rheumatologie (TDR), UniversitätsSpital Zürich | Mitgliedschaften: Schweizerische Gesellschaft für Rheumatologie Zürich, Board Mitglied European Scleroderma Trials and Research Group (EUSTAR), Walter-Siegenthaler-Gesellschaft, Chefärztegesellschaft des Kantons Zürich, Stiftungsrat, Swiss Clinical Quality Management in Rheumatic Diseases in Zürich, Stiftungsrat Hartmann-Müller-Stiftung, Schweizerische Akademie der Medizinischen Wissenschaften (SAMW), Schweizerische Akademie der Medizinischen Wissenschaften in Bern. B. Maurer: finanzielle Interessen: Forschungsförderung zur persönlichen Verfügung (finanziell oder als geldwerte Leistungen): OPO-Stiftung, Hartmann-Müller-Stiftung, EMDO Stiftung (Forschungsförderung), Vontobel-Stiftung, Herzog-Egli-Stiftung, Olga-Mayenfisch-Stiftung, Lunge Zürich, Zuschuss der Deutschen Gesellschaft für systemische Sklerose, Filling-the-Gap-Zuschuss, Prof.-Max-Cloetta-Stiftung, AbbVie, Protagen, Novartis, Protagen – Kongressunterstützung durch Pfizer, Roche, MSD SHARP & DOHME und Actelion. Nichtfinanzielle Interessen: leitende Ärztin am UniversitätsSpital Zürich (Rheumatologie) | Mitgliedschaften: Zürcher/Schweizer Gesellschaft für Rheumatologie, Foederatio Medicorum Helveticorum (FMH), Verband Schweizerischer Assitenz- und Oberärztinnen und -ärzte (VSAO), Deutschen Gesellschaft für Innere Medizin, EuMyoNet, IMACS, European Scleroderma Trials and Research Group.

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.

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

Authors and Affiliations

  1. 1.Klinik für RheumatologieUniversitätsspital ZürichZürichSchweiz

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