Zusammenfassung
Die systemische Sklerose (Sklerodermie) ist eine rheumatologische Erkrankung, die neben Entzündung und Autoimmunität auch durch Fibrose und vaskuläre Komplikationen gekennzeichnet ist. Die Therapie der Patienten richtet sich im Wesentlichen nach den vorliegenden Organbeteiligungen und beinhaltet nicht nur eine Immunsuppression, sondern auch die Behandlung der vaskulären und fibrotischen Krankheitsmanifestationen. Insbesondere letztere können oft nur ungenügend behandelt werden, während für vaskuläre Komplikationen in den letzten Jahren viele Medikamente getestet worden sind. Die aktuellen Therapieoptionen bei systemischer Sklerose werden hier noch einmal zusammengefasst. In der Therapie dieser seltenen Erkrankung hat sich in den letzten 10 Jahren vieles geändert. Auch in Zukunft wird sich dieser Trend fortsetzen.
Abstract
Systemic sclerosis (scleroderma) is a rheumatologic disease characterised not only by inflammation/autoimmunity, but also by tissue fibrosis and vascular lesions. The therapeutic approach to patients is dictated by the organ involvement and includes treatment of vascular and fibrotic disease features beyond mere immunosuppression. Fibrotic features in particular, are still inadequately treated, whereas many drugs have been tested for vascular complications within recent years. In this review, the currently available treatment options for this rare disease are presented. Therapy options in systemic sclerosis have changed over the past 10 years and this trend will also continue in the future.
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Becker, M.O. Systemische Sklerose. Internist 57, 1155–1163 (2016). https://doi.org/10.1007/s00108-016-0148-1
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DOI: https://doi.org/10.1007/s00108-016-0148-1
Schlüsselwörter
- Immunsuppression
- Lungenfibrose
- Hautfibrose
- Hämatopoetische Stammzelltransplantation
- Pulmonal-arterielle Hypertonie