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Therapeutische Strategien im Frühstadium der systemischen Sklerose

Frühe Diagnose – frühe Symptome – frühe Probleme

Therapeutic management in early disease stages of systemic sclerosis

Early diagnosis – early symptoms – early problems

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Zeitschrift für Rheumatologie Aims and scope Submit manuscript

Zusammenfassung

Aufgrund der verbesserten diagnostischen Möglichkeiten der letzten Jahrzehnte kann heute die Erkrankung der systemischen Sklerose (SSc) in immer früheren Erkrankungsstadien diagnostiziert werden. Mit der Frühdiagnose der Erkrankung ergeben sich für den Rheumatologen gleichzeitig neue Herausforderungen im klinischen Alltag. In diesem Artikel wird das therapeutische Management der systemischen Sklerose mit einem Schwerpunkt auf die Frühstadien der Erkrankung erläutert. Diagnostik und Therapie früher Symptome, wie dem Raynaud-Phänomen, „puffy fingers“, digitalen Ulzerationen, Calcinosis cutis sowie einer frühen kardiopulmonalen, nephrologischen oder gastrointestinalen Beteiligung, werden diskutiert. Abschließend werden Möglichkeiten und Grenzen der frühen immunmodulatorischen Therapie und autologen Stammzelltransplantation für Patienten mit einem rasch progressiven und schweren Verlauf der Erkrankung besprochen.

Abstract

Increasing knowledge about the rare disease systemic sclerosis and improved diagnostic methods in the course of recent decades has led to the possibility of diagnosing systemic sclerosis at earlier disease stages. However, earlier diagnosis has an impact on routine clinical care of affected patients, and rheumatologists need to know about early symptoms, their diagnosis, and clinical management. In this review, the therapeutic management of early disease stages is described. In particular, we focus on diagnostic tools which should be included in a “basic assessment” of systemic sclerosis and discuss the diagnosis and treatment options of early symptoms such as Raynaud phenomenon, puffy fingers and hand edema, digital ulcers, calcinosis cutis, and cardiopulmonary, renal, and gastrointestinal involvement. Finally, the options of early immunosuppressive treatment and autologous stem cell transplantation for patients with rapid progressive and severe disease are reviewed.

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Einhaltung ethischer Richtlinien

Interessenkonflikt. Dr. Frerix, Dr. Meier, Dr. Hermann und Prof. Müller-Ladner haben Honorare für eine Referententätigkeit oder im Rahmen von Fortbildungen Reisekostenübernahmen von der Firma Pfizer oder Actelion erhalten. Dr. Meier hat ein Forschungsstipendium von der Firma Pfizer Pharma GmbH erhalten.

Dieser Beitrag enthält keine Studien an Menschen oder Tieren.

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  1. Lehrstuhl für Innere Medizin mit Schwerpunkt Rheumatologie, Justus-Liebig-Universität Gießen, Gießen, Deutschland

    M. Frerix, F.M.P. Meier & U. Müller-Ladner

  2. Abteilung für Rheumatologie und klinische Immunologie, Kerckhoff-Klinik, Benekestr. 2–8, 61231, Bad Nauheim, Deutschland

    M. Frerix, W. Hermann & U. Müller-Ladner

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  1. M. Frerix
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  2. F.M.P. Meier
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  3. W. Hermann
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  4. U. Müller-Ladner
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Correspondence to M. Frerix.

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Frerix, M., Meier, F., Hermann, W. et al. Therapeutische Strategien im Frühstadium der systemischen Sklerose. Z. Rheumatol. 72, 960–969 (2013). https://doi.org/10.1007/s00393-013-1270-2

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