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Evidenz-basierte Therapie der systemischen Sklerose

  • E. Genth

Zusammenfassung

In der Behandlung der verschiedenen klinischen Formen der systemischen Sklerose (SSc) stehen medika-mentöse Therapieverfahren im Vordergrund. In Abhängigkeit von der Krankheitsaktivität sowie der Art und dem Schweregrad der Manifestationen an der Haut (diffuser vs. limitierter Befall), an Blutgefäßen und an verschiedenen inneren Organen werden systemische (antientzündliche, immun-suppressive, antifibrotische) und organspezifische Therapien verwendet. Die Wissensbasis ist in vielen Bereichen noch unzureichend und lückenhaft. Ausreichende Evidenz für eine systemische antientzündliche und antiproliferative Wirkung bei der diffusen kutanen Form der SSc liegt vor für Glukokortikoide, Methotrexat, Cyclophosphamid und für Cyclosporin A. Vasoaktive Therapien haben eine besondere Bedeutung zur Therapie des Ray-naud-Phänomens und kutaner Ul-zera (Nifedipin u. a. Dihydropyridine, Prostaglandinanaloga, Losartan, Prazosin), des arteriellen Hypertonus und der renalen Krise (ACE-Hemmer, AT-1-Antagonisten) und des pulmonalen Hypertonus (Epoprostenol). Cyclophosphamid ist in der Therapie der fibrosierenden Alveolitis wirksam, Prokinetika (Metoclopramid, Domperidon) bei gastro-ösophagealen und Octreotid bei intestinalen (Pseudoobstruktion) Motilitätsstörungen. Nichtmedika-mentöse Behandlungsverfahren wie physikalische Therapien (Lymphdrainage u.a.) sind bisher wenig untersucht. In besonderen Fällen sind chirurgische Maßnahmen (Entfernung von Kalkablagerungen) notwendig.

Schlüsselwörter

Systemische Sklerose Therapie Evidenzbasierte Medizin 

Summary

For the treatment of different forms of systemic sclerosis (SSc), drugs play a predominant role. Depending on disease activity as well as type and severity of cutaneous, vascular and internal organ manifestations, different systemic (antiinflammatory, immunsuppressive, antifibrotic) or organ-specific therapies are used. The scientific basis of most treatment modalities is insufficient and incomplete. There is sufficient evidence for an antiinflammatory and antiproliferative efficacy of glucocorticosteroids, methotrexate, cyclophosphamide and cyclosporine A in the treatment of diffuse cutaneous systemic sclerosis. Vasoactive therapies play an important role in treating Raynaud’s phenomenon (nifedipine or other dihydropyridines, prostaglandin analogs, losartan, prazosine), and arterial (ACE blockers, AT-1 antagonists) or pulmonary (epoprostenol) hypertension. Cyclophosphamide is effective in fibrosing alveolitis, prokinetic substances (metoclo-pramid, domperidone) in gastroesophageal dysmotility or octreotide in intestinal pseudoobstruction. Physical therapies (e.g., massage) are poorly studied. In particular cases, surgical measures (e.g., removal of calcifications) are necessary.

Key words

Systemic sclerosis treatment evidence-based medicine 

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Copyright information

© Steinkopff Verlag 2002

Authors and Affiliations

  • E. Genth
    • 1
  1. 1.Rheumaklinik und RheumaforschungsinstitutAachenGermany

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