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[Systemic sclerosis].

S Kleinert1, H P Tony, C Kneitz

  • 1Schwerpunkt Rheumatologie und Klinische Immunologie, Medizinische Klinik und Poliklinik II, Universität Würzburg, Klinikstrasse 6-8, 97070, Würzburg, Germany. Kleinert_S@klinik.uni-wuerzburg.de

Der Internist
|September 15, 2006
PubMed
Summary
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Systemic sclerosis (SSc) is a rare autoimmune disease affecting women more than men, causing progressive fibrosis and organ damage. Management depends on severity, with immunosuppressants for acute organ involvement and vasodilators for Raynaud's phenomenon.

Area of Science:

  • Rheumatology
  • Autoimmunology
  • Dermatology

Context:

  • Systemic sclerosis (SSc), or systemic scleroderma, is a rare autoimmune disease.
  • It disproportionately affects women, with a prevalence fivefold higher than in men.
  • The disease is characterized by progressive fibrosis and vascular damage, leading to organ involvement.

Purpose:

  • To provide an overview of systemic sclerosis (SSc).
  • To highlight key aspects of its epidemiology, clinical course, and management.
  • To emphasize the impact of SSc on patient survival and quality of life.

Summary:

  • SSc presents with characteristic skin manifestations and progresses slowly, often involving internal organs.
  • The diffuse form of SSc is associated with more severe organ manifestations than the limited form.

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  • Cardiopulmonary involvement significantly impacts mortality, with a 5-year survival rate of approximately 75%.

    • Impact:

    • Understanding SSc progression and organ involvement is crucial for timely diagnosis and intervention.
    • Tailoring treatment based on disease severity, including immunosuppressants and vasodilators, can improve patient outcomes.
    • Further research into SSc pathogenesis and novel therapies is needed to enhance long-term survival and patient well-being.