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Related Experiment Videos

Targeted therapy for systemic sclerosis.

Virginia Steen1

  • 1Medstar Georgetown University, Division of Rheumatology, Immunology, and Allergy, 3800 Reservoir Road NW, LL Gorman, Washington, DC 20007, USA. steenv@georgetown.edu

Autoimmunity Reviews
|January 25, 2006
PubMed
Summary
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Systemic sclerosis involves three main pathways: inflammation, endothelial cell damage leading to complications like pulmonary arterial hypertension and renal crisis, and fibroblast activity causing skin thickening. Treatments are emerging for some complications.

Area of Science:

  • Rheumatology
  • Immunology
  • Pathophysiology

Background:

  • Systemic sclerosis is a complex multisystem autoimmune disease.
  • Pathogenesis involves distinct pathways contributing to organ damage.

Purpose of the Study:

  • To outline the key pathogenic pathways in systemic sclerosis.
  • To discuss current and potential therapeutic targets for organ-specific damage.

Main Methods:

  • Review of established knowledge on systemic sclerosis pathogenesis.
  • Analysis of the roles of T-cells, endothelial cells, and fibroblasts.
  • Examination of specific complications and their treatment.

Main Results:

  • Early disease involves prominent inflammation (e.g., alveolitis).

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  • Endothelial cell damage leads to pulmonary arterial hypertension and renal crisis, with improving treatments.
  • Fibroblast activation causes characteristic skin thickening (cutaneous fibrosis), with limited current treatment.

    • Conclusions:

    • Understanding these pathways is crucial for developing targeted therapies.
    • While B cells and autoantibodies are not primary drivers, they aid in subset classification.
    • Unknown environmental factors and genetic predisposition influence disease development.