Systemic Sclerosis

An Update

Uwe-Frithjof Haustein, MD, PhD


Lab Med. 2011;42(9):562-572. 

In This Article


Therapy must be planned individually due to the inadequate knowledge of the point at which therapeutic action is appropriate and the difficulty of obtaining objective measurements of the treatment results. Beside basic recommendations, physiotherapeutic activities and competent psychological guidance are important. The therapy is directed at 3 pathogenic compartments:

  • Vascular system

  • Immune system (inflammation, immunomodulation, autoimmunity)

  • Fibrosis

Clinical trials in SSc must (semi)quantitatively define disease activity, specific stage of disease, joint motility, and level of internal organ involvement as published by Medsger and colleagues.[94] They developed a severity grading scale from 0 (no documented involvement) to 4 (endstage disease) for 9 potentially affected organ systems and tested it in 579 SSc patients.[94] In 1995, the European Scleroderma Study Group initiated a multicenter prospective 1-year study for this reason. Systemic sclerosis is non-curable but treatable, although the response to therapy is generally slow.


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