Systemic Sclerosis

An Update

Uwe-Frithjof Haustein, MD, PhD

Disclosures

Lab Med. 2011;42(9):562-572. 

In This Article

Clinical Features

In general SSc affects the connective tissue, predominantly of the skin and vessel wall and, to a lesser extent, the gastrointestinal tract, heart, lungs, and kidneys. Cutaneous symptoms, often associated or preceded by Raynauda's phenomenon and finger arthralgias, are usually early signs in the course of SSc and therefore are helpful to establish diagnosis and initiate therapy.

The following 3 phases of dermal involvement can be distinguished:[25] 1) edematous phase (stiff, puffy fingers), 2) indurative phase (hard, tight, hidebound), 3) atrophic phase (softened skin, burned out).

The usefulness of the skin thickness score has been confirmed as a predictor and correlate of the outcome in SSc.[26] An abnormal nailfold capillary pattern on capillaroscopy with dilatation of all 3 parts of the capillary loop (arterial, apical, and venular) and loss of capillaries (drop out) either diffusely, or in localized areas, is an early and important diagnostic sign for SSc.

The prognosis of SSc largely depends on involvement of internal organs, particularly the lungs, heart, and kidneys. Involvement of the gastrointestinal tract, although most frequent, is less life threatening. Severe organ involvement often occurs early in the course of diffuse SSc.[27] As their survival is markedly reduced, these patients should be monitored very closely during the first 3 years and potential disease-modifying therapies must be initiated early.

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