CREST syndrome

The CREST syndrome (synonym: limited systemic scleroderma, lSSc, Thibierge - Weissenbach syndrome) is one of the autoimmune diseases of the so-called collagen diseases. These diseases occur when the immune system begins to attack the body's own structures.

CREST stands for the names:

  • Calcinosis cutis ( " calcification " of the skin )
  • Raynaud's phenomenon ( Raynaud's syndrome)
  • Esophageale dysfunction (reduced function or impaired esophageal motility )
  • Sclerodactyly (thin, pale, indurated and hairless fingers)
  • Telangiectasia ( permanent, morbid enlargement of superficial vessels of the skin )

The CREST syndrome represents a local, so often limited to the above- mentioned phenomena form of scleroderma, a pathological thickening of the skin and changes of internal organs, dar. Since it is often localized, it runs mostly harmless. In addition, a late, but frequent occurrence of pulmonary hypertension, which can result in right heart strain.

The cause is unknown, but one can find a frequent association with HLA -DR 1,4 and 8

Proof is biochemically by antibodies directed against centromere components ( in 70-90 % of cases), and CENP -E ( centromere kinesin -like protein). Other diagnostic possibilities are, for example, a skin biopsy, or radiographic evidence of calcium deposits in the skin.

Swell

  • Gerd Herold, Internal Medicine, 2008
  • Autoimmune
  • Disease in rheumatology
206645
de