CREST syndrome

CREST syndrome
Other namesCalcinosis-Raynaud phenomenon-esophageal involvement-sclerodactyly-telangiectasia syndrome[1]
CREST syndrome (calcinosis and sclerodactyly)
SpecialtyRheumatology

CREST syndrome, also known as the limited cutaneous form of systemic sclerosis (lcSSc), is a multisystem connective tissue disorder. The acronym "CREST" refers to the five main features: calcinosis, Raynaud's phenomenon, esophageal dysmotility, sclerodactyly, and telangiectasia.[2]

CREST syndrome is associated with detectable antibodies against centromeres (a component of the cell nucleus), and usually spares the kidneys (a feature more common in the related condition systemic scleroderma). If the lungs are involved, it is usually in the form of pulmonary arterial hypertension.

  1. ^ RESERVED, INSERM US14-- ALL RIGHTS. "Orphanet: CREST syndrome". www.orpha.net. Retrieved 27 April 2019.{{cite web}}: CS1 maint: numeric names: authors list (link)
  2. ^ Winterbauer RH (1964). "Multiple telangiectasia, Raynaud's phenomenon, sclerodactyly, and subcutanious calcinosis: a syndrome mimicking hereditary hemorrhagic telangiectasia". Bulletin of the Johns Hopkins Hospital. 114: 361–83. PMID 14171636.