CREST Syndrome

The limited cutaneous form of systemic scleroderma (lcSSc) is often referred to as CREST syndrome. "CREST" is an acronym for the five main features:

  • Calcinosis
  • Raynaud's syndrome
  • Esophageal dysmotility
  • Sclerodactyly
  • Telangiectasia

It is a form of systemic scleroderma associated with antibodies against centromeres and usually spares the kidneys. If the lungs are involved it is usually in the form of pulmonary arterial hypertension.

CREST syndrome is a collection of symptoms seen in limited cutaneous systemic scleroderma and is a systemic inflammatory autoimmune connective tissue disease.

Patients with lcSSc commonly induce pulmonary artery hypertension which may result in heart failure. Blood vessel thrombosis and arteriosclerosis has also led to the necessity of amputation of fingers and leg ulceration may result from burst blood vessels and thin skin, leading to chronic infections. Other symptoms of CREST syndrome can be exhaustion, weakness, difficulties with breathing, dizziness and badly healing wounds.

Crest syndrome involves the production of autoimmune anti-nuclear and anti-centromere antibodies, though their etiology is not currently understood. There is no known infectious cause.

Disease progression may be slowed with immunosuppressives and other medications, and esophageal reflux, pulmonary hypertension and Raynaud phenomenon may benefit from symptomatic treatment. However, there is no cure for this disease as there is no cure for scleroderma in general,

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