CREST syndrome

CREST syndrome
Classification and external resources
ICD-10	M34.1
ICD-9	710.1
OMIM	181750
DiseasesDB	29764
MeSH	D017675

The limited cutaneous form of systemic scleroderma (lcSSc) is often referred to as CREST syndrome.^[1] "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 is a systemic inflammatory rheumatic disease and usually results in more pathologies than the five symptoms above.

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

The origin of CREST lies in the immune system. There is production of anti-nuclear and anti-centromere antibodies, although it is not known if these antibodies are involved in the cause of the symptoms of the disease. There is no known infectious cause.

There is no cure for this disease as there is no cure for scleroderma in general. It slowly progresses and should be examined and treated as soon as it has been diagnosed with immunosuppressives and other medications. There are treatments that can ameliorate the symptoms of esophageal reflux, pulmonary hypertension and Raynaud phenomenon.

See also

• Scleroderma

References

1. ^ 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.