CREST syndrome

CREST syndrome, also known as the limited cutaneous form of systemic sclerosis (lcSSc) is a multisystem connective tissue disorder. The initialism "CREST" refers to the five main features: calcinosis, Raynaud's phenomenon, esophageal dysmotility, sclerodactyly and telangiectasia.^[1] It 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.

Signs and symptoms

CREST/lcSSc causes thickening and tightening of the skin with deposition of calcific nodules ("calcinosis"). 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.

Patients with lcSSc commonly induce pulmonary artery hypertension which may result in cor pulmonale (heart failure due to increased pulmonary artery pressure).

Etiology

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.

Treatment

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.

History

The combination of symptoms was first reported in 1964 by R.H. Winterbauer, at that point a medical student at Johns Hopkins School of Medicine.^[1]

See also

• Scleroderma

References