CREST Syndrome

An autoimmune connective tissue disorder associated with anticentromere antibodies. A form of scleroderma associated with esophageal dysmotility. CREST is an acronym for calcinosis, Raynaud phenomenon, esophageal dysmotility, sclerodactyly, telangiectasis.

Limited Scleroderma; Thibièrge-Weissenbach Syndrome (some authors use Thibièrge Weissenbach Syndrome to describe the calcinosis of the hand in combination with ischemia in the context of scleroderma).

First described in 1910 by G. Thibièrge and R.J.E. Weissenbach, two French physicians.

The incidence of CREST syndrome is not known. The incidence of systemic sclerosis in the general population is in the range of approximately 2-7:1,000,000 (with geographical differences). All ethnicities can be affected. Females are affected two to four times more often than males.

Although a few cases with an inheritance pattern have been described, in most cases autoimmune processes appear to be involved in the pathogenesis of this disorder. A genetic predisposition may be present.

The three primary pathologic processes of scleroderma are initially perivascular infiltration with predominantly lymphocytes and macrophages, followed by collagen (including fibronectin and proteoglycans) deposition with tissue thickening and fibrosis secondary to chronic activation of skin fibroblasts, and vascular changes mainly affecting the small arteries and presenting with endothelial injury, intima proliferation, vasospasms, and significantly decreased neovascularization. It has been suggested that several cytokines are involved in the pathogenesis of this disorder, particularly transforming growth factor β and interleukin-4, which are both present in increased concentrations in this disorder, while the level of interferon-γ, which acts as an inhibitor of collagen synthesis, is decreased.

The minimal diagnostic criteria for scleroderma required by the American College of Rheumatology include either the major criterion of proximal cutaneous scleroderma (defined as symmetric thickening, tightening, and induration of the skin of the fingers and the skin proximal to the metacarpophalangeal or metatarsophalangeal joints. The changes may affect the entire limb or spread to head, neck, and trunk) or two of the three minor criteria (i.e., sclerodactyly [aforementioned findings, but strictly limited to the fingers], digital [finger pad] pitting scars, or bilateral pulmonary fibrosis with reticular or reticulonodular pattern on the chest radiograph). The presence of anticentromere antibodies in the serum may be helpful for the diagnosis (positive in approximately 50% of patients with CREST syndrome), while only approximately 20% test positive for scleroderma antibody (SCL-70/topoisomerase I). However, overall the sensitivity and specificity of these antibodies are limited.

Two different forms of scleroderma have been recognized. The localized form includes morphea and linear scleroderma (“sclérodermie en coup de sabre” if located in the facial area) and does not result in visceral organ involvement. The two types of systemic scleroderma are limited scleroderma (80% of patients) and diffuse scleroderma (20%). In limited scleroderma, the disease usually progresses slowly (over several years) and, by definition, the skin involvement remains distal to elbows and knees (however, involvement of the face and neck may occur). In more than two ...

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