Title: CREST Syndrome: Background, Pathophysiology, Etiology
Headings: CREST Syndrome
CREST Syndrome
Background

Content: Frayha et al [ 5] noted the frequent occurrence of esophageal dysmotility and suggested that the acronym CREST may be more appropriate. Velayos et al [ 6] reviewed 13 patients with CREST and CRST syndromes and found the syndromes equivalent. The 1980 American College of Rheumatology Classification Criteria for Rheumatic Diseases is the most widely used system for systemic scleroderma. Because it was designed for research applications and not for clinical diagnosis, it has been criticized for its low sensitivity in identifying early disease and milder forms of systemic scleroderma such as CREST syndrome. Several authors recognized this limitation and responded by categorizing patients with scleroderma syndromes into 2 groups: those with diffuse cutaneous scleroderma and those with a limited form of scleroderma. [ 7, 8, 9]
Others have shown that visceral involvement, poorer prognosis, and higher mortality are all more common in patients with diffuse disease. [ 10, 11, 12, 13] Several new classification systems may better categorize the wide spectrum of systemic scleroderma. In 2004, Nadashkevich et al [ 14] proposed the classification criteria (1) autoantibodies to centromere proteins, Scl-70 (topo I) and fibrillarin; ( 2) bibasilar pulmonary fibrosis; (