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

Content: Jeanie C Yoon, MD; Chief Editor: Dirk M Elston, MD more...
Sections
CREST Syndrome
Sections CREST Syndrome
Overview
Background
Pathophysiology
Etiology
Epidemiology
Prognosis
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Presentation
History
Physical Examination
Complications
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DDx
Workup
Laboratory Studies
Imaging Studies
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Treatment
Medical Care
Surgical Care
Consultations
Diet
Activity
Prevention
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Medication
Medication Summary
Calcium channel blockers
Prostaglandins
Antidepressants
Phosphodiesterase enzyme inhibitors
Histamine H2 antagonists
Proton-pump inhibitors
Antihypertensive agents
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Questions & Answers
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References
Overview
Background
CREST ( calcinosis, Raynaud phenomenon, esophageal dysmotility, sclerodactyly, and telangiectasia) syndrome is a member of the heterogeneous group of sclerodermas, and its name is an acronym for the cardinal clinical features of the syndrome. [ 1, 2]
In 1910, Thibierge and Weissenbach described the first case report of what was later called CRST (calcinosis cutis, Raynaud phenomenon, sclerodactyly, and telangiectasia) syndrome in English by Winterbauer who, in 1964, described a series of 8 patients with the features that make up the abbreviation CRST. [ 3, 4] Although he noted esophageal dysmotility in 4 of 8 patients, he did not include this feature in his original description of CRST syndrome. 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: