CREST syndrome


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CREST syndrome

 
one of the less severe forms of systemic scleroderma, consisting of calcinosis of the skin, raynaud's phenomenon, esophageal dysfunction, sclerodactyly, and telangiectasia. When esophageal dysfunction is not prominent, it is known as CRST syndrome.

CREST syn·drome

a variant of systemic sclerosis characterized by calcinosis, Raynaud phenomenon, esophageal motility disorders, sclerodactyly, and telangiectasia.
An acronym for the pentad of clinical signs seen with a limited form of scleroderma:
Calcinosis cutis
Raynaud’s phenomenon—at least a 2-phase colour change
Esophageal distal hypomotility or reflux esophagitis
Sclerodactyly or nonpitting digital edema
Telangiectasia; CREST has a slightly better prognosis than other connective tissue disorders, but has late complications—e.g., biliary cirrhosis and pulmonary hypertension
Lab Anticentromere antibodies are characteristic of CREST, but may also be seen in progressive systemic sclerosis, older women, or those with HLA-DR1

CREST syn·drome

(krest sin'drōm)
An acronymic designation for a variant of scleroderma characterized by calcinosis, Raynaud phenomenon, esophageal motility disorders, sclerodactyly, and telangiectasia.

CREST syndrome

Acronym for a connective tissue disorder, related to progressive systemic sclerosis, but limited to CALCINOSIS, RAYNAUD'S PHENOMENON, oesophageal involvement, hardness of the fingers (Sclerodactyly) and dilated skin blood vessels (TELANGIECTASIA). The word forming the third letter of the acronym is spelt ‘esophageal’ in the USA.

Thibierge,

Georges, French physician, 1856-1926.
Thibierge-Weissenbach syndrome - calcinosis, Raynaud phenomenon, esophageal motility disorders, sclerodactyly, and telangiectasia. Synonym(s): CREST syndrome

CREST syn·drome

(krest sin'drōm)
Acronym for variant of systemic sclerosis characterized by calcinosis, Raynaud phenomenon, esophageal motility disorders, sclerodactyly, and telangiectasia.
References in periodicals archive ?
Our case presentation confirms the possibility of overlap syndrome between autoimmune thyroiditis and CREST syndrome and highlights the importance of a multidisciplinary approach of the patient with autoimmune disorders.
Moreover, the serologic confirmation of CREST syndrome in a patient without any digestive symptoms could have masked the presence of the gastric malignancy.
It is important to be aware that CREST syndrome and autoimmune thyroiditis can coexist and a thorough evaluation of both conditions and gastric cancer screening may be necessary even in patients who do not complain with digestive symptoms.
Small lymphocytic lymphoma in a patient with CREST syndrome. Hematol Oncol Stem Cell Ther 2011; 4(3):132-135.
To improve sensitivity for milder forms of disease, the condition is often divided into diffuse systemic scleroderma (dSSc) or limited systemic scleroderma (lSSc) (TABLE 1), with CREST syndrome being a variant of the limited form.
If CREST syndrome is suspected, it is important to look for its cardinal features.
Our patient had all the cardinal features of CREST syndrome on history and physical exam.