The CREST syndrome: A distinct serologic entity with anticentromere antibodies

doi:10.1016/0002-9343(80)90462-3

The American Journal of Medicine

Volume 69, Issue 4, October 1980, Pages 520-526

https://doi.org/10.1016/0002-9343(80)90462-3 Get rights and content

Abstract

The CREST syndrome is a variant of systemic sclerosis characterized by the presence of calcinosis, Raynaud's phenomenon, esophageal motility abnormalities, sclerodactyly and telangiectasia.

The serums of 27 patients with the CREST syndrome have been examined for the presence of antinuclear antibodies. Twenty-six of 27 (98 percent) serums contained high titers (> one:80) of an antibody that produces a discrete speckled pattern of immunofluorescence on a human laryngeal carcinoma cell line (HEp-2). The antibody has been shown to react with the centromeric region of metaphase chromosomes. This antibody was also found in three of 14 patients with Raynaud's disease, in one of 60 patients with systemic lupus erythematosus, in three of 26 patients with systemic sclerosis with diffuse scleroderma and in one of 15 patients with mixed connective tissue disease. The antibody was not detected in the serums of patients with rheumatoid arthritis, Sjo¨rgren's sicca complex or linear scleroderma. Patients with osteoarthritis who were age- and sex-matched to the group with the CREST syndrome did not have anti-centromere antibodies. Autoantibodies found in other connective tissue diseases [anti-DNA, anti-RNP, Sjo¨gren's syndrome antigen B (anti-SS-B) were not found in serums from patients with the CREST syndrome. A case report illustrating the appearance of the anticentromere antibody at a time when Raynaud's phenomenon antedated the clinical diagnosis of CREST syndrome is presented.

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: This research was supported by the Medical Research Council of Canada.

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The CREST syndrome: A distinct serologic entity with anticentromere antibodies

Abstract

Am J Med

Clin Immunol Immunopathol

Am J Med

Am J Med

Une forme de concretions calcaires sous-cutanees en relation avec la sclerodermie

Bull Mem Soc Med Hoˆp Paris

Multiple telangiectasia, Raynaud's phenomenon, sclerodactyly, and subcutaneous calcinosis: a syndrome mimicking hereditary hemorrhagic telangiectasia

Bull Johns Hopkins Hosp

CREST syndrome: a benign variant of scleroderma

Arch Dermatol

Calcinosis, Raynaud's Phenomenon, sclerodactyly and telangiectasis. The CREST syndrome

Arch Intern Med

Pulmonary hypertension in the CREST syndrome variant of progressive systemic sclerosis (scleroderma)

Ann Intern Med

Antinuclear and precipitating autoantibodies in progressive systemic sclerosis

Lancet

Serum antinuclear antibodies in systemic sclerosis (scleroderma)

Arthritis Rheum

Profile of antinuclear antibodies in progressive systemic sclerosis (PSS)

Arthritis Rheum

Profiles of anti-nuclear antibodies in systemic diseases

Ann Intern Med

Complexity of anti-nuclear antibodies in scleroderma

Arthritis Rheum

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Ann Intern Med

Preliminary criteria for the classification of systemic lupus erythematosus

Bull Rheum Dis