CREST Syndrome

 
 

Overview

A systemic connective tissue disease characterized by the chronic hardening and tightening of the skin. CREST syndrome is a type of limited scleroderma. 

Common Symptoms

Calcinosis (calcium deposits under the skin), Raynaud’s phenomenon (decreased blood flow to fingers or toes), esophageal dysfunction (acid reflux), sclerodactyly (thickening and tightening of the skin), and telangiectasia (dilated vessels on the skin or inside of the mouth).

Coexisting Diseases and Conditions

Sjögren’s syndrome, SLE, autoimmune thyroiditis, hypothyroidism, rheumatoid arthritis, cardiovascular disease, type 1 diabetes, fibromyalgia, isolated pulmonary artery hypertension, myocardium, and sclerosis of the GI tract.

Risk Factors and Prevalence

Studies have found a greater prevalence of CREST syndrome in females, African Americans, and Choctaw Native Americans. Exposure to silica is an environmental factor associated with the disease.

Sources

  1. Article Sources and Footnotes

    1. Arnett, F. C., Howard, R. F., Tan, F., Moulds, J. M., Bias, W. B., Durban, E., Cameron, H. D., Paxton, G., Hodge, T. J., Weathers, P. E., & Reveille, J. D. (1996). Increased prevalence of systemic sclerosis in a Native American tribe in Oklahoma. Association with an Amerindian HLA haplotype. Arthritis and rheumatism, 39(8), 1362–1370. https://doi.org/10.1002/art.1780390814.

    2. Lescoat, A., Cavalin, C., Ballerie, A., Lecureur, V., Sesé, L., Cazalets, C., Lederlin, M., Coiffier, G., Belhomme, N., Paris, C., Garlantézec, R., Jouneau, S., & Jégo, P. (2020). Silica Exposure and Scleroderma: More Bridges and Collaboration between Disciplines Are Needed. American journal of respiratory and critical care medicine, 201(7), 880–882. https://doi.org/10.1164/rccm.201911-2218LE.

    3. Scleroderma Foundation. (n.d.). What is scleroderma? What is scleroderma? – Scleroderma Foundation. https://www.scleroderma.org/site/SPageServer/?pagename=patients_whatis#.YLf8rH1KiqC.