Overview
A systemic connective tissue disease characterized by the chronic hardening and tightening of the skin. CREST syndrome is a type of limited scleroderma.
CREST Syndrome
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.
Recent Research
- Symptom experience of limited cutaneous systemic sclerosis from the Patients’ perspective: A qualitative study✰,✰✰,★,★★ (2021)
- Phenotype of limited cutaneous systemic sclerosis patients with positive anti-topoisomerase I antibodies: data from the EUSTAR cohort (2022)
- [18F]Sodium Fluoride PET has the potential to identify active formation of calcinosis cutis in limited cutaneous systemic sclerosis (2022)
- Hemorrhagic Cardiac Tamponade as a Complication of Limited Cutaneous Systemic Sclerosis (2023)
Sources
- Article Sources
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.
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.
Scleroderma Foundation. (n.d.). What is scleroderma? What is scleroderma? – Scleroderma Foundation. https://www.scleroderma.org/site/SPageServer/?pagename=patients_whatis#.YLf8rH1KiqC.