CREST Syndrome
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Description[edit | edit source]
CREST syndrome (also known as Cutaneous systemic sclerosis) is an autoimmune disease that has been defined as a subtype of progressive systemic sclerosis with limited skin involvement. [1]The word "CREST " is an acronym for the clinical features that are seen in a patient with this disease:
- Calcinosis- when calcium salts are deposited into the skin and subcutaneous tissue.[2]
- Raynaud's phenomenon.
- Esophageal dysmotility-which can cause difficulty in swallowing.
- Sclerodactyly- scleroderma in which the fingers become thin and shiny with sclerotic skin at the tip due to subcutaneous and intracutaneous calcinosis and diffused fibrosis of the collagen.[3]
- Telangiectasia-small widened blood vessels on the skin.[4]
Epidemiology[edit | edit source]
Wide variation in prevalence of systemic sclerosis was observed, with slightly higher estimates reported in North America (13.5–44.3 per 100,000 individuals) compared to Europe (7.2–33.9 per 100,000 individuals), which may be a true reflection of epidemiological variation or an artifact of clinical data analyses.[5] The apparent increase in both incidence and prevalence over the last 50 years is most likely due to improved classification, earlier diagnosis, and survival. CREST syndrome may account for 22-25% of all occurrences of systemic sclerosis, according to serum antibody investigations; however, epidemiologic research specifically looking at CREST syndrome are missing.[6] SSc diagnosis was reported to occur at the ages of 33.5-59.8 years in Europe and 46.1-49.1 years in North America, and to occur more commonly in women (female:male ratio of 3.8-11.5:1 in Europe and 4.6-15:1 in North America). Women have continuously greater prevalence and incidence rates, indicating a clinically significant difference in the occurrence of SSc across genders.[5]
Diagnostic Tests/Lab Tests/Lab Values[edit | edit source]
Etiology/Causes[edit | edit source]
Systemic Involvement[edit | edit source]
Medical Management[edit | edit source]
Physical Therapy Management[edit | edit source]
References[edit | edit source]
- ↑ Meyer O. CREST syndrome. Ann Med Interne (Paris) [Internet]. 2002;153(3):183–8. Available from: https://europepmc.org/article/med/12218901
- ↑ Le C, Bedocs PM. Calcinosis Cutis. StatPearls Publishing; 2023.
- ↑ Nelson FRT, Blauvelt CT. The Hand and wrist. In: Nelson FRT, Blauvelt CT, editors. A Manual of Orthopaedic Terminology. Elsevier; 2015. p. 307–41.
- ↑ Telangiectasia (Spider Veins) [Internet]. Pennmedicine.org. [cited 2023 Sep 8]. Available from: https://www.pennmedicine.org/for-patients-and-visitors/patient-information/conditions-treated-a-to-z/telangiectasia-spider-veins
- ↑ 5.0 5.1 Bergamasco A, Hartmann N, Wallace L, Verpillat P. Epidemiology of systemic sclerosis and systemic sclerosis-associated interstitial lung disease. Clin Epidemiol [Internet]. 2019;11:257–73. Available from: http://dx.doi.org/10.2147/clep.s191418
- ↑ Wangkaew S, Euathrongchit J, Wattanawittawas P, Kasitanon N, Louthrenoo W. Incidence and predictors of interstitial lung disease (ILD) in Thai patients with early systemic sclerosis: Inception cohort study. Mod Rheumatol [Internet]. 2016;26(4):588–93. Available from: https://academic.oup.com/mr/article-pdf/26/4/588/39351804/mr0588.pdf
