Table of ContentsView AllTable of ContentsAffected SystemsSymptomsCausesDiagnosisTreatmentPrognosis
Table of ContentsView All
View All
Table of Contents
Affected Systems
Symptoms
Causes
Diagnosis
Treatment
Prognosis
CREST syndrome is a connective tissue disorder and a limited form of systemic sclerosis. CREST is the acronym for its clinical features: calcinosis, Raynaud’s phenomenon, esophageal dysfunction, sclerodactyly, and telangiectasia.
Each of these conditions leads to different symptoms in CREST:
CREST syndrome affects different systems in the body and can therefore cause a variety of symptoms. Often, it causes thickening and/or hardening of the skin and internal organs.
Other names for CREST syndrome are limited cutaneoussystemic sclerosisor limited scleroderma.
Scleroderma can appear as a localized issue affecting only small areas of the body at a time (known as linear scleroderma or morphea), or as a systemic problem affecting the entire body. This rareconnective tissuedisease becomes more serious as it progresses to systemic disease.
This article explains how CREST syndrome affects the body, and the symptoms, causes, diagnosis, and treatment options for CREST.
Barb Elkin / iStock / Getty Images
Within these systems, connective tissues are the specific areas affected. For skin, this will appear as a visible thickening or hardening. For other organs, the effect can be more difficult to see. In short, systemic sclerosis causes the lining, or “skin” of organs to harden and not function well.
This dysfunction can cause major problems, such as in the esophagus, where the smooth muscles that line the organ don’t contract and move food effectively.
Symptoms of CREST Syndrome
Symptoms of CRESTcan vary based on the body system being affected. With CREST syndrome, five specific diseases are included which impact the skin, hands, feet, esophagus, and connective tissues of other internal organs. Depending on the body part affected, symptoms can include:
Systemic scleroderma, or the diseases included in CREST syndrome, are most common in femalesand people of African-American descent.
All forms of systemic sclerosis are autoimmune and likely have both genetic and environmental triggers, the latter of which could include infection. A genetic predisposition to this can be inherited or happen spontaneously.
Viruses or environmental factors that have been linked to developing diseases in the sclerosis family include:
Can Natural Remedies Help With Raynaud’s Syndrome?
CREST syndrome diagnosis can be complicated. It may come over time after diagnosis of a number of the conditions associated with the syndrome.
The first step is for your healthcare provider to do a physical assessment. They may suspect CREST syndrome if they see:
If your healthcare provider suspects you have one or more of the diseases in the sclerosis family, or all the elements of CREST syndrome, they will probably send you for additional lab testing and procedures. These tests include:
What Is CREST Syndrome?
Treating CREST syndrome is complicated because the disease process affects each part of the body differently. Also, different organs require different management. There is no universal treatment for the conditions included in CREST syndrome, but early diagnosis is helpful.
Some treatment options include:
Ask your healthcare provider for specific advice to manage your particular symptoms. A healthy diet and exercise regimen may also help keep CREST Syndrome under control.
CREST syndrome is associated with a lower survival rate than other types of systemic sclerosis, like sine scleroderma.
Effects on the heart and lungs are major causes of mortality in CREST. If you have CREST, your healthcare provider should carefully monitor your condition with anechocardiogramandpulmonary function testing.
Prognosis for patients with severe sclerosis has improved over the last several decades, thanks to improved management of organ failure.
A Word From Verywell
CREST syndrome is a term used to describe a number of diseases in the sclerosis family. Skin thickening, hardening, or swelling is usually a hallmark of these diseases, and one of the first noticeable symptoms.
If you have CREST, focus on maintaining a healthy diet and lifestyle. Developing a close relationship with your healthcare provider may help keep your sclerosis from progressing to organ failure.
9 Sources
Verywell Health uses only high-quality sources, including peer-reviewed studies, to support the facts within our articles. Read oureditorial processto learn more about how we fact-check and keep our content accurate, reliable, and trustworthy.
National Institute of Arthritis and Musculoskeletal and Skin Diseases.Scleroderma.
Sobolewski P, Maślińska M, Wieczorek M, et al.Systemic sclerosis - multidisciplinary disease: clinical features and treatment.Reumatologia. 2019;57(4):221-233. doi:10.5114/reum.2019.87619
Gao R, Qing P, Sun X, et al.Prevalence of Sexual Dysfunction in People With Systemic Sclerosis and the Associated Risk Factors: A Systematic Review.Sex Med. 2021;9(4):100392. doi:10.1016/j.esxm.2021.100392
Hughes M, Pauling JD, Armstrong-James L, Denton CP, Galdas P, Flurey C.Gender-related differences in systemic sclerosis.Autoimmun Rev. 2020;19(4):102494. doi:10.1016/j.autrev.2020.102494
Volkmann ER, Steen V, Li N, et al.Racial disparities in systemic sclerosis: short- and long-term outcomes among African American participants of SLS I and II.ACR Open Rheumatol. 2021;3(1):8-16. doi:10.1002/acr2.11206
Arcangeletti MC, Maccari C, Vescovini R, et al.A paradigmatic interplay between human cytomegalovirus and host immune system: possible involvement of viral antigen-driven CD8+ T cell responses in systemic sclerosis.Viruses. 2018;10(9):508. doi:10.3390/v10090508
De Almeida Chaves, S., Porel, T., Mounié, M.et al.Sine scleroderma, limited cutaneous, and diffused cutaneous systemic sclerosis survival and predictors of mortality.Arthritis Res Ther23, 295 (2021). https://doi.org/10.1186/s13075-021-02672-y
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