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CREST Syndrome

Last updated July 26, 2022

Reviewed by: Lester Fahrner, MD

Approved by: Krish Tangella MD, MBA, FCAP

Limited Systemic Scleroderma is a subtype of Scleroderma that affects a variety of organs, such as the gastrointestinal tract, heart, muscles, and joints, potentially causing severe damage.

The topic CREST Syndrome you are seeking is a synonym, or alternative name, or is closely related to the medical condition Limited Systemic Scleroderma.

Quick Summary:

  • Limited Systemic Scleroderma is a subtype of Scleroderma that affects a variety of organs, such as the gastrointestinal tract, heart, muscles, and joints, potentially causing severe damage
  • Scleroderma is a chronic connective tissue disorder of unknown cause. Connective tissues give support to the organs and skin. Individuals with Scleroderma have thick or hard connective tissue

There is currently a debate about how Scleroderma should be classified. A majority believe that it should be classified as an autoimmune rheumatologic disorder. Due to this, many researchers believe that the damage is caused by immune cells mistakenly attacking one’s own healthy tissue.

Scleroderma can be classified into 2 broad categories, which include Localized Scleroderma and Systemic Scleroderma:

  • Localized Scleroderma:
    • It often affects the skin tissues and does not cause harm to the other major organs
    • There are two types of Localized Scleroderma, namely Morphea and Localized Linear Scleroderma
  • Systemic Scleroderma:
    • It affects the skin, tissue beneath the skin, major organs, and blood vessels
    • Systemic Scleroderma also has 2 different types, namely Limited Systemic Scleroderma and Diffuse Systemic Scleroderma

Limited Systemic Scleroderma is a type of Systemic Scleroderma of unknown cause.

  • Commonly, the organs involved include the esophagus (food pipe), stomach, small intestines, large intestines, heart, muscles, joints, and kidneys. However, it may take years for hardening of the tissue to occur, which in turn, can cause a much slower progression of the signs and symptoms
  • Typically, Diffuse Systemic Scleroderma progresses rapidly, resulting in severe signs and symptoms and even life-threatening complications. With Limited Systemic Scleroderma, there is no rapid progression of the condition
  • Limited Systemic Scleroderma is also called as CREST Syndrome. The term CREST stands for:
    • Calcinosis: Calcium deposits on the skin
    • Raynaud’s phenomenon: Blood vessel spasms in response to cold temperatures or stress
    • Esophageal dysfunction: Acid reflux and less motility in the esophagus
    • Sclerodactyly: Thickening and tightening of the skin of the fingers and toes
    • Telangiectasias: Red capillary dilation on the skin of the face and chest
  • There is currently no cure for Limited Systemic Scleroderma, but medications, therapy, and surgery may be used to control symptoms and prevent complications. The treatment measures are dependent upon the tissues or organs affected
  • The way Limited Systemic Scleroderma changes and progresses over time vary among individuals. The rapidity of progression of signs and symptoms and the organs involved affects the prognosis. Typically, individuals who respond to treatment have a better prognosis, than individuals who do not respond to treatment

Please find comprehensive information on Limited Systemic Scleroderma regarding definition, distribution, risk factors, causes, signs & symptoms, diagnosis, complications, treatment, prevention, prognosis, and additional useful information HERE.

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Reviewed and Approved by a member of the DoveMed Editorial Board
First uploaded: June 16, 2017
Last updated: July 26, 2022