CREST syndrome

CREST syndrome
Synonyms	Limited scleroderma
Pronounce	N/A
Specialty	N/A
Symptoms	Calcinosis, Raynaud's phenomenon, Esophageal dysmotility, Sclerodactyly, Telangiectasia
Complications	Pulmonary hypertension, Gastroesophageal reflux disease
Onset	Middle age
Duration	Long term
Types	N/A
Causes	Autoimmune disease
Risks	Family history, Environmental factors
Diagnosis	Clinical evaluation, Antibody testing
Differential diagnosis	N/A
Prevention	N/A
Treatment	Medications, Physical therapy, Lifestyle changes
Medication	N/A
Prognosis	Variable
Frequency	Rare
Deaths	N/A

CREST syndrome is a subtype of systemic sclerosis, also known as limited scleroderma. It is characterized by a specific set of symptoms that form the acronym CREST: Calcinosis, Raynaud's phenomenon, Esophageal dysmotility, Sclerodactyly, and Telangiectasia.

Signs and Symptoms

Calcinosis

Calcinosis refers to the deposition of calcium salts in the skin and tissues. It can lead to painful nodules and skin ulcerations, particularly on the fingers and other pressure points.

Raynaud's Phenomenon

Raynaud's phenomenon is a condition where the blood vessels in the fingers and toes constrict excessively in response to cold or stress, leading to color changes, pain, and sometimes ulceration.

Esophageal Dysmotility

Esophageal dysmotility involves the abnormal movement of the esophagus, which can cause difficulty swallowing, gastroesophageal reflux disease (GERD), and other digestive issues.

Sclerodactyly

Sclerodactyly is the thickening and tightening of the skin on the fingers and toes. This can lead to decreased mobility and function of the digits.

Telangiectasia

Telangiectasia are small, dilated blood vessels near the surface of the skin or mucous membranes, appearing as red spots or lines.

Causes

CREST syndrome is an autoimmune disease, meaning the body's immune system mistakenly attacks its own tissues. The exact cause is unknown, but it is believed to involve a combination of genetic and environmental factors.

Diagnosis

Diagnosis of CREST syndrome is based on clinical evaluation and the presence of specific antibodies in the blood, such as anticentromere antibodies.

Treatment

Treatment for CREST syndrome focuses on managing symptoms and preventing complications. This may include:

• Medications such as calcium channel blockers for Raynaud's phenomenon and proton pump inhibitors for esophageal symptoms.
• Physical therapy to maintain mobility and function.
• Lifestyle changes such as avoiding cold exposure and smoking cessation.

Prognosis

The prognosis for individuals with CREST syndrome varies. Some may experience mild symptoms, while others may develop serious complications such as pulmonary hypertension.

See Also

• Systemic sclerosis
• Autoimmune disease
• Raynaud's phenomenon

External Links

• Scleroderma Foundation

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