CREST syndrome is a subtype of limited scleroderma, an autoimmune disease that affects connective tissue and can involve the skin, blood vessels, muscles and internal organs. CREST is an acronym that refers to the main symptoms of the disease: calcinosis, Raynaud's phenomenon, esophageal dysmotility, sclerodactyly and telangiectasias.
CREST syndrome is a rare form of scleroderma, accounting for approximately 20% of all cases of scleroderma. It predominantly affects middle-aged, Caucasian women.
The exact cause of CREST syndrome is not known, but it is thought to be due to a combination of genetic and environmental factors. Several genes associated with scleroderma have been identified, including HLA genes and genes involved in the regulation of the immune system. It is believed that exposure to certain environmental factors, such as viral or bacterial infections and chemical exposure, may trigger the disease in genetically susceptible individuals.
CREST syndrome is characterized by the presence of the five main symptoms, which are:
- Calcinosis: calcium deposits in the skin or underlying tissues.
- Raynaud's phenomenon: spasms of blood vessels in the hands and feet in response to cold or emotional stress.
- Esophageal dysmotility: difficulty in swallowing or regurgitation of food.
- Sclerodactyly: thickening and hardening of the skin of the fingers.
- Telangiectasias: small dilated veins visible on the surface of the skin.
In addition to these symptoms, patients with CREST syndrome may also experience other symptoms such as joint pain, fatigue, muscle weakness, shortness of breath and heart rhythm disorders.
The diagnosis of CREST syndrome is based on the presence of the five main symptoms. The physician may perform additional tests, such as blood tests, pulmonary function tests, x-rays and echocardiograms, to assess the severity of the disease and to rule out other conditions.
Treatment of CREST syndrome focuses on controlling symptoms and preventing or treating complications associated with the disease. Treatment should be individualized according to the severity and extent of the disease in each patient. Some treatment options include:
- Medications to treat the symptoms of the disease, such as analgesics and non-steroidal anti-inflammatory drugs (NSAIDs), to reduce pain and inflammation.
- Medications to control pulmonary hypertension, if present.
- Immunosuppressive drugs, such as methotrexate, cyclophosphamide and azathioprine, to reduce immune activity and inflammation in the skin and other affected organs.
- Topical treatments for cutaneous scleroderma, such as urea and lactic acid creams, to soften the skin and reduce thickness and stiffness.
- Treatment of skin and digital ulcers, to prevent infections and promote healing.
- Physical therapy to maintain joint mobility and muscle strength and prevent stiffness.
- Occupational therapy to help people adapt to physical limitations and improve quality of life.
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