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Scleroderma (progressive hardening of soft tissue)

Description A rare disease that...


Description

A rare disease that causes the skin and underlying tissue to become hard and inelastic. Scleroderma can affect only the skin or involve other parts of the body. This disorder is felt to be caused by an over active immune system which attacks the body (autoimmune disease). The disease can be very serious leading to fibrous scarring of the esophagus, heart, lungs and kidneys, and carries an average 12 year survival rate from the time of diagnosis. Scleroderma is most often found in patients 30-50 years of age and is more common in women.

Symptoms

Pain in the skin, weakness, decreased urination, fatigue, muscle wasting, intolerance to cold, joint pains, shortness of breath, kidney failure, problems swallowing, abnormal heart beats, palpitations.

Tests

Common tests used for diagnosis and treatment

Workup:
A history and physical exam will be done. Lab tests to measure the immune system response of the patient are usually done. Other tests that identify damage to other body parts include: EKG, blood tests for the kidneys (electrolytes, chem 12), esophagogastroduodenoscopy (EGD), lung function test (pulmonary function test), CT scan, heart ultrasound (echocardiogram), heart catheterization, and bronchoscopy.

Tests:
Antinuclear antibody (ANA), Complete blood count (CBC), Comprehensive metabolic panel (CMP), Rheumatoid factor (RF), Urinalysis (UA), X-ray

Specialists:
Internal Medicine, Rheumatology, Family Practice, Pediatric Rheumatology

Treatment

The goals of treatment are to reduce the symptoms. Medications to soften the skin and reduce the inflammation are used. Applying heat to the affected area can help selected patients. More aggressive treatments such as bone marrow transplant are being studied.
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Mediref: Scleroderma (progressive hardening of soft tissue)
Scleroderma (progressive hardening of soft tissue)
Mediref
https://mediref.blogspot.com/2018/08/scleroderma-progressive-hardening-of.html
https://mediref.blogspot.com/
https://mediref.blogspot.com/
https://mediref.blogspot.com/2018/08/scleroderma-progressive-hardening-of.html
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