Systemic sclerosis (scleroderma, progressive soft tissue hardening)

Description Systemic sclerosis ...

Description

Systemic sclerosis (also called scleroderma) is a rare disease in which scar tissue deposits in the skin and many internal organs. Scleroderma can affect only the skin or involve other parts of the body. This disorder is thought to be an autoimmune disease. This means the immune system mistakenly attacks certain organs of the body. The disease can be serious leading not only to scarring of the skin but also to damage of the esophagus, heart, lungs and kidneys. Scleroderma is most frequently diagnosed in people between the ages of 30 and 50 and is more common among women.

Symptoms

Color changes in the fingers after exposure to cold (Raynaud's phenomenon), thickening of the skin, weakness, fatigue, joint pains, shortness of breath, kidney failure.

Tests

Common tests used for diagnosis and treatment

Workup:
A history and physical exam will be performed. Blood tests and imaging tests (such as a chest x-ray or CT scan) may be performed to help establish the diagnosis.

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

Other Specific Tests: RF (Rhematoid factor), ANA (Antinuclear antibody), Anti-DNA antibodies, ESR (erythrocyte sedimentation rate)

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

Treatment

The goals of treatment are to reduce symptoms and to prevent and treat complications. Medications to dilate (open up) blood vessels, lower blood pressure, reduce inflammation and suppress the immune system may be recommended. More aggressive treatments such as bone marrow transplantation are being studied.