CREST Syndrome

Scleroderma or progressive systemic sclerosis

Scleroderma is an autoimmune disease (body's immune systems mistakenly attacks itself) characterized by diffuse thickening of the skin and internal organs. The cause remains unknown.Scleroderma may be localized, only affecting the skin. The disease can also be systemic, affecting the internal organs.

There are two types of systemic Scleroderma, a limited disease called CREST syndrome (80 percent of cases) and diffuse disease (20 percent of cases). CREST is an acronym for Calcinosis (deposition of lime salts in the tissues), Raynaud's Phenomenon, esophageal involvement, sclerodactyly (hardening of the finger tips or toes), and Telangiectasia. In CREST syndrome, hardening of the skin is limited to the hands and face.

Death from Scleroderma is commonly caused by kidney, heart, or lung disease. The prognosis for CREST syndrome is more positive.Scleroderma is seen between the ages of 30 to 50, and is more prevalent among women.

Skin tightening and thickening is the hallmark symptom

Swollen fingers and toes

Joint pain and stiffness

Hands may turn white when exposed to cold in Raynaud's Phenomenon.

Fever

Fatigue

Swelling

Small, round, red lesions called telangiectasias

Difficulty swallowing

Shortness of breath and wheezing

Chest pain on deep breath

Mild anemia is present.

Urinalysis (urine test) may show red blood cells.

Pulmonary function tests may show a restrictive pattern of lung disease

Esophageal manometry may show abnormalities in esophageal movement

Chest X-Ray may show fibrotic changes.

Elevated sedimentation rate and hypergammaglobinemia are present

ANA may be positive

Rheumatoid factor may be elevated

ACE levels, LE cell test, and cold agglutinins may be altered

Scleroderma anti-body (SCL-70) is positive in up to 30 percent of the patients

Anticentromere antibody is positive in 50 percent of the patients

Currently, there is no cure for this disease. However, there are treatments that exist to alleviate the symptoms.
Treatment of Symptoms:

For Raynaud's Phenomenon, Nifedipine or other Calcium channel blockers may be prescribed.
For sores on the fingers and toes, Iloprost given intravenously may be helpful.
For esophageal Reflux, head elevation, antacid, H2 blockers, proton pump inhibitors may be helpful.
For bacterial overgrowth, the antibiotic Tetracycline may be prescribed.
For hypertensive crisis, ACE inhibitors may be prescribed.
Penicilliamine is prescribed for rapidly progressing systemic Scleroderma.
The medication Cyclophosphamide may improve severe interstitial lung disease.
Support groups for Scleroderma help patients and family better understand how to cope with this disease.