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
- 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.
- Small, round, red lesions called
- Difficulty swallowing
- Shortness of breath and wheezing
- Chest pain on deep
- Mild anemia is present.
- Urinalysis (urine
test) may show red blood cells.
- Pulmonary function tests may show a restrictive pattern of lung
- Esophageal manometry may show
abnormalities in esophageal movement
- Chest X-Ray may show fibrotic
- Elevated sedimentation rate and
hypergammaglobinemia are present
- ANA may be positive
- Rheumatoid factor may
- 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
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
- It has been questioned whether silicon breast implants are associated with Scleroderma. Current
medical studies have not shown a connection.
- If you have silicon breast implants and any symptoms of Scleroderma, consult
a physician or rheumatologist.
Eosinophilia-myalgia syndrome, which is seen in tryptophan toxicity (an old over-the-counter sleep remedy).
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