What is CREST variant of scleroderma?

A less severe form of scleroderma is called limited scleroderma, or CREST syndrome. CREST syndrome is characterized by: Calcinosis: Calcium skin deposits. Raynaud’s phenomenon: A condition in which the blood vessels of the fingers and toes spasm when triggered by factors, such as cold, stress or illness.

What is the life expectancy of someone with CREST?

What Is the Life Expectancy for CREST Syndrome? The survival rate for of CREST syndrome (limited scleroderma) from time of diagnosis is estimated to be as follows: 77.9% at 5 years. 55.1% at 10 years.

What causes CREST syndrome?

Exposure to silica, synthetic adhesives, solvents (including chlorinated solvents, aromatic solvents, white spirit, toluene, trichloroethylene, formaldehyde, vinyl chloride, and cleaning products) have been implicated in a higher risk of developing systemic sclerosis.

Does CREST syndrome affect the brain?

The pathogenesis of cerebral aneurysms associated with collagen diseases, including CREST syndrome, remains unclear. Early treatment of CREST syndrome and other collagen diseases may prevent arteritis from progressing to affect the intracranial arteries and thus reduce the occurrence of aneurysms.

Is scleroderma considered a terminal illness?

It is the most fatal of all the rheumatologic diseases. Systemic scleroderma is very unpredictable although most cases can be classified into one of four different general patterns of disease (see Classification).

What is the life expectancy for someone with scleroderma?

In general, patients with limited scleroderma have a normal life expectancy. Some have problems with their GI tract, especially heartburn; severe Raynaud’s and musculoskeletal pain; and a small subset can develop pulmonary hypertension that can be life-threatening.

Is scleroderma a death sentence?

Scleroderma is “not automatically a death sentence,” says Dr. Chatterjee. A milder form of the disease does not necessarily shorten one’s normal life span. Even if you have a more severe form involving major organs, treatments can manage most symptoms.

What is the life expectancy of people with scleroderma?

People who have localized scleroderma may live an uninterrupted life with only minor symptom experiences and management. On the other hand, those diagnosed with an advanced and systemic version of the disease have a prognosis of anywhere from three to 15 years.

Is CREST syndrome life-threatening?

Without intervention, it may become serious and life-threatening. There is also a form of scleroderma that sits between localized and diffuse. Known as CREST syndrome, or limited scleroderma, this condition does not affect the kidneys.

What environmental triggers cause scleroderma?

Environmental exposure: Some environmental influences can put people at greater risk for developing scleroderma. Men exposed to silica appear to have a higher risk for developing scleroderma. Being around certain solvents and taking certain drugs can also increase your potential for developing the disease.