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Published on: 8/18/2008
Last Visited: 10/5/2008
If this thickening affects the lungs, they can no longer function," explains Carol Feghali-Bostwick, PhD, an assistant professor of medicine in the division of pulmonary, allergy, and critical care medicine at the Simmons Center for Interstitial Lung Disease at the University of Pittsburgh and vice chair of the board of directors of the Scleroderma Foundation.
There are two types of scleroderma: systemic and localized.Systemic scleroderma is more serious and includes diagnoses of diffuse and limited cutaneous forms.It is more common in adults, presenting between the ages of 20 and 50, and approximately four times more common in women."For women, this is right in their childbearing years, and there is a theory that hormones may play a role in the susceptibility to scleroderma.But this is an area where further research is necessary," says Feghali-Bostwick.
Localized scleroderma tends to be less severe.Its forms, including morphea and linear diagnoses, are more common in children and rarely progress to the systemic stage in adulthood, says Feghali-Bostwick.
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In patients with diffuse systemic disease, the progression can be very quick, but in those patients where the disease is caught early enough, it can be managed accordingly and, with an effective symptom management plan, some patients can enjoy a typical life expectancy, explains Feghali-Bostwick.
Diagnosing systemic scleroderma is difficult because of the overlap in symptoms found in more common ailments, including gastrointestinal (GI) reflux disease, GI discomforts such as diarrhea and/or constipation, arthritis, carpal tunnel syndrome, stiffness and pain in joints and tendons, shortness of breath, kidney problems, pulmonary fibrosis, pulmonary hypertension, and Sjögren's syndrome (a chronic disease in which white blood cells attack moisture-producing glands in the body).
However, one of the most common and somewhat telling signs of systemic scleroderma is Raynaud's phenomenon, a condition in which the fingers or toes change color due to the narrowing of blood vessels in the hands and feet."The hand color can be biphasic or triphasic, and the hands become very cold.Other parts of the body that are exposed to the cold can experience Raynaud's phenomenon such as the feet and nose," says Feghali-Bostwick.
The first and most important step to determine if a patient has scleroderma is by performing a thorough physical, including a skin examination to determine its elasticity."In using a skin scoring system, the physician pinches various parts of the patient's body to test for thickness.The less skin that can be pinched equals a higher score in diagnosing scleroderma," says Feghali-Bostwick.
In addition to the physical exam, certain tests can be ordered to determine how much the internal organs have been affected and if the patient has any of the antibodies associated with scleroderma."Currently, there are nine antibodies common to scleroderma that we can test for," says Feghali-Bostwick.
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However, we only have, at best, a 10- to 15-year history on these patients," says Feghali-Bostwick.Most deaths following a transplant are due to transplant complications.Meanwhile, to date, patients who survive do not seem to have any symptoms return to the transplanted lung, she adds.
Scleroderma is not a cancer, nor is it contagious."It is likely a multigenic disease with environmental components serving as triggers," explains Feghali-Bostwick.Some studies have suggested there is an increased risk of developing the disease if a first-degree relative has been afflicted.However, other research disputes that theory."Studies looking at identical twins have found that it is not common for both twins who inherit the same genetic background to develop scleroderma," she says.
One environmental factor that appears to influence the development of the disease and trigger its development in genetically susceptible patients is trichlorethylene, a colorless, poisonous liquid used as an industrial solvent."People who are exposed to trichloroethylene, such as dry cleaners, are at an increased risk of developing sclerodermalike disease., There are several other triggers of sclerodermalike disease.For example, patients who receive the chemotherapy drug bleomycin can develop lung fibrosis similar to that seen in scleroderma patients," says Feghali-Bostwick.
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