Scleroderma

Scleroderma is a disease that affects the skin and internal organs. The literal meaning of the word is "hard skin"; people with Scleroderma experience a hardening and tightening of their skin. The inflammation and scarring associated with Scleroderma can cause damage to the lungs, and it may lead to a condition called pulmonary arterial hypertension (PAH).

Pulmonary hypertension is a state of high blood pressure in the lungs, and according to the University of Michigan Health System, close to half of people who have Scleroderma face PAH as well.

The most common symptom of PAH is shortness of breath with physical activity. Fainting or near fainting with physical activity may occur as well as other symptoms such as fluid retention and chest discomfort. The diagnosis of PAH is often overlooked by the community physician. It is sufficiently common and of such high impact that all patients with Scleroderma should be screened for its presence on a regular basis.

As with many features of Scleroderma, the basic underlying cause of PAH is the progressive scarring of the inner lining of the small artery. The changes in the lung blood vessels look remarkably similar to those in the fingers, kidneys and gastrointestinal tract. Many in the international research community think of Scleroderma as a blood vessel narrowing disease and view the immune system activation and tissue scarring as secondary events.

Treating PAH involves medication and monitoring of organ function, and can be pursued as outlined below.

Step 1:
Treat pulmonary hypertension associated with Scleroderma with a class of medication called vasodilators. Medications of this kind are used to open blood vessels and can lead to a lowering of the blood pressure. Some drugs used to treat PAH may be given intravenously at a medical facility.

Step 2:
Prevent blood clots in the lungs, one of the dangers of scleroderma-related pulmonary hypertension, with blood thinners and diuretics. Blood thinning medications prevent the blood cells from sticking together. Diuretics promote urination and may also prevent the clotting of the blood.

Step 3:
Block the body's production of endothelin with a medication called bosentan. The Scleroderma Foundation explains that people with PAH have a higher-than-normal level of endothelin in their bodies. Endothelin causes the blood vessels to constrict. Bosentan is a drug specifically approved to treat pulmonary hypertension, but it should not be used by pregnant women due to the risk of birth defects.

Step 4:
Monitor liver function while taking medications to treat PAH. An average of 11 percent of Scleroderma patients show abnormal liver function while undergoing treatment for PAH. Liver function should be tested before a drug regimen begins and on a regular basis during the course of treatment.

For further information on PAH, visit the University of Michigan Health System's page here, and the Scleroderma Foundation's page here.