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RELATED CONDITIONS: PULMONARY

Pulmonary (lung) involvement in systemic scleroderma can result from multiple factors.

Collagen build-up thickens lung tissue and causes fibrosis or scarring, thus oxygen transport into the bloodstream becomes more difficult. Symptoms include shortness of breath, decreased tolerance for exercise, and a persistent cough. To detect or confirm pulmonary involvement, the physician may order a chest X-ray, a CAT scan of the lungs, an echocardiogram (ultrasound of the heart), or special breathing tests (pulmonary function tests).

Pulmonary fibrosis is a buildup of scar tissue in the lungs that has progressed to the point that the patient’s breathing is affected. 

Pulmonary fibrosis is a type of Interstitial Lung Disease (ILD). The scarring blocks the transfer of oxygen from the lungs to the bloodstream. The physician may give medication to decrease inflammation, which is thought to lead to the scarring.

Many investigations are underway, but there currently are no proven medications to reverse lung damage once it has occurred. 

Therefore, scleroderma patients must take every possible measure to avoid further lung damage. In addition to avoiding smoking, a major cause of lung disease, patients should avoid exposure to air pollutants, which may worsen breathing problems. Your physician may suggest deep breathing exercises, a graduated aerobic exercise program, or medications to make breathing easier.

Pulmonary hypertension (PH) is high blood pressure in the blood vessels of the lungs. 

Pulmonary arterial hypertension (PAH) is pulmonary hypertension caused by the narrowing of pulmonary arteries. When the pulmonary vessels have high blood pressure, the right side of the heart pumps harder to move blood into the lungs and pick up oxygen; this can lead to heart failure on the right side of the heart.

People with scleroderma have an increased likelihood for developing PH from several mechanisms, and they often have multiple causes of their PH. 
Those with PAH may be given special medications that dilate or open up the pulmonary vessels, possibly changing the disease’s underlying nature. 

This is one part of scleroderma for which recent medications have proven successful; now, treatment possibilities include three different classes of medications.