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Lung Involvement

Multiple factors can cause pulmonary (lung) involvement in systemic scleroderma. Build-up of collagen thickens lung tissue and causes fibrosis or scarring, making the transport of oxygen into the bloodstream more difficult.

Symptoms of lung involvement include shortness of breath, a decreased tolerance for exercise and a persistent cough. The physician may order a chest X-ray, an echocardiogram (ultrasound of the heart), special breathing tests (pulmonary function tests) or a CAT scan of the lungs to detect or confirm lung involvement.

Pulmonary fibrosis, a type of Interstitial Lung Disease (ILD), is a buildup of scar tissue in the lungs that progresses to a point that a person’s breathing is affected. The scarring blocks the lungs ability to transfer oxygen into the blood stream. Medications may be given to decrease the inflammation which is thought to lead to lung scarring.

Although many investigations are under way, there currently are no proven medications to reverse lung changes once they have occurred. It is important, therefore, for the person with scleroderma to take whatever measures are within his or her control to avoid further damage to the lungs. It is essential to avoid smoking, a major cause of lung disease. Exposure to air pollutants may worsen breathing problems and should be avoided to the extent possible. Your doctor may recommend medications to make breathing easier and may also suggest deep breathing exercises and a graduated aerobic exercise program.

Pulmonary hypertension (PH) is high blood pressure in the blood vessels of the lungs. If the high blood pressure in the lungs is due to narrowing of the pulmonary arteries, it is known as pulmonary arterial hypertension (PAH). When the blood pressure inside the pulmonary vessels is high, the right side of the heart has to pump harder to move blood into the lungs to pick up oxygen. This can lead to failure of the right side of the heart. Individuals with scleroderma are at increased risk for developing PH from several mechanisms. Frequently individuals with scleroderma have multiple causes of their PH.

People with pulmonary arterial hypertension may be treated with special medications targeted at dilating or opening up the blood vessels of the lungs, and possibly changing the underlying nature of the disease. This is one complication of scleroderma for which new medications have proven successful; there are now three different classes of medications which can be used.


Heart Involvement

If the heart muscle becomes thickened and fibrous scar tissue accumulates, the force of heart contractions may be decreased, which may ultimately result in heart failure. Spasm of the coronary arteries (the main blood vessels to the heart), may cause chest pain and, rarely, lead to a heart attack. The spasm appears similar to that involving the fingers in Raynaud Phenomenon. Inflammation of the outer heart lining (pericarditis) may cause pain and accumulation of fluid around the heart. An irregular heartbeat may also occur. These conditions require careful evaluation and treatment by the physician.


American Thoracic Society: Interstitial Lung Disease and Pulmonary Hypertension Webinar >>