Pulmonary Hypertension and Scleroderma

Scleroderma can cause scar tissue in the lungs and thickening in the walls of blood vessels that carry blood to and from the heart to the lungs. When the blood is less able to flow smoothly it can lead to pulmonary arterial hypertension (PAH) which is a life-threatening condition. PAH is a leading cause of disability and death among these patients with systemic sclerosis. It occurs in 8%-17% of patients and accounts for 30% of deaths because PAH symptoms are often not recognized early.

Part of the problem is that there are few very early symptoms of PAH. These are often mistaken for a general lack of fitness. Regular screening is very important, because people with scleroderma are at high risk for PAH.

Symptoms that develop are often typical of other conditions (such as asthma). This can cause a further delay in diagnosis. Patients may experience:

• Shortness of breath during normal activities
• Tiredness or fatigue
• Chest pain
• Rapid heartbeat
• Pain in the upper right side of the abdomen
• Decreased appetite
• Swelling of feet, ankles, legs
• Fainting, dizziness, light-headedness
• A bluish color in the lips and skin

Treatments for Pulmonary Arterial Hypertension (PAH)

Treatment for PAH in scleroderma is more effective if it is identified in earlier stages. Sadly, patients rarely report early symptoms such as shortness of breath and fatigue. They may be severely affected by the time their PAH is diagnosed.

Your doctor should be monitoring you regularly to check on the health of your heart and lungs. It is important to have regular screening for heart and lung function. Pulmonary function tests, echocardiography, chest X-rays, or CT scans can help identify problems. You may be referred to a pulmonary physician for evaluation and treatment recommendations.

There is no cure for PAH, but treatment can help to relieve symptoms, improve function, and slow the progression of PAH in scleroderma. Endothelial cells have many functions in our blood vessels. Endothelial cell dysfunction was discovered to be a key trigger for the development of vascular damage in systemic sclerosis. PAH is a major consequence of this damage. This discovery led to development of several treatment options over the past 20 years.

If you do develop PAH, your doctor can recommend a treatment that suits you. Medications for scleroderma-related PAH may be injected, inhaled, or taken by mouth. The goal of these PAH treatments is to relax the blood vessels in the lungs and reduce excess cell growth, so that blood can flow more easily.