Scleroderma is a type of autoimmune disease. If it involves more than just the skin, doctors refer to as systemic sclerosis (SSc). In cases of SSC the body’s immune system can attack the lungs, kidneys, heart, blood vessels, joints, muscles and gastrointestinal tract.
If an SSc patient experiences shortness of breath and tiredness, he or she might have lung fibrosis or Group 1 PH (pulmonary arterial hypertension, or PAH). In patients with PAH, the arteries become stiff and narrow, making it difficult for the right side of the heart to pump blood through the lungs. Between 8-12% of all SSc patients develop PAH. Because the complication is so common in SSc, all scleroderma patients should be screened for PAH, even if they do not have any symptoms. There are multiple medications for PAH and early diagnosis is vital in order to treat the disease most effectively.
Download the Pulmonary Hypertension Association (PHA)’s free patient brochure to learn more about PH risks and treatment options.