Pulmonary arterial hypertension (PAH) is a rare but very complex and serious lung disease, which is progressive and potentially fatal. When an individual has PAH, the arteries of their lungs become narrowed and scarred. Those affected by PAH suffer from continuous high blood pressure in the lungs, which results in an enlargement of the heart and almost complete closing of the arteries, which ultimately leads to heart failure and death.
If left untreated, those diagnosed with PAH live an average of only two to three years.
PAH is a common complication of systemic scleroderma (SSc), a chronic hardening and thickening of the skin and internal organs, and can be very severe in patients affected by this progressive connective tissue disease. This type of PAH is known as systemic scleroderma-associated PAH (SSc-PAH).
There is no cure for PAH, including SSc-PAH, and while the prognosis for patients has improved somewhat in recent years, it remains poor despite currently available treatments. The average survival in adults following a PAH diagnosis is estimated at only five to seven years. For patients with SSc-PAH, the average survival in adults following diagnosis is estimated at three years.