Scleroderma-Associated Pulmonary Arterial Hypertension: Early Detection for Better Outcomes (Archived)

Scleroderma (encompassing localized scleroderma and the more serious form of systemic sclerosis) is a relatively rare autoimmune connective tissue disease (CTD) that predominantly affects women aged 30 to 50 years. It affects between 75,000 and 100,000 people in the US and is more common in people who have family members with other autoimmune CTDs.[American College of Rheumatology. Scleroderma. Fast Facts.] Scleroderma, particularly systemic sclerosis (SSc), is associated with substantial morbidity and mortality. Of great concern is an elevated risk of developing the potentially life-threatening complication of pulmonary arterial hypertension (PAH). PAH is a chronic, progressive type of pulmonary hypertension (PH) characterized by abnormally high pressure in the pulmonary vasculature. This rare cardiovascular disease results from restricted blood flow through the pulmonary arterial circulation. Progression of PAH is characterized by increased pulmonary vascular resistance, vasculature reconstruction, narrowing of the pulmonary arterioles, and right ventricular dysfunction, which ultimately leads to right heart failure and early mortality.[2015 ESC/ERS Guidelines for the diagnosis and treatment of pulmonary hypertension: The Joint Task Force for the Diagnosis and Treatment of Pulmonary Hypertension of the European Society of Cardiology (ESC) and the European Respiratory Society (ERS)] Rates of PAH in patients with SSc are substantially higher than rates in the general population. The overall prevalence of PAH is about 5 to 15 cases per million and is more common in women than men. The leading cause of PAH is CTDs, and particularly SSc. PAH has a prevalence of 7% to 19% in patients with SSc and accounts for about 30% of the mortality in SSc. Originally associated with younger adults aged 35 to 50 years, current data indicates a growing increase in PAH diagnoses among older patients (aged 50 to 65 years), with a closing gender gap. However, owing to nonspecific manifestations (dyspnea, exercise intolerance, and fatigue), patients are typically diagnosed at advanced stages with poorer outcomes. Early screening and diagnosis of PAH followed by prompt initiation of risk-based treatment have been shown to improve outcomes and delay the progression of PAH.[2015 ESC/ERS Guidelines for the diagnosis and treatment of pulmonary hypertension: The Joint Task Force for the Diagnosis and Treatment of Pulmonary Hypertension of the European Society of Cardiology (ESC) and the European Respiratory Society (ERS)] Until the development of current treatment strategies, the average survival for a patient with PAH was 2.8 years.