Funding outcome

Scleroderma is a devastating life-threatening disease that primarily afflicts women and causes inflammation and blood vessel abnormalities. Patients with scleroderma are at a high risk of developing pulmonary arterial hypertension (i.e., increased blood pressure in the lungs) - a known lethal complication. Early in the disease, many patients report exertional breathlessness and an inability to exercise. Importantly, while these symptoms have a large toll on quality of life, little is known about the causes of breathlessness and poor exercise capacity in patients with scleroderma. Furthermore, it is well established that in healthy adults, sex-related differences lung anatomy predisposes women to having greater exertional breathlessness than men. While it is possible that scleroderma may heighten breathlessness, and consequently exercise intolerance in women, no study has directly evaluated this phenomenon or its underlying mechanisms. As such, the proposed project will conduct precise measurements of how the heart and the lungs affect the delivery and utilization of oxygen at rest and during exercise in women with and without scleroderma to determine the causes and extent of breathlessness and poor exercise capacity in this patient population. The information obtained from this study may inform future therapeutic targets to prevent the progression of scleroderma, ultimately resulting in better care for women with this chronic condition.