Spirometric Plus Left Ventricular and Pulmonary Blood Flow Echocardiographic Findings in Patients with Scleroderma before and after Sildenafil

Background: Systemic sclerosis (scleroderma) is a chronic debilitating disease that is caused by the occurrence of fibrotic changes and vascular abnormalities at various levels such as: Skin, lungs, kidneys or heart. Lung involvement in scleroderma is one of the leading causes of mortality in this disease. Sildenafil inhibits phosphodiesterase type 5, an enzyme that metabolizes cyclic guanosine monophosphate, thereby enhancing the cyclic guanosine monophosphate-mediated relaxation and growth inhibition of vascular smooth-muscle cells, including those in the lung. We hypothesized that chronic oral administration of the phosphodiesterase-5 inhibitor sildenafil could improve the pulmonary haemodynamics in Systemic sclerosis (Ssc) patients.