Systemic sclerosis (SS) is an uncommon disease characterized by small blood vessel vasculopathy and increased connective tissue in the skin and in other organs. The pulmonary involvement is common in SS; however, pulmonary hypertension without interstitial fibrosis is rare. The authors present a case of the diffuse form of SS with isolated pulmonary hypertension. The diagnosis of pulmonary hypertension was suggested by physical examination and confirmed by doppler-echocardiography which allowed for the estimation of the pulmonary artery systolic pressure in 80 mmHg. The authors started treatment with nifedipine; however, as the patient presented side effects, it was not possible to increase the dose beyond 30 mg/day. In this dosage, nifedipine did not decrease the pulmonary arterial pressure.

Keywords: Systemic scleroderma. Nifedipine. Pulmonary hypertension. Vasodilatador agents. Doppler echocardiography.