Pulmonary Hypertension Associated with Pulmonary Fibrosis: A Case of Systemic Sclerosis Successfully Treated with Bosentan

Abstract

We report a case of 50-year-old female with an 11-year history of systemic sclerosis that developed worsening dyspnea due to long standing pulmonary fibrosis (PF) and pulmonary hypertension associated with PF. In addition, she had severe esophageal dysfunction and refractory digital ulcers. Her previous treatment regimens had included corticosteroids, beraprost, and oxygen therapy. In September of 2005, a Doppler echcardiogram demonstrated an elevated right ventricular pressure (RVP) of 54 mmHg and her 6-min walk distance was 342 m. We concluded that her pulmonary hypertension was progressing and started bosentan therapy. After 4 weeks of treatment with bosentan (62.5 mg twice daily), the patient’s clinical symptoms and laboratory findings improved significantly: her RVP (estimated by Doppler echocardiogram) had decreased to 30 mmHg and her 6-min walk distance had increased to 389 m. Thus, pulmonary hypertension associated with PF was successfully treated with bosentan in our patient.