Researchers from the Nippon Medical School in Tokyo published a report in the journal Respiratory Medicine Case Reports highlighting an individual case study of a patient with pulmonary hypertension associated with idiopathic pulmonary fibrosis that had a good overall improvement after treatment with bosentan.
The prevalence of pulmonary hypertension (PH) complicating the course of patients with idiopathic pulmonary fibrosis (IPF) has been reported to be between 32%–85% of the patient population. At the moment there are no available guidelines on how to treat the condition. In this study, researchers Naomi Onda and colleagues reported a case were a patient with idiopathic pulmonary fibrosis had a remarkable improvement in pulmonary hypertension with a drug called bosentan, an endothelin antagonist.
In the study titled “Bosentan for pulmonary hypertension secondary to idiopathic pulmonary fibrosis,” the authors refer to a case of a woman aged 81 years with a diagnosis of idiopathic pulmonary fibrosis that was admitted to the hospital because she had severe dyspnea and decreased oxygen saturation. Furthermore, at clinical assessment, the patient was found to have orthopnoea and edema that was rapidly progressing. Results from the transthoracic echocardiography and from the right heart catheterization revealed a pulmonary artery pressure of a mean 39mmHg and 9mHg mean of pulmonary capillary wedge pressure.
Based on this scenario, the clinicians made the diagnosis of pulmonary hypertension due to idiopathic pulmonary fibrosis plus congestive heart failure secondary to pulmonary hypertension. Doctors immediately started a diuretic therapy, however, there was no improvement in the patient’s overall condition.
The clinicians then tried oral bosentan, and the patient revealed an overall improvement in functioning. After one year of follow-up, there was no worsening of arterial blood gases and there was a good improvement in pulmonary hypertension, World Health Organization class, maximum exercise performance, right heart catheterization and transthoracic echocardiography. In this individual case, Bosentan was effective for pulmonary hypertension associated with the underlying lung disease, and did not cause any adverse effects such as worsening of the patient’s respiratory condition.
Based on these findings the researchers suggest that bosentan might be a successful treatment for pulmonary hypertension in patients with idiopathic pulmonary fibrosis, but that further, large-scale studies are necessary to establish its efficacy.