Bosentan Improves Pulmonary Hypertension Secondary to IPF in Individual Case Study

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antidepressants and iPAHA study published in the journal Respiratory Medicine Case Reports revealed a case where a patient with idiopathic pulmonary fibrosis (IPF) was successfully treated for severe pulmonary hypertension (PH) with bosentan. The study is entitled “Bosentan for pulmonary hypertension secondary to idiopathic pulmonary fibrosis.”

Bosentan is a drug prescribed in the treatment of pulmonary arterial hypertension. It is an endothelin receptor antagonist that works to decrease the blood pressure in the lungs. Bosentan is usually prescribed for the purpose of improving a PH patient’s exercise capacity, and decreasing gradual clinical deterioration.

The study reports a case of an 81-year-old woman suffering from IPF. She was admitted to a hospital in Japan because of aggravated shortness of breath (dyspnea), low levels of oxygen saturation, orthopnea (an abnormal condition in which the individual is forced to sit or stand in an erect position in order to breathe comfortably) and progressing edema (accumulation of fluids). After several exams, the patient was diagnosed with PH secondary to her IPF, and PH-related congestive heart failure. The patient was submitted to diuretic therapy, but it proved to be unsuccessful; subsequently, she started treatment with oral bosentan at 125 mg/day and it remarkably resulted in a rapid improvement of pulmonary circulation.

One year later, as part of a follow-up evaluation, physicians found that the pulmonary function of the patient was maintained, PH had improved, and the patient’s exercise capacity was significantly enhanced. Bosentan, therefore, presents a promising effective therapy for PH secondary to IPF.

In other developments in pulmonary hypertension, a recent study showed a “paradoxical” decrease in morbidity and mortality with increasing body mass index (BMI) in patients with heart failure. However, the impact of BMI in patients with idiopathic pulmonary arterial hypertension (IPAH) remains poorly understood. This study revealed that the overweight and obese group had a higher survival rate compared to the normal weight and underweight groups. In addition, the researchers found that a lower BMI, higher functioning, lower cardiac index, smaller LVEDDs and absence of targeted medication were independent predictors of mortality. Based on these findings, the research group concluded that an excess in body mass might be a protective factor against death in patients with IPAH.


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Patrícia holds her PhD in Medical Microbiology and Infectious Diseases from the Leiden University Medical Center in Leiden, The Netherlands. She has studied Applied Biology at Universidade do Minho and was a postdoctoral research fellow at Instituto de Medicina Molecular in Lisbon, Portugal. Her work has been focused on molecular genetic traits of infectious agents such as viruses and parasites.
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