Systemic Sclerosis Patients May Prevent PAH with Long-Term Tracleer Treatment

A recent study reported that long-term treatment with Tracleer (bosentan) can be beneficial for patients with systemic sclerosis by reducing the risk or delaying the development of pulmonary arterial hypertension (PAH).

Systemic sclerosis disease progression can induce PAH and interstitial lung disease, which leads to poor prognosis.

The study “Beneficial effects of long-term treatment with bosentan on the development of pulmonary arterial hypertension in patients with systemic sclerosis,” was published in the Journal of International Medical Research.

Tracleer is approved by the U.S. Food and Drug Administration (FDA) and European Medicines Agency (EMA) to treat pulmonary hypertension (high blood pressure in the lungs) and to thwart digital ulcer occurrence (usually at the fingertips due to poor circulation).

The drug is an endothelin receptor antagonist (ERA) that is able to block endothelin-1, a compound produced by blood vessel cells. When in excess, endothelin-1 is known to induce tightening of blood vessels which causes many PAH symptoms.

For the study, 69 adult patients diagnosed with systemic sclerosis were monitored for 12 years. The authors reported that 25 patients developed digital ulcers and were treated with Tracleer 62.5mg twice daily for 4 weeks, then 125mg twice a day. The remaining patients who did not develop digital ulcers were included in the control group.

Treatment duration with Tracleer averaged 6.75 years. None of the treated patients developed PAH during the 12-year follow-up period.

Patients who received Tracleer were reported to have an overall better respiratory capability and ability for exercise according to evaluations from the 6-min walking distance test, the Borg dyspnoea index values, and other measurements. Treated patients also showed decreased levels of NT-proBNP, a heart failure risk.

In contrast, the control group showed a degradation of all tested parameters. Within the non-treated systemic sclerosis patient group, seven developed PAH which was confirmed by right heart catheterization.

“Long-term treatment with bosentan improves endothelial function and reduces the risk of PAH development in patients with systemic sclerosis. Early initiation of bosentan treatment may reduce the progression of pulmonary arterial impairment in these patients,” the researchers concluded.