Soliris(R) Reduced Measures of Thrombosis and Inflammation, and Decreased Indicators of Pulmonary Hypertension, in Studies of Patient...( Bradford United Kingdom. Using...)

Using data from the Phase III TRIUMPH study of Soliris, Dr. Hill and her colleagues evaluated the efficacy of Soliris in the regulation of cell-free plasma hemoglobin levels, nitric oxide depletion and subsequent cardiovascular morbidities in patients with PNH. This analysis found that 47 percent of patients with PNH (34 of 73) suffered from pulmonary hypertension before starting the trial. In this study, PAH was measured by an elevated blood level of NT-proBNP, which has been shown to be highly predictive of PAH and an independent predictor of mortality in other hemolytic diseases. (2) At the start of the study, levels of hemolysis and nitric oxide consumption were shown to be much greater in PNH (more than 6- and 10-fold, respectively) than in patients with other hemolytic diseases.

Patients treated with Soliris experienced a 50 percent reduction in the incidence of PAH over the course of the 26-week treatment period, from 52.5 percent to 26.3 percent, while PAH did not change with placebo (39.4% to 43.8%) (P<0.001). Additionally, Soliris-treated PNH patients experienced significantly improved shortness of breath compared to placebo, as measured by the EORTC QLQ-C30 quality of life survey (P<0.001).

"A careful analysis of blood levels in PNH patients shows that hemolysis, the red blood cell destruction that defines the disease, consumes nitric oxide in the blood which is likely to result in an increase in cardiovascular complications as shown in other hemolytic diseases," said Dr. Hill. "This study confirms that PAH is common in hemolytic PNH patients and also suggests that the anti-hemolytic effect of Soliris treatment significantly increases nitric oxide and consequently reduces pulmonary hypertension in patients with PNH."

Pulmonary artery hypertension is a rare, progressive disorder characterized by high blood pressure (hypertension) of the pulmon
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