Royal Adelaide Hospital Release: Bosentan Improves Quality Of Life And Prognosis For Patients With Pulmonary Arterial Hypertension

ADELAIDE, Australia, Oct. 21 /PRNewswire-FirstCall/ -- Results showing that the use of bosentan (Tracleer(R)) in the treatment of pulmonary arterial hypertension (PAH) related to connective tissue diseases significantly improves patient quality of life (QoL)(1). These data were presented today at the annual scientific meeting of the American College of Rheumatology (ACR) in San Antonio, Texas.

PAH, a life-threatening disease is a serious complication of connective tissue diseases like scleroderma (Ssc) and causes marked reduction in a patient's ability to function and overall survival. In PAH associated with scleroderma (PAH-Ssc), before the approval of PAH treatments, the median survival of patients has been reported to be only one year following diagnosis.(2)

The VITAL study(1) (Quality of LiVes Improved with BosenTan in Australian Open Label Study), was an Australian multi-centre, open label, single-arm trial, designed to collect further data on the safety and efficacy of bosentan therapy in patients with PAH (idiopathic and related to connective tissue disease) and to assess changes in QoL associated with bosentan.(1) When the study began in 2001, there were no PAH-specific therapies available in Australia, consequently this study also provided treatment access to PAH patients in need. Today, the VITAL study is the largest study in a clinical setting to assess the impact of quality of life in PAH patients.

Study Results

177 patients PAH (class III or IV), treated with bosentan (62.5 mg BID for 1 month, then 125mg BID), were recruited in the study of which 62 (35%) had PAH-Ssc (11 or 6% with PAH related to systemic lupus and 104 or 59% with idiopathic PAH). QoL measures were collected utilising the SF-36 questionnaire, the most widely used scale in health surveys. Results showed that PAH-SSc patients treated with bosentan significantly improved at 3 months compared to baseline measures, particularly in terms of physical function (p<0.0001), social function (p=0.002), vitality (P=0.02), emotional role (p=0.03) and physical role (p<0.0001) and that the results were maintained or improved out to 6 months(1). Patients also reported significantly improved general health compared with 12 months earlier (p <0.001)(1). Furthermore, at the end of the study 81% of the PAH-Ssc patients treated with bosentan improved or remained stable in terms of their baseline WHO functional class.

"The results from the VITAL study reveal the positive impact bosentan can have on the lives of PAH patients, with regards to both their physical and mental well-being," explained Dr Susanna Proudman, Senior Visiting Rheumatologist at the Royal Adelaide Hospital in Adelaide, Australia who presented the data on behalf of the V.I.T.A.L. study investigators. "In a disease such as PAH, prolonging a patient's life is positive but prolonging life with an improved quality of life is vital to improving prognosis over the long-term."

Endothelin has been shown to have a fundamental role in the pathophysiology of PAH and to correlate with disease severity and outcome. Bosentan, an oral, dual endothelin receptor antagonist, blocks the deleterious effects (inflammation, hypertrophy, fibrosis and vasoconstriction) of endothelin, and has been shown to improve exercise capacity and haemodynamics and delays clinical worsening in patients with PAH. Bosentan was safe and well tolerated in the VITAL study and was approved in Australia in March 2004.

In PAH, diagnosis can often be significantly delayed because initial symptoms such as breathlessness are unspecific, and therefore go undetected or are attributed to other diseases. By the time a diagnosis is made, patients have often experienced a decline in their ability to function normally in simple daily tasks such getting dressed or walking stairs. Without treatment, this deterioration may continue rapidly, and may lead to death within two to three years of diagnosis. Consequently early detection of PAH and intervention with effective therapy may improve the prognosis for these patients.

References (1) Proudman, S. (Tracleer (bosentan), a Dual Endothelin Receptor Antagonist (ERA), for the Treatment of Pulmonary Arterial Hypertension (PAH) related to Connecttive Tissue Diseases: Effect on Quality of Life (QoL). Presented at the American College of Rheumatology, October 21, 2004. Abstract #1853. (2) Koh ET, et al. Br J Rheumatol 1996; 35:989-93 and Kawut SM, et al. Chest 2003; 123:344-50.

CONTACT: John Zollo, Royal Adelaide Hospital, +61-8-8222-5671,jZollo@mail.rah.sa.gov.au; or Lucy Heaton or Jim Baxter, both of Cohn & Wolfe,+44-20-7331-5371, for Royal Adelaide Hospital