Advanced lung disease—medical aspectLong-Term Outcome of Bosentan Treatment in Idiopathic Pulmonary Arterial Hypertension and Pulmonary Arterial Hypertension Associated with the Scleroderma Spectrum of Diseases
Section snippets
Methods
This project was reviewed by the Johns Hopkins University institutional review board and determined to meet criteria for exempt research. We reviewed our pulmonary hypertension clinical database to identify all patients with the following:
- 1
a diagnosis of either idiopathic PAH (IPAH), formerly known as primary pulmonary hypertension,6 or APAH-SSD;
- 2
the absence in patients with APAH-SSD of significant interstitial lung disease defined as a total lung capacity of 70% or more of predicted, or between
Subjects
We identified 19 IPAH and 17 APAH-SSD consecutive subjects who met the study inclusion criteria. Table 1 summarizes their demographic and baseline clinical characteristics. All variables were similar between the 2 groups with the exception of a trend for a lower diffusion capacity of the lung for carbon monoxide in the APAH-SSD group (p = 0.08). One patient, originally thought to have IPAH and treated with bosentan as initial therapy, was later shown to have had pulmonary veno-occlusive disease
Discussion
We believe this study is the first report comparing the long-term outcomes of bosentan treatment in patients with IPAH and APAH-SSD. Although a large proportion in both groups (37% and 47%, respectively) failed bosentan as single-agent therapy during the mean follow-up period of 20 months owing to a lack of clinical efficacy or hepatotoxicity, functional class improved in 58% of IPAH patients compared with only 25% of APAH-SSD patients. WHO class was significantly decreased in the IPAH group,
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Pharmacological treatment of systemic sclerosis-associated pulmonary hypertension: A systematic literature review
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2016, Autoimmunity ReviewsCitation Excerpt :Endothelin receptor antagonists such as bosentan and ambrisentan are associated with improved exercise capacity, hemodynamics and time to clinical worsening in idiopathic-PAH. The efficacy of bosentan in SSc patients seems to be less important, with poorer survival rates compared to I-PAH patients [82]. In the SERAPHIN study, macitentan was shown to reduce a composite endpoint that included death, atrial septostomy, lung transplantation, initiation of intravenous prostanoids and worsening of PAH [83].
Inhibition of vascular endothelial growth factor receptor under hypoxia causes severe, human-like pulmonary arterial hypertension in mice: Potential roles of interleukin-6 and endothelin
2014, Life SciencesCitation Excerpt :This relative inefficacy highlights a strong demand in animal modeling with recapitulative pathophysiological changes of severe PAH. Although the prognosis of patients with inflammation-associated PAH (i.e. connective tissue disease- (CTD-) associated PAH, particularly systemic sclerosis- (SSc-) associated PAH) improved recently, it is still worse than that of idiopathic PAH (iPAH) (Condliffe et al., 2009; Fisher et al., 2006; Girgis et al., 2005; Kawut et al., 2003). While inflammatory disturbances are described in both iPAH and SSc-PAH, these aberrancies are more severe in SSc-PAH, which explains the clinical and outcome discrepancies between the two syndromes (Fisher et al., 2006; Hassoun et al., 2009; Kawut et al., 2003).
Update in systemic sclerosis-associated pulmonary arterial hypertension
2014, Presse MedicaleImproved survival of Korean patients with idiopathic pulmonary arterial hypertension after the introduction of targeted therapies
2014, Heart and Lung: Journal of Acute and Critical CareCitation Excerpt :Although several studies have now shown that beraprost improves hemodynamic parameters in PAH patients,36,37,38 including functional class, systolic PAP, PVR, and 6MWD, no studies to date have addressed the survival benefits of this drug. Targeted therapies, such as ambrisentan,15 bosentan,16 sitaxsentan,17 sildenafil,39 epoprostenol,40 iloprost,41 and treprostinil,42 have been shown to increase patient survival in previous reports. Previous reports,7,12 along with the findings from our study (Fig. 4), prove that the survival benefits of beraprost are inferior to those of other targeted therapies.