Abstract: Poster Presentations |

Bosentan in Pulmonary Artery Hypertension Secondary to Systemic Sclerosis FREE TO VIEW

A. Joglekar, MD; F. B. Fausan, BS; D. A. McCloskey, RN; J. E. Wilson, RN; J. R. Seibold, MD; D. J. Riley, MD
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UMDNJ-Robert Wood Johnson Medical School, New Brunswick, NJ


Chest. 2003;124(4_MeetingAbstracts):223S. doi:10.1378/chest.124.4_MeetingAbstracts.223S-b
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INTRODUCTION:  Systemic sclerosis (SSc) was underrepresented (52 patients) in preapproval trials for bosentan in pulmonary artery hypertension (PAH). PURPOSE: Increase the experience of bosentan in PAH secondary to SSc (SSc-PAH) including those with concomitant interstitial lung disease (ILD).

METHODS:  Retrospective review of 48 SSc patients with PAH (> 35 mm Hg pulmonary artery systolic pressure [PASP] by echocardiogram), WHO classes II-IV, and data available for ≥ 9 months. Bosentan dose was 62.5 mg bid 4 wk than 125 mg bid thereafter. Outcomes were WHO class, PASP and PFTs at baseline, 3, 6 and 9 months.

RESULTS:  WHO class (± SD): baseline, 3.0 ± 0.6 (n = 41); 3 months 2.3 ± 0.7* (n=37); 6 months 2.3 ± 0.8* (n=37); 9 months 2.1 ± 0.8* (n=32) (* P<0.001 vs. baseline by Mann-Whitney U test), indicating a significant improvement in clinical status at 3 months that was sustained during therapy. Reduction in WHO class ≥ one rank occurred at 3 months in 20/41 (49%), none worsened; at 6 months, 18/37 (49%) improved, 3% worsened; at 9 months, 19/32 (59%) improved, 3% worsened. Mean PSAP at baseline (n=38) was 46 ± 13 mm Hg (± SD), and there was no significant change during therapy. Mean PFTs at baseline (all % predicted ± SD) were TLC 72 ± 16 (n=48), FVC 69 ± 14 (n=43), and DLco 39 ± 13 (n=48), and none changed significantly during therapy. Abnormal transaminases occurred 8 times in 4 patients (10%), necessitating discontinuation of drug in two. No deaths were ascribed to bosentan therapy.CONCLUSIONS: Bosentan is beneficial in SSc-PAH including patients with ILD. Pulmonary hemodynamics (PASP, DLco) remain stable during treatment.

CLINICAL IMPLICATIONS:  The lack of change in PFTs and PASP does not support an antiproliferative or antifibrotic benefit of endothelin receptor antagonism during 9 months treatment of SSc-PAH.

DISCLOSURE:  A. Joglekar, None.

Wednesday, October 29, 2003

12:30 PM - 2:00 PM




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