Bosentan: A considerable increase in the survival of patients with pulmonary hypertension associated with systemic rheumatic diseases

AIM: To evaluate the short-term efficacy of the nonselective endothelin receptor antagonist bosentan in the treatment of pulmonary hypertension (PH) associated with diffuse connective tissue diseases (CTD), as well as its effect on survival in both monotherapy and in combination with other PH-specif...

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Main Authors: A V Volkov, N N Iudkina, E V Nikolaeva, I A Kurmukov, S I Glukhova, E L Nasonov
Format: Article
Language:Russian
Published: "Consilium Medicum" Publishing house 2014-05-01
Series:Терапевтический архив
Subjects:
Online Access:https://ter-arkhiv.ru/0040-3660/article/view/31477
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author A V Volkov
N N Iudkina
E V Nikolaeva
I A Kurmukov
S I Glukhova
E L Nasonov
author_facet A V Volkov
N N Iudkina
E V Nikolaeva
I A Kurmukov
S I Glukhova
E L Nasonov
author_sort A V Volkov
collection DOAJ
description AIM: To evaluate the short-term efficacy of the nonselective endothelin receptor antagonist bosentan in the treatment of pulmonary hypertension (PH) associated with diffuse connective tissue diseases (CTD), as well as its effect on survival in both monotherapy and in combination with other PH-specific agents/MATERIAL AND METHODS: The study included 20 CDT-associated PH patients who had been hospitalized in 2009-2013. All the patients had valid diagnoses of scleroderma systematica (SDS) (n=18) or systemic lupus erythematosus (SLE) (n=2). Bosentan was given in an initial dose of 62.5 mg/day twice for 4 weeks, then 125 mg/day twice/RESULTS: Eighteen patents completed therapy at 16 weeks. One patient with Functional Class (FC) IV PH associated with SDS died after 10 weeks of treatment because of PH progression; bosentan was discontinued in another patient following 4 weeks because of the enhanced activity of transaminases. The patients who had completed the investigation showed a significant FC decrease (from 2.9±1.0 to 2.4±1.0 following 16 weeks; р=0.03), an increase in 6-minute walking distance (from 298±140 to 375±94 m; р
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spelling doaj.art-a7b182bd00b044f0aff9c6a5d2c4a27e2022-12-21T23:53:26Zrus"Consilium Medicum" Publishing houseТерапевтический архив0040-36602309-53422014-05-01865323928493Bosentan: A considerable increase in the survival of patients with pulmonary hypertension associated with systemic rheumatic diseasesA V VolkovN N IudkinaE V NikolaevaI A KurmukovS I GlukhovaE L NasonovAIM: To evaluate the short-term efficacy of the nonselective endothelin receptor antagonist bosentan in the treatment of pulmonary hypertension (PH) associated with diffuse connective tissue diseases (CTD), as well as its effect on survival in both monotherapy and in combination with other PH-specific agents/MATERIAL AND METHODS: The study included 20 CDT-associated PH patients who had been hospitalized in 2009-2013. All the patients had valid diagnoses of scleroderma systematica (SDS) (n=18) or systemic lupus erythematosus (SLE) (n=2). Bosentan was given in an initial dose of 62.5 mg/day twice for 4 weeks, then 125 mg/day twice/RESULTS: Eighteen patents completed therapy at 16 weeks. One patient with Functional Class (FC) IV PH associated with SDS died after 10 weeks of treatment because of PH progression; bosentan was discontinued in another patient following 4 weeks because of the enhanced activity of transaminases. The patients who had completed the investigation showed a significant FC decrease (from 2.9±1.0 to 2.4±1.0 following 16 weeks; р=0.03), an increase in 6-minute walking distance (from 298±140 to 375±94 m; рhttps://ter-arkhiv.ru/0040-3660/article/view/31477scleroderma systematicapulmonary hypertensionbosentansurvivalright heart catheterization
spellingShingle A V Volkov
N N Iudkina
E V Nikolaeva
I A Kurmukov
S I Glukhova
E L Nasonov
Bosentan: A considerable increase in the survival of patients with pulmonary hypertension associated with systemic rheumatic diseases
Терапевтический архив
scleroderma systematica
pulmonary hypertension
bosentan
survival
right heart catheterization
title Bosentan: A considerable increase in the survival of patients with pulmonary hypertension associated with systemic rheumatic diseases
title_full Bosentan: A considerable increase in the survival of patients with pulmonary hypertension associated with systemic rheumatic diseases
title_fullStr Bosentan: A considerable increase in the survival of patients with pulmonary hypertension associated with systemic rheumatic diseases
title_full_unstemmed Bosentan: A considerable increase in the survival of patients with pulmonary hypertension associated with systemic rheumatic diseases
title_short Bosentan: A considerable increase in the survival of patients with pulmonary hypertension associated with systemic rheumatic diseases
title_sort bosentan a considerable increase in the survival of patients with pulmonary hypertension associated with systemic rheumatic diseases
topic scleroderma systematica
pulmonary hypertension
bosentan
survival
right heart catheterization
url https://ter-arkhiv.ru/0040-3660/article/view/31477
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AT nniudkina bosentanaconsiderableincreaseinthesurvivalofpatientswithpulmonaryhypertensionassociatedwithsystemicrheumaticdiseases
AT evnikolaeva bosentanaconsiderableincreaseinthesurvivalofpatientswithpulmonaryhypertensionassociatedwithsystemicrheumaticdiseases
AT iakurmukov bosentanaconsiderableincreaseinthesurvivalofpatientswithpulmonaryhypertensionassociatedwithsystemicrheumaticdiseases
AT siglukhova bosentanaconsiderableincreaseinthesurvivalofpatientswithpulmonaryhypertensionassociatedwithsystemicrheumaticdiseases
AT elnasonov bosentanaconsiderableincreaseinthesurvivalofpatientswithpulmonaryhypertensionassociatedwithsystemicrheumaticdiseases