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Long-term effects of intravenous iloprost in patients with idiopathic pulmonary arterial hypertension deteriorating on non-parenteral therapy

BACKGROUND: The majority of patients with idiopathic pulmonary arterial hypertension (IPAH) in functional classes II and III are currently being treated with non-parenteral therapies, including endothelin receptor antagonists (ERA), phosphodiesterase (PDE)-5 inhibitors, inhaled iloprost or combinati...

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Autores principales: Knudsen, Lars, Schurawlew, Alexander, Nickel, Nils, Tiede, Henning, Ghofrani, Hossein A, Wilkens, Heinrike, Ewert, Ralf, Halank, Michael, Klose, Hans, Bäzner, Carlos, Behr, Jürgen, Hoeper, Marius M
Formato: Online Artículo Texto
Lenguaje:English
Publicado: BioMed Central 2011
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3247176/
https://www.ncbi.nlm.nih.gov/pubmed/22133492
http://dx.doi.org/10.1186/1471-2466-11-56
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author Knudsen, Lars
Schurawlew, Alexander
Nickel, Nils
Tiede, Henning
Ghofrani, Hossein A
Wilkens, Heinrike
Ewert, Ralf
Halank, Michael
Klose, Hans
Bäzner, Carlos
Behr, Jürgen
Hoeper, Marius M
author_facet Knudsen, Lars
Schurawlew, Alexander
Nickel, Nils
Tiede, Henning
Ghofrani, Hossein A
Wilkens, Heinrike
Ewert, Ralf
Halank, Michael
Klose, Hans
Bäzner, Carlos
Behr, Jürgen
Hoeper, Marius M
author_sort Knudsen, Lars
collection PubMed
description BACKGROUND: The majority of patients with idiopathic pulmonary arterial hypertension (IPAH) in functional classes II and III are currently being treated with non-parenteral therapies, including endothelin receptor antagonists (ERA), phosphodiesterase (PDE)-5 inhibitors, inhaled iloprost or combinations of these substances. If these treatments fail, current guidelines recommend the addition of parenteral prostanoid therapy. There is, however, limited evidence for the efficacy of parenteral prostanoids when added to combinations of non-parenteral therapies. METHODS: In this retrospective, multicentre study we collected data from consecutive IPAH patients receiving intravenous iloprost in addition to optimized non-parenteral therapy between Jan 2002 and Dec 2009. Analyses included 6 min walk distance (6MWD), functional class, need for transplantation, and survival. RESULTS: During the observation period, 50 patients were treated with intravenous iloprost in addition to non-parenteral therapy; 44% of the patients were on dual combination therapy and 52% on triple combination. Three months after initiation of iloprost, functional class had improved in 24% of the patients and the median 6MWD had increased from 289 m to 298 m (n.s.). During the observation period, 22 patients (44%) died and 14 (28%) underwent lung transplantation. The probabilities of LuTx-free survival at 1, 3 and 5 years following iloprost initiation were 38%, 17% and 17%, respectively. A 6MWD < 300 m and persistent functional class IV at 3 months after initiation of intravenous iloprost were predictors of an adverse outcome. CONCLUSION: In essence, late initiation of intravenous iloprost in IPAH patients who previously failed to respond to non-parenteral therapies appears to be of limited efficacy in the majority patients. Alternative therapeutic options are currently not available, underlying the need for the development of new drugs.
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spelling pubmed-32471762011-12-30 Long-term effects of intravenous iloprost in patients with idiopathic pulmonary arterial hypertension deteriorating on non-parenteral therapy Knudsen, Lars Schurawlew, Alexander Nickel, Nils Tiede, Henning Ghofrani, Hossein A Wilkens, Heinrike Ewert, Ralf Halank, Michael Klose, Hans Bäzner, Carlos Behr, Jürgen Hoeper, Marius M BMC Pulm Med Research Article BACKGROUND: The majority of patients with idiopathic pulmonary arterial hypertension (IPAH) in functional classes II and III are currently being treated with non-parenteral therapies, including endothelin receptor antagonists (ERA), phosphodiesterase (PDE)-5 inhibitors, inhaled iloprost or combinations of these substances. If these treatments fail, current guidelines recommend the addition of parenteral prostanoid therapy. There is, however, limited evidence for the efficacy of parenteral prostanoids when added to combinations of non-parenteral therapies. METHODS: In this retrospective, multicentre study we collected data from consecutive IPAH patients receiving intravenous iloprost in addition to optimized non-parenteral therapy between Jan 2002 and Dec 2009. Analyses included 6 min walk distance (6MWD), functional class, need for transplantation, and survival. RESULTS: During the observation period, 50 patients were treated with intravenous iloprost in addition to non-parenteral therapy; 44% of the patients were on dual combination therapy and 52% on triple combination. Three months after initiation of iloprost, functional class had improved in 24% of the patients and the median 6MWD had increased from 289 m to 298 m (n.s.). During the observation period, 22 patients (44%) died and 14 (28%) underwent lung transplantation. The probabilities of LuTx-free survival at 1, 3 and 5 years following iloprost initiation were 38%, 17% and 17%, respectively. A 6MWD < 300 m and persistent functional class IV at 3 months after initiation of intravenous iloprost were predictors of an adverse outcome. CONCLUSION: In essence, late initiation of intravenous iloprost in IPAH patients who previously failed to respond to non-parenteral therapies appears to be of limited efficacy in the majority patients. Alternative therapeutic options are currently not available, underlying the need for the development of new drugs. BioMed Central 2011-12-01 /pmc/articles/PMC3247176/ /pubmed/22133492 http://dx.doi.org/10.1186/1471-2466-11-56 Text en Copyright ©2011 Knudsen et al; licensee BioMed Central Ltd. http://creativecommons.org/licenses/by/2.0 This is an Open Access article distributed under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/by/2.0), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.
spellingShingle Research Article
Knudsen, Lars
Schurawlew, Alexander
Nickel, Nils
Tiede, Henning
Ghofrani, Hossein A
Wilkens, Heinrike
Ewert, Ralf
Halank, Michael
Klose, Hans
Bäzner, Carlos
Behr, Jürgen
Hoeper, Marius M
Long-term effects of intravenous iloprost in patients with idiopathic pulmonary arterial hypertension deteriorating on non-parenteral therapy
title Long-term effects of intravenous iloprost in patients with idiopathic pulmonary arterial hypertension deteriorating on non-parenteral therapy
title_full Long-term effects of intravenous iloprost in patients with idiopathic pulmonary arterial hypertension deteriorating on non-parenteral therapy
title_fullStr Long-term effects of intravenous iloprost in patients with idiopathic pulmonary arterial hypertension deteriorating on non-parenteral therapy
title_full_unstemmed Long-term effects of intravenous iloprost in patients with idiopathic pulmonary arterial hypertension deteriorating on non-parenteral therapy
title_short Long-term effects of intravenous iloprost in patients with idiopathic pulmonary arterial hypertension deteriorating on non-parenteral therapy
title_sort long-term effects of intravenous iloprost in patients with idiopathic pulmonary arterial hypertension deteriorating on non-parenteral therapy
topic Research Article
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3247176/
https://www.ncbi.nlm.nih.gov/pubmed/22133492
http://dx.doi.org/10.1186/1471-2466-11-56
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