Abstract
Idiopathic pulmonary fibrosis (IPF) is a chronic condition of unknown etiology with deteriorating respiratory function leading to respiratory failure. Corticosteroids, alone or in combination with immunosuppressive drugs such as azathioprine, colchicine, and cyclophosphamide, have been used with limited success. Interferon-gamma-1b showed a significant improvement in pulmonary function only in one study. Pirfenidone, cyclosporine and acetylcysteine may also be of benefit but data from studies are limited. Novel drugs, mainly antifibrotic, anticytokine and immunoregulatory, are currently being investigated in various trial phases. Endothelin receptor antagonists have been shown to have possible beneficial effects in early stages of IPF. However, most recently, the so-called triple combination therapy, anticoagulation therapy and endothelin receptor antagonists, especially ambrisentan, are either harmful or ineffective in IPF and are not recommended. We report a brief review on the present and possible future therapeutic options in IPF.
Keywords: Ambrisentan, bosentan, cyclophosphamide, hypertension, idiopatic pulmonary fibrosis, iloprost, pulmonary pulmonary fibrosis, pirfenidone, sildenafil.
Cardiovascular & Hematological Agents in Medicinal Chemistry
Title:Novelty in Treatment of Pulmonary Fibrosis: Pulmonary Hypertension Drugs and Others
Volume: 11 Issue: 3
Author(s): Michele Correale, Antonio Totaro, Donato Lacedonia, Deodata Montrone, Matteo Di Biase, Maria Pia Barbaro Foschino and Natale Daniele Brunetti
Affiliation:
Keywords: Ambrisentan, bosentan, cyclophosphamide, hypertension, idiopatic pulmonary fibrosis, iloprost, pulmonary pulmonary fibrosis, pirfenidone, sildenafil.
Abstract: Idiopathic pulmonary fibrosis (IPF) is a chronic condition of unknown etiology with deteriorating respiratory function leading to respiratory failure. Corticosteroids, alone or in combination with immunosuppressive drugs such as azathioprine, colchicine, and cyclophosphamide, have been used with limited success. Interferon-gamma-1b showed a significant improvement in pulmonary function only in one study. Pirfenidone, cyclosporine and acetylcysteine may also be of benefit but data from studies are limited. Novel drugs, mainly antifibrotic, anticytokine and immunoregulatory, are currently being investigated in various trial phases. Endothelin receptor antagonists have been shown to have possible beneficial effects in early stages of IPF. However, most recently, the so-called triple combination therapy, anticoagulation therapy and endothelin receptor antagonists, especially ambrisentan, are either harmful or ineffective in IPF and are not recommended. We report a brief review on the present and possible future therapeutic options in IPF.
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Cite this article as:
Correale Michele, Totaro Antonio, Lacedonia Donato, Montrone Deodata, Biase Di Matteo, Foschino Pia Barbaro Maria and Brunetti Daniele Natale, Novelty in Treatment of Pulmonary Fibrosis: Pulmonary Hypertension Drugs and Others , Cardiovascular & Hematological Agents in Medicinal Chemistry 2013; 11 (3) . https://dx.doi.org/10.2174/187152571131100086
DOI https://dx.doi.org/10.2174/187152571131100086 |
Print ISSN 1871-5257 |
Publisher Name Bentham Science Publisher |
Online ISSN 1875-6182 |
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