Novelty in Treatment of Pulmonary Fibrosis: Pulmonary Hypertension Drugs and Others

ISSN: 1875-6182 (Online)
ISSN: 1871-5257 (Print)

Volume 15, 3 Issues, 2017

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Cardiovascular & Hematological Agents in Medicinal Chemistry

Formerly: Current Medicinal Chemistry - Cardiovascular & Hematological Agents

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Debabrata Mukherjee
Department of Internal Medicine Texas Tech University
El Paso, TX

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Novelty in Treatment of Pulmonary Fibrosis: Pulmonary Hypertension Drugs and Others

Cardiovascular & Hematological Agents in Medicinal Chemistry, 11(3): 169-178.

Author(s): Michele Correale, Antonio Totaro, Donato Lacedonia, Deodata Montrone, Matteo Di Biase, Maria Pia Barbaro Foschino and Natale Daniele Brunetti.

Affiliation: Department of Cardiology, “Ospedali Riuniti” OO.RR, viale L Pinto, 1. 71100 Foggia, Italy.


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.


Ambrisentan, bosentan, cyclophosphamide, hypertension, idiopatic pulmonary fibrosis, iloprost, pulmonary pulmonary fibrosis, pirfenidone, sildenafil.

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Article Details

Volume: 11
Issue Number: 3
First Page: 169
Last Page: 178
Page Count: 10
DOI: 10.2174/187152571131100086
Price: $58
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