title: Novelty in treatment of pulmonary fibrosis: pulmonary hypertension drugs and others.
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.
authors.forename: Michele
authors.initials: M
authors.lastname: Correale
authors.forename: Antonio
authors.initials: A
authors.lastname: Totaro
authors.forename: Donato
authors.initials: D
authors.lastname: Lacedonia
authors.forename: Deodata
authors.initials: D
authors.lastname: Montrone
authors.forename: Matteo
authors.initials: M
authors.lastname: Di Biase
authors.forename: Maria Pia
authors.initials: MP
authors.lastname: Barbaro Foschino
authors.affiliation: Department of Cardiology, "Ospedali Riuniti" OO.RR, viale L Pinto, 1. 71100 Foggia, Italy. opsfco@tin.it.
authors.forename: Natale Daniele
authors.initials: ND
authors.lastname: Brunetti
mesh_terms: D000959:Antihypertensive Agents / Q000627:therapeutic use*; D000077300:Bosentan; D006801:Humans; D007555:Isoxazoles / Q000627:therapeutic use; D010666:Phenylpropionates / Q000627:therapeutic use; D010726:Phosphodiesterase Inhibitors / Q000627:therapeutic use; D011658:Pulmonary Fibrosis / Q000188:drug therapy*; D011724:Pyridazines / Q000627:therapeutic use; D013449:Sulfonamides / Q000627:therapeutic use; D013876:Thiophenes / Q000627:therapeutic use
chemical_list: D000959:Antihypertensive Agents; D007555:Isoxazoles; D010666:Phenylpropionates; D010726:Phosphodiesterase Inhibitors; D011724:Pyridazines; D013449:Sulfonamides; D013876:Thiophenes; C467894:ambrisentan; C106276:sitaxsentan; D000077300:Bosentan