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Vol. 48. Issue S2.
Actualización en fibrosis pulmonar idiopática
Pages 13-15 (October 2012)
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Vol. 48. Issue S2.
Actualización en fibrosis pulmonar idiopática
Pages 13-15 (October 2012)
Actualización en fibrosis pulmonar idiopática
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Perspectivas en el tratamiento de la fibrosis pulmonar idiopática
Future prospects in the treatment of idiopathic pulmonary fibrosis
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Ferran Morell
Corresponding author
fmorell@vhebron.net

Autor para correspondencia.
, Ana Villar
Servei de Pneumologia, Hospital Universitari Vall d’Hebron, Universitat Autònoma de Barcelona, Barcelona, España
CIBER de Enfermedades Respiratorias (CibeRes)
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Resumen

Después de las tres revisiones Cochrane realizadas en los últimos diez años, se ha demostrado que la mayoría de los ensayos clínicos llevados a cabo con corticosteroides, inmunomoduladores y antifibróticos muestran resultados no concluyentes para el tratamiento de la fibrosis pulmonar idiopática. Hasta la fecha, pirfenidona es el único fármaco que en ensayos clínicos controlados ha demostrado eficacia contrastada en el tratamiento de esta enfermedad. Otros fármacos se están ensayando en la actualidad, lo que ofrece una perspectiva optimista para el tratamiento futuro de esta entidad.

Palabras clave:
Fibrosis pulmonar
Fármacos
N-acetilcisteína
Pirfenidona
Abstract

After the three revisions done by Cochrane for the last ten years, it has been demonstrated that most of the clinical assays performed whit corticosteroids, immunomodulators and antifibrotic drugs have shown inconclusive results for the treatment of idiopathic pulmonary fibrosis. To date, only pirfenidone has proved to have significant efficacy in controlled clinical trials in the treatment of this disease. At present, a large number of new drugs are being tested, which offers a new perspective in some ways optimistic concerning the future treatment of this disease.

Keywords:
Pulmonary Fibrosis
Drugs
N-acetylcysteine
Pirfenidone
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Bibliografía
[1.]
American Thoracic Society.
Idiopathic pulmonary fibrosis: diagnosis and treatment. International consensus statement. American Thoracic Society (ATS), and the European Respiratory Society (ERS).
Am J Respir Crit Care Med, 161 (2000), pp. 646-664
[2.]
L. Richeldi, H.R. Davies, G. Ferrara, F. Franco.
Corticosteroids for idiopathic pulmonary fibrosis.
Cochrane Database Syst Rev, (2003), pp. CD002880
[3.]
H.R. Davies, L. Richeldi, E.H. Walters.
Immunomodulatory agents for idiopathic pulmonary fibrosis.
Cochrane Database Syst Rev, (2003), pp. CD003134
[4.]
P. Spagnolo, C. Del Giovane, F. Luppi, S. Cerri, S. Balduzzi, E.H. Walters, et al.
Non-steroid agents for idiopathic pulmonary fibrosis.
Cochrane Database Syst Rev, (2010), pp. CD003134
[5.]
M.A. Johnson, S. Kwan, N.J. Snell, A.J. Nunn, J.H. Darbyshire, M. Turner-Warwick.
Randomised controlled trial comparing prednisolone alone with cyclophosphamide and low dose prednisolone in combination in cryptogenic fibrosing alveolitis.
Thorax, 44 (1989), pp. 280-288
[6.]
G. Raghu, W.J. Depaso, K. Cain, S.P. Hammar, C.E. Wetzel, D.F. Dreis, et al.
Azathioprine combined with prednisone in the treatment of idiopathic pulmonary fibrosis: a prospective double-blind, randomized, placebo-controlled clinical trial.
Am Rev Respir Dis, 144 (1991), pp. 291-296
[7.]
W.W. Douglas, J.H. Ryu, S.J. Swensen, K.P. Offord, D.R. Schroeder, G.M. Caron, et al.
Colchicine versus prednisone in the treatment of idiopathic pulmonary fibrosis. A randomized prospective study. Members of the Lung Study Group.
Am J Respir Crit Care Med, 158 (1998), pp. 220-225
[8.]
R. Ziesche, E. Hofbauer, K. Wittmann, V. Petkov, L.H. Block.
A preliminary study of long-term treatment with interferon gamma-1b and low-dose prednisolone in patients with idiopathic pulmonary fibrosis.
N Engl J Med, 341 (1999), pp. 1264-1269
[9.]
G. Raghu, K.K. Brown, W.Z. Bradford, K. Starko, P.W. Noble, D.A. Schwartz, et al.
Idiopathic Pulmonary Fibrosis Study Group. A placebo-controlled trial of interferon gamma- 1b in patients with idiopathic pulmonary fibrosis.
N Engl J Med, 350 (2004), pp. 125-133
[10.]
T.E. King, C. Albera, W.Z. Bradford, U. Costabel, P. Hormel, L. Lancaster, et al.
Effect of interferon gamma 1-b on survival in patients with idiopathic pulmonary fibrosis (INSPIRE): a multicentre, randomized, placebo, controlled trial.
[11.]
H. Kubo, K. Nakayama, M. Yanai, T. Suzuki, M. Yamaya, M. Watanabe, et al.
Anticoagulant therapy for idiopathic pulmonary fibrosis.
Chest, 128 (2005), pp. 1475-1482
[12.]
A. Azuma, T. Nukiwa, E. Tsuboi, M. Suga, S. Abe, K. Nakata, et al.
Double-blind, placebocontrolled trial of pirfenidone in patients with idiopathic pulmonary fibrosis.
Am J Respir Crit Care Med, 171 (2005), pp. 1040-1047
[13.]
M. Demedts, J. Behr, R. Buhl, U. Costabel, R. Dekhuijzen, H.M. Jansen, IFIGENIA Study Group, et al.
High-dose acetylcysteine in idiopathic pulmonary fibrosis.
N Engl J Med, 353 (2005), pp. 2229-2242
[14.]
G. Raghu, K.K. Brown, U. Costabel, V. Cottin, R.M. Du Bois, J.A. Lasky, et al.
Treatment of idiopathic pulmonary fibrosis with etanercept: an exploratory, placebo-controlled trial.
Am J Respir Crit Care Med, 178 (2008), pp. 948-955
[15.]
C.E. Daniels, J.A. Lasky, A.H. Limper, K. Mieras, E. Gabor, D.R. Schroeder, Imatinib-IPF Study Investigators.
Imatinib treatment for idiopathic pulmonary fibrosis: Randomized placebo-controlled trial results.
Am J Respir Crit Care Med, 181 (2010), pp. 604-610
[16.]
T.E. King Jr., J. Behr, K.K. Brown, R.M. Du Bois, L. Lancaster, J.A. De Andrade, et al.
BUILD-1: a randomized placebo-controlled trial of bosentan in idiopathic pulmonary fibrosis.
Am J Respir Crit Care Med, 177 (2008), pp. 75-81
[17.]
P.W. Noble, C. Albera, W.Z. Bradford, U. Costabel, M.K. Glassberg, D. Kardatzke, CAPACITY Study Group, et al.
Pirfenidone in patients with idiopathic pulmonary fibrosis (CAPACITY): two randomised trials.
Lancet, 377 (2011), pp. 1760-1769
[18.]
H. Taniguchi, M. Ebina, Y. Kondoh, T. Ogura, A. Azuma, M. Suga, Pirfenidone Clinical Study Group in Japan, et al.
Pirfenidone in idiopathic pulmonary fibrosis.
Eur Respir J., 35 (2010), pp. 821-829
[19.]
J.J. Swigris, K.K. Brown.
Evaluation of bosentan for idiopathic pulmonary fibrosis.
Expert Rev Respir Med, 2 (2008), pp. 315-321
[20.]
BUILD 3. Open label extension study in patients with Idiopathic Pulmonary Fibrosis who completed Protocol AC-052-321. Clinical Trials.gov.
[21.]
ARTEMIS-PH - Study of Ambrisentan in Subjects With Pulmonary Hypertension Associated With Idiopathic Pulmonary Fibrosis. Clinical Trials.gov processed this record on February 14, 2012.
[22.]
Matitentan. Safety and tolerability study of Matitentan in patients with Idiopathic Pulmonary Fibrosis. Clinical Trials.gov.
[23.]
D.A. Zisman, M. Schwarz, K.J. Anstrom, H.R. Collard, K.R. Flaherty, G.W. Hunninghake.
Idiopathic Pulmonary Fibrosis Clinical Research Network. A controlled trial of sildenafil in advanced idiopathic pulmonary fibrosis.
N Engl J Med, 363 (2010), pp. 620-628
[24.]
L. Richeldi, U. Costabel, M. Selman, D.S. Kim, D.M. Hansell, A.G. Nicholson, et al.
Efficacy of a tyrosine kinase inhibitor in idiopathic pulmonary fibrosis.
N Engl J Med, 365 (2011), pp. 1079-1087
[25.]
E.E. McGrath, A.B. Millar.
Hot off the breath: triple therapy for idiopathic pulmonary fibrosis--hear the PANTHER roar.
[26.]
J. Behr, K. Maier, B. Degenkolb, F. Krombach, C. Vogelmeier.
Antioxidative and clinical effects of high-dose N-acetylcysteine in fibrosing alveolitis. Adjunctive therapy to maintenance immunosuppression.
Am J Respir Crit Care Med, 156 (1997), pp. 1897-1901
[27.]
S. Homma, A. Azuma, H. Taniguchi, T. Ogura, Y. Mochiduki, Y. Sugiyama, and the Japan NAC Clinical Study Group, et al.
Efficacy of inhaled N-acetylcysteine monotherapy in patients with early stage idiopathic pulmonary fibrosis.
Respirology, (2012),
[28.]
A. Azuma, Y. Taguchi, T. Ogura, M. Ebina, H. Taniguchi, Y. Kondoh, Pirfenidone Clinical Study Group in Japan, et al.
Exploratory analysis of a phase III trial of pirfenidone identifies a subpopulation of patients with idiopathic pulmonary fibrosis as benefiting from treatment.
Respir Res, 12 (2011), pp. 143
Copyright © 2012. Sociedad Española de Neumología y Cirugía Torácica
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