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Vol. 50. Issue 6.
Pages 260-261 (June 2014)
Vol. 50. Issue 6.
Pages 260-261 (June 2014)
Letter to the Editor
DOI: 10.1016/j.arbr.2013.08.010
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Triple Therapy in Idiopathic Pulmonary Fibrosis
Triple terapia en fibrosis pulmonar idiopática
Diego Castillo Villegasa,b,??
Corresponding author

Corresponding author.
, Silvia Barril Farréb
a Royal Brompton Hospital, Londres, United Kingdom
b Servicio de Neumología, Hospital de la Santa Creu i Sant Pau, Barcelona, Spain
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To the Editor:

We have read with particular interest the new “Guidelines for the diagnosis and treatment of idiopathic pulmonary fibrosis (IPF)”.1 It is clear that, as in the previous guidelines published in 2003 (one of the most widely read articles of this journal), Xaubet et al. have made a brilliant summary of the most important aspects of this disease.

One of the main novelties compared to the previous guidelines is regarding treatment. In the past 10 years there has been a shift in the treatment of the IPF patient from immunomodulators to the new antifibrotic drugs, and this is clearly reflected in the document. However, there is one clinical situation that has not been clarified: how should patients already diagnosed with IPF that are stable on triple therapy (N-acetylcysteine, low-dose prednisone and azathioprine) be managed? Certainly, many other clinicians like ourselves will have asked the same question when reading the guidelines. This is a problem that we would like to examine further.

Although the provisional results of the PANTHER study were widely received with alarm, as Wells et al. recently pointed out, this does not mean a permanent farewell to immunomodulators in IPF.2,3 It should be remembered that the IFIGENIA study data showed a reduction in disease progression in patients receiving triple therapy.4 Moreover, the adverse effects reported in the PANTHER study were associated with the use of high-dose steroids. For this reason, many experts believe that it is reasonable to maintain triple therapy with low-dose steroids in patients who have remained stable on this regimen.5

Accordingly, each case can be evaluated by following three simple steps:

  • 1.

    IPF diagnosis: this is another great step forward reflected in the guidelines. Diagnostic criteria are becoming ever more specific. And we know that patients with an incorrect diagnosis of IPF tend to respond better to immunosuppressive treatment, particularly those with non-specific interstitial pneumonia. Thus, the first step consists of reviewing the clinical records to confirm that the patient meets the diagnostic criteria specified in the new guidelines.

  • 2.

    Clinical stability: patients often continue to receive a certain treatment indefinitely, despite complete lack of response. For this reason, the next step would be to review clinical, radiological and functional parameters to ensure that the patient does not present significant deterioration.

  • 3.

    Tolerance and complications from immunosuppressive treatment: the final step would consist of determining the symptoms caused by the treatment, the extent of the side effects, and particularly the effect of the immunosuppressive treatment on the patient (repeated or severe infections, leukopenia, cancer, etc.).

These three simple steps could be useful for correctly weighing up the situation of the patient and the effectiveness of the treatment. Nevertheless, the most important factor is indubitably missing from this equation: the opinion of the patient. Another essential step is to discuss the new therapeutic options, and the risks and benefits compared to the previous ones, since, as in life, two heads are generally better than one in coming to the right decision.

A. Xaubet, J. Ancoechea, E. Bollo, E. Fernádez-Fabrellas, T. Franquet, M. Molina-Molina, et al.
Normativa sobre el diagnóstico y tratamiento de la fibrosis pulmonar idiopática.
Arch Bronconeumol, 49 (2013), pp. 343-353
G. Raghu, K.J. Anstrom, T.E. King Jr., J.A. Lasky, F.J. Martinez.
Idiopathic pulmonary fibrosis clinical research network. Prednisone, azathioprine, and N-acetylcysteine for pulmonary fibrosis.
N Engl J Med, 366 (2012), pp. 1968-1977
A.U. Wells, W.P. Kelleher.
Idiopathic pulmonary fibrosis pathogenesis and novel approaches to immunomodulation: we must not be tyrannized by the PANTHER data.
Am J Respir Crit Care Med, 187 (2013), pp. 677-679
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
A.U. Wells, J. Behr, U. Costabel, V. Cottin, V. Poletti.
Triple therapy in idiopathic pulmonary fibrosis: an alarming press release.
Eur Respir J, 39 (2012), pp. 805-806

Please cite this article as: Castillo Villegas D, Barril Farré S. Triple terapia en fibrosis pulmonar idiopática. Arch Bronconeumol. 2014;50:260–261.

Copyright © 2014. SEPAR
Archivos de Bronconeumología (English Edition)

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