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We thank Dr Wuyts et al for their letter and appreciate their comments on the importance of our observations. Clinicians have to make a number of difficult decisions when deciding on treatment options in patients with pulmonary fibrosis. These include whether to commence classical treatment which offers little therapeutic advantage, or whether to enter patients into a clinical trial. Importantly, in those eligible, identification of the optimal timing for referral for lung transplantation assessment is critical as this is the only treatment to be of proven benefit.
We welcome the algorithm presented by Wuyts et al as a simple guide for all clinicians involved in the management of patients with pulmonary fibrosis. It emphasises the pivotal role that the early identification of potential lung transplantation candidates plays, as well as considering eligibility for entry into a clinical trial. We would, however, suggest that this algorithm could be modified to allow those assessed and listed for transplantation to be considered for inclusion in such trials as a possible “bridge to transplant”.
While this algorithm seems to relate to only those with idiopathic pulmonary fibrosis, we would like to suggest that it might be applied to all patients with pulmonary fibrosis. Our study highlighted that patients with pulmonary fibrosis may be misclassified on pretransplant histology and radiology or on clinical grounds, and that other forms may present with a phenotype that mimics usual interstitial pneumonia. We therefore believe that phenotype based on rate of disease progression seems to be more predictive of poor survival than histological classification or any one physiological measure.
In summary, we welcome this algorithm which challenges the conventional approach to treatment options in pulmonary fibrosis by considering first the need and suitability for transplantation and thereafter considering classical treatment or entry into a clinical trial. Such a radical change in the approach to the management may bring about considerable advances without the need for an exhaustive search for the precise histological classification.
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