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We read with interest the paper by Mackay et al on the use of disease progression in patients with pulmonary fibrosis as a trigger for referral for lung transplantation.1 The authors attempt to identify the reasons why patients with idiopathic pulmonary fibrosis (IPF) have a higher mortality on the waiting list for lung transplantation than non-IPF patients. Although mortality on the waiting list is a major concern, physicians dealing with these patients face even more difficulties such as deciding which treatment to administer to a given patient: transplantation, inclusion in a clinical trial or classical treatment (corticosteroids, immunosuppressives and N-acetylcysteine). In order to tackle these questions we have developed an algorithm that deals with these key issues (fig 1).
Confronted with a patient with IPF, every physician needs to consider whether the patient would be suitable for transplantation as this is the only treatment that has been shown to have survival benefit.2 Early referral is important to decrease mortality on the waiting list. If there are no overt contraindications such as age >65–70 years, recent untreatable malignancy or major vascular disease, the patient needs to be referred to a transplant centre.3 If there the patient is found to be an appropriate candidate, the next question is whether the patient needs to be listed immediately. Key issues are blood group (as blood group O increases the waiting time significantly) and height and sex of the patient (as smaller female patients tend to have a longer waiting time on the list). Progression of disease is a chief determinant in this decision, as highlighted by the studies by Mackay et al1 and Collard et al.4 Another study5 found that the clinical course of patients with IPF was characterised by clinical parameters such as deterioration in forced vital capacity, carbon monoxide transfer factor and alveolar–arterial oxygen gradient, worsening dyspnoea over 72 weeks, the number of hospital admissions and acute exacerbations of IPF. If the patient has rapidly progressing disease, he/she will be listed without delay or otherwise we perform a follow-up from close by. In this case it might be useful to include the patient in a clinical trial with a promising antifibrotic drug. As there is insufficient evidence for a significant effect of classical treatment in IPF, it is our opinion that, for proven IPF, the classical anti-inflammatory treatment needs to be reserved for patients not suitable for transplantation and those for whom inclusion in a clinical trial is not possible owing to non-compatibility with the inclusion and exclusion criteria of the protocols.
In conclusion, the article by Mackay et al once more points to the fact that we need to choose protocols carefully to determine what to do with patients with IPF in order to provide every patient with the most effective treatment at the best possible time. We consider that this algorithm is easy but effective in dealing with these problems.
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