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Letters Idiopathic pulmonary fibrosis

Underestimating treatment benefit of lung transplantation for idiopathic pulmonary fibrosis

BMJ 2014; 348 doi: https://doi.org/10.1136/bmj.g24 (Published 06 January 2014) Cite this as: BMJ 2014;348:g24

This article has a correction. Please see:

  1. P Riddell, respiratory registrar1,
  2. D Eaton, consultant in lung transplant surgery1,
  3. A B Miller, professor of respiratory medicine2,
  4. A U Wells, professor of respiratory medicine3,
  5. J J Egan, professor of respiratory/transplant medicine1
  1. 1Advanced Lung Disease and Transplant Programme, Mater Misericordiae University Hospital, Dublin, Republic of Ireland
  2. 2Academic Respiratory Unit, School of Clinical Sciences, Bristol University, Bristol, UK
  3. 3Interstitial Lung Disease Unit, Royal Brompton Hospital and National Heart and Lung Institute, London, UK
  1. priddell{at}mater.ie

Dempsey and Miller drew attention to the inadequacy of medical treatments for idiopathic pulmonary fibrosis (IPF) and the optimism provided by the emergence of pirfenidone.1However, only a cursory note was given to lung transplantation, which seems to have been dismissed by the authors, who describe patients as “too frail or old” with “serious comorbidity.” We are worried that this viewpoint will foster a passive attitude to this successful treatment.

International Society for Heart and Lung Transplantation registry data from 2012 show that 37% of North American and 31% of worldwide lung transplants were performed for IPF, compared with only 13% in Europe. This discrepancy is hard to explain. However, the proportion of patients with IPF given transplants in the US increased after the introduction of the lung allocation scoring system,2 which prioritises patients on the basis of treatment benefit.

Although IPF has been associated with higher perioperative mortality than other conditions, this is probably because of delayed referral—long term outcomes have been shown to be excellent.3 In Ireland, five year survival in these patients is 79% after transplantation. Outcomes are similar in all age groups, including those over 65.4 5

Lung transplantation is the only intervention with a proved survival benefit in this disease. Although future drug development should be viewed optimistically, treatments that are known to enhance survival should be focused on. Increased referrals to transplant centres, allied to improved organ utilisation for these patients, should be measured as key performance indicators. Advocacy aimed at achieving transplant rates of 30%, akin to the US, would provide considerable benefits.

Notes

Cite this as: BMJ 2014;348:g24

Footnotes

  • Competing interests: None declared.

References

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