Idiopathic pulmonary fibrosis
BMJ 2013; 347 doi: https://doi.org/10.1136/bmj.f6579 (Published 07 November 2013) Cite this as: BMJ 2013;347:f6579
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In the subtitle of their editorial on idiopathic pulmonary fibrosis (IPF) (1) Dempsey and Miller claim that the condition was first described 150 years ago by Austin Flint (2).
Unfortunately this is not true. Flint indeed described acute lobar pneumonitis and further “the different stages of acute pneumonia furnishing different therapeutical indications” including circumscribed pneumonitis and chronic pneumonitis. He precised that “the term chronic pneumonitis might perhaps with propriety be applied to occasional cases in which, after an acute attack, more or less solidification of lung continues for several or even many weeks, and final resolution is accomplished”.
JM Charcot (3) as well as W Osler (4) also described unilateral chronic pneumonia following infectious acute pneumonia.
Genuine IPF has been described only in the second half of the XXth century and is related to the cigarette smoking epidemic (5).
Jean-François Cordier
Vincent Cottin
National Reference Center for Rare Pulmonary Diseases, Claude Bernard University, Lyon, France
1. Dempsey OJ, Miller D. Idiopathic pulmonary fibrosis. Modest progress nearly 150 years after the condition was first described. Br Med J 2013 ; 347 : 8
2. Flint A. A treatise on the principles and practice of Medicine. Philadelphia, HC Lea, 1868
3. Charcot JM. Des pneumonies chroniques [Chronic pneumonias]. Rev Mensuelle Med Chir 1878; 2: 776–790
4. Osler W. The principles and practice of medicine. New York, D Appleton and Company, 1892
5. Cordier JF, Cottin V. Neglected evidence in idiopathic pulmonary fibrosis: from history to earlier diagnosis. Eur Respir J 2013 ; 42 : 916-23
Competing interests: No competing interests
Underestimating the Treatment Benefit of Lung Transplantation for Idiopathic Pulmonary Fibrosis
P Riddell 1; D Eaton 1; AB Miller 2; AU Wells 3; JJ Egan 1.
1. Advanced Lung Disease and Transplant Programme, Mater Misericordiae University Hospital, Ireland
2. Academic Respiratory Unit, School of Clinical Sciences, Bristol University, UK
3. Interstitial Lung Disease Unit, Royal Brompton Hospital and National Heart and Lung Institute, London, UK
It is laudable that, in its recent editorial, the BMJ has drawn attention to the inadequacy of medical therapies for Idiopathic Pulmonary Fibrosis and the optimism that has been provided by the emergence of Pirfenidone therapy (1).
However, it was disappointing to read the cursory note that was given to lung transplantation. This treatment option appears to have been dismissed by the authors, who describe patients as "too frail or old" with "serious co-morbidity". We are concerned that this viewpoint will foster a passive attitude to a very successful treatment. Lung transplantation is the only intervention to date with a proven survival benefit in this disease.
ISHLT registry data from 2012 shows that 37.2% of N. American and 31.1% of worldwide lung transplants were performed for IPF. This compares to only 13% in Europe, a discrepancy which is hard to explain. However, it is interesting to note that the proportion of IPF patients being transplanted in the USA has increased following the introduction of the Lung Allocation Scoring system (2). This system prioritises patients based on treatment benefit.
Although IPF has been associated with a higher peri-operative mortality than other conditions, this likely reflects a pattern of delayed referral. Indeed, long term outcomes have been shown to be excellent (3). In Ireland, a 5 year survival of 79% can be offered to IPF patients following transplantation. This survival outcome is similar in patients of all age groups, including those over the age of 65 years (4, 5).
It is important to reiterate that lung transplantation is the only intervention with a proven survival benefit in this disease. Whilst future drug development should be looked upon with optimism, it is critical to focus our attention on therapies that have been proven to offer enhanced survival. Increased referrals to transplant centres, allied to improved organ utilisation for IPF patients should be measured as key performance indicators. Advocacy aimed at achieving transplant rates of 30% akin to the USA will provide significant benefits to IPF patients.
Bibliography:
1. O J Dempsey, D Miller. Idiopathic Pulmonary Fibrosis: Modest progress nearly 150 years after the condition was first described. BMJ 2013; 347: f6579
2. Merlo CA et al. Impact of the Lung Allocation Score on Survival After Lung Transplantation. The Journal of Heart and Lung Transplantation 2009 Aug;28(8):769-75
3. Keating D, et al. Lung transplantation in Pulmonary Fibrosis: Challenging Early Outcomes Counterbalanced by Surprisingly Good Outcomes Beyond 15 years. Transplantation Proceedings. 2009 Jan-Feb; 41(1):289-91
4. Adamali HI, Judge EP, Healy D, Nolke L, Redmond KC, Bartosik W, McCarthy J, Egan JJ. International collaboration: a retrospective study examining the survival of Irish citizens following lung transplantation in both the UK and Ireland. BMJ Open. 2012 Mar 28; 2(2).
5. P Riddell, I Lawrie, S Winward, K Redmond, JJ Egan. Lung Transplantation and Survival in Idiopathic Pulmonary Fibrosis. Thorax. 2013; 68(Suppl 3):A168.
Competing interests: No competing interests
Re: Idiopathic pulmonary fibrosis
We thank Professors Cordier and Cottin for their helpful comments in which they rebut our suggestion that Flint was “probably” the first to describe idiopathic pulmonary fibrosis (IPF). The history of medicine is often keenly debated, but we can agree that a disease similar to what we now call “idiopathic pulmonary fibrosis” (IPF) was first described in the late 19th Century. To add further fuel to the debate, while Flint certainly did describe the clinical association between finger clubbing and pneumonitis [1] it could even be argued that it was an Irish Cardiologist (rather than a Chest Physician!), who provided the first documented account of pulmonary fibrosis. Sir Dominic Corrigan (also credited for first describing the collapsing pulse of aortic incompetence, Corrigan’s pulse) described a condition that he termed “cirrhosis” of the lungs.[2] For the general reader these discussions, however interesting, are somewhat academic, and the main thrust of our editorial was to increase disease awareness and also highlight new developments, which we hope we have achieved.
We also thank Dr Riddell and colleagues for their important comments emphasising the role of lung transplantation in patients with IPF. Editorial space precluded significant discussion of this topic, but it is tackled in the latest IPF guidelines , the main message being that transplantation should be considered in all patients without any absolute contraindications with referral ideally in the first 6 months after a confident diagnosis.[3] With regard to the maximal age of patient in whom lung transplantation should be considered, the British Thoracic Guidelines on IPF (published in 2008) suggested that transplantation for IPF is considered in “those who fulfil established selection criteria… thus generally excluding those over the age of 65 years of age and / or those with significant co-morbidity.”[4] Dr Riddell raises the important point, however, that in carefully selected patients over the age of 65 years, there may still be clinical benefit. This is highly relevant given the median age of onset for IPF is approximately 70 years and we routinely see chronologically “elderly” but biologically youthful patients with this devastating disease. Worldwide there has been a striking increase in the number of transplants now being performed in those > 65 years of age - only 138 lung transplants for IPF were performed between 2000-2005, whereas in 2006-2012 this had risen to 1061.[5] Median survival (the estimated time point by which 50% of transplant recipients had died) was only marginally reduced in the > 65 years group (3.8 years) compared to the younger 50-65 years group (4.3 years) and perhaps this is a point that requires more emphasis in routine clinical practice in addition to more timely referral for transplant.
REFERENCES
1.Flint A. A treatise on the principles and practice of medicine. Henry C Lea, 1868:193
2.Corrigan DJ. On cirrhosis of the lung. Dublin J Med Sci 1838;13:266.
3.National Institute for Health and Care Excellence. Idiopathic pulmonary fibrosis: the diagnosis and management of suspected idiopathic pulmonary fibrosis. 2013. http://guidance.nice.org.uk/CG163.
4.Bradley B, Branley HM, Egan JJ, Greaves MS, Hansell DM, Harrison NK, et al. Interstitial lung disease guideline: the British Thoracic Society in collaboration with the Thoracic Society of Australia and New Zealand and the Irish Thoracic Society. Thorax 2008;63(suppl 5):v1-58.
5.International Society for Heart and Lung Transplanation website accessed 31st December 2013 at http://www.ishlt.org/registries/slides.asp?slides=heartLungRegistry
Competing interests: No competing interests