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Clinical judgement in the diagnosis of IPF

Prof Athol Wells, Consultant Respiratory Physician in the Royal Brompton Hospital and the National Heart and Lung Institute Imperial College London, UK, believes the current guidelines in the treatment of idiopathic pulmonary fibrosis (IPF) are “broken”.

Prof Wells made the remark about the guidelines, which were produced by the American Thoracic Society and Japanese Respiratory Society and the Latin American Thoracic Society in 2011, at the 2015 Irish Thoracic Society Annual Scientific Meeting, which took place in Cork in November.

He said that the guidelines were developed at a time before definitive treatment for IPF was available and when the IFIGENIA study and pirfenidone data were inconclusive. Also inclusion in trials was considered best management at the time, which is referenced in the 2011 guidelines.

During this time triple therapy (prednisone, azathioprine, and N-acetylcysteine (NAC)) was widely used to treat IPF and in patients with probable or possible IPF and differentials of chronic hypersensitivity or chronic fibrotic nonspecific interstitial pneumonia (NSIP). However, the PANTHER-IPF study found that currently used triple-therapy is a potentially harmful combination for people with IPF.

“There was a problem in the design of the study because far too much steroid was used in elderly patients and I think you can see where bad events happened it was during that period of high-dose steroid,” Prof Wells told the Medical Independent. “Nevertheless, any idea that the treatment was good in IPF, even with lower-dose steroid, now looks rather unlikely. Until then you could say it doesn’t matter too much if you can’t diagnose IPF because you would use triple therapy. It is what people would use in IPF and by the way it is the right sort of treatment approach for the alternatives. Now you are in a position where if it is IPF you shouldn’t use this treatment.”

…the guidelines were developed at a time before definitive treatment for IPF was available and when IFIGENIA and pirfenidone data were inconclusive. 

Prof Wells said that following the PANTHER findings, it is more important to be able to definitely diagnose IPF. However, he maintained that the guidelines are too restrictive and, if followed, leave up to half of patients unclassified.

“I think for those patients that satisfy guideline criteria it makes the diagnosis very efficient and for those patients the guidelines are a good thing,” Prof Wells said.

“The trouble is it disenfranchises half of IPF patients. You can’t reach the diagnosis, you can’t diagnose anything else. So they have got diseases without a name.”

The only way that the patients can be diagnosed, in accordance with the guidelines, is by doing a biopsy, which Dr Wells said is not always possible because of the risks involved.

“What you are really saying is that in these cases it is very rare for the diagnosis to be 50/50, it is IPF, it is not IPF,” according to Prof Wells.

“The important thing to remember is that you can talk about diagnosis, meaning not just is it IPF, but can you be exact about the alternatives? But the alternatives you could group together, they broadly get treated the same way. Although very accurate diagnosis means you should also focus even if you are sure it is not IPF on whether you can be accurate as to what it is. The thing that matters practically is, is it IPF or not? The treatments are quite different, the outcomes are quite different. So really it is rare to say it is 50/50, what you often say is it is 60/70 per cent likely it is IPF and then try to push that up to 90/95 per cent through close examination and other processes.”

In arriving at a diagnosis, Prof Wells said that clinical judgement, along with multidisciplinary team input is key. Observing the history of disease, using the bronchoalveolar lavage test, and examining whether there is subtle evidence of auto-immune disease are ways to arrive at a diagnosis of IPF even if the guidelines say it is unclassifiable.

Prof Wells was part of the team behind a recent study, published online in the American of Journal Respiratory and Critical Care Medicine, showing the benefit of bronchoscopic lung cryobiopsy. The method allows tissue to be removed without undue trauma and much larger biopsies than those with the traditional transbronchial biopsy.

“What this means is two things,” according to Prof Wells.

“You can now present different risk information to patients. And they are much more likely to accept the much lower risk with the benefits of the diagnosis. And you can actually biopsy patients that are more severe without the same severity risk as you would with a VATS [video-assisted thoracic surgery] biopsy.

“What that means in effect is quite a large subgroup of those patients who had a disease without a name you are going to be able to show now have tissue that is IPF-like. That is going to be enough to allow you to diagnose IPF efficiently.  That is still not going to be all patients, and you still going to have to use clinical judgement and clinical reasoning,” he concluded.

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