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IPF diagnosis and management starting to move forward


The relative excitement of having medications to slow disease progression in patients with idiopathic pulmonary fibrosis (IPF) is offset by the reality that they only help some patients.

According to a summary of the evidence, anti-fibrotic medications have a clinically meaningful impact in mild to moderate IPF but there is little data to guide treatment for other patients.

Lead author Dr Helen Jo, a respiratory physician at the Royal Prince Alfred Hospital, told the limbic there was obviously a lot more work to be done.

 “These are promising times for IPF but it’s all relative. There was nothing to stop the relentless progression of this disease but now, this year, we have two medications approved for use in IPF.”

She said there was possibly some off-label use of the drugs for more severe disease but it was expensive and not encouraged given the lack of evidence.

“Everyone is just excited about new medications but we want to temper that with the fact that these medications are not for everyone and that we need to approach patients as a whole.”

Importantly, management of common co-morbidities such as reflux, pulmonary hypertension and sleep disordered breathing was sometimes overlooked as an opportunity to prolong and improve quality of life.

Dr Jo said the evidence for benefit from pulmonary rehabilitation was quite good and especially for patients with milder disease.

And while the evidence for supplemental oxygen was derived from the literature for COPD, she said there was no reason to believe it wouldn’t also be beneficial in hypoxic IPF patients (PaO2 <55 mm Hg).

“The grey zone is for people who have oxygen levels above that but who desaturate on exercise,” she said.

“If ambulatory oxygen helps patients to be more active and do more then it should be considered on an individual basis however the few trials in ILD and IPF have had mixed results and not a big benefit.”

The review highlighted the importance of referring patients early for lung transplantation given the unpredictable disease course.

Dr Jo said there was no evidence to suggest IPF recurs in the transplanted lung but outcomes were obviously not as good as for cystic fibrosis given the older age of IPF patients and the presence of comorbidities.

She said there was promising work being done on both diagnostic and prognostic biomarkers for IPF, including locally through the Australian IPF Registry.

“The other thing that’s promising is cryobiopsy to confirm a diagnosis versus surgical lung biopsy which can be quite a risky in IPF.”

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