Dr Lavanya Raman

The antifibrotic nintedanib significantly slows lung function decline in non-IPF progressive pulmonary fibrosis (PPF), according to ‘real-world’ data from the UK.

A retrospective study reviewed outcomes of treatment in eight specialist centres and 126 non-IPF PPF patients who commenced nintedanib in 2019/2020 via an early access program.

At the start of treatment the most prescribed immunosuppressive drugs were prednisolone (68%; median dose 10mg) and mycophenolate mofetil (56%; 2mg). Also, 69% of all patients were prescribed a steroid sparing agent and 63% oxygen.

The research team, led by respiratory physician Dr Lavanya Raman from the Royal Brompton Hospital, London, found that in 123 patients with available data lung function decline was significantly lower in the 12 months after starting nintedanib treatment than in the 12 months before, with FVC values of -88.8ml vs -239.9mL, respectively (p=0.004).

Similarly, in 114 patients with data an absolute decline in DLco of -2.1% was recorded after nintedanib treatment compared to -6.1% before (p=0.004), according to the study published in ERJ Open Research.

Sensitivity analyses showed that 51% of (126) patients had complete lung function. Those with incomplete lung function were found to be older in age (mean age 62.7 versus 57.8 years, p=0.034) with more severe MRC dyspnoea scores (p=0.002), but there were no differences in gender, baseline FVC or ppDLco, diagnosis or CT pattern, the researchers noted.

Data from patients who survived to follow up (n=108/126) revealed an increase in the number reporting symptom improvement after starting treatment with nintedanib, including: shortness of breath – 26% after 12 months’ treatment versus 12% in the 12 months before; and exercise tolerance – 30% versus 13%, respectively.

However, there was no such impact on cough, a finding which the authors said highlights an unmet need for PPF patients.

On safety and tolerability, diarrhoea was the most common side effect (39%), and most commonly linked to a reduction in nintedanib dose, and hepatotoxicity was reported 6% patients.

No serious adverse events

But there were no serious adverse events linked to the drug, and 80% of surviving patients were still taking it 12 months after treatment initiation.

Also of note, a reduction in nintedanib dose did not seem to impact its effect on lung function stabilisation, and similarly a change in immunosuppressive treatment following treatment initiation was not associated with a difference in rate of lung function change, according to the paper.

The study, which has provided the first ‘real-world’ look at the antifibrotic’s performance in patients with non-IPF PPF, has shown that “the decline of lung function is significantly attenuated following the introduction of nintedanib”, the investigators concluded.

In addition, nintedanib “did not appear to have excess deleterious effects when taken in combination with other immunosuppressants, supporting its use for the pharmacological treatment of non-IPF ILD where progression has occurred despite optimal management,” they added.