Idiopathic pulmonary fibrosis (IPF) is a progressive fibrosing interstitial lung disease (ILD) characterised by a decline in lung function. 

Measuring its decline effectively may guide better treatment decisions and further optimise disease management. Assessing disease progression accurately is another challenge and due to the lack of effective tools available to reliably predict lung function decline, Dr Marlies Wijsenbeek from the Erasmus University Medical Centre Rotterdam, The Netherlands, presented insights into forced vital capacity (FVC) monitoring using home spirometry at the European Respiratory Society (ERS) in Madrid recently.

Monitoring FVC using home spirometry can actively engage patients to see their data which will facilitate a more collaborative patient-clinician relationship. This also enables a patient-tailored detection of disease decline and will negate the burden of standard clinic visits.

The STARLINER study (NCT03261037) is an ongoing, international, multicenter study designed to characterise disease behaviour during the peri-diagnostic period in patients with suspected ILD. 

Dr Wijsenbeek presented data from a pre-planned interim analysis of STARLINER, which aimed to investigate changes in lung function, physical functional activity, symptoms and health-related quality of life during the peri-diagnostic period in patients with IPF and non-IPF ILD. It emphasised how disease behaviour can be used as a tool to facilitate diagnosis where patients can provide accurate and acceptable reading via home spirometry. Of the 6,734 spirometry blows provided by patients with IPF, 53.8% were ‘good’, 18.5% were ‘acceptable’ and 27.7% were ‘bad’. Although it was reported that a ‘majority was good’, compliance with home spirometry fluctuated in over 3000 patients making it a relatively high variable. 

Dr Toby Maher, consultant respiratory physician at the Royal Brompton Hospital, discussed the correlation between home and clinic spirometry in subjects with IPF: results from the INMARK® trial. In subjects with IPF and preserved FVC, home and clinic measurements of FVC and Forced expiratory volume in 6 seconds (FEV6) at individual visits over 52 weeks were strongly correlated, but there was a weak correlation between home- and clinic-based measurements of changes in lung function. This appeared to be due to the variability in changes from baseline in FVC measured using home spirometry, which was greater than the variability seen using clinic spirometry. These findings suggest that home spirometry is less robust than clinic spirometry as a measure of changes in lung function over time.

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Characterising disease behaviour is highly relevant to the entire spectrum of ILD and has been a key subject throughout the ERS congress. Its significance in ILD evolution is increasing as it provides a conceptual basis for evaluating treatment interventions. GlobalData believes that solely focusing on disease behaviour must not overshadow decades of experience which has been the basis of the current idiopathic interstitial pneumonia (IIP) classification. These approaches are not competitive, they are designed to complement disease progression and pharmacological therapies. 

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