Study finds interstitial lung disease often misdiagnosed in early stages

By Melanie Hinze

More than 95% of patients with interstitial lung disease (ILD) are misdiagnosed with an alternative respiratory condition or heart failure before their ILD diagnosis, according to research published in Respirology.

The study authors also found that these patients presented with key signs and symptoms, often multiple times, even 10 years before their diagnosis.

The research identified incident ILD cases (n=18,914) from the UK Optimum Patient Care Research Database, a longitudinal healthcare database comprising anonymised electronic health records for more than 25 million patients who attended more than 1000 primary care practices across the UK. They then investigated the prevalence and timing of symptoms and compared signs and health patterns between patients with ILD and 60,156 matched controls in the 10 years preceding diagnosis.

The authors found that patients with ILD had symptoms of dyspnoea, cough, fatigue, weight loss and loss of appetite recorded more often than control patients.

In total, 73% of patients with ILD reported cough or dyspnoea at least once at primary care visits. Furthermore, 13% had dyspnoea recorded on five or more occasions, compared with 5% of controls, and 17% had cough recorded on five or more occasions, compared with 7% of controls.

One of the authors, Professor Tamera Corte, Director of Interstitial Lung Disease, Royal Prince Alfred Hospital, and Chief Investigator at the Centre of Research Excellence for Pulmonary Fibrosis, The University of Sydney, Sydney, said the main finding was that patients with ILD were presenting to their GPs with cough and breathlessness more than control patients, up to 10 years prior to diagnosis.

‘The presentations increased in the two years prior to diagnosis,’ she said. ‘It is really important for GPs to think about pulmonary fibrosis when patients are presenting, especially when they have repeat presentations with cough and breathlessness,’ she added.

‘These data provide a major opportunity for intervention with education and awareness campaigns aiming to improve early diagnosis and prevent treatment delays and ultimately improve the care of people living with ILD,’ wrote the authors, led by Dr Caitlin Fermoyle, a postdoctoral researcher at the Centre of Research Excellence for Pulmonary Fibrosis in Sydney.

Professor Corte told Medicine Today that nowadays there was good evidence that early diagnosis made a difference, and early referral for treatment improved survival.

‘I really hope this [study] will encourage GPs to think about pulmonary fibrosis and consider referring their patients for a CT scan early.’

Respirology 2026; doi: 10.1002/resp.70230.