Scientists from the Karolinska University Hospital, Sweden, reported findings from the first year of a national registry of idiopathic pulmonary fibrosis patients (IPF), which showed that Swedish IPF patients have a poor quality of life and poor perception of their disease — despite a relatively modest level of disease severity.
The study, “Idiopathic pulmonary fibrosis in Sweden: report from the first year of activity of the Swedish IPF-Registry,“ published in the European Clinical Respiratory Journal, underscored the importance of the web-based registry in identifying the needs of patients as well as in enabling future research.
While pulmonary fibrosis care is standardized in Sweden and in line with international guidelines, the high cost of treatment with pirfenidone (Esbriet) and nintedanib (Ofev), paired with weak evidence of outcomes on mortality, have prompted a discussion of how resources for pulmonary fibrosis patients should best be allocated within the healthcare system. A national registry will more easily identify these patients’ needs, allowing for both a better allocation of resources and more targeted research.
The registry uses a web-based platform, and collects data on demographics, lung function, radiology, quality of life, ongoing treatments, adverse events, follow-up, and outcomes such as death and lung transplantation.
Study findings reported that in the registry’s first year of implementation, from September 2014 to October 2015, a total 41 percent of the country’s respiratory units connected to it, and 33 percent started reporting data.
During that time, 71 patients were registered, of which 70.4 percent were male. The findings also showed that diagnoses were based on radiological features in 72 percent of the patients, while 14 percent had a lung biopsy, and an additional 20 percent had a diagnosis after a clinical conference.
Patient characteristics were similar to what has been reported in other registries and studies, with a median age of 70 at diagnosis but patients ranging from 47 to 86 years old, and a high proportion of ex-smokers. While most patients in the registry had mild or moderate disease severity, they nonetheless reported a low quality of life.
The study underscores that implementing a nationwide and web-based tool can be rapidly executed, and that such tools could considerably assist future research assessments of pulmonary fibrosis in a country.