Australia’s national registry of patients with idiopathic pulmonary fibrosis (IPF) includes patients of diverse ages, disease severity and co-morbidities, and shows that baseline lung function and GAP (gender, age, physiology) stage are important prognostic markers for mortality.
Large patient registries help physicians understand the natural history and clinical management of IPF patients. The Australian IPF Registry (AIPFR) collects data from IPF patients across Australia.
Using AIPFR, authors assessed the clinical environment and long-term outcomes of 647 IPF patients and compared their findings to other registries and clinical trial cohorts. These include the INSIGHTS-IPF Registry, which includes 502 patients in Germany, 111 in Finland and 71 in Sweden, as well as two clinical trials, the ASCEND study of 278 patients (NCT01366209) and the INPULSIS-2 study of 329 patients (NCT01335477).
Their analysis showed that contrary to many cohorts in clinical trials, patients in the AIPFR are diverse, showing a wide range of age, severity of lung function impairment and co-morbidities. The cumulative mortality rate — or proportion of a group that dies over a specified time interval — was 5 percent in the first year, 24 percent in the second, 37 percent in the third and 44 percent in the fourth year.
This was the first time researchers collected such data from a national IPF registry. Researchers also observed that baseline lung function, a result of several parameters (forced vital capacity, diffusing capacity of the lung for carbon monoxide, composite physiological index), and GAP were strong predictors of mortality.
Patients who received anti-fibrotic medications lived longer compared to those who did not, independently of the severity of their disease, researchers said. Overall, they concluded, “these data support the crucial role that large longitudinal registries such as the AIPFR can play in better understanding the spectrum, natural history and clinical management of IPF in a real-world setting.”