Patients in the Australian Idiopathic Pulmonary Fibrosis Registry who take anti-fibrotic therapies tend to live longer than those who do not, according to an analysis of the records.

Another finding was there is a broad range of age, lung-function impairment and additional diseases among those in the registry.

The analysis, “Baseline characteristics of idiopathic pulmonary fibrosis: analysis from the Australian Idiopathic Pulmonary Fibrosis Registry,” was published in the European Respiratory Journal.

Scientists created the registry in 2012 to get a real-world sense of the characteristics of IPF patients. The picture the registry offers contrasts with that provided by clinical trials of selected patient populations.

It is difficult to obtain statistics on features of the disease because relatively few people develop it. The idea of the registry is to gather as much information as possible in one place to obtain a better understanding of the disease and its course.

Six hundred forty-seven people were listed in the registry at the time of the analysis. Their average age was 70.9 years, and 67.7% were men.  One hundred eighty-five patients died and 26 had a lung transplant during a post-analysis follow-up period. The follow-up period ranged from six month to 4 1/2 years, depending on the patient.

Compared with clinical trials, the registry offered a wide range of patients’ ages, the severity of their disease, and co-morbidity, or the presence of other diseases. As an example, the INPULSIS (NCT03047031) and ASCEND (NCT01366209) trials assessed much more specific groups of IPF patients.

The death rates of those in the registry were 5 percent in the first year of the analysis, 24 percent in the second, 37 percent in the third and 44 in the fourth.

Poorer lung function before the analysis began and a higher risk of death were strong predictors of mortality, researchers said. The team used a measure called GAP stage to determine risk of death. It is a screening method based on gender, age, and measures of lung function.

The most common co-morbidity among patients in the registry was gastro-esophageal reflux disease, seen in 233 or 41 percent of participants. It was followed by chronic obstructive pulmonary disease (COPD) in 197 or 36 percent and coronary artery disease in 161 or 28 percent. Connective tissue disease was reported in 55 or 10 percent of patients.

Although Esbriet (pirfenidone) and Ofev (nintedanib) — two anti-fibrotic IPF therapies approved in the United States — are not available through government-funded programs in Australia, 146 patients, or 23 percent, obtained them through other means. They were younger, more often men, and able to walk further on a measure of exercise capacity called the six-minute walk test.

“In conclusion, we report a large national IPF cohort with outcome data extending up to 4 years,” the researchers said. “Patients from the AIPFR [registry] are diverse, with a broad range of age, severity of lung function impairment and co-morbidities. Baseline lung function, GAP stage and PROMs [patient-reported outcome measures] were strong prognostic indicators in our cohort, and those on anti-fibrotic therapy did better independent of their underlying disease severity.

“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,” the team concluded.