Idiopathic Pulmonary Fibrosis update, comparing the Australasian Interstitial Lung Disease Registry to the Australian Idiopathic Pulmonary Fibrosis Registry.

BACKGROUND AND AIMS: Real-world evaluation of clinical characteristics and treatment profile of patients with idiopathic pulmonary fibrosis (IPF) is needed to establish areas for improvement according to evidence-based recommendations. This study evaluated the clinical characteristics and treatment profile of patients with IPF from the Australasian ILD Registry (AILDR), comparing them to the Australian IPF Registry (AIPFR) cohort, to identify differences in baseline characteristics among IPF patients treated with antifibrotics and immunosuppressants.

METHODS

Consecutive patients with IPF enrolled between May 2016 and December 2023 from the AILDR and data of patients enrolled in the AIPFR between 2012 and 2016 were included.

RESULTS

A total of 842 and 647 patients with IPF were included from the AILDR and the AIPFR respectively. Both cohorts were male predominant (AILDR: 72.0%; AIPFR: 67.7%) of similar mean body mass index (AILDR: 29.2 ± 4.9 kg/m2; AIPFR: 28.7 ± 4.8 kg/m2). With regard to disease-targeted treatment, 572 (67.9%) and 108 (12.8%) participants of the AILDR cohort were on antifibrotic medications and immunosuppressants respectively. There were a total of 35.6% (n = 300) IPF patients on pirfenidone and 40.4% (n = 340) on nintedanib in AILDR. Only 146 (23%) of the AIPFR cohort were receiving antifibrotics. Of newly registered patients in 2016 (n = 18), 2020 (n = 71) and 2023 (n = 151), 50.0%, 18.3% and 15.2% respectively had received immunosuppressive therapy.

CONCLUSION

Baseline demographics from the IPF cohorts for both AILDR and AIPFR are largely comparable, with increasing antifibrotic and decreasing use of immunosuppressants since 2016. Immunosuppressive therapy in IPF remains relatively higher than expected despite guideline recommendations.