Baseline characteristics of idiopathic pulmonary fibrosis: analysis from the Australian Idiopathic Pulmonary Fibrosis Registry

Abstract

7The prevalence of idiopathic pulmonary fibrosis (IPF), a fatal and progressive lung disease, is estimated at 1.25-63 out of 100 000, making large population studies difficult. Recently, the need for large longitudinal registries to study IPF has been recognised.The Australian IPF Registry (AIPFR) is a national registry collating comprehensive longitudinal data of IPF patients across Australia. We explored the characteristics of this IPF cohort and the effect of demographic and physiological parameters and specific management on mortality.Participants in the AIPFR (n=647, mean age 70.9±8.5 years, 67.7% male, median follow up 2 years, range 6 months-4.5 years) displayed a wide range of age, disease severity and co-morbidities that is not present in clinical trial cohorts. The cumulative mortality rate in year one, two, three and four was 5%, 24%, 37% and 44% respectively. Baseline lung function (forced vital capacity, diffusing capacity of the lung for carbon monoxide, composite physiological index) and GAP (gender, age, physiology) stage (hazard ratio 4.64, 95% CI 3.33-6.47, p<0.001) were strong predictors of mortality. Patients receiving anti-fibrotic medications had better survival (hazard ratio 0.56, 95% CI 0.34-0.92, p=0.022) than those not on anti-fibrotic medications, independent of underlying disease severity.The AIPFR provides important insights into the understanding of the natural history and clinical management of IPF.

Staging of idiopathic pulmonary fibrosis

“…These difference confirmed both derivation cohort and validation cohort. (Table 1) This index is simple and applicable not only tertiary center but also general hospital (52,53).And GAP index was valuable as a measure of IPF severity (54)(55)(56)(57)(58)(59). Presence of Velcro Crackles is associated with high score of GAP in IPF (60).…”

Design, Rationale, Methodology, and Aims of a Greek Prospective Idiopathic Pulmonary Fibrosis Registry: Investigating Idiopathic Pulmonary Fibrosis in Greece (INDULGE IPF)

“…Long-term data on the natural course of IPF are missing. RCTs have proved the efficacy of novel agents after a short period of follow-up (up to 1 year), but epidemiological data show that the median survival of IPF patients may reach up to 5 years [4]. Therefore, the appropriate duration of treatment with novel agents as well as the possible benefit by sequential treatment with these agents cannot be determined by published RCTs.…”

“…However, only a small percentage of patients included in these registries were treated with novel agents – all as part of clinical trials or specialized access programs –, therefore further recording of patients is needed in order to assess the effect of novel agents in current clinical practice. Such registries are already ongoing [4, 26-28]. The last issue regarding the heterogeneity of a disease with relative low incidence and prevalence is the acquisition of data for the treatment of special subpopulations.…”

“…Furthermore, there is a lack of information on detailed patient characteristics. Registries conducted in Germany and Australia [4, 25] have provided valuable insights into the baseline characteristics and the natural course of IPF patients diagnosed after the publication of the consensus guidelines in 2011. However, only a small percentage of patients included in these registries were treated with novel agents – all as part of clinical trials or specialized access programs –, therefore further recording of patients is needed in order to assess the effect of novel agents in current clinical practice.…”

“…The decline in forced vital capacity (FVC) though is predictive of mortality. In general, IPF is the most severe among the idiopathic interstitial pneumonias and has a poor prognosis, with a median overall survival of 5 years [4]. …”