Comorbidity burden and survival in patients with idiopathic pulmonary fibrosis: the EMPIRE registry study

Abstract

Background Patients with idiopathic pulmonary fibrosis (IPF) frequently have multiple comorbidities, which may influence survival but go under-recognised in clinical practice. We therefore report comorbidity, antifibrotic treatment use and survival of patients with IPF observed in the multi-national EMPIRE registry. Methods For this prospective IPF cohort, demographics, comorbidities, survival and causes of death were analysed. Comorbidities were noted by the treating physician based on the patient’s past medical history or as reported during follow-up. Comorbidities were defined as prevalent when noted at enrolment, or as incident when recorded during follow-up. Survival was analysed by Kaplan–Meier estimates, log-rank test, and Cox proportional hazards models. Hazard ratios (HR) were adjusted for gender, age, smoking status and FVC at enrolment. Results A population of 3,580 patients with IPF from 11 Central and Eastern European countries was followed every 6 months for up to 6 years. At enrolment, 91.3% of patients reported at least one comorbidity, whereas more than one-third (37.8%) reported four or more comorbidities. Five-year survival was 53.7% in patients with no prevalent comorbidities, whereas it was 48.4%, 47.0%, 43.8% and 41.1% in patients with 1, 2, 3 and ≥ 4 comorbidities, respectively. The presence of multiple comorbidities at enrolment was associated with significantly worse survival (log-rank test P = 0.007). Adjusted HRs indicate that risk of death was increased by 44% in patients with IPF reporting ≥ 4 comorbidities at baseline compared with no comorbidity (P = 0.021). The relationship between number of comorbidities and decreased survival was also seen in patients receiving antifibrotic treatment (63% of all patients; log-rank test P < 0.001). Comorbidity as cause of death was identified in at least 26.1% of deaths. Conclusions The majority of patients with IPF demonstrate comorbidities, and many have comorbidity-related deaths. Increasing numbers of comorbidities are associated with worse survival; and this pattern is also present in patients receiving antifibrotic therapy.

Delineating excess comorbidities in idiopathic pulmonary fibrosis: an observational study

“…The burden of comorbidity in patients with IPF has not been studied as extensively as it has in COPD patients. Estimates of comorbidity prevalence vary widely between studies, but the rates of multiple prevalent comorbidity identified in our study are in line with those documented in prior registry studies [ 9 , 27 ]. Regarding individual comorbidities, previous studies have generated comparable estimates of the prevalence of hypertension [ 9 , 28 ], cardiac disease [ 9 , 27 ], thyroid disease [ 27 ], GERD [ 9 ], cerebrovascular disease [ 10 ], heart failure [ 10 ] in IPF populations (Table 1 ).…”

“…Estimates of comorbidity prevalence vary widely between studies, but the rates of multiple prevalent comorbidity identified in our study are in line with those documented in prior registry studies [ 9 , 27 ]. Regarding individual comorbidities, previous studies have generated comparable estimates of the prevalence of hypertension [ 9 , 28 ], cardiac disease [ 9 , 27 ], thyroid disease [ 27 ], GERD [ 9 ], cerebrovascular disease [ 10 ], heart failure [ 10 ] in IPF populations (Table 1 ). The prevalence of diabetes was lower in our IPF cohort than prior studies (11% versus 23–45%) [ 9 , 10 , 29 ], but depression was more commonly seen in our study than in prior reports (23% versus 3–15%) [ 9 , 10 , 29 ].…”

Ectopic and visceral fat deposition in aging, obesity, and idiopathic pulmonary fibrosis: an interconnected role

“…The EMPIRE registry study included 3,580 IPF patients from multiple countries, and at the time of enrollment, 91.3% of patients had been diagnosed with at least one comorbidity, with over one-third (37.8%) reporting four or more comorbidities. The 5-year survival rates for patients without common complications and with 1, 2, 3, and ≥ 4 complications were 53.7%, 48.4%, 47.0%, 43.8%, and 41.1%, respectively [120].…”

The Impact of Chronic Comorbidities on Outcomes in Acute Exacerbations of Idiopathic Pulmonary Fibrosis

“…Our study addresses a gap in knowledge by identifying comorbidities associated with poor prognosis specifically in hospitalized patients with AE-IPF. The burden of comorbidities is noted to be high among IPF patients, and increasing number of comorbidities has been associated with worse survival [37]. However, to our knowledge, our study is unique in examining the impact of comorbidity on mortality in AE-IPF.…”