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Abstract
Introduction and Objectives Idiopathic pulmonary fibrosis (IPF) is a progressive and invariably fatal disease. Historical cohort studies have reported a median survival of 2.8–3.2 years1. The UK has seen a number of important developments for IPF patients in the last year which it is hoped will translate to improved outcomes for individuals with this devastating disease. These include: the licensing and approval by NICE of the first anti fibrotic therapy, pirfenidone; specialist commissioning of ILD services; and publication of NICE IPF guidelines. If the impact of these important developments is to be measured it is vital that current IPF disease burden is understood.
Methods The incidence and mortality of IPF-Clinical Syndrome, using broad and narrow disease definitions, was assessed via the Clinical Practice Research Datalink (CPRD) GOLD dataset.
Results The incidence of broadly defined IPF-CS between 2000–2012 was 8.65 (95% CI 8.40–8.90) per 100,000 person years and significantly increased over time (incidence rate ratio adjusted by gender, region and age category = 1.02, p for linear trend <0.0001). Kaplan-Meier survival estimates show no significant survival difference by year of diagnosis (log rank test for equality of survivor functions, p = 0.17) with a median survival of 3.0 years (95% CI 2.8–3.1). There was no significant survival difference for broad and narrow definitions (log rank test for equality of survivor functions, p = 0.06) validating the definition of IPF-CS.
Conclusions In keeping with previous data the incidence of IPF-CS continues to rise and the survival time from diagnosis remains unchanged. The mortality burden of IPF-CS is therefore increasing. The results of this study provide an important benchmark against which the effects of changes in the management and delivery of care for individuals with IPF can be measured.
References
Navaratnam V, Fleming KM, West J, Smith CJ, Jenkins RG, Fogarty A, Hubbard RB. The rising incidence of idiopathic pulmonary fibrosis in the U. K. Thorax. 2011;66:462–7.