Prevalence of progressive fibrosing interstitial lung disease

A. Olson (Denver, Colorado, United States of America), N. Hartmann (Ingelheim am Rhein, Germany), R. Schlenker-Herceg (Ingelheim am Rhein, Germany), L. Wallace (Ridgefield, Connecticut, United States of America)

Source: International Congress 2018 – Pitfalls in the diagnosis and management of rare diffuse parenchymal lung diseases (DPLDs)

Session: Pitfalls in the diagnosis and management of rare diffuse parenchymal lung diseases (DPLDs)
Session type: Thematic Poster
Number: 3030

Disease area: Interstitial lung diseases

Abstract

Background: There is an important group of patients with a progressive fibrosing form of interstitial lung disease (PF-ILD) (Flaherty, K.R. et al. BMJ Open Respir Res 2017;4:e000212) who do not meet idiopathic pulmonary fibrosis (IPF) diagnostic criteria but have a similar natural history and prognosis. Compared to IPF, the relative prevalence of PF-ILD is unknown.

Aims and objectives: To estimate the prevalence of PF-ILD in Europe and the USA.

Methods: Systematic literature review in Medline and Embase (1990–2017) focused on the prevalence of ILD and the forms of ILD known to be at risk for a progressive fibrosing phenotype. Supplemented by data from physician surveys and interviews, prevalence estimates were generated for each subtype and then combined to estimate overall PF-ILD prevalence. Sensitivity analyses were performed to determine the upper bounds of the estimate.

Results: The overall prevalence of ILD (per 10,000 persons) was reported as 0.63–7.6 in 4 studies in Europe and 7.43 in the USA. Prevalence estimates for individual progressive fibrosing ILDs and PF-ILD overall are presented in the Table. PF-ILD prevalence (per 10,000 persons) ranged from 0.22–2.0 in Europe and was 2.80 in the USA.

Conclusions: PF-ILD affects fewer than 5 in 10,000 persons in Europe and the USA, a patient population with an unmet need for treatment.

Funding: Boehringer Ingelheim. Editorial assistance: Complete HealthVizion.

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A. Olson (Denver, Colorado, United States of America), N. Hartmann (Ingelheim am Rhein, Germany), R. Schlenker-Herceg (Ingelheim am Rhein, Germany), L. Wallace (Ridgefield, Connecticut, United States of America). Prevalence of progressive fibrosing interstitial lung disease. 3030

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