Differing Clinical Courses of Ipf at an Acute District General Hospital

Introduction IPF is more prevalent in males and smokers and tends to present in the 6th and 7th decades, with a 2 to 3 year prognosis. Data suggests rapid and slow progressors exist but phenotypic differences are yet to be identified (Raghu et al, Am J Respir Crit Care Med. 2011; 183: 788-824). Aims and Methods Patients with pulmonary fibrosis who died between 2006 and 2012 were identified(n=76). 18 were excluded: 11 had no evidence of fibrosis and 7 had diseases associated with ILD. Of those remaining (n=58), demographics, disease associations and course were examined. Patients were separated into rapid progressors (top quartile) and slow progressors (lower 3 quartiles) by time from diagnosis to death. Our aim was to determine if phenotypic differences were apparent. Results Mean age at onset was 74(± 1.47) years. Male:Female ratio was 2:1 and 56.9% had a smoking history. Survival from diagnosis until death was 34(± 5.13) months. Rapid and slow progressor phenotypes are shown below. ![Figure][1] Conclusion In comparison to published IPF data on peak incidence, patients were older, however survival was similar to that described. Longer survival is associated with younger age at diagnosis, smoking history, use of proton pump inhibitors, drug treatment and respiratory follow up. Identifying characteristics of rapid progressors may begin to help us understand factors influencing progression. These insights will help shape services for our current IPF population. [1]: pending:yes