Abstract
Introduction: Idiopathic pulmonary fibrosis (IPF) is a progressive lung disease leading to respiratory failure and death. Treatment with Pirfenidone reduces %FVC decline and improves progression-free survival at 1 year. A subset of patients remain on treatment for ≥3 years; it is unclear whether specific characteristics within this group are identifiable at baseline.
Aim: Identify the proportion and characteristics of patients in a UK ILD centre maintained on Pirfenidone ≥3 years. Risk stratification based on these characteristics is examined as a method to target nursing support.
Methodology: This retrospective study examined IPF patients with potential Pirfenidone exposure of ≥3 years (n=103). Data derived from the clinical records of the Oxford ILD service.
Results: 21 patients had uninterrupted treatment for ≥3 years; initiation FVC 70% predicted, BMI 30.5 and TLco 54%. This group demonstrated stabilisation of lung function; mean FVC 66% and TLco 49% at end of year 3.
82 patients failed to complete 3 years; no difference in age or baseline FVC, with lower BMI and TLco more prevalent. 38 patients; mean baseline BMI 27.3 and TLco 45.9% stopped treatment due to side effects. 23 patients died on treatment; mean starting BMI of 27.7 and TLco 37.7%. 21 patients experienced disease progression while on full treatment dose.
Conclusion: A subset of patients tolerate Pirfendone well, with reduced burden of disease (TLco >50%) and BMI>30 at start. Lower TLco and BMI reflects a higher risk of side effect or death during the first year. Nursing resource is stratified to target those at higher risk, with 2 weekly phone contact, bespoke titration regime and proactive side effect management.
Footnotes
Cite this article as: European Respiratory Journal 2020; 56: Suppl. 64, 780.
This abstract was presented at the 2020 ERS International Congress, in session “Respiratory viruses in the "pre COVID-19" era”.
This is an ERS International Congress abstract. No full-text version is available. Further material to accompany this abstract may be available at www.ers-education.org (ERS member access only).
- Copyright ©the authors 2020