Background Nintedanib was shown to significantly slow the annual rate of decline in FVC in patients
with chronic progressive fibrosing interstitial lung diseases (PF-ILD/PPF). The effects
on the patient reported outcomes (PRO) dyspnea or cough have only been investigated
scarcely, especially in a real-world cohort.
Aim We hypothesize that in a 1-year observational study a correlation between changes
in lung function (FVC) and changes in the PROs dyspnea and cough of the Living with
Pulmonary Fibrosis (L-PF) questionnaire will be confirmed in a real-world patient
population as shown in the recent INBUILD trial.
Study Design After a shared decision making in a patient with PF-ILD/PPF nintedanib will be initiated
as standard of care. Within this prospective, single-arm, open-label, non-interventional
(NIS), real-world study 100 patients with PF-ILD/PPF in approximately 20 specialized
centers (pulmonologists, rheumatologists) throughout Germany will be recruited. Adults≥18
years with physician’s diagnosis of PF-ILD, (except idiopathic pulmonary fibrosis)
with a need of antifibrotic treatment, not currently hospitalized and with a life
expectancy>12 months per investigator’s assessment will be included. Exclusion criteria
are physician diagnosed exacerbation of ILD, current lung cancer or respiratory failure
(pH<7.35 and/or respiratory rate>30/min). Changes from baseline over 52 weeks after
initiation of nintedanib treatment in FVC (Δ FVC% predicted and mL) and changes in
cough and dyspnea scores (Δ cough, Δ dyspnea, absolute change) as measured with the
L-PF questionnaire will be correlated to baseline values, including a snapshot-analysis
at the time of last patient in (NCT04702893).
Outlook The study started in 02/2021 and first results are expected for 2024. Main baseline
characteristics will be described. Data from this real-world patient population with
PF-ILD will help to understand the impact of antifibrotic treatment on PROs in patients
with PF-ILD/PPF.
* Presenting on behalf of the authors
