Treatment of idiopathic pulmonary fibrosis (IPF) with nintedanib: an experience from the compassionate use program (CUP) in Estonia

Abstract

Background: Nintedanib was the first approved anti-IPF drug available in Estonia in the frame of CUP. Aim: To evaluate the clinical efficacy and safety of nintedanib in a CUP cohort of IPF patients in Estonia. Methods: Treatment-naive patients aged ≥40 years with IPF diagnosed according to the ATS/ERS/JRS/ALAT IPF 2011 guidelines, forced vital capacity (FVC) ≥50% predicted and diffusing capacity of the lung (DLco) 30%-79% predicted were successively assigned for treatment with nintedanib. Clinical, lung function and adverse event (AE) data were recorded. Results: Seventeen patients (13 males/82.3%, 8 prevalent cases/47.1%, 8 having the diagnosis proven with lung biopsy/47.1%) aged 67.0±1.6 years (mean±SEM) with IPF (FVC 71.5±4.1%, DLco 38.7±2.7%, GAP score 4.3±0.3) started treatment with nintedanib at 2 centers 22.4±7.5 months after the diagnosis. The CUP period lasted 10.4±0.9 months and was followed by routine treatment with nintedanib or pirfenidone. FVC was stable (changed by 10%, p=0.029). The most common AEs, diarrhea (52.9%), increased hepatic aminotransferases (23.5%) and stomachache (17.7%), reduced over time. Four patients (23.5%) had >1 side effects. Dose reduction and treatment interruption were necessary in 35.3% and 35.3% of the CUP participants, respectively. Conclusions: Nintedanib stabilized lung function in the majority of IPF patients and was moderately well-tolerated. The current results are in line with those obtained by former authors. Funded by: Boehringer Ingelheim International GmbH