| Institution: | 1. Department of Pulmonary Medicine, Thoracic Center, St. Luke''s International Hospital, St. Luke''s International University, 9-1 Akashi-cho, Chuo-ku, Tokyo, 104-8560, Japan;2. Graduate School of Public Health, St. Luke''s International University, 9-1 Akashi-cho, Chuo-ku, Tokyo, 104-8560, Japan;3. Department of Social Medicine, National Center for Child Health and Development, 2-10-1 Okura, Setagaya-ku, Tokyo, 157-8535, Japan;1. Department of Radiology, The Jikei University School of Medicine, Tokyo, Japan;2. Division of Thoracic Surgery, Department of Surgery, The Jikei University School of Medicine, Tokyo, Japan;1. Department of Clinical Infectious Diseases, Aichi Medical University, Nagakute, Aichi, Japan;2. Department of Pathology, University of Michigan, Ann Arbor, MI, 48109, USA;3. Department of Rheumatology & Allergy, Shonan Fujisawa Tokushukai Hospital, Fujisawa, Kanagawa, Japan;4. Department of Pulmonology, Kameda Medical Center, Kamogawa, Chiba, Japan;1. Department of Respiratory Medicine, Miyakonojo Medical Association Hospital, Miyazaki, Japan;2. Department of Respiratory Medicine, Fukuoka University Chikushi Hospital, Fukuoka, Japan |
| Abstract: | BackgroundThe antifibrotic agent nintedanib has been reported to effectively prevent the decline in forced vital capacity (FVC) in a broad range of interstitial lung diseases. However, the efficacy of nintedanib against idiopathic pleuroparenchymal fibroelastosis (iPPFE) remains unclear.MethodsWe retrospectively examined patients with idiopathic PPFE or idiopathic pulmonary fibrosis (IPF) who received nintedanib for more than 6 months. We evaluated annual changes in %FVC, radiological PPFE lesions, and body weight before and during nintedanib treatment. To investigate radiological PPFE lesions, we examined the fibrosis score, which was defined as the mean percentage of the high attenuation area in the whole lung parenchyma using three axial computed tomography images.ResultsOverall, 15 patients with iPPFE and 27 patients with IPF were included in the present study. In patients with IPF, the annual rate of decline in %FVC was significantly lower during nintedanib treatment than that before treatment (?2.01%/year ?7.64 to 3.21] versus ?7.64%/year ?10.8 to ?4.44], p = 0.031). Meanwhile, in patients with iPPFE, the annual rate of decline in %FVC during nintedanib treatment was higher than that before treatment (?18.0%/year ?21.6 to ?12.7] versus ?9.40%/year ?12.3 to ?8.23], p = 0.109). In addition, nintedanib treatment failed to inhibit the annual rate of increase in fibrosis score in patients with iPPFE (6.53/year 1.18–15.3] during treatment versus 2.70/year 0.27–12.2] before treatment, p = 0.175).ConclusionsNintedanib efficacy may be limited in patients with iPPFE. |
