The patient journey and burden of disease in progressive pulmonary fibrosis in Japan: a cross-sectional survey
BackgroundFor patients with interstitial lung diseases (ILDs) other than idiopathic pulmonary fibrosis (IPF) with a progressive pulmonary fibrosis (PPF) phenotype, current knowledge of patient experience and symptom burden is limited. This study aimed to describe the patient journey for patients wit...
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Frontiers Media S.A.
2025-04-01
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| Series: | Frontiers in Medicine |
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| Online Access: | https://www.frontiersin.org/articles/10.3389/fmed.2025.1526530/full |
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| author | Yuko Kaneko Yuko Waseda Hidekata Yasuoka Masateru Okazaki Shoko Nagata Ryoko Iwasaki Mark Small Haruyuki Ishii |
| author_facet | Yuko Kaneko Yuko Waseda Hidekata Yasuoka Masateru Okazaki Shoko Nagata Ryoko Iwasaki Mark Small Haruyuki Ishii |
| author_sort | Yuko Kaneko |
| collection | DOAJ |
| description | BackgroundFor patients with interstitial lung diseases (ILDs) other than idiopathic pulmonary fibrosis (IPF) with a progressive pulmonary fibrosis (PPF) phenotype, current knowledge of patient experience and symptom burden is limited. This study aimed to describe the patient journey for patients with PPF and IPF in a real-world setting in Japan.MethodsData were analyzed from the Adelphi Real World PPF-ILD Disease Specific Programme™, a cross-sectional survey with elements of retrospective data collection of pulmonologists and rheumatologists in Japan from April to October 2022. Participants provided data for up to 12 consecutive patients with physician-confirmed ILD with a progressive phenotype. Analyses were descriptive, except Kappa (κ) statistic was used to measure the alignment between physician- and patient-reported symptom burden in the 4 weeks prior to survey date (poor agreement: κ =<0.00; slight 0.00–0.20; fair 0.21–0.40; moderate 0.41–0.60; substantial 0.61–0.80; almost perfect 1.00).ResultsA total of 63 physicians (43 pulmonologists and 20 rheumatologists) provided data on 382 patients (312 with PPF and 70 with IPF). These patients were also asked to complete a voluntary survey on their experience and symptoms. Mean time from first symptom to consultation was 14.1 months for IPF, 8.0 months for non-connective tissue disease (CTD)-associated ILDs, and 10.7 months for CTD-ILDs. Mean times from consultation to diagnosis were 7.1, 4.8, and 3.6 months, respectively. Perception of symptoms differed between physicians and patients with alignment ranging from poor (dysphagia, κ = –0.0296, p = 0.6217) to substantial (weight loss, κ = 0.6174, p = 0.001). Health-related quality of life (HRQoL) was consistently impaired in patients overall, but too few patients completed HRQoL instruments to compare IPF with other forms of ILD.ConclusionsThis real-world study expands our understanding of the patient journey for patients with PPF and IPF in Japan. Greater communication between patients and physicians is needed to shorten diagnostic delays and target treatment strategies to improve patient experience and overall outcomes. |
| format | Article |
| id | doaj-art-4ac1e9744cff4d49b85bdce569bea09a |
| institution | DOAJ |
| issn | 2296-858X |
| language | English |
| publishDate | 2025-04-01 |
| publisher | Frontiers Media S.A. |
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| series | Frontiers in Medicine |
| spelling | doaj-art-4ac1e9744cff4d49b85bdce569bea09a2025-08-20T03:06:20ZengFrontiers Media S.A.Frontiers in Medicine2296-858X2025-04-011210.3389/fmed.2025.15265301526530The patient journey and burden of disease in progressive pulmonary fibrosis in Japan: a cross-sectional surveyYuko Kaneko0Yuko Waseda1Hidekata Yasuoka2Masateru Okazaki3Shoko Nagata4Ryoko Iwasaki5Mark Small6Haruyuki Ishii7Division of Rheumatology, Department of Internal Medicine, School of Medicine, Keio University, Tokyo, JapanDivision of Respiratory Medicine, University of Fukui Hospital, Eiheiji, JapanDivision of Rheumatology, Department of Internal Medicine, School of Medicine, Fujita Health University, Toyoake, JapanNippon Boehringer Ingelheim Co., Ltd, Tokyo, JapanNippon Boehringer Ingelheim Co., Ltd, Tokyo, JapanNippon Boehringer Ingelheim Co., Ltd, Tokyo, JapanAdelphi Real World, Bollington, United KingdomDepartment of Respiratory Medicine, Faculty of Medicine, Kyorin University, Tokyo, JapanBackgroundFor patients with interstitial lung diseases (ILDs) other than idiopathic pulmonary fibrosis (IPF) with a progressive pulmonary fibrosis (PPF) phenotype, current knowledge of patient experience and symptom burden is limited. This study aimed to describe the patient journey for patients with PPF and IPF in a real-world setting in Japan.MethodsData were analyzed from the Adelphi Real World PPF-ILD Disease Specific Programme™, a cross-sectional survey with elements of retrospective data collection of pulmonologists and rheumatologists in Japan from April to October 2022. Participants provided data for up to 12 consecutive patients with physician-confirmed ILD with a progressive phenotype. Analyses were descriptive, except Kappa (κ) statistic was used to measure the alignment between physician- and patient-reported symptom burden in the 4 weeks prior to survey date (poor agreement: κ =<0.00; slight 0.00–0.20; fair 0.21–0.40; moderate 0.41–0.60; substantial 0.61–0.80; almost perfect 1.00).ResultsA total of 63 physicians (43 pulmonologists and 20 rheumatologists) provided data on 382 patients (312 with PPF and 70 with IPF). These patients were also asked to complete a voluntary survey on their experience and symptoms. Mean time from first symptom to consultation was 14.1 months for IPF, 8.0 months for non-connective tissue disease (CTD)-associated ILDs, and 10.7 months for CTD-ILDs. Mean times from consultation to diagnosis were 7.1, 4.8, and 3.6 months, respectively. Perception of symptoms differed between physicians and patients with alignment ranging from poor (dysphagia, κ = –0.0296, p = 0.6217) to substantial (weight loss, κ = 0.6174, p = 0.001). Health-related quality of life (HRQoL) was consistently impaired in patients overall, but too few patients completed HRQoL instruments to compare IPF with other forms of ILD.ConclusionsThis real-world study expands our understanding of the patient journey for patients with PPF and IPF in Japan. Greater communication between patients and physicians is needed to shorten diagnostic delays and target treatment strategies to improve patient experience and overall outcomes.https://www.frontiersin.org/articles/10.3389/fmed.2025.1526530/fullidiopathic pulmonary fibrosisprogressive pulmonary fibrosisinterstitial lung diseaseantifibroticstreatmentreal-world data |
| spellingShingle | Yuko Kaneko Yuko Waseda Hidekata Yasuoka Masateru Okazaki Shoko Nagata Ryoko Iwasaki Mark Small Haruyuki Ishii The patient journey and burden of disease in progressive pulmonary fibrosis in Japan: a cross-sectional survey Frontiers in Medicine idiopathic pulmonary fibrosis progressive pulmonary fibrosis interstitial lung disease antifibrotics treatment real-world data |
| title | The patient journey and burden of disease in progressive pulmonary fibrosis in Japan: a cross-sectional survey |
| title_full | The patient journey and burden of disease in progressive pulmonary fibrosis in Japan: a cross-sectional survey |
| title_fullStr | The patient journey and burden of disease in progressive pulmonary fibrosis in Japan: a cross-sectional survey |
| title_full_unstemmed | The patient journey and burden of disease in progressive pulmonary fibrosis in Japan: a cross-sectional survey |
| title_short | The patient journey and burden of disease in progressive pulmonary fibrosis in Japan: a cross-sectional survey |
| title_sort | patient journey and burden of disease in progressive pulmonary fibrosis in japan a cross sectional survey |
| topic | idiopathic pulmonary fibrosis progressive pulmonary fibrosis interstitial lung disease antifibrotics treatment real-world data |
| url | https://www.frontiersin.org/articles/10.3389/fmed.2025.1526530/full |
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