Platythorax progresses with lung involvement in pleuroparenchymal fibroelastosis |
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| Affiliation: | 1. Department of Medical Oncology, Izumi City General Hospital, 4-5-1 Wake, Izumi, Osaka, 594-0073, Japan;2. Department of Medical Oncology, Kindai University Faculty of Medicine, 377-2, Ohno-higashi, Osaka-Sayama, Osaka, 589-8511, Japan;3. Department of Hematology, Izumi City General Hospital, 4-5-1, Wake, Izumi, Osaka, 594-0073, Japan;4. Department of General Thoracic Surgery, Osaka University Graduate School of Medicine, 2-2-L5 Yamadaoka, Suita, Osaka, 565- 0871, Japan;5. Department of Pathology, Nagasaki University Graduate School of Biomedical Sciences, 1-7-1, Sakamoto, Nagasaki, 852-8501, Japan;6. Department of Diagnostic Pathology, Tokyo Medical University Hachioji Medical Center, 1163 Tatemachi, Hachioji-shi, Tokyo, 193-0998, Japan;7. Department of Pathology, Keiyu Hospital, 3-7-3 Minatomirai Nishi-ku, Yokohama-shi, Kanagawa, 220-8521, Japan;8. Department of Pathology, School of Medicine, International University of Health and Welfare, 1-4-3 Mita, Minato-ku, Tokyo, 108-8329, Japan;1. Department of Respiratory Medicine, Iizuka Hospital, Iizuka, Japan;2. Department of Respiratory Medicine, Juntendo University, School of Medicine, Tokyo, Japan;1. Department of Respiratory Medicine, Kobe City Medical Center General Hospital, 2-1-1, Minatojimaminamimachi, Chuo-ku, Kobe-city, Hyogo, 650-0047, Japan;2. Department of Respiratory Medicine, Ichinomiyanishi Hospital, 1 Kaimeihira, Ichinomiya-shi, Aichi, 494-0001, Japan;3. Post Graduate Education Center, Kameda Medical Center, 929 Higashi-cho, Kamogawa City, Chiba, 296-8602, Japan;4. Department of Thoracic Medicine, Saiseikai Yokohamashi Tobu Hospital, 3-6-1 Shimosueyoshi Tsurumi-ku, Yokohama-city, Yokohama, 230-8765, Japan;5. Department of Internal Medicine, Kyoto Min-Iren Asukai Hospital, Asukai-cho 89, Tanaka, Sakyo-ku, Kyoto-city, Kyoto, 606-8226, Japan;6. Section of Clinical Epidemiology, Department of Community Medicine, Kyoto University Graduate School of Medicine, Yoshida Konoe-cho, Sakyo-ku, Kyoto-city, Kyoto, 606-8501, Japan;7. Department of Healthcare Epidemiology, Graduate School of Medicine and Public Health, Kyoto University, Yoshida Konoe-cho, Sakyo-ku, Kyoto-city, Kyoto, 606-8501, Japan;1. School of Medical Technology, Faculty of Health and Medical Care, Saitama Medical University, 1397-1 Yamane, Hidaka, Saitama 350-1241, Japan;2. Department of Respiratory Medicine, Saitama Medical University Hospital, 38 Morohongo, Moroyama-machi, Iruma-gun, Saitama 350-0400, Japan;1. Department of Respiratory Medicine, Okayama City Hospital, 3-20-1, Kitanagaseomote-cho, Kita-Ku, Okayama, 700-8557, Japan;2. Department of Cardiovascular Medicine, Okayama City Hospital, 3-20-1, Kitanagaseomote-cho, Kita-Ku, Okayama, 700-8557, Japan;3. Department of Radiology, Okayama City Hospital, 3-20-1, Kitanagaseomote-cho, Kita-Ku, Okayama, 700-8557, Japan |
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| Abstract: | ![]()
BackgroundPatients diagnosed with pleuroparenchymal fibroelastosis (PPFE) exhibit unique clinical features, including upper lobe-dominant lung involvement and platythorax (or flattened thoracic cage). Although platythorax have been shown to be a sign of disease progression, the temporal relationship between the progression of platythorax and the extent of lung involvement has not been closely investigated.MethodsWe retrospectively investigated patients diagnosed with PPFE, who did not exhibit fibrotic lesions other than PPFE in the lower lobes. We estimated the fibrosis score, which is a visual score indicating the percentage of lung parenchyma occupied by the disease on computed tomography images selected every 2 cm from the lung apex to the lung base, and the flat chest index (the ratio of the anteroposterior diameter of the thoracic cage to the transverse diameter of the thoracic cage). Additionally, we investigated serial changes in the flat chest index and fibrosis score.ResultsA total of 29 patients were included in this study. The fibrosis score was found to be weakly and inversely correlated with forced vital capacity %predicted at the diagnosis (r = ?0.40, p = 0.038). Furthermore, the annual changes in the flat chest index and fibrosis score was found to be moderately and inversely correlated (r = ?0.663, p = 0.0037).ConclusionsThese results indicate that there is a causal relationship between the progression of fibroelastosis and that of platythorax in patients with PPFE. |
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| Keywords: | Platythorax Flat chest index Thoracic cage Pleuroparenchymal fibroelastosis BMI" },{" #name" :" keyword" ," $" :{" id" :" kwrd0035" }," $$" :[{" #name" :" text" ," _" :" body mass index CT" },{" #name" :" keyword" ," $" :{" id" :" kwrd0045" }," $$" :[{" #name" :" text" ," _" :" computed tomography diffusing capacity of the lung for carbon monoxide FVC" },{" #name" :" keyword" ," $" :{" id" :" kwrd0065" }," $$" :[{" #name" :" text" ," _" :" forced vital capacity KL-6" },{" #name" :" keyword" ," $" :{" id" :" kwrd0075" }," $$" :[{" #name" :" text" ," _" :" Krebs von den Lungen-6 PPFE" },{" #name" :" keyword" ," $" :{" id" :" kwrd0085" }," $$" :[{" #name" :" text" ," _" :" pleuroparenchymal fibroelastosis RV" },{" #name" :" keyword" ," $" :{" id" :" kwrd0095" }," $$" :[{" #name" :" text" ," _" :" residual volume SP" },{" #name" :" keyword" ," $" :{" id" :" kwrd0105" }," $$" :[{" #name" :" text" ," _" :" surfactant protein TLC" },{" #name" :" keyword" ," $" :{" id" :" kwrd0115" }," $$" :[{" #name" :" text" ," _" :" total lung capacity |
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