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上葉優位型肺線維症(PPFE)に関する業績

更新日:4月16日

福岡大学呼吸器内科の渡辺憲太朗前教授による2012年の報告(”PPFE”におけるFVCの急速な低下)を先駆けとして、私どもはこの10年間 PPFEに関する論文を世に出してきました。ここ数年は全てここ筑紫病院からの発信です。渡辺先生が厚労省難治性疾患研究班(びまん班)のPPFE部会長だった事もあり、全国規模の研究会や診断基準案の作成に関わることが出来ました。その後石井が副部会長を拝命し、部会長が所属する東京医科歯科大学とも協働して、さらに踏み込んだ研究が進むことが期待されます。



  1. Rapid decrease in forced vital capacity in patients with idiopathic pulmonary upper lobe fibrosis. Watanabe K, Nagata N, Kitasato Y, Wakamatsu K, Nabeshima K, Harada T, Hirota T, Shiraishi M, Fujita M. Respir Investig. 2012 Sep;50(3):88-97.

  2. Pleuroparenchymal fibroelastosis as a manifestation of chronic lung rejection? Hirota T, Fujita M, Matsumoto T, Higuchi T, Shiraishi T, Minami M, Okumura M, Nabeshima K, Watanabe K. Eur Respir J. 2013 Jan;41(1):243-5.

  3. Pleuroparenchymal Fibroelastosis: Its Clinical Characteristics. Watanabe K. Curr Respir Med Rev. 2013 Jun;9(4):299-237.

  4. The thoracic cage becomes flattened in the progression of pleuroparenchymal fibroelastosis. Harada T, Yoshida Y, Kitasato Y, Tsuruta N, Wakamatsu K, Hirota T, Tanaka M, Tashiro N, Ishii H, Shiraishi M, Fujita M, Nagata N, Watanabe K. Eur Respir Rev. 2014 Jun;23(132):263-6.

  5. Histological evolution of pleuroparenchymal fibroelastosis. Hirota T, Yoshida Y, Kitasato Y, Yoshimi M, Koga T, Tsuruta N, Minami M, Harada T, Ishii H, Fujita M, Nabeshima K, Nagata N, Watanabe K. Histopathology. 2015 Mar;66(4):545-54.

  6. Heterogeneous clinical features in patients with pulmonary fibrosis showing histology of pleuroparenchymal fibroelastosis. Yoshida Y, Nagata N, Tsuruta N, Kitasato Y, Wakamatsu K, Yoshimi M, Ishii H, Hirota T, Hamada N, Fujita M, Nabeshima K, Kiyomi F, Watanabe K. Respir Investig. 2016 May;54(3):162-9.

  7. Two cases of pleuroparenchymal fibroelastosis diagnosed with transbronchial lung biopsy. Kushima H, Hidaka K, Ishii H, Nakao A, On R, Kinoshita Y, Fujita M, Nabashima K, Watanabe K. Respir Med Case Rep. 2016 Jul 18;19:71-3

  8. Two Different Aspects of Pleuroparenchymal Fibroelastosis: A Disease of Pulmonary Fibrosis, and of the Chest Wall. Watanabe K. J Med Surg Pathol. 2016 1:111.

  9. Evaluation of urinary desmosines as a noninvasive diagnostic biomarker in patients with idiopathic pleuroparenchymal fibroelastosis (PPFE). Oyama Y, Enomoto N, Suzuki Y, Kono M, Fujisawa T, Inui N, Nakamura Y, Kuroishi S, Yokomura K, Toyoshima M, Imokawa S, Oishi K, Watanabe S, Kasahara K, Baba T, Ogura T, Ishii H, Watanabe K, Nishioka Y, Suda T. Respir Med. 2017 Feb;123:63-70.

  10. Proliferation of elastic fibres in idiopathic pulmonary fibrosis: a whole-slide image analysis and comparison with pleuroparenchymal fibroelastosis. Kinoshita Y, Watanabe K, Ishii H, Kushima H, Fujita M, Nabeshima K. Histopathology. 2017 Dec;71(6):934-942.

  11. 肺癌を合併したpleuroparenchymal fibroelastosisの1例.木下義晃、渡辺憲太朗、石井 寛、吉村雅代、濱崎 慎、鍋島一樹.診断病理 2018;35:66-71.

  12. Is idiopathic PPFE an established subset of idiopathic interstitial pneumonias? Watanabe K. Respir Investig. 2018 Jan;56(1):3-4.

  13. Significant increases in the density and number of lymphatic vessels in pleuroparenchymal fibroelastosis. Kinoshita Y, Watanabe K, Ishii H, Kushima H, Fujita M, Nabeshima K. Histopathology. 2018 Sep;73(3):417-427.

  14. Pleuroparenchymal fibroelastosis diagnosed by multidisciplinary discussions in Japan. Ishii H, Watanabe K, Kushima H, Baba T, Watanabe S, Yamada Y, Arai T, Tsushima K, Kondoh Y, Nakamura Y, Terasaki Y, Hebisawa A, Johkoh T, Sakai F, Takemura T, Kawabata Y, Ogura T; Tokyo Diffuse Lung Disease Study Group. Respir Med. 2018 Aug;141:190-197.

  15. Pleuroparenchymal fibroelastosis as a histological background of autoimmune diseases. Kinoshita Y, Watanabe K, Ishii H, Kushima H, Hamasaki M, Fujita M, Nabeshima K. Virchows Arch. 2019 Jan;474(1):97-104.

  16. Chronic Pulmonary Aspergillosis with Pleuroparenchymal Fibroelastosis-like Features. Kushima H, Ishii H, Kinoshita Y, Fujita M, Watanabe K. Intern Med. 2019 Apr 15;58(8):1137-1140.

  17. The upward shift of hilar structures and tracheal deviation in pleuroparenchymal fibroelastosis. Ishii H, Kinoshita Y, Kushima H, Ogura T, Watanabe K. Multidiscip Respir Med. 2019 Mar 7;14:10.

  18. Criteria for the diagnosis of idiopathic pleuroparenchymal fibroelastosis: A proposal. Watanabe K, Ishii H, Kiyomi F, Terasaki Y, Hebisawa A, Kawabata Y, Johkoh T, Sakai F, Kondoh Y, Inoue Y, Azuma A, Suda T, Ogura T, Inase N, Homma S; Study Group on Diffuse Pulmonary Disorders, Scientific Research/Research on Intractable Diseases in Japan. Respir Investig. 2019 Jul;57(4):312-320.

  19. Lower-lobe predominant pleuroparenchymal fibroelastosis. Kinoshita Y, Watanabe K, Ishii H, Kushima H, Nabeshima K. Pathol Int. 2019 Sep;69(9):536-540.

  20. The similarities and differences between pleuroparenchymal fibroelastosis and idiopathic pulmonary fibrosis. Ishii H, Kinoshita Y, Kushima H, Nagata N, Watanabe K. Chron Respir Dis. 2019 Jan-Dec;16:1479973119867945.

  21. Remodeling of the pulmonary artery in idiopathic pleuroparenchymal fibroelastosis. Kinoshita Y, Ishii H, Kushima H, Johkoh T, Yabuuchi H, Fujita M, Nabeshima K, Watanabe K. Sci Rep. 2020 Jan 15;10(1):306.

  22. Role of alveolar collapse in idiopathic pleuroparenchymal fibroelastosis. Kinoshita Y, Ishii H, Kushima H, Fujita M, Nabeshima K, Watanabe K. Sarcoidosis Vasc Diffuse Lung Dis. 2020;37(2):212-217.

  23. Serum latent transforming growth factor-β binding protein 4 as a novel biomarker for idiopathic pleuroparenchymal fibroelastosis. Kinoshita Y, Ikeda T, Kushima H, Fujita M, Nakamura T, Nabeshima K, Ishii H. Respir Med. 2020 Sep;171:106077.

  24. The pathogenesis and pathology of idiopathic pleuroparenchymal fibroelastosis. Kinoshita Y, Ishii H, Nabeshima K, Watanabe K. Histol Histopathol. 2021 Mar;36(3):291-303.

  25. Physiological Criteria Are Useful for the Diagnosis of Idiopathic Pleuroparenchymal Fibroelastosis. Ikeda T, Kinoshita Y, Ueda Y, Sasaki T, Kushima H, Ishii H. J Clin Med. 2020 Nov 22;9(11):3761.

  26. Alveolar Epithelial Denudation Is a Major Factor in the Pathogenesis of Pleuroparenchymal Fibroelastosis. Zaizen Y, Tachibana Y, Kashima Y, Bychkov A, Tabata K, Otani K, Kinoshita Y, Yamano Y, Kataoka K, Ichikado K, Okamoto M, Kishaba T, Mito R, Nishimura K, Yamasue M, Nabeshima K, Watanabe K, Kondoh Y, Fukuoka J. J Clin Med. 2021 Feb 24;10(5):895.

  27. A proposed prognostic prediction score for pleuroparenchymal fibroelastosis. Kinoshita Y, Ikeda T, Miyamura T, Ueda Y, Yoshida Y, Kushima H, Fujita M, Ogura T, Watanabe K, Ishii H. Respir Res. 2021 Jul 30;22(1):215.

  28. Severe and progressive platythorax disproportionate to lung fibrosis: A rare variant of idiopathic pleuroparenchymal fibroelastosis. Ikeda T, Kinoshita Y, Ueda Y, Sasaki T, Kushima H, Ishii H. Respir Med Case Rep. 2021 Mar 19;33:101395.

  29. Platythorax progresses with lung involvement in pleuroparenchymal fibroelastosis. Ikeda T, Kinoshita Y, Miyamura T, Ueda Y, Yoshida Y, Kushima H, Ishii H. Respir Investig. 2022 Mar;60(2):293-299.

  30. Limited efficacy of nintedanib for idiopathic pleuroparenchymal fibroelastosis. Kinoshita Y, Miyamura T, Ikeda T, Ueda Y, Yoshida Y, Kushima H, Ishii H. Respir Investig. 2022 Jul;60(4):562-569.

  31. Lung involvement during the prediagnostic phase of idiopathic pleuroparenchymal fibroelastosis. Miyamura T, Kinoshita Y, Kushima H, Mukae H, Ishii H. ERJ Open Res. 2022 May 9;8(2):00548-2021.

  32. Idiopathic pleuroparenchymal fibroelastosis. Kinoshita Y, Kushima H, Ishii H. BMJ Case Rep. 2022 Jul 5;15(7):e251483.

  33. Familial pleuroparenchymal fibroelastosis: an entity to be recognized. Kinoshita Y, Utsunomiya T, Koide Y, Wada K, Ueda Y, Yoshida Y, Kushima H, Ishii H. Respir Med and Res. 2022 82: 100954.

  34. Change in body weight reflects disease progression in pleuroparenchymal fibroelastosis. Kinoshita Y, Ishii H, et al. Respir Med and Res. (in press)

  35. Partial pressure of carbon dioxide levels reflect disease severity in idiopathic pleuroparenchymal fibroelastosis. (under submission)

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