International Epidemiological Study for Idiopathic Pulmonary Alveolar Proteinosis

• Project/Area Number: 18406031
• Research Category: Grant-in-Aid for Scientific Research (B)
• Allocation Type: Single-year Grants
• Section: 海外学術
• Research Field: Respiratory organ internal medicine
• Research Institution: Clinical Research Center, National Hospital Organization, Kinki-Chuo Chest Medical Center
• Principal Investigator: INOUE Yoshikazu Clinical Research Center, National Hospital Organization, Kinki-Chuo Chest Medical Center, 臨床研究センター・呼吸不全・難治性肺疾患研究, 部長 (90240895)
• Co-Investigator(Kenkyū-buntansha): NAKATA Koh 新潟大学, 大学医歯学総合病院・生命科学医療センター, 教授 (80207802)
• Project Period (FY): 2006 – 2008
• Project Status: Completed (Fiscal Year 2008)
• Budget Amount: ¥15,800,000 (Direct Cost: ¥13,400,000、Indirect Cost: ¥2,400,000); Fiscal Year 2008: ¥4,810,000 (Direct Cost: ¥3,700,000、Indirect Cost: ¥1,110,000); Fiscal Year 2007: ¥5,590,000 (Direct Cost: ¥4,300,000、Indirect Cost: ¥1,290,000); Fiscal Year 2006: ¥5,400,000 (Direct Cost: ¥5,400,000)
• Keywords: 肺胞蛋白症 / 特発性肺胞蛋白症 / 自己免疫性肺胞蛋白症 / 抗GM-CSF自己抗体 / 国際データベース

Research Abstract

我々は、抗GM-CSF自己抗体を基盤とした肺胞蛋白症(PAP)診断のアルゴリズムを確立し、国内PAP患者の集積を行った。新潟大学と国立病院機構近畿中央胸部疾患センターで症例データベースを作成(248例)、米国シンシナテイ子供医療センター等と国際共同疫学調査として解析を行った。わが国初、世界最大規模の特発性PAP(多くは自己免疫性PAP)の疫学データを発表し臨床像を明らかにした。更に欧米と共同で国際登録及び比較を開始するに至った。

Research Products

• [Journal Article] Comparative study of high-resolution CT findings between autoimmune and secondary pulmonary alveolar proteinosis (2009)
  • Author(s): Ishii H, Inoue Y, Nakata K, et al.
  • Journal Title: Chest (in press)
• [Journal Article] Usual interstitial pneumonia and nonspecific interstitial pneumonia with and without concurrent emphysema : thin-section CT findings (2009)
  • Author(s): Akira M, Inoue Y, Kitaichi M, Yamamoto S, Arai T, Toyokawa K
  • Journal Title: Radiology 251 Pages: 271-279
• [Journal Article] Quatitative CT in chronic obstructive pulmonary disease : Inspiratory and expiratory assessment (2009)
  • Author(s): Akira M, Toyokawa K, Inoue Y, Arai T
  • Journal Title: AJR 192 Pages: 267-272
• [Journal Article] Granulocyte/macrophage-colony-stimulating factor autoantibodies and myeloid cell immune functions in healthy subjects (2009)
  • Author(s): Uchida K, Nakata K, Suzuki T, et al.
  • Journal Title: Blood 113 Pages: 2547-2556
• [Journal Article] Characteristics of a large cohort of patients with autoimmune pulmonary alveolar proteinosis in Japan (2008)
  • Author(s): Inoue Y, Trapnell BC, Nakata K, et al.
  • Journal Title: Am J Respir Crit Care Med. 177 Pages: 752-762
• [Journal Article] Diagnostic potential of serum VEGF-D for lymphangioleiomyomatosis (2008)
  • Author(s): Young L, Inoue Y, McCormack FX
  • Journal Title: N Engl J Med. 358 Pages: 199-200
• [Journal Article] Propionibacterium acnes in granulomas of apatient with necrotising sarcoid granulomatosis (2008)
  • Author(s): Arai T, Inoue Y, Eishi Y, et. Al.
  • Journal Title: Thorax 63 Pages: 90-91
• [Journal Article] A combination therapy of whole lung lavage and GM-CSF inhalation In pulmonary alveolar proteinosis (2008)
  • Author(s): Yamamoto H, Yamaguchi E, Nakata K, et al.
  • Journal Title: Pediatr Pulmonol. 43 Pages: 828-830
• [Journal Article] A large cohort of patients with Autoimmune pulmonary Alveolar proteinosis in Japan (2008)
  • Author(s): Inoue Y, Trapnell BC, Tazawa R, et al.
  • Journal Title: Am J Respir Crit Care Med 177 Pages: 752-762
  • Peer Reviewed
• [Journal Article] Diagnostic potential of serum VEGF-D for lymphangioleiomyomatosis (2008)
  • Author(s): Young L, Inoue Y, McCormack FX
  • Journal Title: N Engl J Med 358 Pages: 199-200
  • Peer Reviewed
• [Journal Article] PropionibacteriumAcnes inGranulomas ofA patient with necrotising sarcoid granulomatosis (2008)
  • Author(s): Arai T, Inoue Y, Eichi Y, et al.
  • Journal Title: Thorax 63 Pages: 90-91
  • Peer Reviewed
• [Journal Article] Characteristics of a Large Cohort of Autoimmune Pulmonary Alveolar Proteinosis Patients in Japan (2008)
  • Author(s): Inoue Y, Trapnell BC, Tazawa R, et. al.
  • Journal Title: Am J Respir Crit Care Med. 177(7) Pages: 752-762
  • Peer Reviewed
• [Journal Article] Diagnostic Potential of Serum VEGF-D for Lymphangioleiomyomatosis (2008)
  • Author(s): Young LR, Inoue Y, McCormack FX
  • Journal Title: N Engl J Med 358 Pages: 199-200
  • Peer Reviewed
• [Journal Article] Propionibacerium acnes in granulomas of a patient with necrotising Sarcoid granulomatosis (2008)
  • Author(s): Arai T, Inoue Y, Eishi Y, et. al.
  • Journal Title: Thorax 63 Pages: 90-91
  • Peer Reviewed
• [Journal Article] The epidemiology of lymphangioleiomyomatosis in Japan :A nationwide cross-sectional study of presenting features And prognostic factors (2007)
  • Author(s): Hayashida M, Seyama K, Inoue Y, et. Al.
  • Journal Title: Respirology 12 Pages: 523-530
• [Journal Article] Enhanced mast cell chymase expression in human Idiopathic interstitial pneumonia (2007)
  • Author(s): Hirata K, Sugame Y, Inoue Y, et. Al.
  • Journal Title: Int J Mol Med. 19 Pages: 565-570
• [Journal Article] Pulmonary MycobacteriumAvium complex in fection Associated with the IVS8-T5Allele of the CFTRGene (2007)
  • Author(s): Mai HN, Hijikata M, Inoue Y, et. Al.
  • Journal Title: Int J Tuberc Lung Dis. 11 Pages: 808-813
• [Journal Article] Mutations in type IIb sodium phosphate co-transporter (SLC34A2) cause pulmonary Alveolar microlithiasis (2007)
  • Author(s): Huqun, Izumi S, Inoue Y, et. Al.
  • Journal Title: Am J Respir Crit Care Med. 175 Pages: 263-268
• [Journal Article] Enhanced expression of angiotensin II type 1 receptor in usual interstitial pneumonia (2007)
  • Author(s): Sugama Y, Ikura Y, Inoue Y, et. Al.
  • Journal Title: Osaka City Med J. 53 Pages: 87-95
• [Journal Article] Anti-cytokine autoantibodies are ubiquitous in healthy individuals (2007)
  • Author(s): Watanabe M, Uchida K, Nakata K
  • Journal Title: FEBS Lett. 581 Pages: 17-21
• [Journal Article] Expression of PU.1 and terminal differentiation of alveolar macrophages in newborn rats (2007)
  • Author(s): Iwabuchi H, Kawasaki T, Nakata K, et al.
  • Journal Title: Cell Tissue Res. 329 Pages: 71-79
• [Journal Article] Mutations in type IIb sodium phosphate co-transporter (SLC34A2) cause pulmonary alveolar microlithiasis (2007)
  • Author(s): Huqun, Izumi S, Miyazawa H, Inoue Y. et al.
  • Journal Title: Am J Respir Crit Care Med 175 Pages: 263-268
• [Journal Article] 肺胞蛋白症の診断と治療 (2007)
  • Author(s): 井上義一, 新井徹, 大塚淳司
  • Journal Title: 内科 99 Pages: 279-289
• [Journal Article] Granulocyte-macrophage colony-stimulating factor inhalation therapy for patients with idiopathic pulmonary alveolar proteinosis : a pilot study; and long-term treatment with aerosolized granulocyte-macrophage colony-stimulating factor : a case report (2006)
  • Author(s): Tazawa R, Nakata K, Inoue Y, et. Al.
  • Journal Title: Respirology 11 Pages: 561-564
• [Journal Article] Epidemiological and clinical feature of idiopathic pulmonary alveolar proteinosis in Japan (2006)
  • Author(s): Inoue Y, Nakata K, Arai T, et. Al.
  • Journal Title: Respirology 11 Pages: 555-560
• [Journal Article] Granulocyte-macrophage colony-stimulating factor inhalation therapy for patients with idiopathic pulmonary alveolar proteinosis: a pilot study; and long-term treatment with aerosolized granulocyte-macrophage colony-stimulating factor : a case report (2006)
  • Author(s): Tazawa R, Nakata K, Inoue Y, et. al.
  • Journal Title: Respirology 11 Pages: 561-564
• [Journal Article] NSIP in a curry sauce factory worker (2006)
  • Author(s): Ando S, Arai T, Inoue Y, et. Al.
  • Journal Title: Thorax 61 Pages: 1012-1013
• [Journal Article] Intractable desquamative interstitial pneumonia in a tattooed man (2006)
  • Author(s): Arai T, Inoue Y, Hayashi S, et. Al.
  • Journal Title: Intern Med. 45 Pages: 1055-1058
  • NAID: 130000076440
• [Journal Article] A New Tool to Assess Sarcoidosis Disease Severity (2006)
  • Author(s): Wasfi Y, Inoue Y, et. Al.
  • Journal Title: Chest 129 Pages: 1234-1245
• [Journal Article] DNA vaccine using hemagglutinating virus of Japan-liposome encapsulating combination encoding mycobacterial heat shock protein 65 and interleukin-12 confers protection against Mycobacterium tuberculosis by T cell activation (2006)
  • Author(s): Yoshida S, Tanaka T, Inoue Y, et. Al.
  • Journal Title: Vaccine 24 Pages: 1191-1204
• [Journal Article] Pathogenesis and Pathology of Pulmonary Alveolar Proteinosis (2006)
  • Author(s): Ishii H, Nakata K
  • Journal Title: Pathology and Clinical Medicine 24 Pages: 921-927
• [Journal Article] Epidemiological and clinical feature of idiopathic pulmonary alveolar proteinosis in Japan (2006)
  • Author(s): Inoue Y, Nakata K, Arai T., et al.
  • Journal Title: Respirplogy 11
• [Journal Article] Granulocyte-macrophage colony-stimulating factor inhalation therapy for patients with idiopathic pulmonary alveolar proteinosis : a pilot study; and long-term treatment with aerosolized granulocyte-macrophage colony-stimulating factor : a case report (2006)
  • Author(s): Tazawa R, Nakata K, Inoue Y, et al.
  • Journal Title: Respirplogy 11
• [Journal Article] 我が国に於ける肺胞蛋白症患者データベースを構築する (2006)
  • Author(s): 井上義一
  • Journal Title: 分子呼吸器病 10 Pages: 27-31
• [Journal Article] 肺胞蛋白症 (2006)
  • Author(s): 井上義一
  • Journal Title: Medicina 44 Pages: 330-331
• [Journal Article] Successful cord blood transplantation for myelodysplastic syndrome resulting in resolution of pulmonary alveolar proteinosis
  • Author(s): Fukuno K, Tomonari A, Nakata K, et al.
  • Journal Title: Bone Marrow Transplant. 38 Pages: 581-582
• [Presentation] Th1/Th2 cytokines and VEGF-D profile in sera from patients with lymphangioleiomyomatosis (2008)
  • Author(s): Inoue Y, Hirose M, Matsumuro A, et al.
  • Organizer: The LAM Foudation / NHLBI Lymphan gioleiomyomatosis. International Research Conference 2008
  • Place of Presentation: The West in Cincinnati, Ohio, USA
• [Presentation] Serum VEGF-D is diagnostic test for lymphangioleiomyomatosis(LAM) (2008)
  • Author(s): Young LR, Inoue Y, McCormack FX, et al.
  • Organizer: The LAM Foudation / NHLBI Lymphangioleiomyomatosis. International Research Conference 2008
  • Place of Presentation: The Westin Cincinnati, Ohio, USA
• [Presentation] Natural history of primary (Autoimmune) PAP. Basic clinical translational. Scientific symposium. Session D89 Rare lung disease research: Pulmonary alveolar proteinosis in 2008 (2008)
  • Author(s): Inoue Y.
  • Organizer: ATS 2008 Toronto International conference
  • Place of Presentation: Toronto Ontario Canada
• [Presentation] P. Serum VEGF-D is diagnostic test for lymphangioleiomyomatosis (LAM). A48 Lymphangioleiomyomatosis/ The matic Poster Session (2008)
  • Author(s): L.R.Young, Inoue Y, McCormack FX. Et al.
  • Organizer: ATS 2008 Toronto International conference
• [Presentation] Characteristics of a Large Cohort of Autoimmune Pulmonary Alveolar Proteinosis Patients in Japan. D35 Pulmonary Alveolar Proteinosis / Thematic Poster Session (2008)
  • Author(s): Inoue Y, B.C. Trapnell, T. Nakata K, et al.
  • Organizer: ATS 2008 Toronto International conference (American Journal of Respiratory and Critical Care Medicine. Vol. 177, Abstracts issue, A878)
• [Presentation] Strategy for Diagnosis Pulmonary Alveolar Proteinosis. D35 Pulmonary Alveolar Proteinosis / Thematic Poster Session (2008)
  • Author(s): Ishii H, Inoue Y, R. Nakata K, et al.
  • Organizer: ATS 2008 Toronto International conference
• [Presentation] Pulmonary Alveolar Proteinosis (PAP) A Clinicopathologic Study of 48 cases in the Second Osaka Symposium on Respiratory Diseases. EMS16-1 ILD3 (2008)
  • Author(s): Kitaichi M, Inoue Y, Nakata K, et al.
  • Organizer: The 48th Japanese Respiratory Society Conference (The Journal of the Japanese Respiratory Society, Vol. 46, May 2008, P.365)
  • Place of Presentation: Kobe, Japan
• [Presentation] Characteristics of a Large Cohort of Autoimmune Pulmonary Alveolar Proteinosis Patients in Japan (2008)
  • Author(s): Inoue Y, et al.
  • Organizer: American Thoracic Society
  • Place of Presentation: Toronto Ontario Canad
• [Presentation] Longitudinal evaluation of autoimmune pulmonany alveolar proteinosis in Japan : Five years cohort study (2007)
  • Author(s): Inoue Y, Nakata K, Tazawa R, et al.
  • Organizer: Fifth annual international pulmonany alveolar proteinosis scientific conference
  • Place of Presentation: Osaka, Japan
  • Year and Date: 2007-05-08
• [Presentation] Evaluating pulmonary alveolar proteinosis on a global scale (2007)
  • Author(s): Trapnell B, Inoue Y, Nakata K, et al.
  • Organizer: Fifth annual international pulmonany alveolar proteinosis scientific conference
  • Place of Presentation: Osaka, Japan
  • Year and Date: 2007-05-08
• [Presentation] Pulmonary alveolar proteinosis (PAP) in Japan : Crossectional study of autoimmune PAP (2007)
  • Author(s): Nishiyama A , Inoue Y, Nakata K. et al.
  • Organizer: Fifth annual international pulmonany alveolar proteinosis scientific conference
  • Place of Presentation: Osaka, Japan
  • Year and Date: 2007-05-08
• [Presentation] Maintenance GM-CSF inhalation therapy for autoimmune PAP (2007)
  • Author(s): Tazawa T, Inoue Y, Nakata K, et al.
  • Organizer: Fifth annual international pulmonany alveolar proteinosis scientific conference
  • Place of Presentation: Osaka, Japan
  • Year and Date: 2007-05-08
• [Presentation] Physiological aspects of autoimmune PAP (2007)
  • Author(s): Arai T, Inoue Y, Ohtsuka J, et al.
  • Organizer: Fifth annual international pulmonany alveolar proteinosis scientific conference
  • Place of Presentation: Osaka, Japan
  • Year and Date: 2007-05-08
• [Presentation] Lymphocyte abnormalities in autoimmune Pulmonary Alveolar Proteinosis (a PAP) (2007)
  • Author(s): Kanazawa H, Inoue Y, Nakata K. et al.
  • Organizer: Fifth annual international pulmonany alveolar proteinosis scientific conference
  • Place of Presentation: Osaka, Japan
  • Year and Date: 2007-05-08
• [Presentation] Psychosocial condition in patients with Lymphangioleiomyomatosis (2007)
  • Author(s): Inoue Y, MD, Ohya A, Tokoro H, et al.
  • Organizer: The LAM Foundation / NHLBI international reseach conference
  • Place of Presentation: Hilton Cincinnati Netherland Plaza hotel
• [Presentation] Hypoxia is correlated with reduction of CD4+T Cell in the peripheral blood of idiopathic pulmonary alveolar protenosis (iPAP) (2007)
  • Author(s): Kanazawa H, Inoue Y, Nakata K et al.
  • Organizer: ATS 2007 international conference
• [Presentation] Five years cohort study of 53 patients with idiopathic pulmonary alveolar proteinosis. Outcome of therapeutic lavage (2007)
  • Author(s): Inoue Y, Nakata K, Tazawa R, et al.
  • Organizer: ATS 2007 international conference
  • Place of Presentation: San Francisco, California
• [Presentation] Maintenance Therapy with intermittent granulocyte-macrophage colony-stimulating factor (GM-CSF) inhalation for pulmonary plveolar proteinosis (2007)
  • Author(s): Tazawa R, Inoue Y, Nakata K, et al.
  • Organizer: ATS 2007 international conference
  • Place of Presentation: San Francisco, California
• [Presentation] Multicenter phase IItraial of inhaled aerosolized glanulocyte-macrophage colony-stimulation factor for patients with idiopathic pulmonary alveolar proteinosis. Mini-symposium (2007)
  • Author(s): Nakata K, Inoue Y, Nukiwa T, et al.
  • Organizer: ATS 2007 international conference
  • Place of Presentation: San Francisco, California
• [Presentation] Five years cohort study of 53 patients with idiopathic pulmonary alveolar proteinosis. Outcome of therapeutic lavage. (2007)
  • Author(s): Y. Inoue, K. Nakata, R. Tazawa, et. al.
  • Organizer: American Thoracic Society 2007 International conference
  • Place of Presentation: San Francisco, California, USA
• [Presentation] Repeated Therapies with GM-CSF Inhalation for Pulmonary Alveolar Proteinosis (2006)
  • Author(s): Tazawa R, Inoue Y, Nakata K, et al.
  • Organizer: ATS International Conference (Abstracts ATS International Conference)
  • Place of Presentation: California
• [Presentation] Psychosocial conditions in pulmonary Lymphangioleimyomatosis (2006)
  • Author(s): Inoue Y, A. Ohya, A. Tokoro, Y. et al.
  • Organizer: ATS International Conference (Abstracts ATS International Conference)
  • Place of Presentation: California
• [Presentation] A Cross- sectional Study of Patients with Idiopathic Pulmonary Alveolar Proteinosis in Japan (2006)
  • Author(s): Inoue Y, Nakata K, Arai T, et al.
  • Organizer: the 11th Congress of the Asian Pacific Society of Respirology
  • Place of Presentation: Kyoto International Conference Hall
• [Presentation] High concentration of GM-CSF-Autoantibody Immune Complex in Bronchoalveolar LavageFluid and Serum from Patient with Idiopathic Pulmonary Alveolar Proteinosis (2006)
  • Author(s): Watanabe M, Nakata K, Inoue Y, et al.
  • Organizer: the 11th Congress of the Asian Pacific Society of Respirology
  • Place of Presentation: Kyoto International Conference Hall
• [Presentation] GM-CSF Inhalation Therapy for Recurrence of Pulmonary Alveolar Proteinosis (2006)
  • Author(s): Tazawa R, Inoue Y, Nakata K et al.
  • Organizer: the 11th Congress of the Asian Pacific Society of Respirology
  • Place of Presentation: Kyoto International Conference Hall
• [Book] 50^<th> Anniversary Today's Therapy 2008 今日の治療指針2008年 (Volume50) 私はこう治療している (2008)
  • Author(s): 井上義一, 総編集 山口徹、北原光夫、福井次矢
  • Publisher: (株)医学書院
• [Book] C. BAL 法の手技: 1. 洗浄液の採取. 気管支肺胞洗浄〔BAL〕法の手引き (2008)
  • Author(s): 井上義一, 山田佳子
  • Publisher: (株)克誠堂出版
• [Book] C. BAL 法の手技: 2. 回収洗浄液の処理. 気管支肺胞洗浄〔BAL〕法の手引き (2008)
  • Author(s): 井上義一, 山田佳子
  • Total Pages: 11
  • Publisher: (株)克誠堂出版
• [Book] C. BAL 法の手技: 3. 細胞数算定. 気管支肺胞洗浄〔BAL〕法の手引き (2008)
  • Author(s): 井上義一, 山田佳子
  • Total Pages: 11
  • Publisher: (株)克誠堂出版
• [Book] 肺胞蛋白症. 第XI章 各種疾患の気管支鏡所見17. 気管支鏡-臨床医のためのテクニックと画像診断 第2版 (2008)
  • Author(s): 井上義一
  • Publisher: (株)医学書院
• [Book] 特別報告 リンパ脈管筋腫症 lymphangioleiomyomatosis (LAM) の診断基準 (日本呼吸器学会雑誌 第46巻/第6号 別冊)ioleiomyomatosis(LAM)の診断基準. (2008)
  • Author(s): 林田美江, 久保惠嗣, 瀬山邦明, 熊坂利夫, 井上義一, 北市正則, 審良正則
• [Book] 特別報告 リンパ脈管筋腫症 lymphangioleiomyomatosis (LAM) の治療と管理の手引き (日本呼吸器学会雑誌 第46巻/第6号 別冊) (2008)
  • Author(s): 林田美江, 藤本圭作, 久保惠嗣, 瀬山邦明, 井上義一
• [Book] まれなびまん性肺疾患 呼吸器疾患診療マニュアル (日本医師会雑誌 第137巻・特別号(2)) (2008)
  • Author(s): 井上義一
• [Book] 今日の治療指針2008年 (2008)
  • Author(s): 井上義一
  • Total Pages: 1
  • Publisher: 医学書院
• [Book] 肺胞蛋白症 (Medicina 44) (2007)
  • Author(s): 井上義一
• [Book] リンパ脈管筋腫症 特集COPDと鑑別を要する疾患・合併しうる疾患 (COPD Frontier. Vol.6 No.2) (2007)
  • Author(s): 井上義一
  • Publisher: メディカルレビュ-社
• [Book] LAMposium2007 に参加して (J-LAMの会ニュースレター「羽のたより」, 2007年6月号) (2007)
  • Author(s): 井上義一
  • Total Pages: 4
• [Book] わが国におけるLAMの疫学 (日本胸部臨床 65) (2006)
  • Author(s): 林田美江、藤本圭作、久保惠嗣、瀬山邦明、井上義一
• [Book] 本邦におけるLAMの治療、予後の現状と問題点 (日本胸部臨床 65) (2006)
  • Author(s): 久保惠嗣、井上義一
• [Book] 我が国に於ける肺胞蛋白症患者データベースを構築する (分子呼吸器病 10) (2006)
  • Author(s): 井上義一
• [Remarks]
  • URL: http://www.hosp.go.jp/~kch/
• [Remarks]
  • URL: http://www.hosp.go.jp/~kch/