Pathogenesis of pleuroparenchymal fibroelastosis

2021 Fiscal Year Final Research Report

• Project/Area Number: 19K08648
• Research Category: Grant-in-Aid for Scientific Research (C)
• Allocation Type: Multi-year Fund
• Section: 一般
• Review Section: Basic Section 53030:Respiratory medicine-related
• Research Institution: Kyoto University
• Principal Investigator: Tanizawa Kiminobu 京都大学, 医学研究科, 助教 (20639140)
• Co-Investigator(Kenkyū-buntansha): 松田 文彦 京都大学, 医学研究科, 教授 (50212220) ; 伊達 洋至 京都大学, 医学研究科, 教授 (60252962)
• Project Period (FY): 2019-04-01 – 2022-03-31
• Keywords: 上葉優位型肺線維症 / PPFE / 肺移植 / 造血幹細胞移植 / サルコイドーシス

Outline of Final Research Achievements

(1) Of 59 lung transplantation recipients with radiological PPFE-like lesion, 28 had pathological PPFE, which was characterized by intra-alveolar fibrosis and the deposition of elastic fibers. Radiological findings and post-transplant survival were similar between idiopathic and secondary cases with pathological PPFE. (2) There were 106 genes, including ADAMTS4, IL-6, selectin E, and EGR3, whose expression was increased in PPFE lung specimens compared to UIP. (3) Patients with late-onset non-infectious pulmonary complications after hematopoietic stem cell transplantation had a similar outcome to those with interstitial lung diseases and a worse outcome than those with non-ILD diseases. (4) Lung function decline was associated with shorter survival time in patients with sarcoidosis. Basal interventricular septum thinning was late-onset left ventricular dysfunction in sarcoidosis.

Free Research Field

臨床呼吸器学

Academic Significance and Societal Importance of the Research Achievements

(1)画像的なPPFEと病理学的なPPFEを照合し、今後の診断基準の作成の基礎データとなりうる結果を明らかにした。(2)PPFEに特徴的な発現亢進を示す遺伝子を同定し、バイオマーカーや治療標的の可能性を示した。(3)肺移植待機患者群において、造血幹細胞移植後の晩期性肺障害患者の予後を明らかにした。(4)サルコイドーシスにおいて、呼吸機能の経時的な評価や心臓超音波検査の臨床的な意義を示した。