Multidimensional study of PPFE

2023 Fiscal Year Final Research Report

• Project/Area Number: 19K08638
• 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: Fukuoka University
• Principal Investigator: Ishii Hiroshi 福岡大学, 医学部, 教授 (60398146)
• Project Period (FY): 2019-04-01 – 2024-03-31
• Keywords: びまん性肺疾患 / 肺線維症 / 間質性肺炎

Outline of Final Research Achievements

We presented the results of our analysis of clinical, imaging and pathological findings in 52 cases of upper lobe predominant pulmonary fibrosis (PPFE), proposed diagnostic criteria based on these data, and reported the results of validation of these proposed diagnostic criteria. Subsequently, we reported the pathological features of pulmonary arteries in PPFE, promising serum biomarkers for PPFE, the mechanism of alveolar collapse in the histopathology of PPFE, and a summary of pulmonary histopathology in PPFE. In addition, we made prognostic models for PPFE, and reported the progression of thoracic flattening, limited effect of antifibrotic agents, presence of pre-diagnostic lesions, familial cases, and the studies on weight loss and prognosis. Finally, this year, we published papers on partial pressure of carbon dioxide and prognosis, and on a comparison of two clinical diagnostic criteria for PPFE.

Free Research Field

びまん性肺疾患

Academic Significance and Societal Importance of the Research Achievements

われわれは、指定難病の特発性間質性肺炎の一亜型として令和6年度に新たに加わった上葉優位型肺線維症（PPFE）に関して約10年間研究に取り組んできた。多くの臨床・画像・病理所見の解析結果をもとにした臨床診断基準案、この診断基準案の検証結果を英文論文として世界に発表した。その後、有望な血清バイオマーカーになりうる物質、PPFEにおける肺胞虚脱の機序、予後予測モデル、胸郭扁平の進行、抗線維化薬の限定的効果、診断前の病変の存在、体重減少や二酸化炭素分圧と予後に関する研究などを報告し、病態解明に向けた一助になったと考える。