主な結果: 10種類の異なる薬を対象とした15の試験が含まれた。1156人の患者を対象にインターフェロンガンマ-1ベータとプラセボを比較した2つの試験では、インターフェロンガンマ-1ベータによる生存の有意な改善は認められなかった（HR 0.88, 95% CI 0.47から1.64; P = 0.68）。1155人の患者を対象にピルフェニドンとプラセボを比較した4つの試験がある。1046人の患者を対象に行われた3つの試験は、ピルフェニドンが疾患進行のリスクを30％減少させることを示した（HR 0.70, 95% CI 0.56から0.88, P = 0.002）。
1. The PANTHER-IPF Trial 参考論文: Raghu G, Anstrom KJ, King TE, Jr, Lasky JA, Martinez FJ, on behalf of the Idiopathic Pulmonary Fibrosis Clinical Research Network. Prednisone, azathioprine, and N-acetylcysteine for pulmonary fibrosis. N Engl J Med. 2012;366(21):1968-77. 概要: PANTHER-IPF試験は、ステロイド（プレドニゾン）、免疫抑制剤（アザチオプリン）、および抗酸化剤（N-アセチルシステイン）の組み合わせ治療がIPFの進行を遅らせるかどうかを評価するために設計されました。しかし、この3剤組み合わせ療法は、プラセボ群と比較して死亡率、入院率、および副作用の増加を示したため、早期に中止されました。 結論: この試験は、IPFにおけるプレドニゾン（ステロイド）を含む従来の免疫抑制療法の有害な影響を強く示唆しています。
2. Cochrane Review on Non-steroid Treatments for IPF 参考論文: Spagnolo P, Tonelli R, Cocconcelli E, Stefani A, Richeldi L. Non-steroid agents for idiopathic pulmonary fibrosis. Cochrane Database Syst Rev. 2020. 概要: このシステマティックレビューは、IPF治療のための非ステロイド治療法を評価し、特に抗線維化療法の効果に焦点を当てました。レビューは、ステロイド治療の代替として、ピルフェニドンやニンテダニブなどの抗線維化薬がIPFの進行を遅らせる可能性があることを示しました。 結論: ステロイド以外の治療法がIPF管理においてより効果的であることを支持する証拠は、ステロイドの使用を推奨しない理由の一つです。
ATS/ERS/JRS/ALAT Guidelines: "An Official ATS/ERS/JRS/ALAT Clinical Practice Guideline: Treatment of Idiopathic Pulmonary Fibrosis. An Update of the 2011 Clinical Practice Guideline." These guidelines provide comprehensive recommendations on the management of IPF, including the use of corticosteroids.
Systematic Reviews and Meta-Analyses: Extensive literature reviews and meta-analyses have assessed the efficacy of corticosteroids in IPF, providing a robust evidence base for current treatment recommendations.
… While idiopathic interstitial pneumonia (IIP) centering on idiopathicpulmonaryfibrosis(IPF) is the most prevalent interstitial lung disease (ILD), especially in the older adult population, connective tissue disease (CTD)-related ILD is the second …
G Juliá-Serdá, J Navarro-Esteva, L Doreste-Salgado… - Cureus, 2024
… (HP), both fibrotic (f-HP) and nonfibrotic (nf-HP), and to compare them with those in patients with idiopathicpulmonaryfibrosis (IPF). … In most cases, IPF was diagnosed based on HRCT findings. Results: We identified 84 patients: 46 with IPF …
… Idiopathicpulmonaryfibrosis (IPF) is a life-limiting chronic interstitial lung disease with symptoms of cough, shortness of breath, fatigue, anxiety, and depression. Palliative care (PC) aims to treat such symptoms for seriously ill patients. … Using …
J Xia, C Wu, X Jin, M Ding, C Zhang, G Hou, C Hao… - … and Environmental Safety, 2024
… To test this hypothesis, we screened the genetic changes in patients with idiopathicfibrosis … We identified 9 cuproptosis-related genes among the differential genes in patients with IPF at … to the possibility of cuproptosis in the …
… ILDs with unknown aetiology include the various idiopathic interstitial pneumonias and the rare forms of ILDs like Lymphangiomyomatosis (LAM) & the various vasculitis. Idiopathic interstitial pneumonias are further classified into …
Y Tsuchiya, T Seki, H Nakano, M Tanaka, R Takahashi - Yakugaku zasshi: Journal of …, 2024
… Additionally, we provide a brief summary of the two currently approved anti-fibrotic drugs for idiopathicpulmonaryfibrosis, nintedanib and pirfenidone, as well as their possibility of future expansion of application toward other fibrotic diseases. …
Y Bian, M Deng, Q Gao, G Zhou, R Tong, L Zhao, M Liu… - 2024
… TBLC is also included in clinical practice guidelines for hypersensitivity pneumonitis (7) and idiopathicpulmonaryfibrosis (IPF) (8) as a … Only a small number of patients were diagnosed with IPF and nonspecific interstitial pneumonia …
間質性肺疾患の診断における 1.1 mm 凍結プローブを使用した経気管支肺凍結生検の診断効率と安全性
S Arnold, AR Kitching, V Witko-Sarsat, T Wiech… - The Lancet Rheumatology, 2024
… About 5–10% of individuals diagnosed with idiopathicpulmonaryfibrosis and usual interstitial pneumonia are MPO-… idiopathicpulmonaryfibrosis after being ANCAnegative at the time of first evaluation. Finally, approximately 25% of …
… Additionally, we provide a brief summary of the two currently approved anti-fibrotic drugs for idiopathicpulmonaryfibrosis, nintedanib and … しかし本邦で承認されている 抗線維化薬は,特発性肺線維症(idiopathicpulmonaryfibrosis: IPF)に対するニンテダニブ …
SS Zamparelli, C Lombardi, C Candia, PR Iovine… - Journal of Clinical Medicine, 2024
… Idiopathicpulmonaryfibrosis (IPF) is a chronic and irreversible fibrotic disease whose natural history is … and IPF in particular, this comprehensive approach remains underused and under-resourced. In this review, we will discuss the …
PXL Gan, S Zhang, WSF Wong - Biochemical Pharmacology, 2024
… diseases such as asthma, idiopathicpulmonaryfibrosis (IPF), and chronic … , IPF, and COPD, supports targeting metabolism as a therapeutic approach for respiratory diseases. Correspondingly, inhibiting glycolysis has largely attested effective …
I Roger, P Montero, J Milara, J Cortijo - European Journal of Pharmacology, 2024
… Idiopathicpulmonaryfibrosis (IPF) associated to pulmonary hypertension (PH) portends a poor … Serum and parenchyma levels of Interleukin 11 (IL-11) are elevated in IPF-PH patients and … Idiopathicpulmonaryfibrosis (IPF) is a disease …
C Mao, Y Du, K Niu, F Yang, C Bai, B Zhou, Y Niu - Natural Product Communications, 2024
… is effective for the treatment of idiopathicpulmonaryfibrosis (IPF). In this study, we explored the anti-pulmonaryfibrosis effect of QZJFD and … In total, 3875 disease action targets were related to IPF, with 308 common targets shared by drugs and …
D Ishii, T Kawasaki, H Sato, K Tatsumi, T Imamoto… - International Journal of …, 2024
… Idiopathicpulmonaryfibrosis (IPF) is a specific form of chronic progressive fibrosing interstitial pneumonia of … IPF remain unclear. This study explored the potential PBMC signature in patients with IPF and the effects of anti-fibrotic drugs on …
… Idiopathicpulmonaryfibrosis (IPF), one of the most prevalent forms of interstitial lung disease (ILD), is a chronic and progressive … , IPF results in substantial respiratory impairment and poor prognoses, with a median survival of 3–5 years post-diagnosis …
L Yao, L Qu, J Deng, C Zhu, R Fu, M Jiang, D Fan - Food Frontiers
… ginsenoside Rh4 on idiopathicpulmonaryfibrosis (IPF): (a… IPF mice; (c) impact of ginsenoside Rh4 on mortality in IPF mice; (d) influence of ginsenoside Rh4 on hydroxyproline (HYP) content in the lungs of IPF mice; (e) effect of ginsenoside Rh4 …
… Idiopathicpulmonaryfibrosis (IPF), a form of this … in pulmonaryfibrosis through paracrine signalling, thus fostering epithelial-mesenchymal transition (EMT). Autophagy, a cellular recycling process, is a potential treatment strategy in several …
JV Pugashetti, JS Kim, MP Combs, SF Ma… - The Journal of Heart and …, 2024
… Lung transplantation remains the sole curative option for patients with idiopathicpulmonaryfibrosis (IPF), but donor organs remain scarce, and many eligible patients die prior to transplant. Tools to optimize the timing of transplant referral are urgently …
A Dare, SD King, SY Chen - Scientific Reports, 2024
… Surfactant protein A (SPA) regulates macrophage function, strongly binds to lipids, and is implicated in renal and idiopathicpulmonaryfibrosis (IPF). However, the role of SPA in lipid accumulation, inflammation, and hepatic fibrosis that characterize …
