Review
Biochemistry & Molecular Biology
Francesco Amati, Anna Stainer, Veronica Polelli, Marco Mantero, Andrea Gramegna, Francesco Blasi, Stefano Aliberti
Summary: Pirfenidone and nintedanib are effective in slowing disease progression in interstitial lung diseases other than idiopathic pulmonary fibrosis. Two well-designed trials demonstrate the efficacy of nintedanib, while caution should be taken in interpreting the results of pirfenidone due to trial limitations. Ongoing randomized control trials aim to improve the quality of evidence in this field.
INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES
(2023)
Article
Biochemistry & Molecular Biology
Nora Melinda Toth, Veronika Muller, Tamas Nagy, Lorinc Polivka, Peter Horvath, Aniko Bohacs, Noemi Eszes
Summary: Elevated serum progranulin (PGRN) levels may help differentiate between idiopathic pulmonary fibrosis (IPF) and other interstitial lung diseases (ILDs). Non-IPF ILD patients had significantly higher serum PGRN levels compared to healthy subjects and IPF patients. Non-UIP patterns on high-resolution computed tomography (HRCT) were associated with significantly elevated PGRN levels, while UIP patterns were associated with normal PGRN levels.
INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES
(2023)
Review
Medicine, General & Internal
John N. Shumar, Abhimanyu Chandel, Christopher S. King
Summary: Progressive fibrosing interstitial lung disease (PF-ILD) is a subset of interstitial lung diseases characterized by progressive and intractable lung fibrosis, with similarities to idiopathic pulmonary fibrosis. Recent studies have shown the clinical efficacy of antifibrotic therapy in PF-ILD, changing the treatment landscape for fibrotic lung diseases and opening the door for combined therapies targeting both fibrotic and inflammatory components.
JOURNAL OF CLINICAL MEDICINE
(2021)
Review
Respiratory System
James Patrick Finnerty, Aravind Ponnuswamy, Prosjenjit Dutta, Ammar Abdelaziz, Hafiz Kamil
Summary: This study compared the efficacy of nintedanib and pirfenidone in the treatment of progressive pulmonary fibrosis, finding that anti-fibrotic therapy can slow the decline in forced vital capacity in both IPF and non-IPF patients. However, there was insufficient data to confirm a reduction in mortality in non-IPF patients with anti-fibrotic therapy.
BMC PULMONARY MEDICINE
(2021)
Article
Medicine, General & Internal
Sara Tomassetti, Claudia Ravaglia, Sara Piciucchi, Jay Ryu, Athol Wells, Luca Donati, Alessandra Dubini, Catherine Klersy, Valentina Luzzi, Leonardo Gori, Elisabetta Rosi, Federico Lavorini, Venerino Poletti
Summary: This study evaluated the survival of IPF patients over the past 15 years, and found that antifibrotic therapy significantly improved patient survival, along with the introduction of cryobiopsy and antifibrotic drugs, leading to an earlier detection and better prognosis of IPF.
FRONTIERS IN MEDICINE
(2023)
Article
Medicine, General & Internal
Junyu Liang, Heng Cao, Yang Yang, Yini Ke, Ye Yu, Chuanyin Sun, Lihuan Yue, Jin Lin
Summary: This study aimed to clarify the efficacy and tolerability of nintedanib in patients with IIM-ILD, and results showed that nintedanib therapy could reduce the incidence of RP-ILD and improve survival in these patients. Anti-MDA5 antibody and disease activity were significant factors associated with unfavorable outcomes, while hepatic insufficiency was closely related to dosage reduction or therapy discontinuation.
FRONTIERS IN MEDICINE
(2021)
Article
Immunology
Yuxue Chen, Zhiqian Bai, Ziyun Zhang, Qiongjie Hu, Jixin Zhong, Lingli Dong
Summary: The study aimed to examine the efficacy of tacrolimus in the treatment of idiopathic inflammatory myopathies-associated interstitial lung disease (IIM-ILD) on top of glucocorticoids and assess the therapeutic benefit and safety of low-dose pirfenidone as an add-on treatment. The results showed that tacrolimus was well-tolerated and effective in the treatment of IIM-ILD, and the combination of tacrolimus and low-dose pirfenidone resulted in favorable improvements in pulmonary involvements for the patients.
FRONTIERS IN IMMUNOLOGY
(2022)
Article
Medicine, Research & Experimental
Vincent Cottin, Sonia Gueguen, Hilario Nunes, Stephane Jouneau, Bruno Crestani, Philippe Bonniaud, Lidwine Wemeau, Dominique Israel-Biet, Martine Reynaud-Gaubert, Anne Gondouin, Jacques Cadranel, Sylvain Marchand-Adam, Marie Chevereau, Isabelle Dufaure-Gare, Serge Amselem, Annick Clement
Summary: This study in France on IPF patients using pirfenidone capsules and tablets showed good acceptability of the tablet formulation among patients, suggesting possible advantages over the capsules. The study highlighted no new adverse events and lower discontinuation rates in the tablet and substitution subgroups compared to the capsules-only subgroup.
ADVANCES IN THERAPY
(2022)
Article
Respiratory System
Toby M. Maher, Kevin K. Brown, Michael Kreuter, Anand Devaraj, Simon L. F. Walsh, Lisa H. Lancaster, Elizabeth A. Belloli, Maria Padilla, Juergen Behr, Rainer-Georg Goeldner, Kay Tetzlaff, Rozsa Schlenker-Herceg, Kevin R. Flaherty
Summary: The study investigated the effect of nintedanib on progressive fibrosing interstitial lung diseases (ILDs). The inclusion criteria for ILD progression were used to classify patients into different subgroups. The results showed that nintedanib can reduce the rate of decline in forced vital capacity (FVC) across all subgroups, indicating its effectiveness in treating progressive fibrosing ILDs.
EUROPEAN RESPIRATORY JOURNAL
(2022)
Article
Respiratory System
Tamera J. Corte, Lisa Lancaster, Jeffrey J. Swigris, Toby M. Maher, Jonathan G. Goldin, Scott M. Palmer, Takafumi Suda, Takashi Ogura, Anne Minnich, Xiaojiang Zhan, Giridhar S. Tirucherai, Brandon Elpers, Hong Xiao, Hideaki Watanabe, R. Adam Smith, Edgar D. Charles, Aryeh Fischer
Summary: IPF and PF-ILD are progressive fibrotic lung diseases associated with a gradual loss of lung function and poor prognosis. Treatment with antifibrotic agents can slow disease progression, but treatment discontinuation due to adverse events is common. BMS-986278, a second-generation LPA(1) antagonist, is currently being developed as a therapy for these diseases in a phase 2 trial.
BMJ OPEN RESPIRATORY RESEARCH
(2021)
Article
Medicine, General & Internal
Elisabetta Zanatta, Andrea Martini, Roberto Depascale, Anna Gamba, Marta Tonello, Mariele Gatto, Chiara Giraudo, Elisabetta Balestro, Andrea Doria, Luca Iaccarino
Summary: By assessing the role of CCL18 and OX40L as biomarkers of interstitial lung disease (ILD) and/or progressive fibrosing (PF-ILD) in idiopathic inflammatory myopathies (IIMs), we found that CCL18 may be a useful biomarker in IIMs-ILD, particularly in the early identification of patients at risk of developing PF-ILD.
Article
Pharmacology & Pharmacy
K. M. Roach, E. Castells, K. Dixon, S. Mason, G. Elliott, H. Marshall, M. A. Poblocka, S. Macip, M. Richardson, L. Khalfaoui, P. Bradding
Summary: Pirfenidone and nintedanib show modest anti-fibrotic effects in a human model of lung fibrogenesis, while the K(Ca)3.1 blocker senicapoc may have greater benefits in treating IPF based on its attenuation of fibrosis-associated genes expression.
FRONTIERS IN PHARMACOLOGY
(2021)
Article
Respiratory System
Michael T. Durheim, Elisabeth Bendstrup, Lisa Carlson, Eva M. Sutinen, Charlotte Hyldgaard, Dimitrios Kalafatis, Marjukka Myllarniemi, C. Magnus Skold, Tone Sjaheim
Summary: The study found that patients with advanced idiopathic pulmonary fibrosis (IPF) at the initiation of antifibrotic therapy did not experience greater decline in lung function over time compared to those with mild-moderate IPF, but had significantly higher mortality rates. Prospective studies are needed to determine the effect of antifibrotic therapy in patients with advanced IPF.
Article
Respiratory System
Lavanya Raman, Iain Stewart, Shaney L. Barratt, Felix Chua, Nazia Chaudhuri, Anjali Crawshaw, Michael Gibbons, Charlotte Hogben, Rachel Hoyles, Vasilis Kouranos, Jennifer Martinovic, Sarah Mulholland, Katherine J. Myall, Marium Naqvi, Elisabetta A. Renzoni, Peter Saunders, Matthew Steward, Dharmic Suresh, Muhunthan Thillai, Athol U. Wells, Alex West, Jane A. Mitchell, Peter M. George
Summary: In this retrospective cohort study, nintedanib was found to significantly attenuate lung function decline for patients with progressive pulmonary fibrosis (PPF). The real-world efficacy of nintedanib replicated that of clinical trials, and it was also shown to be safe and well tolerated.
Article
Cell Biology
Kazutaka Takehara, Yasuhiko Koga, Yoshimasa Hachisu, Mitsuyoshi Utsugi, Yuri Sawada, Yasuyuki Saito, Seishi Yoshimi, Masakiyo Yatomi, Yuki Shin, Ikuo Wakamatsu, Kazue Umetsu, Shunichi Kouno, Junichi Nakagawa, Noriaki Sunaga, Toshitaka Maeno, Takeshi Hisada
Summary: In patients with IPF, the discontinuation rate of nintedanib treatment due to adverse events is higher than that of pirfenidone treatment. Better management of adverse events with nintedanib leads to more continuous treatment, preventing disease progression and acute exacerbations, thus improving prognosis for IPF patients.