Review
Medicine, General & Internal
Carla R. Copeland, Lisa H. Lancaster
Summary: Progressive fibrosing interstitial lung diseases (PF-ILD) is a diverse group of interstitial lung diseases characterized by accelerated respiratory failure, frequent disease exacerbation, and earlier mortality. Available therapeutic approaches include antifibrotic therapy, immunosuppression, novel therapies, and lung transplantation.
FRONTIERS IN MEDICINE
(2021)
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)
Article
Pharmacology & Pharmacy
Sebastiano Emanuele Torrisi, Nicolas Kahn, Julia Waelscher, Markus Polke, Joyce S. Lee, Philip L. Molyneaux, Francesca Maria Sambataro, Claus Peter Heussel, Carlo Vancheri, Michael Kreuter
Summary: Almost one-third of fibrosing ILD (fILDs) have a clinical disease behavior similar to IPF, but there are no globally accepted criteria on the definition of a progressive phenotype in non-IPF fILD. Different definitions identify different groups of patients with progressive fILD, which may have important prognostic and therapeutic implications.
FRONTIERS IN PHARMACOLOGY
(2021)
Article
Biochemistry & Molecular Biology
Patrycja Rzepka-Wrona, Szymon Skoczynski, Adam Barczyk
Summary: This pilot study aimed to determine the differences in pathogenetic pathways between interstitial pneumonia with autoimmune features (IPAF), connective-tissue-disease-associated interstitial lung diseases (CTD-ILDs), and idiopathic interstitial pneumonias (IIPs). The study revealed that IL-8 and TGF-beta 1 concentrations were significantly lower in the IPAF group compared to the CTD-ILD group, but similar to the IIP group. IL-8 and CXCL1 were significantly correlated with BAL total cell count (TCC), and IL-8 and CXCL1 were found to be significant predictors of BAL TCC.
INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES
(2022)
Article
Respiratory System
Noriyuki Enomoto, Hyogo Naoi, Yasutaka Mochizuka, Takuya Isayama, Yuko Tanaka, Atsuki Fukada, Yuya Aono, Mineo Katsumata, Hideki Yasui, Kazutaka Mori, Masato Karayama, Hironao Hozumi, Yuzo Suzuki, Kazuki Furuhashi, Tomoyuki Fujisawa, Naoki Inui, Yutaro Nakamura, Takafumi Suda
Summary: This retrospective study analyzed data of patients with SAID-ILD between 1999 and 2020 to determine the incidences of AE-ILD in each SAID and the proportion of PF-ILD in AE-SAID-ILD. The study found that the incidence of AE-SAID-ILD was significantly higher in patients with RA, MPA, or SSc than in patients with other SAID. Additionally, the proportion of PF-ILD just before AE in AE-SAID-ILD was not high (20%).
BMC PULMONARY MEDICINE
(2022)
Article
Medicine, General & Internal
Maria Boutel, Afroditi Boutou, Georgia Pitsiou, Alexandros Garyfallos, Theodoros Dimitroulas
Summary: This study assessed the effectiveness and safety of nintedanib in CTD-ILD patients, and found that it could be beneficial in combination with immunosuppressives in slowing the rate of lung function decline.
Article
Cardiac & Cardiovascular Systems
Dilber Ademhan Tural, Nagehan Emiralioglu, Beste Ozsezen, Birce Sunman, Halime Nayir Buyuksahin, Ismail Guzelkas, Berna Oguz, Yelda Bilginer, Diclehan Orhan, Ebru Yalcin, Deniz Dogru, Ugur Ozcelik, Seza Ozen, Nural Kiper
Summary: This study evaluated 17 ILD patients for IPAF criteria, with 6 patients being diagnosed with IPAF. The incidence of IPAF in children with ILD was 4.5%, and the study reviewed the demographic, clinical features, and treatment of these patients.
RESPIRATORY MEDICINE
(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
Medicine, General & Internal
Francesco Bozzao, Paola Tomietto, Elisa Baratella, Metka Kodric, Rossella Cifaldi, Rossana Della Porta, Ilaria Prearo, Silvia Maria Grazia Pirronello, Paola Confalonieri, Barbara Ruaro, Fabio Fischetti, Bruno Fabris
Summary: This study aimed to describe the clinical characteristics and predictive factors for progression of ILD patients with autoimmune features. The results showed that IPAF patients had a lower prevalence of UIP pattern compared to CTD-ILD and UIPAF patients. ILD progression was less frequent in the IPAF group compared to CTD-ILD and UIPAF groups. The definition of UIPAF should be separated from ILD classification and include sicca syndrome for better prognosis prediction.
MEDICINA-LITHUANIA
(2023)
Review
Cardiac & Cardiovascular Systems
Mei Yang, Yanqing Wu, Xuemei Liu, Chen Zhao, Ting Li, Tingqian Li, Xiawei Zhang, Hongli Jiang, Bing Mao, Wei Liu
Summary: This study investigated the efficacy and safety of antifibrotic agents in the treatment of CTD-ILD and RA-ILD. The results suggest that antifibrotic treatment can significantly slow the decline of FVC. However, more high-quality trials are needed to provide further evidence.
RESPIRATORY MEDICINE
(2023)
Article
Rheumatology
Nikhil Jiwrajka, Giorgos Loizidis, Karen C. Patterson, Maryl E. Kreider, Cheilonda R. Johnson, Wallace T. Miller, Eduardo Jose Mortani Barbosa, Namrata Patel, Michael F. Beers, Leslie A. Litzky, Michael D. George, Mary K. Porteous
Summary: This study aimed to identify patients with IPAF, CTD-ILD, and IPF and evaluate the prognosis of IPAF patients. The results showed that IPAF patients had similar transplant-free survival compared to CTD-ILD and IPF patients. Male sex may be associated with worse prognosis in IPAF.
JCR-JOURNAL OF CLINICAL RHEUMATOLOGY
(2022)
Review
Medicine, General & Internal
Willis S. Bowman, Gabrielle A. Echt, Justin M. Oldham
Summary: Interstitial lung disease (ILD) is a group of diverse lung disorders that can lead to irreversible pulmonary fibrosis. While idiopathic pulmonary fibrosis (IPF) is a common subtype, other ILD patients may develop a progressive fibrosing phenotype. Biomarker investigation has identified molecular markers that can predict disease endpoints, suggesting potential for biomarker implementation in ILD management.
FRONTIERS IN MEDICINE
(2021)
Article
Rheumatology
Michael R. Allen, Michail K. Alevizos, David Zhang, Elana J. Bernstein
Summary: This study assessed the ability of two risk prediction models (GAP and ILD-GAP) to predict death or lung transplantation in patients with interstitial pneumonia with autoimmune features (IPAF). The results showed that these models performed well, especially for patients with GAP stage 1 and GAP stage 2 disease.
Article
Respiratory System
Kyung-In Joung, Hyemin Park, Sunyoung Park, Ju-Young Shin, Yong Hyun Kim
Summary: This study aimed to estimate the prevalence, incidence, and mortality of fibrosing interstitial lung disease (F-ILD) in Korea. The results showed that the prevalence and incidence of idiopathic pulmonary fibrosis (IPF) and progressive fibrosing ILD (PF-ILD) have been steadily increasing in recent years. The mortality rate of PF-ILD remained consistently high and exceeded that of IPF in all years.
BMC PULMONARY MEDICINE
(2023)
Review
Biochemistry & Molecular Biology
Giuliana Cerro Chiang, Tanyalak Parimon
Summary: CTD-ILD is a group of systemic autoimmune disorders that cause lung interstitial abnormalities or lung fibrosis. The pathogenesis of CTD-ILD is not well understood, but it shares common risk factors with idiopathic pulmonary fibrosis. The primary mechanism involves dysfunction of alveolar type II cells, triggering inflammatory cascades and leading to abnormal lung remodeling and fibrosis. Understanding these mechanisms is important for developing targeted therapies and identifying disease biomarkers.
INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES
(2023)