Article
Allergy
Wanlong Wu, Li Guo, Yakai Fu, Kaiwen Wang, Danting Zhang, Wenwen Xu, Zhiwei Chen, Shuang Ye
Summary: MDA5(+) DM is a rare autoimmune disease predominantly reported in East Asia, characterized by high mortality due to rapid-progressive interstitial lung disease. The prognosis factors and treatment regimens for this disease still require further research.
CLINICAL REVIEWS IN ALLERGY & IMMUNOLOGY
(2021)
Review
Medical Laboratory Technology
Xiaomeng Li, Yongmei Liu, Linlin Cheng, Yuan Huang, Songxin Yan, Haolong Li, Haoting Zhan, Yongzhe Li
Summary: Studies have shown that dysregulated expression of blood biomarkers related to ILD, such as ferritin, KL-6, SP-D, cytokines, and some tumor markers in MDA5(+)DM, may provide valuable information for disease presence, activity, treatment response, and prognosis.
JOURNAL OF CLINICAL LABORATORY ANALYSIS
(2022)
Review
Medicine, General & Internal
Hideaki Yamakawa, Yuko Toyoda, Tomohisa Baba, Tomoo Kishaba, Taiki Fukuda, Tamiko Takemura, Kazuyoshi Kuwano
Summary: The presence of lung lesions in microscopic polyangiitis (MPA) and interstitial lung disease (ILD) can lead to a poor prognosis. The relationship between myeloperoxidase antineutrophil cytoplasmic antibodies (MPO-ANCA), MPA, and idiopathic ILD remains unclear. Treatment strategies for MPO-ANCA-positive ILD patients without systemic vasculitis should be carefully considered.
JOURNAL OF CLINICAL MEDICINE
(2022)
Article
Immunology
Yu Zuo, Lifang Ye, Fang Chen, Yawen Shen, Xin Lu, Guochun Wang, Xiaoming Shu
Summary: RP-ILD is more prevalent in MDA5(+)DM patients compared to ASS patients, with lower survival rates. Independent risk factors for RP-ILD in MDA5(+)DM patients include fever, lymphopenia, decreased T cell levels, elevated ALT, high LDH, hyper-ferritin, and elevated CEA, among others. In contrast, independent risk factors for RP-ILD in ASS patients are elevated CEA, CA125, and NSE.
FRONTIERS IN IMMUNOLOGY
(2022)
Article
Cell Biology
Lin Liang, Ya-Mei Zhang, Ya-Wen Shen, Ai-Ping Song, Wen-Li Li, Li-Fang Ye, Xin Lu, Guo-Chun Wang, Qing-Lin Peng
Summary: The study revealed that serum Gal-9 levels were significantly elevated in DM patients and were associated with the severity of RP-ILD in anti-MDA5-positive patients. Gal-9 may serve as a promising biomarker for monitoring disease activity in DM patients, particularly in anti-MDA5-positive patients with RP-ILD. Aberrant expression of the Gal-9/Tim-3 axis may be involved in the immunopathogenesis of DM-ILD.
FRONTIERS IN CELL AND DEVELOPMENTAL BIOLOGY
(2021)
Article
Rheumatology
Wanlong Wu, Wenwen Xu, Wenjia Sun, Danting Zhang, Jiangfeng Zhao, Qun Luo, Xiaodong Wang, Feng Zhu, Yu Zheng, Yu Xue, Weiguo Wan, Huaxiang Wu, Qian Han, Shuang Ye
Summary: This study aimed to establish and validate a clinical prediction model for 6-month all-cause mortality in Chinese patients with anti-MDA5 positive DM-ILD. The results showed that a categorical predictor based on forced vital capacity (FVC)% was identified and validated as an independent prognostic factor, which could effectively distinguish the risk stages in patients and facilitate risk stratification in clinical practice.
Article
Immunology
Xixia Chen, Lu Zhang, Qiwen Jin, Xin Lu, Jieping Lei, Qinglin Peng, Guochun Wang, Yongpeng Ge
Summary: This study explores the clinical features and prognoses of patients with dermatomyositis who have both positive anti-MDA5 and anti-ARS antibodies. The results show that these patients have distinct clinical and radiological characteristics compared to other subgroups, and they may respond well to glucocorticoid therapy combined with immunosuppressants.
FRONTIERS IN IMMUNOLOGY
(2022)
Article
Rheumatology
Masahiro Kogami, Yoshiyuki Abe, Taiki Ando, Ayako Makiyama, Ken Yamaji, Naoto Tamura
Summary: The anti-MDA5 antibody titre at diagnosis may predict the clinical course of anti-melanoma differentiation-associated gene 5 antibody-positive dermatomyositis. Levels of macrophage-associated cytokines significantly decline at 6 months post-treatment, and they may increase after anti-MDA5 antibody titre positivity.
Article
Medicine, General & Internal
Xia Lv, Yuyang Jin, Danting Zhang, Yixuan Li, Yakai Fu, Suli Wang, Yan Ye, Wanlong Wu, Shuang Ye, Bing Yan, Xiaoxiang Chen
Summary: This study revealed that in patients with anti-MDA5 antibody-positive dermatomyositis, lymphocyte and monocyte counts early in the disease course may serve as predictive biomarkers for 6-month all-cause mortality, with lower counts indicating higher risk of death.
FRONTIERS IN MEDICINE
(2022)
Article
Immunology
Meiqi Li, Xuli Zhao, Baocheng Liu, Yaqi Zhao, Xinya Li, Zhenzhen Ma, Qingrui Yang
Summary: In patients with anti-MDA5+ DM, elevated LDH and PNI were identified as independent prognostic factors, with increased mortality associated with elevated LDH (p = 0.01) and reduced mortality associated with elevated PNI (p < 0.001). RP-ILD, the most serious complication of anti-MDA5+ DM, was characterized by significantly increased white blood cell count and LDH concentration (p = 0.007 and p = 0.019, respectively), while PNI was significantly lower (p < 0.001). Measurement of LDH and PNI could be valuable for diagnosis, assessment of lung involvement, and prognosis prediction in patients with anti-MDA5+ DM.
FRONTIERS IN IMMUNOLOGY
(2023)
Article
Immunology
Xianhua Gui, Wangzhong Li, Yanzhe Yu, Tingting Zhao, Ziyi Jin, Kaifang Meng, Rujia Wang, Shenyun Shi, Min Yu, Miao Ma, Lulu Chen, Wei Luan, Xiaoyan Xin, Yuying Qiu, Xiaohua Qiu, Yingwei Zhang, Min Cao, Mengshu Cao, Jinghong Dai, Hourong Cai, Mei Huang, Yonglong Xiao
Summary: We developed and validated a mortality risk prediction tool with good discrimination and calibration for Asian patients with anti-MDA5 DM-ILD. This tool can offer individualized mortality risk estimation and inform clinical decision-making.
FRONTIERS IN IMMUNOLOGY
(2022)
Article
Rheumatology
Antao Xu, Yan Ye, Qiong Fu, Xinyue Lian, Sheng Chen, Qiang Guo, Liang-jing Lu, Min Dai, Xia Lv, Chunde Bao
Summary: Anti-Ro52 antibody is highly prevalent in anti-MDA5-positive CADM-ILD patients and their coexistence correlates with a subgroup of patients with more aggressive phenotypes. The combination of anti-MDA5 antibody levels and anti-Ro52 antibody status could help to predict patients' prognosis and guide risk-based therapy.
Article
Rheumatology
Kenichiro Hata, Takuya Kotani, Shogo Matsuda, Youhei Fujiki, Takayasu Suzuka, Takao Kiboshi, Yumiko Wada, Hideyuki Shiba, Takeshi Shoda, Maki Kagitani, Tohru Takeuchi
Summary: Aggressive multi-combination therapy may improve the survival outcome of patients with anti-MDA5 antibody-positive DM-RPILD. Group B exhibited a lower corticosteroid dose during the treatment period compared to Group A.
INTERNATIONAL JOURNAL OF RHEUMATIC DISEASES
(2023)
Article
Rheumatology
Xinyue Lian, Yan Ye, Jing Zou, Chunmei Wu, Shuang Ye, Qiang Guo, Sheng Chen, Liangjing Lu, Ran Wang, Qiong Fu, Chunde Bao
Summary: This study conducted an 8-year longitudinal study on MDA5+ DM-ILD patients, and found that the mortality rate was still high despite aggressive treatment. Patients who survived the first year usually showed a significant improvement in serological markers and pulmonary function during long-term follow-up.
Article
Immunology
Zong Jiang, Xiaoling Yao, Fang Tang, Wukai Ma
Summary: This study reports a case of anti-MDA5 antibody-positive DM-ILD, in which tofacitinib and tacrolimus were used as treatment options after conventional immunosuppressive therapy and hormone therapy. The patient showed remission of clinical symptoms, reversal of lung imaging tests, and conversion of anti-MDA5 antibody from positive to negative.
IMMUNITY INFLAMMATION AND DISEASE
(2023)
Article
Critical Care Medicine
Laurens J. De Sadeleer, John E. McDonough, Jonas C. Schupp, Xiting Yan, Arno Vanstapel, Anke Van Herck, Stephanie Everaerts, Vincent Geudens, Annelore Sacreas, Tinne Goos, Celine Aelbrecht, Tim S. Nawrot, Dries S. Martens, Dominique Schols, Sandra Claes, Johny A. Verschakelen, Eric K. Verbeken, Maximilian Ackermann, Anabelle Decottignies, Manon Mahieu, Tillie-Louise Hackett, James C. Hogg, Bart M. Vanaudenaerde, Stijn E. Verleden, Naftali Kaminski, Wim A. Wuyts
Summary: This study identified molecular traits that characterize the morphological progression of fibrotic hypersensitivity pneumonitis (fHP) and their associations with clinical behavior. Comparing with idiopathic pulmonary fibrosis (IPF), the transcriptome landscape is determined by local disease extent rather than diagnosis alone.
AMERICAN JOURNAL OF RESPIRATORY AND CRITICAL CARE MEDICINE
(2022)
Article
Critical Care Medicine
Laurens J. De Sadeleer, Stijn E. Verleden, Jonas C. Schupp, John E. McDonough, Tinne Goos, Jonas Yserbyt, Elena Bargagli, Paola Rottoli, Naftali Kaminski, Antje Prasse, Wim A. Wuyts
Summary: Gene expression-based endotyping is feasible in IPF and can inform clinical evolution, opening up the possibility of personalized therapy.
Article
Multidisciplinary Sciences
Maor Sauler, John E. McDonough, Taylor S. Adams, Neeharika Kothapalli, Thomas Barnthaler, Rhiannon B. Werder, Jonas C. Schupp, Jessica Nouws, Matthew J. Robertson, Cristian Coarfa, Tao Yang, Maurizio Chioccioli, Norihito Omote, Carlos Cosme, Sergio Poli, Ehab A. Ayaub, Sarah G. Chu, Klaus H. Jensen, Jose L. Gomez, Clemente J. Britto, Micha Sam B. Raredon, Laura E. Niklason, Andrew A. Wilson, Pascal N. Timshel, Naftali Kaminski, Ivan O. Rosas
Summary: Chronic obstructive pulmonary disease (COPD) is a leading cause of death worldwide, but our understanding of cell-specific mechanisms underlying COPD pathobiology remains incomplete. In this study, the authors used single-cell RNA sequencing to analyze human lung tissue and identified transcriptional changes in alveolar niche cells associated with the disease.
NATURE COMMUNICATIONS
(2022)
Article
Multidisciplinary Sciences
Avraham Unterman, Tomokazu S. Sumida, Nima Nouri, Xiting Yan, Amy Y. Zhao, Victor Gasque, Jonas C. Schupp, Hiromitsu Asashima, Yunqing Liu, Carlos Cosme, Wenxuan Deng, Ming Chen, Micha Sam Brickman Raredon, Kenneth B. Hoehn, Guilin Wang, Zuoheng Wang, Giuseppe DeIuliis, Neal G. Ravindra, Ningshan Li, Christopher Castaldi, Patrick Wong, John Fournier, Santos Bermejo, Lokesh Sharma, Arnau Casanovas-Massana, Chantal B. F. Vogels, Anne L. Wyllie, Nathan D. Grubaugh, Anthony Melillo, Hailong Meng, Yan Stein, Maksym Minasyan, Subhasis Mohanty, William E. Ruff, Inessa Cohen, Khadir Raddassi, Laura E. Niklason, Albert Ko, Ruth R. Montgomery, Shelli F. Farhadian, Akiko Iwasaki, Albert C. Shaw, David van Dijk, Hongyu Zhao, Steven H. Kleinstein, David A. Hafler, Naftali Kaminski, Charles S. Dela Cruz
Summary: Using multi-omics single-cell analysis, this study reveals the immune abnormalities associated with progressive COVID-19, including S100A(hi)/HLA-DRlo classical monocytes and activated LAG-3(hi) T cells. It also finds a correlation between T cell receptor repertoire and B cell somatic hypermutation frequency with disease progression.
NATURE COMMUNICATIONS
(2022)
Article
Biology
Christopher J. Brereton, Liudi Yao, Elizabeth R. Davies, Yilu Zhou, Milica Vukmirovic, Joseph A. Bell, Siyuan Wang, Robert A. Ridley, Lareb S. N. Dean, Orestis G. Andriotis, Franco Conforti, Lennart Brewitz, Soran Mohammed, Timothy Wallis, Ali Tavassoli, Rob M. Ewing, Aiman Alzetani, Benjamin G. Marshall, Sophie Fletcher, Philipp J. Thurner, Aurelie Fabre, Naftali Kaminski, Luca Richeldi, Atul Bhaskar, Christopher J. Schofield, Matthew Loxham, Donna E. Davies, Yihua Wang, Mark G. Jones
Summary: This study provides evidence that the activation of the hypoxia-inducible factor (HIF) pathway is a critical regulator of pathogenetic collagen structure-function in fibrosis. Oxidative stress can drive the activation of pseudohypoxic HIF pathway, which alters collagen nanostructure and increases tissue stiffness.
Article
Physiology
Dinesh Babu Uthaya Kumar, Efthymios Motakis, Marina Yurieva, Vivek Kohar, Jan Martinek, Te-Chia Wu, Johad Khoury, Jessica Grassmann, Mingyang Lu, Karolina Palucka, Naftali Kaminski, Jonathan L. Koff, Adam Williams
Summary: This study identified a novel epithelial cell type called aberrant basaloid cells in fibrotic lung tissue. The role of epithelial-mesenchymal transition (EMT) in the development of these cells was investigated, and it was found that EMT induction led to transcriptional changes and a partial EMT gene signature in the cells. The study highlights the importance of using physiologically relevant models, such as air-liquid interface (ALI) cultures, to study aberrant basaloid-like cells and EMT mechanisms.
AMERICAN JOURNAL OF PHYSIOLOGY-LUNG CELLULAR AND MOLECULAR PHYSIOLOGY
(2022)
Article
Multidisciplinary Sciences
Xu Zhou, Ruth A. Franklin, Miri Adler, Trevor S. Carter, Emily Condiff, Taylor S. Adams, Scott D. Pope, Naomi H. Philip, Matthew L. Meizlish, Naftali Kaminski, Ruslan Medzhitov
Summary: This article reports on the mechanisms controlling the population numbers of different cell types in animal tissues. The study shows that fibroblasts are limited in proliferation due to space availability, while macrophages are constrained by growth factor availability. The transcriptional coactivator YAP1 in fibroblasts directly regulates the expression of the macrophage-specific growth factor Csf1. The findings suggest that tissue environment sensing is a general strategy for controlling tissue composition.
PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA
(2022)
Article
Respiratory System
Ming Chen, Yiliang Zhang, Taylor Adams, Dingjue Ji, Wei Jiang, Louise Wain, Michael Cho, Naftali Kaminski, Hongyu Zhao
Summary: Our study identified new genes associated with IPF susceptibility through integrative analysis, expanding the understanding of the complex genetic architecture and disease mechanism of IPF.
Article
Respiratory System
Rene Cortese, Taylor S. Adams, Kylie H. Cataldo, Justin Hummel, Naftali Kaminski, Leila Kheirandish-Gozal, David Gozal
Summary: Obstructive sleep apnoea (OSA) is a prevalent disease that affects neurocognitive, behavioural, metabolic, and cardiovascular function. This study used single-cell transcriptomics analysis to show the impact of OSA on the composition of immune cells in peripheral blood mononuclear cells (PBMCs), and identified severity-dependent differences in cell lineages. A molecular signature consisting of 32 genes was also identified, which could accurately distinguish OSA patients from controls.
EUROPEAN RESPIRATORY JOURNAL
(2023)
Article
Medicine, Research & Experimental
Bianca M. . Vamesu, Teodora Nicola, Rui Li, Snehashis Hazra, Sadis Matalon, Naftali Kaminski, Namasivayam Ambalavanan, Jegen Kandasamy
Summary: Mitochondrial dysfunction at birth is associated with the development of bronchopulmonary dysplasia (BPD) in extremely low-birth weight (ELBW) infants. Nebulized thyroid hormone (TH) has shown potential to improve mitochondrial function and reduce pulmonary fibrosis in adult animals. This study investigated the effects of intranasal T3 on neonatal lung injury and mitochondrial dysfunction in newborn mice, as well as its impact on lung fibroblasts and mesenchymal stem cells from ELBW infants. The results demonstrated that inhaled T3 attenuated hyperoxia-induced lung injury and improved mitochondrial function in mice, suggesting that TH supplementation might be a valuable therapeutic strategy for BPD.
Article
Cell & Tissue Engineering
Katherine L. Leiby, Yifan Yuan, Ronald Ng, Micha Sam Brickman Raredon, Taylor S. Adams, Pavlina Baevova, Allison M. Greaney, Karen K. Hirschi, Stuart G. Campbell, Naftali Kaminski, Erica L. Herzog, Laura E. Niklason
Summary: By modulating the components of the alveolar epithelial cell niche, researchers have successfully guided the regeneration of functional alveolar cells, potentially expanding treatment options for patients with end-stage lung disease.
NPJ REGENERATIVE MEDICINE
(2023)
Article
Critical Care Medicine
John C. Huston, Naftali Kaminski
Summary: With digital technology, medical education has transitioned from chalk talks to digital formats, and now artificial intelligence-generated art is being introduced as a powerful tool. This tool can improve medical education in narrative medicine and educational imagery.
Editorial Material
Critical Care Medicine
Jonas C. Schupp, Naftali Kaminski
AMERICAN JOURNAL OF RESPIRATORY AND CRITICAL CARE MEDICINE
(2023)
Letter
Critical Care Medicine
Jonas C. Schupp, Edward P. Manning, Maurizio Chioccioli, Jan C. Kamp, Leonard Christian, Changwan Ryu, Erica Herzog, Mark P. Kuehnel, Antje Prasse, Naftali Kaminski, Danny D. Jonigk, Robert J. Homer
AMERICAN JOURNAL OF RESPIRATORY AND CRITICAL CARE MEDICINE
(2023)
Article
Respiratory System
Alison G. Lee, Jason Maley, Kathryn Hibbert, Kathleen M. Akgun, Katrina E. Hauschildt, Anica Law, Naftali Kaminski, Margaret Hayes, Yaron Gesthalter, Gabriel T. Bosslet, Lekshmi Santhosh, Alison Witkin, Kelsey Hills-Dunlap, Basak Coruh, Hayley B. Gershengorn, C. Corey Hardin
ANNALS OF THE AMERICAN THORACIC SOCIETY
(2023)