Class 1. Pulmonary Hypertension Associated with Inflammation

Bronchiectasis in Inborn Errors of Immunity: Prevalence, Predictors, and Cardiopulmonary Complications in a Genetically Characterized Cohort

Diana Marangu-Boore, Katherine Myint-Hpu, Esther Kang, Luigi D. Notarangelo, Ottavia M. DelmonteNational Institutes of Health. University of Nairobi. United States and Kenya Journal of Clinical ImmunologyJ Clin Immunol 2026; DOI: 10.1007/s10875-026-02044-8 AbstractPurpose: Bronchiectasis poses a serious but incompletely defined burden in patients with inborn errors of immunity (IEI). We determined its prevalence, independent predictors, and cardiopulmonary complications […]

Bronchiectasis in Inborn Errors of Immunity: Prevalence, Predictors, and Cardiopulmonary Complications in a Genetically Characterized Cohort Read More »

Adventitial Niches, Complement and Inflammation in Pulmonary Vascular Disease: Current Status and Future Directions

Hui Zhang, Ram Raj Prasad, Sushil Kumar, Min Li, Dallas Jones, Cheng-Jun Hu, Claudia Mickael, Yen-Rei Yu, Rubin M. Tuder, Kurt R. StenmarkUniversity of Colorado Anschutz Medical Campus.United States Comprehensive PhysiologyCompre Physiol 2026; 16: DOI: 10.1002/cph4.70133 AbstractThere is strong evidence supporting inflammatory and autoimmune processes in the pathogenesis of pulmonary arterial hypertension (PAH), although the initiating

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Neonatal pulmonary vascular remodeling induced by increased blood flow is associated with an antiviral-like immune signature

Sixie Zheng, Hao Li, Siqi She, Yiting Xue, Debao Li, Jiapei Wang, Jing Wang, Yuqing Hu, Lincai YeShanghai Children’s Medical Center and Shanghai Jiao Tong University School of Medicine. Children’s Hospital of Fudan University and National Children’s Medical Center. Affiliated Women and Children’s Hospital of Ningbo University. China Frontiers in ImmunologyFront Immunol 2026; 17: DOI: 10.3389/fimmu.2026.1780303

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GM-CSF exacerbates pulmonary arterial hypertension via CCL2/CCR2-axis-mediated macrophage NLRP3 inflammasome activation

Ruoxuan Jiang, Liuyi Li, Yuhang Luo, Wen Wang, Xiaoya Zeng, Cheng Qian, Chen Gong, Jianlong ShenSecond Affiliated Hospital of Anhui Medical University. First Affiliated Hospital of Anhui Medical University. China International ImmunopharmacologyInt Immunopharmacol 2026; DOI: 10.1016/j.intimp.2026.116394 AbstractPulmonary arterial hypertension (PAH) is a fatal disease marked by pulmonary vascular remodeling. Although endothelial dysfunction and immune cell infiltration

GM-CSF exacerbates pulmonary arterial hypertension via CCL2/CCR2-axis-mediated macrophage NLRP3 inflammasome activation Read More »

Mixed effects of complement in a chronic murine model of inflammatory erosive arthritis and pulmonary vascular disease

Kiana L. Chen, Stacey Duemmel, Michael Christof, Gaochan Wang, H. Mark Kenney, Marc Nuzzo, Qingfu Xu, Benjamin Korman, Homaira RahimUniversity of Rochester Medical Center. United States Public Library of Science OnePLOS One 2026; 21: DOI: 10.1371/journal.pone.0340677 AbstractComplement’s role in the pathology of rheumatoid arthritis and pulmonary hypertension (PH) is not fully understood. We aimed to determine

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A Novel Rat Model for Group 2 Pulmonary Hypertension by Total Pulmonary Vein Banding: Multi-Omics Insights into Pathophysiological Mechanisms

Jin Shentu, Wenxuan Dai, Chang Chen, Jiawei Huang, Lijun Chen, Yi Yan, Han Zhang, Zhongqun Zhu, Guocheng Shi, Huiwen ChenShanghai Children’s Medical Center and Shanghai Jiao Tong University School of Medicine. China Journal of Thoracic and Cardiovascular SurgeryJ Thorac Cardiovasc Surg 2026; DOI: 10.1016/j.jtcvs.2026.01.017 AbstractObjectives: Group 2 pulmonary hypertension (PH) remains a highly morbid disease, yet no

A Novel Rat Model for Group 2 Pulmonary Hypertension by Total Pulmonary Vein Banding: Multi-Omics Insights into Pathophysiological Mechanisms Read More »

Gut Microbiota in Pulmonary Arterial Hypertension: Murine Models and Human Microbial Signatures, Pathogenic Mechanisms, and Emerging Therapeutic Avenues

Yudan Qiu, Xiaojiang Lyu, Dashuang Zhang, Hong Xu, Xu He, Jiao Chen, Hanmin Liu, Yang Liu, Liang XieWest China Second University Hospital and Sichuan University. School of Life Sciences of Fudan University and West China Institute of Women and Children’s Health. Affiliated Hospital of North Sichuan Medical College. Affiliated Hospital of Southwest Medical University. Xizang

Gut Microbiota in Pulmonary Arterial Hypertension: Murine Models and Human Microbial Signatures, Pathogenic Mechanisms, and Emerging Therapeutic Avenues Read More »

Urinary leukotriene E4 in pediatric pulmonary arterial hypertension: a pilot case control study

Hamdy El-Sayed, Hala Elmarsafawy, Basma Shouman, Mostafa Mansour, Amal OsmanMansoura University.Egypt Archive de PédiatrieArch Pediatr 2025; DOI: 10.1016/j.arcped.2025.08.004 AbstractBackground: Pediatric pulmonary arterial hypertension (PAH) is a potentially fatal disease. New targeted therapies based on the underlying pathogenesis are still needed.Objective: This study aims to evaluate the role of leukotriene E4, an inflammatory mediator, in developing pediatric pulmonary arterial

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IDO-1 Promotes Pulmonary Vascular Remodeling Via Kynurenine Pathway in Pulmonary Arterial Hypertension

Zongye Cai, Ly Tu, Siyu Tian, Lin Deng, Yahong Fu, Carole Phan, Thierry P. P. van den Bosch, Karin Tran‐Lundmark, Bence M. Nagy, Horst Olschewski, Xiaoxuan Li, Sihao Wang, Danan Wang, Yi Yan, Lijun Fu, Karin A. Boomars, Christophe Guignabert, Daphne MerkusSecond Affiliated Hospital and Zhejiang University School of Medicine. Erasmus MC and University Medical Center. Pathophysiologie et Innovation Thérapeutique (HPPIT) and Université Paris-Saclay, School of Medicine. Eighth Affiliated Hospital of Sun Yat-sen University. Lund University and Skane University Hospital. Medical University

IDO-1 Promotes Pulmonary Vascular Remodeling Via Kynurenine Pathway in Pulmonary Arterial Hypertension Read More »

Response to two Janus kinase inhibitors in a boy with SAVI during 2-year follow-up: case report and literature review

Yiting Chen, Wenhe Zang, Haoyuan Zhong, Xianqin Deng, Wenting Zhong, Lianyu Wang, Xinying ChenSecond Affiliated Hospital of Guangzhou University of Chinese Medicine and Guangdong Provincial Hospital of Chinese Medicine.China Frontiers in ImmunologyFront Immunol 2025; 15: DOI: 10.3389/fimmu.2025.1615075 AbstractSTING-associated vasculopathy with onset in infancy (SAVI) represents an identified rare type I interferonopathy, triggered by gain-of-function mutations in

Response to two Janus kinase inhibitors in a boy with SAVI during 2-year follow-up: case report and literature review Read More »

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