APDS patients with immune-complex vasculitis and resolution with leniolisib
Clin Immunol. 2024 Mar 8:110176. doi: 10.1016/j.clim.2024.110176. Online ahead of print.ABSTRACTActivated phosphoinositide 3-kinase delta syndrome (APDS) is an inborn error of immunity with heterogeneity in the clinical manifestations of immune dysregulation, including recurrent sinopulmonary infections, bronchiectasis, chronic herpesvirus infections, autoimmunity, lymphoproliferation, and malignancy. Immune complexmediated vasculitides have not yet been described in APDS patients. Here we offer a case series of three patients with APDS who have refractory IgA vasculitis (also called Henoch-Schönlein purpura), a form of i...
Source: Clinical Immunology - March 10, 2024 Category: Allergy & Immunology Authors: Mohammad-Ali Dorodouchi Alexis V Stephens Ziwei Wang Jaspreet Dhami Manish J Butte Source Type: research
Deep immune profiling of chronic rhinosinusitis in allergic and non-allergic cohorts using mass cytometry
In conclusion, there are distinct immunological features in PBMCs of CRS phenotypes and allergy.PMID:38462155 | DOI:10.1016/j.clim.2024.110174 (Source: Clinical Immunology)
Source: Clinical Immunology - March 10, 2024 Category: Allergy & Immunology Authors: Fana Alem Kidane Lena M üller Marianne Rocha-Hasler Aldine Tu Victoria Stanek Nicholas Campion Tina Bartosik Mohammed Zghaebi Slagjana Stoshikj Daniela Gompelmann Andreas Spittler Marco Idzko Julia Eckl-Dorna Sven Schneider Source Type: research
From mice to men: An assessment of preclinical model systems for the study of vitiligo
Clin Immunol. 2024 Mar 8:110171. doi: 10.1016/j.clim.2024.110171. Online ahead of print.ABSTRACTVitiligo is an autoimmune skin disease of multiple etiology, for which there is no complete cure. This chronic depigmentation is characterized by epidermal melanocyte loss, and causes disfigurement and significant psychosocial distress. Mouse models have been extensively employed to further our understanding of complex disease mechanisms in vitiligo, as well as to provide a preclinical platform for clinical interventional research on potential treatment strategies in humans. The current mouse models can be categorized into three...
Source: Clinical Immunology - March 10, 2024 Category: Allergy & Immunology Authors: Wei Wu Xinju Wang Kaiqiao He Chunying Li Shuli Li Source Type: research
The role of podocytes in lupus nephritis: Insights and implications
Clin Immunol. 2024 Mar 8:110180. doi: 10.1016/j.clim.2024.110180. Online ahead of print.ABSTRACTLupus nephritis (LN) is a severe complication of systemic lupus erythematosus, with high mortality rates despite medical advancements. The complexity of its pathogenesis, including the pivotal role of podocytes - kidney-localized cells - remains a challenge, lacking effective treatments and biomarkers. Recent studies highlight the significant contribution of these cells to LN's development, particularly through their immune-related functions and interaction with other kidney cells. This new understanding opens possibilities for ...
Source: Clinical Immunology - March 10, 2024 Category: Allergy & Immunology Authors: Kunihiro Ichinose Source Type: research
Intestinal permeability correlates with disease activity and DNA methylation changes in lupus patients
CONCLUSION: Our data suggest a correlation between increased intestinal permeability and disease activity in lupus patients. Further, increased intestinal permeability might be associated with epigenetic changes that could play a role in the pathogenesis of lupus.PMID:38460891 | DOI:10.1016/j.clim.2024.110173 (Source: Clinical Immunology)
Source: Clinical Immunology - March 9, 2024 Category: Allergy & Immunology Authors: Mckenna M Bowes Desir é Casares-Marfil Amr H Sawalha Source Type: research
The critical role of RAGE in severe influenza infection: A target for control of inflammatory response in the disease
Clin Immunol. 2024 Mar 7:110178. doi: 10.1016/j.clim.2024.110178. Online ahead of print.ABSTRACTControlling the excessive inflammatory response is one of the key ways to reduce the severity and mortality of severe influenza virus infections. RAGE is involved in inflammatory responses and acute lung injuries. Here, we investigated the role of RAGE and its potential application as a target for severe influenza treatment through serological correlation analysis for influenza patients, and treatment with the RAGE inhibitor FPS-ZM1 on A549 cells or mice with influenza A (H1N1) infection. The results showed high levels of RAGE w...
Source: Clinical Immunology - March 9, 2024 Category: Allergy & Immunology Authors: Wenhui Guo Junhao Luo Song Zhao Li Li Wenge Xing Rongbao Gao Source Type: research
The landscape of immune dysregulation in pediatric sepsis at a single-cell resolution
Clin Immunol. 2024 Mar 7:110175. doi: 10.1016/j.clim.2024.110175. Online ahead of print.ABSTRACTRecognizing immune dysregulation as a hallmark of sepsis pathophysiology, leukocytes have attracted major attention of investigation. While adult and pediatric sepsis are clinically distinct, their immunological delineation remains limited. Single cell technologies facilitated the characterization of immune signatures. We tackled to delineate immunological profiles of pediatric sepsis at a single-cell level by analyzing blood samples from six septic children, at both acute and recovery phases, and four healthy children. 16 singl...
Source: Clinical Immunology - March 9, 2024 Category: Allergy & Immunology Authors: Fahd Alhamdan Sophia Koutsogiannaki Koichi Yuki Source Type: research
Understanding the pathogenic significance of altered calcium-calmodulin signaling in T cells in autoimmune diseases
Clin Immunol. 2024 Mar 7:110177. doi: 10.1016/j.clim.2024.110177. Online ahead of print.ABSTRACTCalcium/calmodulin-dependent protein kinase IV (CaMK4) serves as a pivotal mediator in the regulation of gene expression, influencing the activity of transcription factors within a variety of immune cells, including T cells. Altered CaMK4 signaling is implicated in autoimmune diseases such as systemic lupus erythematosus, rheumatoid arthritis, and psoriasis, which are characterized by dysregulated immune responses and clinical complexity. These conditions share common disturbances in immune cell functionality, cytokine productio...
Source: Clinical Immunology - March 9, 2024 Category: Allergy & Immunology Authors: Tomohiro Koga Source Type: research
Tumor-necrosis factor α-rich environment alters type-I interferon response to viral stimuli in patients with juvenile idiopathic arthritis by altering myeloid dendritic cell phenotype
Clin Immunol. 2024 Mar 7:110170. doi: 10.1016/j.clim.2024.110170. Online ahead of print.ABSTRACTThe balance between the tumor-necrosis factor α (TNFα) and type-I interferon (T1IFN) pathways is crucial for proper immune function. Dysregulation of either pathway can contribute to autoimmune diseases development. Even though TNFα blockade has shown promising results in various autoimmune diseases, the effect on the balance between TNFα and T1IFN is elusive. We used targeted anti-TNFα therapies in juvenile idiopathic arthritis (JIA) as an experimental approach to study the cross-regulation between TNFα and type-I IFN. We...
Source: Clinical Immunology - March 9, 2024 Category: Allergy & Immunology Authors: Irena Zentsova Adam Klocperk Marketa Bloomfield Helena Kubesova Hana Malcova Dita Cebecauerova Rudolf Horvath Anna Sediva Zuzana Parackova Source Type: research
The role of cAMP dependent gene transcription in lupus pathophysiology
Clin Immunol. 2024 Mar 7:110179. doi: 10.1016/j.clim.2024.110179. Online ahead of print.ABSTRACTT lymphocytes play a major role in the pathophysiology of systemic lupus erythematosus. T cellular dysregulation includes significant alterations in signal transduction, cytokine production and metabolic pathways. The cAMP dependent transcription factors like CREB and CREM exert pleiotropic functions as they are critically involved in epigenetic conformational changes and gene regulation of different key effector cytokines in CD4+ T cells including that of IL2, IL17 and IL21 genes. In the present review we review current knowled...
Source: Clinical Immunology - March 9, 2024 Category: Allergy & Immunology Authors: Rauen Thomas Tenbrock Klaus Source Type: research
Intestinal permeability correlates with disease activity and DNA methylation changes in lupus patients
CONCLUSION: Our data suggest a correlation between increased intestinal permeability and disease activity in lupus patients. Further, increased intestinal permeability might be associated with epigenetic changes that could play a role in the pathogenesis of lupus.PMID:38460891 | DOI:10.1016/j.clim.2024.110173 (Source: Clinical Immunology)
Source: Clinical Immunology - March 9, 2024 Category: Allergy & Immunology Authors: Mckenna M Bowes Desir é Casares-Marfil Amr H Sawalha Source Type: research
The critical role of RAGE in severe influenza infection: A target for control of inflammatory response in the disease
Clin Immunol. 2024 Mar 7:110178. doi: 10.1016/j.clim.2024.110178. Online ahead of print.ABSTRACTControlling the excessive inflammatory response is one of the key ways to reduce the severity and mortality of severe influenza virus infections. RAGE is involved in inflammatory responses and acute lung injuries. Here, we investigated the role of RAGE and its potential application as a target for severe influenza treatment through serological correlation analysis for influenza patients, and treatment with the RAGE inhibitor FPS-ZM1 on A549 cells or mice with influenza A (H1N1) infection. The results showed high levels of RAGE w...
Source: Clinical Immunology - March 9, 2024 Category: Allergy & Immunology Authors: Wenhui Guo Junhao Luo Song Zhao Li Li Wenge Xing Rongbao Gao Source Type: research
The landscape of immune dysregulation in pediatric sepsis at a single-cell resolution
Clin Immunol. 2024 Mar 7:110175. doi: 10.1016/j.clim.2024.110175. Online ahead of print.ABSTRACTRecognizing immune dysregulation as a hallmark of sepsis pathophysiology, leukocytes have attracted major attention of investigation. While adult and pediatric sepsis are clinically distinct, their immunological delineation remains limited. Single cell technologies facilitated the characterization of immune signatures. We tackled to delineate immunological profiles of pediatric sepsis at a single-cell level by analyzing blood samples from six septic children, at both acute and recovery phases, and four healthy children. 16 singl...
Source: Clinical Immunology - March 9, 2024 Category: Allergy & Immunology Authors: Fahd Alhamdan Sophia Koutsogiannaki Koichi Yuki Source Type: research
Understanding the pathogenic significance of altered calcium-calmodulin signaling in T cells in autoimmune diseases
Clin Immunol. 2024 Mar 7:110177. doi: 10.1016/j.clim.2024.110177. Online ahead of print.ABSTRACTCalcium/calmodulin-dependent protein kinase IV (CaMK4) serves as a pivotal mediator in the regulation of gene expression, influencing the activity of transcription factors within a variety of immune cells, including T cells. Altered CaMK4 signaling is implicated in autoimmune diseases such as systemic lupus erythematosus, rheumatoid arthritis, and psoriasis, which are characterized by dysregulated immune responses and clinical complexity. These conditions share common disturbances in immune cell functionality, cytokine productio...
Source: Clinical Immunology - March 9, 2024 Category: Allergy & Immunology Authors: Tomohiro Koga Source Type: research
Tumor-necrosis factor α-rich environment alters type-I interferon response to viral stimuli in patients with juvenile idiopathic arthritis by altering myeloid dendritic cell phenotype
Clin Immunol. 2024 Mar 7:110170. doi: 10.1016/j.clim.2024.110170. Online ahead of print.ABSTRACTThe balance between the tumor-necrosis factor α (TNFα) and type-I interferon (T1IFN) pathways is crucial for proper immune function. Dysregulation of either pathway can contribute to autoimmune diseases development. Even though TNFα blockade has shown promising results in various autoimmune diseases, the effect on the balance between TNFα and T1IFN is elusive. We used targeted anti-TNFα therapies in juvenile idiopathic arthritis (JIA) as an experimental approach to study the cross-regulation between TNFα and type-I IFN. We...
Source: Clinical Immunology - March 9, 2024 Category: Allergy & Immunology Authors: Irena Zentsova Adam Klocperk Marketa Bloomfield Helena Kubesova Hana Malcova Dita Cebecauerova Rudolf Horvath Anna Sediva Zuzana Parackova Source Type: research
