IgG4 Subclass of Immunoglobulins; Immunobiology and Roles in Relation to Human Diseases DOI Creative Commons
Эва Мала, Moeina Afshari, Jan Krejsek

et al.

Acta Medica (Hradec Kralove Czech Republic), Journal Year: 2024, Volume and Issue: 67(4), P. 101 - 106

Published: Jan. 1, 2024

IgG4, a subclass of antibodies known as immunoglobulins have unique structural features, in particular, their Fc regions, that prevents interactions with other receptors on effector cells and thus disabling them activating complements system. IgG4 can undergo process called Fab-arm exchange, wherein they exchange half-molecules antibodies, forming bispecific monovalent antibodies. Isotypic switch mature B germinal centres secondary lymphoid organs is controlled by Tfh subset T cells. Functionally exert immunomodulatory blocking activities, modulating protective inflammation evolved parasitic invasion allergic inflammation. From the pathophysiological point view, autoantibodies are prominently observed autoimmune diseases under umbrella IgG4-autoimmune (IgG4-AID). Furthermore, IgG4-related (IgG4-RD) affecting various characterized lymphoplasmacytic infiltrates storiform fibrosis tissues, together elevated levels blood. A better understanding immunobiology helps us diagnose treat patients suffering from these rare forms diseases.

Language: Английский

Inebilizumab for Treatment of IgG4-Related Disease DOI
James R. Stone, Arezou Khosroshahi, Zhang Wen

et al.

New England Journal of Medicine, Journal Year: 2024, Volume and Issue: unknown

Published: Nov. 14, 2024

IgG4-related disease is a multiorgan, relapsing, fibroinflammatory, immune-mediated disorder with no approved therapy. Inebilizumab targets and depletes CD19+ B cells may be effective for treating patients disease. In this phase 3, multicenter, double-blind, randomized, placebo-controlled trial, adults active underwent randomization in 1:1 ratio to receive inebilizumab (300-mg intravenous infusions on days 1 15 week 26) or placebo 52-week treatment period. Participants both groups received identical glucocorticoid tapers. Glucocorticoids were allowed treat flares, but background immunosuppressants not permitted. The primary end point was the first treated, adjudicated flare during period, assessed time-to-event analysis. Key secondary points annualized rate treatment-free glucocorticoid-free complete remission. A total of 135 participants randomization: 68 assigned 67 placebo. Treatment reduced risk; 7 (10%) group had at least one flare, as compared 40 (60%) (hazard ratio, 0.13; 95% confidence interval [CI], 0.06 0.28; P<0.001). lower than (rate 0.14; CI, 0.31; More flare-free, remission (odds 4.68; 2.21 9.91; P<0.001) 4.96; 2.34 10.52; Serious adverse events occurred period 12 (18%) who 6 (9%) risk flares increased likelihood flare-free year, confirming role CD19-targeted B-cell depletion potential (Funded by Amgen; MITIGATE ClinicalTrials.gov number, NCT04540497.).

Language: Английский

Citations

17
The Platelet-Specific Gene Signature in the Immunoglobulin G4-Related Disease Transcriptome DOI Creative Commons
Ali Kemal Oğuz,

Çağdaş Şahap Oygür,

Bala Gür Dedeoğlu

et al.

Medicina, Journal Year: 2025, Volume and Issue: 61(1), P. 162 - 162

Published: Jan. 19, 2025

Background and Objectives: Immunoglobulin G4-related disease (IgG4-RD) is an immune-mediated, fibroinflammatory, multiorgan with obscure pathogenesis. Findings indicating excessive platelet activation have been reported in systemic sclerosis, which another autoimmune, multisystemic fibrotic disorder. The inflammatory, fibrosing intersections of IgG4-RD sclerosis raised a question about platelets’ role IgG4-RD. Materials Methods: By borrowing transcriptomic data from Nakajima et al. (GEO repository, GSE66465) we sought contribution to the pathogenesis GEO2R BRB-ArrayTools were used for class comparisons, WebGestalt functional enrichment analysis. During selection differentially expressed genes (DEGs), translationally active but significantly low amount mRNA was specifically considered. platelet-specific gene signature derived cluster analysis patient control groups. Results: When patients compared controls, 268 DEGs (204 increased 64 decreased expression) detected. Among these, molecular 22 harbored important leukocyte–platelet aggregate formation (i.e., CLEC1B, GP1BA, ITGA2B, ITGB3, SELP, TREML1) extracellular matrix synthesis CLU, PF4, PPBP, SPARC, THBS1). Functional documented enriched terms related platelets, including not limited “platelet reactivity”, degranulation”, aggregation”, activation”. clustering, signatures successfully discriminated before after treatment Conclusions: Patients apparently display activated phenotype potential immunopathogenesis. If validated through further carefully designed research, therapeutic potentials selected conventional and/or novel antiplatelet agents remain be evaluated Transcriptomics proteomics research platelets should take into account relatively amounts mRNA, miRNA, protein. Secondary omics sets has great reveal new valuable information.

Language: Английский

Citations

0
A 69-year-old man with generalised lymphadenopathy, glandular swelling and pleural effusion DOI Open Access
Shan Kai Ing, Young Lee,

Nga Hung Ngu

et al.

Breathe, Journal Year: 2025, Volume and Issue: 21(1), P. 240125 - 240125

Published: Jan. 1, 2025

Patients with multisystemic presentation including respiratory symptoms and generalised lymphadenopathy should alert the clinician to this potential diagnosis https://bit.ly/4eJ0PHT.

Language: Английский

Citations

0
Beyond Glucocorticoids for IgG4-Related Disease DOI
Robert Spiera

New England Journal of Medicine, Journal Year: 2025, Volume and Issue: 392(12), P. 1232 - 1233

Published: March 26, 2025

IgG4-related disease is an increasingly recognized fibroinflammatory condition with protean manifestations. The disorder characterized by inflammatory mass lesions that can involve many different organs and organ systems — including the pancreas biliary tract, orbital structures, salivary lacrimal glands, lungs, retroperitoneum, kidneys also cause aortitis periaortitis.1 effects of relate to resultant damage in affected systems. It often be a relapsing disorder, optimal approach inducing or maintaining remission has not been well defined. etiologic factors are uncertain, but histopathological characteristics .

Language: Английский

Citations

0
Zielorgan Gefäße – vom Morbus Ormond zu Immunglobulin-G4-assoziierten Erkrankungen DOI
Marco Krasselt, Bimba F. Hoyer

Deleted Journal, Journal Year: 2025, Volume and Issue: unknown

Published: April 1, 2025

Immunoglobulin G4-related disease (IgG4-RD) is a rare and clinically diverse entity with an estimated incidence of 0.8-1.4 per 100,000 person-years. Typical sites manifestation include the head neck area, hepato-pancreato-biliary system, retroperitoneum. Perhaps best-known variant retroperitoneal fibrosis, formerly known as Ormond's disease. Biopsy histological examination are great diagnostic importance, relying solely on serum IgG4 problematic not sufficiently specific. Therapeutically, in addition to initial administration glucocorticoids, treatment B cell-directed antibodies, particularly rituximab, has become established. Initial studies demonstrate effectiveness other therapies targeting cells. Although received more clinical attention recent years, it still necessary further increase awareness IgG4-RD enable optimal for patients.

Language: Английский

Citations

0
Enfermedad relacionada con IgG4 DOI

Grisell Starita Fajardo,

David Lucena López,

Coral Arévalo-Cañas

et al.

Medicine - Programa de Formación Médica Continuada Acreditado, Journal Year: 2025, Volume and Issue: 14(31), P. 1861 - 1870

Published: April 1, 2025

Citations

0
Comparative Analysis of Classification Criteria in IgG4-Related Disease and Evaluating Diagnostic Accuracy from Retrospective Cohort in Clinical Practice DOI Creative Commons

Marta López-Gómez,

Patricia Moya, Hye Sang Park

et al.

Diagnostics, Journal Year: 2024, Volume and Issue: 14(22), P. 2583 - 2583

Published: Nov. 17, 2024

Introduction: We conducted a comprehensive comparative analysis of the Okazaki, Umehara, and American College Rheumatology/European League Against Rheumatism (ACR/EULAR) classification criteria for diagnosing immunoglobulin G4-related disease (IgG4-RD). Materials Methods: A retrospective study was in single tertiary hospital, using expert clinical judgment as gold standard. compared diagnostic accuracy ACR/EULAR cohort 41 patients with suspected IgG4-RD. assessed sensitivity, specificity, positive negative predictive values each criterion, separate based on four IgG4-RD subtypes. Results: total 30 were confirmed to have 11 identified mimickers. The Umehara demonstrated highest sensitivity (83.33%), followed by 2019 (66.67%) Okazaki (60.0%) criteria. All three exhibited 100% overall ranging from 70% 88%. areas under curve (AUC) 0.917 (Umehara), 0.800 (Okazaki), 0.833 (ACR/EULAR 2019), indicating significant effectiveness (p < 0.000). Subtype revealed that more effective pancreato-hepato-biliary involvement (subtype 1), while retroperitoneal fibrosis and/or aortitis 2). Conclusions: Our provides valuable insights into performance criterion suggesting its potential utility screening purposes, all showed consistent specificity.

Language: Английский

Citations

1
When Dumbo meets IKAROS: Fear and hubris DOI
V. Koneti Rao

Journal of Allergy and Clinical Immunology, Journal Year: 2024, Volume and Issue: 154(3), P. 552 - 553

Published: June 27, 2024

Language: Английский

Citations

0
IgG4-Related Disease: Leading International Co-Authorship Networks and Future Research Directions DOI Creative Commons
Naruaki Ogasawara

Research Square (Research Square), Journal Year: 2024, Volume and Issue: unknown

Published: Sept. 2, 2024

Abstract Aim This study aims to analyze the structure of co-author networks in IgG4-related disease (IgG4-RD) research from 2000 2023, using data Web Science (WoS) Core Collection. The goal is identify collaborative relationships, key researchers, and trends within community over time. Method I conducted a comprehensive network analysis 5,310 articles on IgG4-RD published between as indexed WoS was performed Python (Version 3.10.5) PyCharm integrated development environment (IDE) (Software Version 2022.1.3). Macro-level indicators, including density, clustering coefficient, number components, average path length, were used assess overall structure. Micro-level such degree centrality, closeness betweenness employed evaluate influence connectivity individual researchers network. Result co-authorship revealed fragmented with isolated clusters throughout studied periods: 2000–2009, 2010–2019, 2020–2023. Network density remained low, reflecting limited direct collaborations among while high coefficients indicated formation tight-knit groups. components decreased slightly time, suggesting gradual improvement connectivity. Key John H. Stone, Mitsuhiro Kawano, Kazuichi Okazaki, consistently exhibited centrality metrics, highlighting their pivotal roles bridging fostering collaboration IgG4-RD. Conclusion 2023 reveals field characterized by strong localized but low cohesion. While have played significant connecting various clusters, network's nature suggests opportunities for enhancing broader efforts. Improving international interdisciplinary connections could foster more accelerate advancements understanding treatment

Language: Английский

Citations

0
IgG4-related disease presented with a neck nodule; a case report DOI

Fatima Alghawe,

Maysoun Kudsi,

Lilianne Haj Hassan

et al.

International Journal of Surgery Global Health, Journal Year: 2024, Volume and Issue: 7(6)

Published: Nov. 1, 2024

Introduction and importance: Immunoglobulin G4 (IgG4)-related disease is an infrequent systemic condition that affects males more than females. Fatigue, arthralgia, salivary lacrimal gland enlargement, symptoms of pancreatitis, tubulointerstitial nephritis, retroperitoneal fibrosis are the frequent manifestations. The spectrum IgG4-related thyroid includes Hashimoto thyroiditis, Graves’ disease, Riedel thyroiditis. Case presentation: We presented a 49‐year‐old male with progressive painless swallowing neck for year without any accompanying symptoms. Conclusion: Expanding goiter common, but its severity leads to rare diagnosis

Language: Английский

Citations

0