Basic Research and Clinical Reports Associated with Low Serum IgG4 Concentrations

Tian, Xinyu; Deng, Zhenling; Wang, Song; Wang, Yue

doi:10.1159/000503967

Cited by 8 publications

(7 citation statements)

References 71 publications

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“…In our paper, we found that correct immunization was achieved by children with higher levels of IgG4. Our findings could explain Tian et al findings that link recurrent respiratory infections -particularly pulmonaryand bronchiectasis with IgG4 serum deficiency 27 .…”

Section: Discussionsupporting

confidence: 77%

Study of pneumococcal vaccine response in children with underlying medical conditions

Jms

et al. 2021

Preprint

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Background: Pneumococcal infection causes serious illness in children, especially those with underlying conditions. Although pneumococcal vaccination in the pediatric population is recommended, the immune response to pneumococcal vaccines in this group is usually deficient. The aim of this study was to study the response to pneumococcal vaccines in children with underlying conditions. Methods: A one-year retrospective study on children with underlying conditions attended at the Pediatric Immunology Unit of La Fe University Hospital (Valencia, Spain). Patient demographics, immunosuppressive diseases, pneumococcal vaccination coverage, serum levels of C3, C4, IgA, IgM, IgG, IgG1, IgG2, IgG3, IgG4, blood levels of leukocytes, neutrophils and CD4 and CD8 T lymphocyte were analyzed. Results: A total of 85 children were included in the study. Only 29.41% of them had the complete vaccination schedule, most under 5 years of age (76.00%). Protective levels of pneumococcal antibodies were found only in 48% of the correctly vaccinated patients, whose serum levels of IgG4 and CD4/CD8 ratio were higher than in children with deficient response. Low vaccine response was mostly found among leukemic patients, who exhibited the lowest level of CD4 T lymphocyte. Patients’ age, immunosuppressive therapy, and serum levels of C3, C4, IgG3 and lymphocytes were associated with pneumococcal vaccine response. Conclusion: Most of the patients lacked some dose of pneumococcal vaccine and had an inadequate response. Mathematical models can be useful in clinical practice to detect non responders. Emphasis should be placed on completing a vaccination schedule and, if necessary, revaccination with a booster dose of pneumococcal vaccines.

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Section: Discussionsupporting

confidence: 77%

Study of pneumococcal vaccine response in children with underlying medical conditions

Jms

et al. 2021

Preprint

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“…This raises the possibility that the impact of IgG4 deficiency on mortality may be indirect. Consistent with this notion, recent studies have shown that low serum IgG4 increases the risk of not only respiratory infections but also non-respiratory conditions including allergic, autoimmune, and cardiovascular diseases [ 20 – 26 ]. For these latter conditions, serum IgG levels appear to be inversely related to the severity of disease [ 23 , 25 , 26 ].…”

Section: Discussionmentioning

confidence: 82%

The impact of IgG subclass deficiency on the risk of mortality in hospitalized patients with COPD

et al. 2022

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Background Immunoglobulin G (IgG) deficiency increases the risk of acute exacerbations and mortality in chronic obstructive pulmonary disease (COPD). However, the impact of IgG subclass deficiency on mortality in COPD is unknown. Here, we determined which IgG subclass, if any, is associated with increased risk of mortality in COPD. Methods We measured serum IgG subclass concentrations of 489 hospitalized patients with COPD who were enrolled in the Rapid Transition Program (clinicaltrials.gov identifier NCT02050022). To evaluate the impact of IgG subclass deficiency on 1-year mortality, Cox proportional hazards regression analyses were performed with adjustments for potential confounders. Results Deficiencies in IgG1, IgG2, IgG3, and IgG4 were present in 1.8%, 12.1%, 4.3%, and 11.2% of patients, respectively. One-year mortality was 56% in patients with IgG1 deficiency, 27% in IgG2 deficiency, 24% in IgG3 deficiency, and 31% in IgG4 deficiency. Cox proportional modeling showed that IgG1 and IgG4 deficiencies increased the 1-year mortality risk with an adjusted hazard ratio of 3.92 (95% confidence interval [CI] = 1.55–9.87) and 1.74 (95% CI = 1.02–2.98), respectively. Neither IgG2 nor IgG3 deficiency significantly increased 1-year mortality. Two or more IgG subclass deficiencies were observed in 5.3%. Patients with 2 or more IgG subclass deficiencies had a higher 1-year mortality than those without any deficiencies (46.2% vs. 19.7%, p < 0.001), with an adjusted hazard ratio of 2.22 (95% CI = 1.18–4.17). Conclusions IgG1 and IgG4 deficiency was observed in 1.8% and 11.2% of hospitalized patients with COPD, respectively, and these deficiencies were associated with a significantly increased risk of 1-year mortality.

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“…Although patients with anti-LGI-1 and CASPR2 antibodies are characterized by clinical heterogeneity, they also demonstrate considerable overlap in clinical symptomatology; anti-LGI1 antibodies are most commonly associated with epilepsy and limbic encephalitis, while anti-CASPR2 antibodies are associated with neuromyotonia, Morvan syndrome, and neuropathic pain. Anti-IgLON5 antibodies define a complex syndrome of chronic progressive brainstem symptomatology, gait instability, distinct non-rapid eye movement (REM) and REM parasomnias, sleep-disordered breathing, obstructive sleep apnea, cognitive decline, and movement disorders as recently identified, most commonly craniofacial dyskinesias, chorea, dystonia, and abnormal eye movements [ 3 , 71 , 72 , 73 , 74 , 75 ].…”

Section: Molecular Commonalities Between Musk-mg and Other Autoimmune...mentioning

confidence: 99%

MuSK Myasthenia Gravis—Potential Pathomechanisms and Treatment Directed against Specific Targets

Dziadkowiak,

Baczyńska,

Waliszewska-Prosół

2024

Cells

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Myasthenia gravis (MG) is an autoimmune disease in which autoantibodies target structures within the neuromuscular junction, affecting neuromuscular transmission. Muscle-specific tyrosine kinase receptor-associated MG (MuSK-MG) is a rare, often more severe, subtype of the disease with different pathogenesis and specific clinical features. It is characterized by a more severe clinical course, more frequent complications, and often inadequate response to treatment. Here, we review the current state of knowledge about potential pathomechanisms of the MuSK-MG and their therapeutic implications as well as ongoing research in this field, with reference to key points of immune-mediated processes involved in the background of myasthenia gravis.

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Basic Research and Clinical Reports Associated with Low Serum IgG4 Concentrations

Cited by 8 publications

References 71 publications

Study of pneumococcal vaccine response in children with underlying medical conditions

Study of pneumococcal vaccine response in children with underlying medical conditions

The impact of IgG subclass deficiency on the risk of mortality in hospitalized patients with COPD

MuSK Myasthenia Gravis—Potential Pathomechanisms and Treatment Directed against Specific Targets

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