scholarly journals Does intravenous immunoglobulin therapy prolong immunodeficiency in transient hypogammaglobulinemia of infancy?

2013 ◽  
Vol 5 (3) ◽  
pp. 14 ◽  
Author(s):  
Lale Memmedova ◽  
Elif Azarsiz ◽  
Neslihan Edeer Karaca ◽  
Guzide Aksu ◽  
Necil Kutukculer
Keyword(s):  
Immune System ◽  
Intravenous Immunoglobulin ◽  
Ivig Therapy ◽  
Immunoglobulin Therapy ◽  
Serum Immunoglobulin ◽  
Ivig Treatment ◽  
Intravenous Immunoglobulin Therapy ◽  
Humoral Immune ◽  
Significant Difference ◽  
Ivig Replacement

Transient hypogammaglobulinemia of infancy (THI) is characterized by recurrent infections and one or more reduced serum immunoglobulin levels. Typically, THI patients recover spontaneously, mostly within 30-40 months of age, but sometimes recovery may be delayed until 5-6 years of age. The use of intravenous immunoglobulin (IVIg) as an alternative to antibiotic prophylaxis remains contraversial also in symptomatic THI patients. In fact, some authors believe that IVIg therapy may cause a delay in the maturation of the humoral immune system because of the interference from passively transfered antibodies. The aim of this study was to investigate the effect of IVIg replacement on recovery from immunodeficiency in THI patients and determine new parameters in order to include these patients in IVIg therapy groups. In this retrospective study, 43 patients (65%) received IVIg replacement therapy while 23 patients (34.8%) showed spontaneous normalization without IVIg. The percentages of patients who had more than six times the number of febrile infections in a year decreased from 91% to 21% in the group receiving IVIg treatment. At admission, before being recruited to IVIg therapy, serum immunoglobulin G (IgG) levels and anti-hemophilus B (Hib) antibody titers were found to be significantly low in cases who were selected for IVIg replacement. The percentages of patients who did not have protective levels of anti-Hib, anti-rubella or anti-rubeola-IgG were also significantly high in IVIg cases. There was no statistically significant difference in the age at which IgG levels normalized between the IVIg and the non-IVIg group. Patients in the IVIg group and non-IVIg group reached normal IgG levels at the age of 42.9±22.0 and 40.7±19.8 months, respectively. In conclusion, IVIg infusions do not cause a delay in the maturation of the immune system in THI patients. Besides the well-established criteria, very low and non-protective specific antibody responses against previously applied vaccines are important factors to consider when selecting patients for IVIg therapy.

scholarly journals Defects of CTLA-4 Are Associated with Regulatory T Cells in Myasthenia Gravis Implicated by Intravenous Immunoglobulin Therapy

2020 ◽  
Vol 2020 ◽  
pp. 1-13
Author(s):  
Wenhua Xu ◽  
Mingshan Ren ◽  
Swagata Ghosh ◽  
Kai Qian ◽  
Zhaofeng Luo ◽  
...  
Keyword(s):  
T Cells ◽  
Myasthenia Gravis ◽  
Intravenous Immunoglobulin ◽  
Ivig Therapy ◽  
Immunoglobulin Therapy ◽  
Treg Cells ◽  
Ivig Treatment ◽  
Intravenous Immunoglobulin Therapy ◽  
Circulating Autoantibodies ◽  
Antigen Presenting

Myasthenia gravis (MG) is a CD4+ T cell-dependent autoimmune disease resulting from aberrant immune response mediated by circulating autoantibodies at the neuromuscular junction. Intravenous immunoglobulin (IVIg) is an expensive and commonly used immunotherapeutic approach to treat patients with MG. The mechanisms of actions involved in IVIg treatment, however, remain to be investigated. In an effort to examine the roles of various subsets of CD4+ T cells in the periphery blood of MG and uncover the mechanisms that contribute to the therapeutical effects of IVIg, we first demonstrated that a subset of CD4+ T cells, CTLA-4-expressing regulatory T (Treg) cells, were underrepresented and functionally defective in MG patients. The dynamic profiling during the IVIg therapy course further revealed an inverse relationship between the frequency of CTLA-4+ Treg and the quantitative MG (QMG) score that represents disease severity. Our mechanistic studies indicated that IVIg expands CTLA-4-Treg cells via modulating antigen-presenting dendritic cells (DCs). To determine the molecular defects of CTLA-4 in abnormities of Treg in MG patients, we demonstrated hypermethylation at -658 and -793 CpGs of CTLA-4 promoter in MG Tregs. Interestingly, IVIg therapy significantly reduced the methylation level at these two sites in MG patients. Overall, our study may suggest a role of CTLA-4 in functionally defected Treg cells in MG and its actions involved in IVIg therapy.

ACUTE CORONARY SYNDROME FOLLOWING INTRAVENOUS IMMUNOGLOBULIN THERAPY : A HIDDEN RISK

2021 ◽  
pp. 78-79
Author(s):  
Karthikkeyan Rajachandran ◽  
Giphy Susan Varghese
Keyword(s):  
Myocardial Infarction ◽  
Cardiovascular Disease ◽  
Intravenous Immunoglobulin ◽  
Ivig Therapy ◽  
Immunoglobulin Therapy ◽  
Left Ventricular ◽  
Intravenous Immunoglobulin Therapy ◽  
Severe Left Ventricular Dysfunction ◽  
St Segment Elevation ◽  
Coronary Syndrome

Intravenous immunoglobulin (IVIG) is one of the main line modalities of therapy for chronic inammatory demyelinating polyneuropathy (CIDP). We hereby, report an incidence of acute myocardial infarction probably induced by IVIG during the treatment of CIDP. A 76 year old female with no history suggestive of cardiovascular disease, developed an acute Non ST Segment Elevation Myocardial Infarction (NSTEMI) and severe left ventricular dysfunction after receiving three doses of IVIG. Since hypercoagulability is a concern with IVIG therapy, it was discontinued. Hence, we highlight the importance of cardiac evaluation before initiation and during the course of IVIG therapy in elderly patients as well as in patients with known risk factors for cardiovascular disease and thrombotic events.

scholarly journals High-dose intravenous immunoglobulin therapy induces and maintains complete remission for polyarteritis nodosa

2014 ◽  
Vol 1 (1) ◽  
pp. 13
Author(s):  
Kazu Ode ◽  
Yoshinori Taniguchi ◽  
Yoshitaka Kumon ◽  
Yoshio Terada
Keyword(s):  
Complete Remission ◽  
Intravenous Immunoglobulin ◽  
Polyarteritis Nodosa ◽  
Alternative Therapy ◽  
Ivig Therapy ◽  
Immunoglobulin Therapy ◽  
High Dose ◽  
Intravenous Immunoglobulin Therapy ◽  
First Line Therapy ◽  
High Dose Ivig

We report a case of successful high-dose intravenous immunoglobulin (IVIG) use in a patient with refractory polyarteritis nodosa (PAN). Treatments with prednisolone (PSL) and various types of immunosuppressants including methotrexate (MTX) and intravenous cyclophosphamide (IVCY) were unsuccessful, and then, high-dose IVIG therapy was added. High-dose IVIG therapy improved all symptoms including high fever, arthralgia, mononeuritis multiplex and indurated erythema due to PAN. Moreover, serum inflammatory markers were also normalized. High-dose IVIG is maintaining complete remission for PAN without flare-up for additional 4 years. Therefore, high-dose IVIG therapy might be considered as a first-line therapy in patients with PAN or alternative therapy in refractory PAN.

scholarly journals Effect of Early Intravenous Immunoglobulin Therapy in Kawasaki Disease: A Systematic Review and Meta-Analysis

2020 ◽  
Vol 8 ◽  
Author(s):  
Fan Yan ◽  
Huayong Zhang ◽  
Ruihua Xiong ◽  
Xingfeng Cheng ◽  
Yang Chen ◽  
...  
Keyword(s):  
Systematic Review ◽  
Kawasaki Disease ◽  
Intravenous Immunoglobulin ◽  
Meta Analysis ◽  
The United States ◽  
Cochrane Library ◽  
Ivig Treatment ◽  
Intravenous Immunoglobulin Therapy ◽  
Increased Risk ◽  
Significant Difference

Background: In the latest 2017 American Heart Association guidelines for Kawasaki disease (KD), there are no recommendations regarding the early administration of intravenous immunoglobulin (IVIG). Therefore, the purpose of this systematic review and meta-analysis was to investigate the effects of early IVIG therapy on KD.Methods: We searched databases including the PubMed, Medline, the Cochrane Library, and the Clinicaltrials.gov website until July 2019.Results: Fourteen studies involving a total of 70,396 patients were included. Early treatment with IVIG can lead to an increased risk of IVIG unresponsiveness [OR 2.24; 95% CI (1.76, 2.84); P = 0.000]. In contrast to the studies performed in Japan [OR 1.27; 95% CI (0.98, 1.64); P = 0.074] that found no significant difference in coronary artery lesions (CAL) development, studies conducted in China [OR 0.73; 95% CI (0.66, 0.80); P = 0.000] and the United States [OR 0.50; 95% CI (0.38, 0.66); P = 0.000] showed a reduced risk in the occurrence of CAL with early IVIG treatment.Conclusions: At present, the evidence does not support the treatment with IVIG in the early stage of the onset of KD. But, early IVIG treatment could be a protective factor against the development of CAL, which needs to be further clarified.

Abstract 106: Utility of Ferritin as a Predictor of the Patients with Kawasaki Disease Refractory to Intravenous Immunoglobulin Therapy

Circulation ◽  
2015 ◽  
Vol 131 (suppl_2) ◽  
Author(s):  
Noboru Yamamoto ◽  
Kaoru Sato ◽  
Takayuki Hoshina ◽  
Masumi Kojiro ◽  
Koichi Kusuhara ◽  
...  
Keyword(s):  
Kawasaki Disease ◽  
Intravenous Immunoglobulin ◽  
Serum Ferritin ◽  
Serum Ferritin Level ◽  
Ferritin Level ◽  
Scoring Systems ◽  
Ivig Therapy ◽  
Ivig Treatment ◽  
Operating Characteristics ◽  
Intravenous Immunoglobulin Therapy

Purpose: Several scoring systems for prediction of non-responsiveness to initial course of intravenous immunoglobulin (IVIG) therapy have been available in the patients diagnosed as Kawasaki disease (KD). However, all non-responders cannot be identified completely by these scoring systems. The aim of this study is to investigate whether ferritin can be a useful marker as a predictor of the patients with KD refractory to IVIG therapy. Materials and Methods: This retrospective study enrolled 63 patients with KD hospitalized at Kitakyushu General Hospital during 2010 to 2013. These patients were divided into IVIG responders (n= 41) and non-responders (n=22). Serum ferritin levels and the scoring systems for prediction of non-responsiveness to initial IVIG treatment were compared between these two groups. Results: Serum ferritin level was significantly elevated in non-responders (p=0.01). The area under the receiver-operating-characteristics curve was 0.698, and the sensitivity and specificity in more than 215 ng/ml of serum ferritin levels was 54.5% and 85.4%, respectively. Two of the three scoring systems for prediction of non-responsiveness to initial IVIG treatment in non-responders were also significantly higher scores than that in responders, but many non-responders had a low score of these scoring systems. Approximately half of the patients with low score of these scoring systems had high serum ferritin level (≧ 215 ng/ml). Conclusion: Serum ferritin level can be a useful marker for the prediction of non-responsiveness to initial IVIG treatment and may be an important complementary marker to the scoring systems for prediction of non-responsiveness to initial IVIG treatment.

scholarly journals Chronic Inflammatory Demyelinating Polyneuropathy: Time to Maximal Recovery in Patients Receiving Intravenous Immunoglobulin Therapy

2020 ◽  
Vol 47 (4) ◽  
pp. 531-537
Author(s):  
Adrian R. Opala ◽  
Kevin Kennedy ◽  
Steven K. Baker
Keyword(s):  
Chronic Inflammatory Demyelinating Polyneuropathy ◽  
Retrospective Chart Review ◽  
Ivig Therapy ◽  
Immunoglobulin Therapy ◽  
Demyelinating Polyneuropathy ◽  
Ivig Treatment ◽  
Maximal Strength ◽  
Intravenous Immunoglobulin Therapy ◽  
Days Of Therapy ◽  
Inflammatory Demyelinating Polyneuropathy

ABSTRACTBackground:The response of chronic inflammatory demyelinating polyneuropathy (CIDP) to intravenous immunoglobulins (IVIgs) treatment is well established. However, it remains unclear whether patients not responding to two IVIg treatments or those whose condition stabilizes (ICE trial) may benefit from additional doses. We aim to identify the time period required to reach maximal strength gains from IVIg treatment.Methods:Retrospective chart review of 14 patients with CIDP was performed. Change in handgrip (HG), Knee extension (KE), elbow flexion, and dorsiflexion was analyzed with a dynamometer during IVIg therapy. Strength improvements in Nm or kg, cumulative grams (g) of IVIg, and time in days required for maximal strength recovery were determined per function (± standard error of the mean). Ancillary therapy was recorded for all patients.Results:Improvements in strength of each function were significant (p < 0.05). Earliest improvement was in HG (137.07 ± 21.23) and latest in KE (238.15 ± 38.9). Majority of patients improved by 200 days of therapy. HG required the lowest cumulative grams of IgG (561.71 ± 97.21) and KE the most (798 ± 120.7).Conclusion:Our study has demonstrated the effectiveness of multiple treatments with IVIg to reach significant improvement in strength. Different muscle groups manifested different time dependency, reflecting the requirement of variable amounts of IVIg. Improvement was identified on an ongoing basis, with therapy lasting between 20.2 and 37.3 weeks, requiring between 562 and 798 g of IVIg.

scholarly journals Intravenous Immunoglobulin Therapy Restores the Quantity and Phenotype of Circulating Dendritic Cells and CD4+ T Cells in Children with Acute Kawasaki Disease

2021 ◽  
Author(s):  
Nana Wang ◽  
Zhongyue Chen ◽  
Fan Zhang ◽  
Qianwen Zhang ◽  
Ling Sun ◽  
...  
Keyword(s):  
T Cells ◽  
Dendritic Cells ◽  
Kawasaki Disease ◽  
Intravenous Immunoglobulin ◽  
Peripheral Blood ◽  
Immunoglobulin Therapy ◽  
Ivig Treatment ◽  
Intravenous Immunoglobulin Therapy ◽  
Domain 3 ◽  
Number Percentage

Abstract Background: Intravenous immunoglobulin (IVIG) is widely used to treat Kawasaki disease (KD). However, the mechanisms by which it reduces systemic inflammation are not completely understood. Dendritic cells (DCs) and T cells play critical roles in the pathogenic processes of immune disorders. Assessing the quantity of DC subsets and T cells and identifying functional molecules present on these cells, which provide information about KD, in the peripheral blood may provide new insights into the mechanisms of immunoglobulin therapy. Methods: In total, 54 patients with KD and 27 age-matched healthy controls (HCs) were included in this study. The number, percentage, and phenotype of DC subsets and CD4+ T cells in peripheral blood were analysed through flow cytometry.Results: Patients with KD exhibited fewer peripheral DC subsets and CD4+ T cells than HCs. Human leucocyte antigen-DR (HLA-DR) expression was reduced on myeloid DCs (CD1c+ mDCs), whereas that on plasmacytoid DCs (pDCs) did not change significantly. Both pDCs and CD1c+ mDCs displayed significantly reduced expression of co-stimulatory molecules, including CD40, CD86. Expression of T-cell immunoglobulin and mucin domain 3 (TIM-3) was increased on CD4+ T cells. No significant differences were observed concerning the quantity and phenotype of DC subsets and CD4+ T cells in patients with KD with and without coronary artery lesions. Importantly, these altered quantity and phenotypes on DC subsets and CD4+ T cells were restored to a great extent after IVIG treatment. T helper (Th) subsets including Th1 and Th2 among CD4+ T cells did not show alteration pre- and post-IVIG treatment, although the Th1-related cytokine IFN-γ level increased dramatically in patients with KD pre-IVIG treatment.Conclusions: pDCs and CD1c+ DCs presented an immature or tolerant phenotype in acute stages of KD, IVIG treatment restored the quantity and functional molecules of DCs and CD4+ T cells to distinct levels, indicating the involvement of DCs and CD4+ T cells in the inflammation in KD. The findings provide insights into the immunomodulatory actions of IVIG.

Chronic Inflammatory Demyelinating Polyradiculoneuropathy - An Overview of Intravenous Immunoglobulin Therapy

2009 ◽  
Vol 4 (1) ◽  
pp. 72
Author(s):  
Vera Bril ◽  
Keyword(s):  
Intravenous Immunoglobulin ◽  
Ivig Therapy ◽  
Immunoglobulin Therapy ◽  
Chronic Inflammatory Demyelinating Polyradiculoneuropathy ◽  
Intravenous Immunoglobulin Therapy ◽  
Short And Long Term ◽  
Term Data ◽  
Ivig Administration

Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is a significant source of disability, and early diagnosis and immunomodulatory therapy administration are critical to minimise disease progression and axonal degeneration. Intravenous immunoglobulin (IVIg) therapy is considered to be a first-line treatment for CIDP. Comparative short- and long-term data of IVIg versus corticosteroids in CIDP patients are limited. Of the five published placebo-controlled studies in CIDP, four reported only on short-term improvements in disability (≤6 weeks). However, the IGIV CIDP Efficacy (ICE) study, the largest randomised, placebo-controlled CIDP study published to date (n=117), reported significant improvements in disability, functional impairment and quality of life with IVIg (Gamunex®) 1g/kg maintenance therapy every three weeks for up to 48 weeks. Furthermore, long-term IVIg administration was safe and well tolerated, particularly given the short duration of the infusions. Data suggest that a long-term scheduled maintenance regimen of IVIg in appropriate patients may provide substantial benefit and reduce the risk of CIDP relapse.

scholarly journals Intravenous Immunoglobulin Therapy Eliminates Candida albicans and Maintains Intestinal Homeostasis in a Murine Model of Dextran Sulfate Sodium-Induced Colitis

2019 ◽  
Vol 20 (6) ◽  
pp. 1473 ◽  
Author(s):  
Rogatien Charlet ◽  
Boualem Sendid ◽  
Srini Kaveri ◽  
Daniel Poulain ◽  
Jagadeesh Bayry ◽  
...  
Keyword(s):  
Candida Albicans ◽  
Intravenous Immunoglobulin ◽  
Murine Model ◽  
Dextran Sulfate ◽  
Dextran Sulfate Sodium ◽  
Intestinal Inflammation ◽  
Ivig Therapy ◽  
Ivig Treatment ◽  
Intravenous Immunoglobulin Therapy ◽  
Anti Inflammatory

Intravenous immunoglobulin (IVIg) therapy has diverse anti-inflammatory and immunomodulatory effects and has been employed successfully in autoimmune and inflammatory diseases. The role of IVIg therapy in the modulation of intestinal inflammation and fungal elimination has not been yet investigated. We studied IVIg therapy in a murine model of dextran sulfate sodium (DSS)-induced colitis. Mice received a single oral inoculum of Candida albicans and were exposed to DSS treatment for 2 weeks to induce colitis. All mice received daily IVIg therapy starting on day 1 for 7 days. IVIg therapy not only prevented a loss of body weight caused by the development of colitis but also reduced the severity of intestinal inflammation, as determined by clinical and histological scores. IVIg treatment significantly reduced the Escherichia coli, Enterococcus faecalis, and C. albicans populations in mice. The beneficial effects of IVIg were associated with the suppression of inflammatory cytokine interleukin (IL)-6 and enhancement of IL-10 in the gut. IVIg therapy also led to an increased expression of peroxisome proliferator-activated receptor gamma (PPARγ), while toll-like receptor 4 (TLR-4) expression was reduced. IVIg treatment reduces intestinal inflammation in mice and eliminates C. albicans overgrowth from the gut in association with down-regulation of pro-inflammatory mediators combined with up-regulation of anti-inflammatory cytokines.

scholarly journals Intravenous immunoglobulin therapy in COVID-19-related encephalopathy

2020 ◽  
Author(s):  
Lorenzo Muccioli ◽  
Umberto Pensato ◽  
Giorgia Bernabè ◽  
Lorenzo Ferri ◽  
Maria Tappatà ◽  
...  
Keyword(s):  
Intravenous Immunoglobulin ◽  
Neuropsychiatric Symptoms ◽  
Brain Mri ◽  
Invasive Mechanical Ventilation ◽  
Case Series ◽  
Ivig Therapy ◽  
Immunoglobulin Therapy ◽  
Severe Respiratory Distress ◽  
Intravenous Immunoglobulin Therapy ◽  
Extrapyramidal Signs

Abstract Objective To report on efficacy and safety of intravenous immunoglobulin (IVIg) therapy in a case series of patients with COVID-19-related encephalopathy. Methods We retrospectively collected data on all patients with COVID-19 hospitalized at two Italian hospitals who developed encephalopathy during disease course and were treated with IVIg. Results Five patients (two females, mean age 66.8 years) developed encephalopathy after a mean of 12.6 days, since the onset of respiratory/constitutional symptoms related to COVID-19. Four patients suffered severe respiratory distress, three of which required invasive mechanical ventilation. Neurological manifestations included impaired consciousness, agitation, delirium, pyramidal and extrapyramidal signs. EEG demonstrated diffuse slowing in all patients. Brain MRI showed non-specific findings. CSF analysis revealed normal cell count and protein levels. In all subjects, RT-PCR for SARS-CoV-2 in CSF tested negative. IVIg at 0.4 g/kg/die was commenced 29.8 days (mean, range: 19–55 days) after encephalopathy onset, leading to complete electroclinical recovery in all patients, with an initial improvement of neuropsychiatric symptoms observed in 3.4 days (mean, range: 1–10 days). No adverse events related to IVIg were observed. Conclusions Our preliminary findings suggest that IVIg may represent a safe and effective treatment for COVID-19-associated encephalopathy. Clinical efficacy may be driven by the anti-inflammatory action of IVIg, associated with its anti-cytokine qualities.

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