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Background: Severe coronavirus disease 2019 (COVID-19) may lead to the cytokine storm syndrome which may cause acute respiratory failure syndrome and death. Our aim was to investigate the therapeutic effects of infliximab, intravenous gammaglobulin (IVIg) or combination therapy in patients with severe COVID-19 disease admitted to the intensive care unit (ICU). Methods: In this observational research, we studied 104 intubated adult patients with severe COVID-19 infection (based on clinical symptoms, and radiographic or CT scan parameters) who were admitted to the ICU of a multispecialty hospital during March 2020 in Tehran, Iran. All cases received standard treatment regimens as local protocol (Oseltamivir + hydroxychloroquine + lopinavir/ritonavir or sofosbuvir or atazanavir ± ribavirin). The cases were grouped as controls (n = 43), infliximab (n = 27), IVIg (n = 23) and combination (n = 11). Results: There was no significant difference between controls and treatment groups in terms of underlying diseases or the number of underlying diseases. The mean age (SD) of cases was 72.42 (16.06) in the control group, 64.52 (12.965) in IVIg, 63.40 (17.57) in infliximab and 64.00 (11.679) in combination therapy; (P = 0.047, 0.031 and 0.11, respectively). Also, 37% in the infliximab group, 26.1% in IVIg, 45.5% in combination therapy, and 62.8% in the control group expired (all P < 0.05). Hazard ratios were 0.31 in IVIg (95% CI: 0.12-0.76, P = 0.01), 0.30 in infliximab (95% CI: 0.13-0.67, P = 0.004), 0.39 in combination therapy (95% CI: 0.12-1.09, P = 0.071). Conclusion: According to the findings of this study, it seems that infliximab and IVIg, alone or together, in patients with severe COVID-19 disease can be considered an effective treatment. [ABSTRACT FROM AUTHOR]

Lack of reliable laboratory parameters is the main challenge in the management of fetal and neonatal alloimmune thrombocytopenia ( FNAIT). Despite the long-known association between the HLA- DRB3*01:01 allele and human platelet antigen 1a ( HPA-1a) alloimmunisation, maternal human leucocyte antigen ( HLA) typing has been of little clinical value. Recently, other DRB3 allele variants have been suggested to predict the severity of FNAIT. In this nationwide population-based retrospective cohort study, we performed extensive HLA typing of 96 women, accounting for 87% of our cohort of 110 families with confirmed or possible HPA-1a-immunisation. The HLA type was compared with anti- HPA-1a levels, severity of neonatal disease and responsiveness to maternally administrated intravenous gammaglobulin ( IVIG). HLA haplotypes were constructed to investigate further HLA associations. Despite significantly lower anti- HPA-1a levels in DRB3*01:01-negative women, the carrier status of this particular allele could not be used to confirm or rule out FNAIT in the absence of detectable antibodies. In the haplotype analysis, the DRB3*01:01 allele was the actual factor associated with FNAIT. No other HLA allele was shown to be of additional value as a predictor of severe FNAIT or non-responsiveness to IVIG treatment. Thus, HLA genotyping was not found useful in differentiating high- and low-risk pregnancies or in guiding antenatal treatment in affected families. [ABSTRACT FROM AUTHOR]

Introduction: Clinical observations in patients with specific antibody deficiency treated for periods of time with IgG infusion have suggested that IgG may have a positive immunoregulatory effect on the production of specific antibodies against pneumococcal polysaccharides. We developed an in vitro model to test the effect of an IgIV preparation on the antibody production in response to a pneumococcal polysaccharide serotype and on the antibody and cytokine production in response to both a protein antigen and a pneumococcal polysaccharide antigen. Methods: We studied 37 consecutive patients referred to our clinics for evaluation of their recurrent respiratory infections. Subjects were divided into two groups: 22 patients without SAD and 15 patients with SAD. PBMCs were left unstimulated or were stimulated with tetanus toxoid or pneumococcal polysaccharide serotype 19, in the presence of human albumin or IgIV. IgG anti-Pn-19 antibody, IL-4 and IFN-γ concentrations in culture supernatants were determined by ELISA. Results: An increase in IgG anti-Pn-19 antibodies, associated with an increase in IFN-γ and a decrease in IL-4 production was observed in cultures stimulated with pneumococcal polysaccharide in the presence of IgIV when patients were analyzed together. The enhancing effect of IgIV was more significant for both IgG anti-Pn19 antibodies and IFN-γ in patients without SAD. In contrast, IgIV caused a significant decrease in IL-4 secretion in patients with SAD, which was associated with an increase in IgG anti-Pn19 antibodies in 3 of 7 of these patients. Conclusions: These results suggest that IgIV has some immunomodulatory effect on the in vitro production of IgG anti-Pn19 antibodies and cytokine production in cell cultures stimulated with Pn-19 antigen and that this modulation may be associated with a Th1/Th2 regulatory mechanism. Further studies at a cellular and molecular level are in progress to examine if the differences in the in vitro modulatory response to IgIV in these two groups of patients may point to a functional defect in patients with SAD. [ABSTRACT FROM AUTHOR]

Objective: To explore the clinical efficacy of high-dose intravenous gammaglobulin (IVIG) in acute Guillain-Barre syndrome (GBS) and its effect on serum concentrations of inflammatory factors., Methods: A total of 111 patients with acute GBS were enrolled in this retrospective study. They were admitted to Ji'nan City People's Hospital from January 2019 to December 2020. According to the treatment method, the patients were divided into a control group (n=53, received routine treatment) and an observation group (n=58, received high-dose IVIG in addition to routine treatment). The clinical efficacy, Barthel index for activities of daily living (ADL), serum concentrations of inflammatory factors (IL-6, TNF-α, NO) in peripheral blood, potential of electromyography signals, abnormal rates of motor and sensory conduction velocity, and F wave abnormality rate were compared. Also, the risk factors affecting IVIG treatment efficacy were analyzed., Results: The overall response rate, and Barthel index for ADL were higher, while serum concentrations of IL-6, TNF-α, and NO were lower in the observation group than the control group (all P<0.05). There were differences in spontaneous potential and motor potential before and after treatment in both groups (both P<0.05). The observation group showed lower abnormal rates of motor and sensory conduction velocity, F wave abnormality rate, and prolonged latency rate than the control group (all P<0.05). Concomitant lung infection, respiratory muscle involvement, and treatment with high-dose IVIG >2 weeks from onset were independent risk factors for treatment efficacy., Conclusion: High-dose IVIG has good clinical efficacy in treating acute GBS by reducing the serum concentrations of IL-6, TNF-α, and NO, improving patients' abnormal muscle electrical condition, and promoting recovery. It is recommended for use clinically at an early stage. At the same time, lung infection must be prevented., Competing Interests: None., (AJTR Copyright © 2022.)

Objectives: The clinical and therapeutic aspects of primary Sjögren syndrome (PSS) in patients with peripheral neuropathy were analyzed and the specifics of individual case studies are discussed. Methods: We retrospectively studied six patients (four women, two men; mean age 64.5 years) presenting with PSS with peripheral neurological involvement over a five-year period (2008-2012). All patients had neurological examinations, including nerve conduction studies, somatosensory evoked potentials, and sural nerve biopsies. Treatment regimens included corticosteroids, intravenous gammaglobulin, or immunosuppressive treatment. Results: Peripheral neuropathy was observed in six (7.9 %) of 76 patients with SS as the underlying disease; three were cases of multiple mononeuropathy, two cases had sensory ataxic neuropathy, one of which was autonomic neuropathy, and one case was diagnosed as painful sensory neuropathy without sensory ataxia. Four of the six patients were diagnosed with SS after the onset of neurological symptoms. Individual peripheral neuropathies had distinct neurological, electrophysiological, and pathological characteristics. The effect of steroids and intravenous gammaglobulin differed depending on the case. Conclusions: In PSS patients, a precise diagnosis is important, because the therapeutic strategy and response varies depending on the type of neuropathy. In clinical practice, it is important to consider a diagnosis of SS when patients present with peripheral neuropathy. [ABSTRACT FROM AUTHOR]

Background: The clinical significance and efficacy of treating patients who have immunoglobulin (Ig) G subclass deficiency and/or antibody deficiency with Ig-replacement therapy has been debated. There are no clear guidelines to recommend intravenous gammaglobulin (IgIV) in these patients as there are few published studies documenting its efficacy. Methods: We studied in an open-label protocol 10 adult patients with recurrent respiratory infections and IgG subclass and/or antibody deficiency. All patients received monthly IgIV for 12 months and then were observed for 3 months without IgIV infusions. We studied quality of life, incidence of infections, need for antibiotics, frequency of hospitalizations due to infections, IgG subclass and antibody (tetanus and pneumococcal) levels. Innate immunity was evaluated by studying the status of Toll-like receptors and polymorphisms, mannan-binding lectin levels and genotypes. Correction of the immune defects during IgIV therapy was evaluated. Results: Monthly IgIV significantly improved quality of life, decreased the number of infections and the need for antibiotics, and improved IgG subclass and antibody serum levels. No consistent finding of innate immunity could be detected. Conclusions: IgIV could be beneficial in patients with IgG subclass or antibody deficiency. Copyright © 2009 S. Karger AG, Basel [ABSTRACT FROM AUTHOR]

Objectives: Scleromyxedema, a rare systemic disease of unknown etiology, presents with progressive dermal mucin deposition, causing skin thickening and a variety of gastrointestinal, neurologic, pulmonary, cardiac, and renal complications. Scleromyxedema is notoriously difficult to treat. Our objective was to suggest a new treatment option and to propose a novel tool, musculoskeletal ultrasound, for diagnosis and disease monitoring. Methods: We present a patient with a severe scleromyxedema with cutaneous, gastrointestinal, hematologic, and neurologic manifestations. Having failed multiple therapeutic attempts, he responded to treatment with intravenous gammaglobulin (IVIG) and oral thalidomide. Using high-frequency (13 Hz) ultrasound and electronic calipers, we obtained 3 measurements of skin thickness before and after treatment to demonstrate response to treatment. A focused review of the published literature on scleromyxedema, with an emphasis on treatment, was conducted and the results were reviewed in this article. Results: Following treatment with IVIG, the patient showed dramatic improvement in his clinical symptoms and remained in remission through combination IVIG infusions (2 g/kg) and oral thalidomide (150 mg/d). Literature review revealed the absence of any controlled studies for the treatment of scleromyxedema. Various agents have been proposed in case reports and small series with variable success. Emerging data on the use of IVIG or thalidomide alone have been encouraging. Conclusion: IVIG and thalidomide in combination may be an effective novel treatment of scleromyxedema. Additionally, skin ultrasonography, a noninvasive test, may become a useful diagnostic and disease activity monitoring tool. [Copyright &y& Elsevier]

The effect of a commercially available intravenous gammaglobulin preparation (IgSRK; Sandoglobulin) on the antigen-non-specific activation of the immune system was examined using pokeweed mitogen (PWM)-induced B cell differentiation. In cultures of peripheral blood mononuclear cells from 16 normal donors. IgSR K (300 μg/ml) inhibited PWM-induced generation of plaque-forming cells by 76% (P &lt 0.001), whereas human serum albumin (300 μg/ml) induced no significant inhibition (5%; P not significant). The IgSRK-mediated suppression was demonstrable in both serum-containing (76%) and serum-free (63%) media, and monomeric IgSRK suppressed as effectively as did heataggregated IgSRK. F(ab')2 fragments exhibited no inhibitory capacity (mean inhibition -- 11%: P not significant) suggesting that the Fc portion of IgSRK may be required for suppression. In addition, IgSRK had lo be added to the cultures at their initiation to effect full inhibition. These studies suggest a potential beneficial pharmacological role for IgSRK in the treatment of disorders characterized by pathogenic autoantibodies, but also warn of a potential deleterious effect of inhibiting the host's humoral response to an infectious challenge. [ABSTRACT FROM AUTHOR]

Nine children with acute typical post-diarrhea hemolytic uremic syndrome (HUS) were treated with intravenous gammaglobulin (IVIG). These children were compared to nine children with HUS who did not receive IVIG. The use of IVIG did not appear to have a beneficial effect on eight of the nine treated children. There were no significant differences found in the duration of hemorrhagic colitis, thrombocytopenia, elevation of the white blood count (WBC), anuria, dialysis, or hospitalization, or the presence of a central nervous system complication or pancreatitis. Although no significant difference was found in the duration of thrombocytopenia, there was a trend towards a longer duration of thrombocytopenia in children treated with IVIG ( P=0.13). One child demonstrated both an increase in her platelet count and a decrease in her WBC count within 24 h of receiving her first dose of IVIG. [ABSTRACT FROM AUTHOR]

Two cases of non-familial haemophagocytic lymphohistiocytosis (HLH) are presented in which treatment with interferon alfa and gammaglobulin was associated with complete clinical remission. In one case, serological evidence of recent Epstein-Barr virus infection was found. Natural killer cell activity was within normal limits in both children, compatible with a secondary form of HLH. The combination of interferon alfa and intravenous gammaglobulin requires further evaluation in the treatment of non-familial HLH. [ABSTRACT FROM AUTHOR]

Abstract: Central nervous system involvement in rheumatoid arthritis is uncommon. In order of frequency, published cases have reported rheumatoid nodules, meningeal vasculitis, and cerebral vasculitis (CV). The frequency of vasculitic cerebral involvement in rheumatoid arthritis is unknown. However, it is known that it is more common in patients with seropositive, long-standing rheumatoid arthritis, and in those with concomitant systemic vasculitis. We report the case of a 47-year-old woman with an 11-year history of seropositive rheumatoid arthritis without any extra-articular manifestations, with the exception of secondary Sjogren''s syndrome, presenting with mental status changes and tonic-clonic seizures as symptoms of isolated CV. Magnetic resonance imaging (T2) showed hyperintense white-matter lesions in the frontal and temporal lobes, as well as in the hippocampus and cerebellum. Transcranial Doppler ultrasound findings were consistent with CV. Other differential diagnoses were ruled out. The patient responded favorably to methylprednisolone and intravenous gammaglobulin therapy. [Copyright &y& Elsevier]

The long-term sequelae and early predictors of sequelae were determined in a chart review of 47 children with Guillain-Barre syndrome (GBS) of whom 30 had been treated with intravenous gammaglobulin (IVIG) at The Hospital for Sick Children, Toronto, Canada.

During the summer of 2000, a countrywide epidemic of West Nile fever (WNF) occurred in Israel, with 417 confirmed cases and 35 deaths. Immunosuppressed patients had a 31% case-fatality rate, which was significantly higher compared to non-immunosuppressed patients (13%). We describe a 42-year-old male lung-transplant recipient with serologically confirmed West Nile virus (WNV) encephalitis and deteriorating level of consciousness. He was treated with 0.4 g/kg intravenous immunoglobulin preparation from Israeli donors that contained a high titer of anti-WNV antibodies (1 : 1600). The patient showed rapid improvement within 24 h and complete disappearance of signs and symptoms within 48 h. This is the second case of an immunosuppressed patient responding to the same preparation of intravenous immunoglobulins. Larger studies are required in order to establish the therapeutic role of immunoglobulins in patients with WNF. [ABSTRACT FROM AUTHOR]

Kawasaki syndrome is one of the commonest vasculitides in pediatric age group with devastating consequences if not treated early. Intravenous Gammaglobulin leads to rapid amelioration of clinical symptoms and significantly decreases the risk of development of coronary artery aneurysms. Occasionally, Kawasaki disease may be resistant to I/V IgG. We present our experience with one such patient who ultimately respondend to infliximab therapy. [ABSTRACT FROM AUTHOR]

The article presents a case study of the treatment of Beh#x00E7;et's disease in a 28-year-old man with intravenous gammaglobulins and aspirin. The disease may be caused due to autoimmune reaction by virus, bacteria or other antigens. The condition can be relieved by immunosuppressants which include selective helper T-cells acting on cyclosporin that provides antibodies against antigens. Women suffering from immunological diseases might transmit the transitorily symptoms to the offspring.

A 3-year old boy who had acquired HIV infection transplacentally and developed AIDS encephalopathy is reported from the Depts of Paediatrics and Immunology, Newcastle General Hospital, Newcastle upon Tyne, England.

Objective: We sought to prevent intracranial hemorrhage (ICH) through antenatal management of alloimmune thrombocytopenia. Study Design: A total of 33 women (37 pregnancies) with alloimmune thrombocytopenia and ICH in a previous child were stratified according to the timing of the previous child''s ICH: extremely high risk (HR) (n = 8) had ICH <28 weeks, very HR (n = 17) between 28-36 weeks, and HR (n = 12) in the perinatal period. Treatment was initiated at 12 weeks with intravenous immunoglobulin 1 or 2 g/kg/wk, and if the fetal platelet count by cordocentesis was <30,000/mL despite treatment, prednisone and/or more intravenous immunoglobulin were added. Results: Five of 37 fetuses suffered ICHs. Two ICHs had platelet counts >100,000/mL, and 1 was grade I. The other 2 ICHs were unequivocal treatment failures; both were grade III-IV and resulted in fetal demise. Conclusion: These findings demonstrate the success of stratified treatment in these HR patients, which tailored interventions according to the timing of the sibling''s ICH. [Copyright &y& Elsevier]