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Összefoglaló. Bevezetés: A Kawasaki-szindróma immunvasculitis, amely kezeletlenül kardiológiai szövődményekhez vezethet. A korai intravénás immunglobulin-terápia mérsékli a szövődményeket, de az esetek 10–20%-a rezisztens a kezelésre. Ennek előrejelzésére világszerte számos rizikóbecslő pontrendszert használnak. Célkitűzés: A Kobayashi- és a Kawanet-pontrendszer prediktív értékének vizsgálata betegeink intravénás immunglobulin-rezisztenciája és kardiológiai szövődményei vonatkozásában. Tudomásunk szerint ez az első magyarországi vizsgálat, amely Kawasaki-szindróma esetében pontrendszerek prediktív értékét méri fel. Módszer: Retrospektív pilotvizsgálatunkban kigyűjtöttük a 2005. január és 2020. április között Kawasaki-szindróma miatt ápolt betegeink adatait. Mindegyiküknél Kobayashi-, illetve Kawanet-pontot számoltunk, valamint megvizsgáltuk azok specificitását, szenzitivitását az intravénás immunglobulin-rezisztencia, illetve a kardiológiai szövődmények előrejelzése szempontjából. A Kobayashi-pontrendszerben 4, a Kawanet-pontrendszerben pedig 2 pont vagy annál magasabb érték jelez rizikót. Eredmények: Kawasaki-szindrómát 28 gyereknél véleményeztünk, 13 esetben észleltünk mérsékelt, 4 esetben súlyos szövődményt. 4 betegünk bizonyult intravénás immunglobulinra rezisztensnek. A rezisztencia szempontjából a Kobayashi-pontrendszer alacsony szenzitivitást (25%), illetve magas specificitást (91,6%), míg a Kawanet-pontrendszer mérsékelt szenzitivitást (50%) és specificitást (50%) mutatott. A szövődmények szempontjából hasonló eredményeket kaptunk, Kobayashi-pontrendszer: szenzitivitás: 17%; specificitás: 100%, illetve Kawanet-pontrendszer: szenzitivitás: 47%; specificitás: 45%. Következtetés: A legtöbb, nem ázsiai országban készült tanulmányhoz hasonlóan az intravénás immunglobulin-rezisztencia előrejelzésében a Kobayashi-pontrendszer vizsgálatunkban sem bizonyult hatékonynak. Ezzel szemben, magasabb szenzitivitása miatt, a Kawanet-pontrendszer intravénás immunglobulin-rezisztenciát előre jelző hatékonyságát érdemes lenne nagyobb esetszámban vizsgálni a hazai populációban is. A kardiológiai szövődmények előrejelzésére egyik pontrendszer sem bizonyult alkalmasnak. Orv Hetil. 2021; 162(47): 1885–1890. Summary. Introduction: Kawasaki disease is an immunovasculitis, which, without treatment, leads to cardiac complications. Early intravenous immunoglobulin therapy moderates complications, however, 10–20% of patients are resistant to the therapy. Numerous risk score systems are used worldwide to predict this. Objective: To assess the predictive value of the Kobayashi and Kawanet score systems regarding intravenous immunoglobulin resistance and cardiac complications in our department’s patient cohort. To our best knowledge, this is the first study in Hungary, which examines the predictive value of score systems in the case of Kawasaki disease. Method: In our study, we identified the patients treated for Kawasaki disease between January 2005 and April 2020. In each case, we calculated both the Kobayashi and the Kawanet score, and we examined their specificity and sensitivity regarding the prediction of intravenous immunoglobulin resistance and cardiac complications. In the Kobayashi score system, values above 4, in the Kawanet score system, values above 2 signal risk. Results: We identified 28 patients with Kawasaki disease. We observed moderate complications in 13, severe complications in 4 cases. 4 of our patients were resistant to intravenous immunoglobulin therapy. Regarding intravenous immunoglobulin resistance in our patient cohort, we detected low sensitivity (25%) and high specificity (91.6%) in the case of Kobayashi score, and moderate sensitivity (50%) and specificity (50%) in the case of Kawanet score. Regarding complications, we found similar results in the case of Kobayashi (sensitivity: 17%; specificity: 100%) and the Kawanet (sensitivity: 47%; specificity: 45%) score system. Conclusion: Similarly to the majority of non-Asian studies, we found the Kobayashi score system ineffective in predicting intravenous immunoglobulin resistance. However, due to its higher sensitivity, the predictive value of the Kawanet score system regarding intravenous immunoglobulin resistance is worth examining in a larger patient population in Hungary. Regarding the prediction of cardiac complications, both score systems were found to be ineffective. Orv Hetil. 2021; 162(47): 1885–1890.

Background Many clinicians are aware that certain therapies administered to their patients can have downstream consequences in the form of clinical laboratory test interferences. This is particularly true of laboratory tests that depend on, or directly involve the use of, antibody-based methodology. Intravenously-administered immunoglobulin therapy is one such treatment that can in theory directly impact the results of particular tests in the area of viral serology. This study can help serve as a reference for clinicians researching the impact of intravenously-administered immunoglobulin therapy in the context of positive results that do not reflect the clinical background of the patient. Case presentation We describe a case whereby an intravenously-administered immunoglobulin therapy led to a series of clinical false positives in viral serology, inconsistent with the known patient history as well as recent laboratory results. The patient presented to hospital with petechiae-type bleeding rashes and was investigated for thrombocytopenia after initial blood investigations indicated very low platelets. Subsequent testing of the potential causes for low-platelet involved several viral serology investigations, including hepatitis, cytomegalovirus and human immunodeficiency virus. Initial testing indicated patient exhibited negative status for all viral antibodies and antigens (except immunity for hepatitis B surface antigen antibody). As part of the thrombocytopenia treatment, intravenously-administered immunoglobulin therapy was administered, and subsequent viral serology was ordered. These investigations indicated a positive status for several hepatitis antibodies as well as cytomegalovirus. Conclusions This case study illustrates the potential for improper diagnosis of previous or ongoing infection status in patients administered IVIg therapy. Caution should be exercised particularly when interpreting results involving cytomegalovirus and hepatitis.

Histobulin is complementary to IVIG therapy but is an essential therapeutic for PWCD. Histoublin is recommended not only in atopic dermatitis and multiple food allergies but also in patients with multiple allergic diseases.

Background Patients whose parents had a history of Kawasaki disease (KD) often show a poor response to intravenous immunoglobulin (IVIG). There are very few reports describing the clinical characteristics and no reports on changes in C-reactive protein (CRP) level after IVIG administration in such cases. Methods A single-center cohort study including 950 patients hospitalized for KD and treated with IVIG was conducted. The patients were divided into two groups: patients whose parents had a history of KD (PHK, N=16) and patients whose parents had no history of KD (NPHK, N=934). The clinical characteristics and responsiveness to IVIG were compared between the two groups. Results The PHK group had higher CRP levels before administering IVIG than the NPHK group (P=0.0010). CRP levels after IVIG administration were higher in the PHK group than in the NPHK group (P=0.0094). The percentage of patients who received a second administration of IVIG on day 2 after the initial IVIG therapy was higher in the PHK group than in the NPHK group (P=0.0016). The percentage of patients who required plasma exchange therapy in the PHK group was significantly higher than that in the NPHK group (P=0.0010). Conclusions Patients in the PHK group had severe KD before IVIG administration, and these patients showed poor responsiveness to IVIG therapy.

Stiffperson syndrome (SPS) is one of the rarest neurological disorders. It is an autoimmune disease with antibodies against glutamic acid decarboxylase - enzyme needed to produce neurotransmitter GABA. This disease is very debilitating and fatal if not managed in time. A 32-year-old man presented with stiffness of abdominal and thoracolumbar paraspinal muscles first noticed 10 months back. There was history of jerkiness of hands. After excluding other mimicking conditions like multiple sclerosis, parkinsonism and axial dystonia, a diagnosis of Stiffperson syndrome was made. Treatment with IVIG therapy along with anti-spasticity drugs resulted in improved patient outcome. SPS is potentially treatable though cannot be cured. Not one treatment fits all patients with SPS and may have to be tailored according to the clinical manifestations. Increasing awareness in medical doctors and identifying the disease early can help the patients escape the disabling effects of SPS and improve the quality of life.

Background Symptomatic COVID-19 cases in children occur mostly in those with primary immunodeficiency (PID), chronic lung diseases, and heart disease. Guidelines recommend that patients with PID continue to use their regular medication during the pandemic. Objectives This study aimed to evaluate anxiety related to COVID-19 in the parents of patients receiving intravenous immunoglobulin (IVIG) treatment in our hospital and to evaluate the effect of their anxiety on the continuity of treatment. Methods The parents of the patients who underwent IVIG therapy in our clinic during the pandemic (between May 15, 2020 and July 1, 2020) were included in our study. Results Twenty-seven patients with PID whose IVIG therapy was initiated before the pandemic and 29 non- PID control subjects were included in the study. All patients received IVIG treatment in our clinic continued treatment during the pandemic at the same dose intervals. Parents in the IVIG group had significantly higher state (p=0.003) and trait (p=0.003) anxiety scores compared to control parents. IVIG group showed statistically significant higher scores in Beck depression inventory, than the control group (p=0.002). Conclusions The parents of PID patients who needed to come to the hospital for IVIG therapy had higher anxiety levels than the parents of similar aged children who presented to our clinic for different complaints between the same dates. Despite their concerns, the parents of all patients under IVIG therapy maintained treatment continuity at the recommended treatment intervals. None of our immunodeficient patients who presented for treatment during the pandemic contracted COVID-19 infection during our study.

Background Immunoglobulin replacement has been standard therapy for patients with primary immunodeficiency diseases (PIDD). Intravenous immunoglobin (IVIg) is delivered at the hospital, whereas subcutaneous immunoglobin (SCIg) is used for home-based treatment. The aim of the study was to determine the advantages and disadvantages of IVIg and SCIg in Polish children aged 1-5 years, with PIDD, and the satisfaction of their parents / caregivers regarding immunoglobulin replacement. Methods The research involved parents of 23 children with PIDD, aged 1-5 years. All children were given IVIg therapy and after at least 6 months they were switched towards home SCIg therapy for at least 6 months. A questionnaire assessing advantages and disadvantages of preferred types of treatment and the quality of life of PIDD patients' families' lives was used. Results The research showed that IVIg therapy was better accepted by parents than SCIg therapy (P = 0.034) for the following reasons: It made it possible for the children to receive treatment once per month (60%); it reduced the fear of injecting the children (60%), and it provided better control of the disease through regular visits to the hospital (53.33%). Parents noticed, however, that IVIg had a significant impact on absence at school or work (70%). Parents who preferred SCIg for their children were guided mainly by the smaller number of side effects (40%), and the fact that the treatment did not interfere with parents' work or the children's school (40%). Conclusion The results showed that IVIg therapy was better accepted by parents than SCIg therapy Parents of children with SCIg are less satisfied with their life, and feel anxiety about their children disease, which is related to administering the medicine by themselves.

Background: Infants younger than 1 year of age with Kawasaki disease (KD) have a high risk for coronary artery lesions (CAL). However, the appropriate acute phase treatment for the infants has not been established. Objective: To ascertain the usefulness of an initial single intravenous immunoglobulin (IVIG) therapy (2g/ kg) with delayed use of aspirin (DUA) for the infants. Materials and Methods: The outcomes in 205 patients who underwent initial single IVIG therapy at 2g/ kg/ dose with DUA for KD were investigated retrospectively. These subjects were divided into those aged

Jeffrey T Guptill,1 M Chris Runken,2 Michael Eaddy,3 Orsolya E Lunacsek,4 Rupali M Fuldeore,4 Christopher M Blanchette,5 Emily Zacherle,5 Joshua M Noone51Department of Neurology, Duke University, Durham, NC, USA; 2Grifols SSNA, Research Triangle Park, Durham, NC, USA; 3Scientific Consulting, Xcenda, Palm Harbor, FL, USA; 4Real-World Evidence, Xcenda, Palm Harbor, FL, USA; 5Department of Public Health Sciences, University of North Carolina at Charlotte, Charlotte, NC, USAPurpose: For chronic inflammatory demyelinating polyneuropathy (CIDP) patients, each branded intravenous immunoglobulin (IVIG) treatment differs in production processes, virus elimination, formulation, and composition. Given the limited availability of real-world data comparing IVIGs for CIDP, this study evaluated switching patterns between IVIG products in 2 separate retrospective databases.Patients and methods: Two independent analytic teams retrospectively evaluated IVIG treatment-naïve patients with an ICD diagnosis code for CIDP. Study 1 used integrated healthcare claims from IMS LifeLink PharMetrics Plus™ and Study 2 used the Truven MarketScan® Database. All analyses were descriptive, with outcomes assessed during the 2-year post-index period.Results: One-quarter of IVIG patients switched therapies within the 2-year study period. In both studies, switching rates were lowest for IVIG-G (Gamunex®-C) (Study 1: 9.8%, Study 2: 8.9%), followed by IVIG-F (Flebogamma®) (Study 1: 25.0%, Study 2: 18.2%), and highest for IVIG-other (Octagam®/Gammaplex®) (Study 1: 50.0%, Study 2: 33.3%). When patients were switched, most switched to IVIG-G (Study 1: 51.6%, Study 2: 54.3%).Conclusion: The small proportion of CIDP switchers in 2 independent studies suggests that IVIG therapy is generally well tolerated. However, differences existed in switch rates for different IVIG products. The reason for low switching rates could not be assessed in this study; therefore, further studies are required to detect possible relevant differences in effectiveness and tolerability.Keywords: intravenous immunoglobulin, chronic inflammatory demyelinating polyneuropathy, treatment patterns

Introduction: Kawasaki disease is a common systemic vasculitis of unknown cause in children and involves various organs but the main damage of this multisystemic vasculitis is mainly in the coronary arteries. Currently, the main treatments for this disease include aspirin therapy and IVIG. In the present study, we investigated the ratio of neutrophils to lymphocytes in patients with Kawasaki disease and its relationship with IVIG resistance in Kawasaki patients.Method: First, we reviewed the clinical records of patients diagnosed with Kawasaki, who received treatment at Motahari Hospital in Urmia from 2008 to 2018 (IVIG therapy). We re-examined Kawasaki diagnostic criteria for each individual before enrolling patients. Results: In our study, initially 460 cases of patients who were admitted to Motahari Hospital in Urmia between 2009 and 2019 with an initial diagnosis of Kawasaki disease were reviewed. Among them, patients who met Kawasaki diagnostic criteria were included in the final review and the rest of the cases were excluded from the study according to the exclusion criteria. Finally, out of 460 cases, 241 patients were included in the final analysis. The results indicated high blood leukocyte count (p-value = 0.013) and low urinary leukocyte count (p-value = 0.01) were directly related to response to treatment in Kawasaki patients, but age, sex, neutrophil ratio to Lymphocytes, neutrophil count, blood lymphocytes, CRP, hypoalbuminemia, ALT levels, anemia, thrombocytosis, and the time interval between onset of symptoms and treatment response to IVIG therapy in Kawasaki patients are not significantly related.Conclusion: High blood leukocyte count and low urinary leukocyte count in patients can be good criteria for predicting the prognosis of Kawasaki patients in response to IVIG, while the ratio of neutrophils to lymphocytes is not effective in this prediction.

International audience; Intravenous immunoglobulin (IVIG) is used to treat several autoimmune and inflammatory diseases, but some patients are refractory to IVIG and require alternative treatments. Identifying a biomarker that could segregate IVIG responders from non-responders has been a subject of intense research. Unfortunately, previous transcriptomic studies aimed at addressing IVIG resistance have failed to predict a biomarker that could identify IVIG-nonresponders. Therefore, we used a novel data mining technique on the publicly available transcriptomic data of Kawasaki disease (KD) patients treated with IVIG to identify potential biomarkers of IVIG response. By studying the boolean patterns hidden in the expression profiles of KD patients undergoing IVIG therapy, we have identified new metabolic pathways implicated in IVIG resistance in KD. These pathways could be used as biomarkers to segregate IVIG nonresponders from responders prior to IVIG infusion. Also, boolean analysis of the transcriptomic data could be further extended to identify a universal biomarker that might predict IVIG response in other autoimmune diseases.

Although the treatment of the hyperinflammatory response due to COVID-19 has not yet been found, high-dose corticosteroids, interleukin receptor blockers and intravenous immunoglobulin (IVIG) are used to improve the hyperinflammatory response. In this article, we aimed to share our experiences with 3 patients who received IVIG therapy in ICU. (English) [ABSTRACT FROM AUTHOR] COVID-19’a bagli gelisen hiperinflamatuvar yanitin kesin bir tedavisinin henuz bulunamamis olmasina ragmen, yuksek doz kortikosteroid, interlokin reseptor blokerleri ve intravenoz immunoglobulin (IVIG) tedavisi bugunlerde kullanilmaktadir. Biz bu makalemizde, yogun bakimimizda IVIG tedavisi uyguladigimiz 3 Covid-19 hastasindaki tecrubelerimizi paylasmayi amacladik. (Turkish) [ABSTRACT FROM AUTHOR] Copyright of Anestezi Dergisi (JARSS) is the property of Logos Medical Publishing and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

Coronavirus disease 2019 (COVID-19) pandemic has drawn attention over old immunotherapeutic agents such as convalescent (immune) plasma (CIP). Here, an adolescent with severe COVID-19 case requiring CIP and intravenous immunoglobulin (IVIG) treatments is described. A 17-year-old male patient was brought to the emergency room with complaints of fever, fatigue, and severe cough. Two doses of CIP were infused to the patient because of the increase in persisting fever, dyspnea, and acute phase reactant levels after the third day of routine protocol treatment. IVIG therapy was begun for 2 days at a dose of 1 g/kg/day due to resurgence in acute phase reactants and progressing radiological findings following CIP transfusion. Our patient avoided mechanical ventilation and showed immediate clinical and radiological improvement with CIP transfusion followed by IVIG therapy. Timely initiation of CIP treatment followed by IVIG prevented the disease from worsening and helped to reduce the requirement for mechanical ventilation.

The outbreak of a severe acute respiratory syndrome caused by a novel coronavirus (COVID-19), has raised health concerns for patients with chronic inflammatory demyelinating polyradiculoneuropathy (CIDP), who are frequently on long-term immunotherapies. Treatment with IVIg does not increase the risk of contracting COVID-19, and the IVIg administration may have a protective role. However, infusions can expose patients to an increased risk of contracting SARS-CoV-2 due to repeated access to Health Facilities. In this report we analyzed the short-term follow-up of CIDP patients who modified their chronic IVIg therapy during pandemic. About half of CIDP patients regularly treated with IVIg tried to stop treatment and about 10% shifted to SCIg. Forty-two percent of the patients who stopped the treatment reported a clinical deterioration after suspension and had to restart IVIg. This study demonstrated that in selected cases it is possible to successfully stop the chronic IVIg treatment, even in patients who have been treated for several years.

Intravenous immunoglobulin (IVIG) is a standard therapy for Kawasaki disease (KD), because it prevents formation of coronary artery aneurysm, a major complication of KD. Herein, we report a 3-year-old boy with KD complicated by haemolytic anaemia (HA) which developed following two courses of IVIG. Although both direct and indirect antiglobulin tests and anti-M antibodies were positive in his blood obtained after the onset of HA, indirect antiglobulin tests and anti-M antibodies were negative either in the blood sample before the treatment or the same lot of IVIG products as those used for the therapy, suggesting autoimmune mechanism. This is, to our knowledge, the first report of autoimmune HA caused by anti-M autoantibodies after IVIG therapy in KD.

A steroid- and cyclosporine A- resistant case of Pfeiffer-Weber-Christian disease (PWCD) was remitted completely with sequential treatment with Histobulin and IVIG. The multiple food allergies and the atopic dermatitis of this PWCD patient were also improved with Histobulin treatment. The upper respiratory infections were improved by Histobulin therapy also.

Intravenous immunoglobulin (IVIg) is increasingly used for the treatment of inflammatory and autoimmune diseases. Although skin reactions to IVIg therapy are usually minor, rare, and not life-threatening, dermatologists need to recognize the nature of these adverse reactions. We describe a 33-year-old man suffering from demyelinating polyneuropathy who developed dyshidrotic eczema on the palms and flaky grayish-white scales on an erythematous base on his face after the administration of IVIg.

Intravenous immunoglobulin (IVIG) is an immune modulating treatment used in a variety of immune mediated diseases. We report, two patients of different demographics, treated with IVIG for different neurological diagnoses who reported significant hair growth after starting IVIG treatments. To date, there have been no reports of using IVIG as a treatment for hair loss due to androgenic alopecia (AGA).