Intravenous Immunoglobulin Regimen Research Articles

Dose, exposure, and treatment regimen of intravenous immunoglobulin G in multifocal motor neuropathy.

Intravenous immunoglobulin (IVIG) is the only approved treatment for multifocal motor neuropathy (MMN), a rare, chronic, immune-mediated demyelinating neuropathy. There is a significant gap in understanding of the role of serum immunoglobulin G (IgG) levels in the efficacy of IVIG in affected patients. We aimed to characterize the interplay between dose and exposure of IVIG and the effects of patient factors on individual variabilities. Serum IgG trough concentration data from a phase 3, randomized, double-blind, placebo-controlled, crossover trial of IVIG 10% in 44 patients with MMN (NCT00666263) were analyzed using fit-for-purpose population PK modeling. Patient factors were tested as covariates, and IgG PK profiles following various dosing regimens were simulated. Serum IgG levels, with significant inter-patient variability, correlated with dose and treatment interruptions at the individual patient level. Simulated data for various dosing regimens (0.4-2 g/kg once every 1-4 weeks [Q1-4W]) revealed that more frequent dosing provided more stable IgG levels than less frequent dosing, and dose splitting over multiple days had no significant effects on PK. In patients with MMN, stable dosing and consistent serum IgG levels are crucial to avoid negative responses owing to treatment interruptions. Dosing intervals more frequent than Q4W may alleviate periodic symptom deterioration. Dose splitting potentially offers flexibility for patients requiring large volumes of IVIG without negatively affecting serum IgG PK, while maintaining treatment efficacy. Variability in serum IgG levels between patients suggests that individualizing IVIG treatment regimens and target IgG levels may play a key role in managing MMN.

Prevention of potential delayed hemolytic transfusion reaction in two sickle cell patients using intravenous immunoglobulins and steroids before and after red blood cell exchange with antigen positive units and review literature

Emergent Red Blood Cell (RBC) exchange is indicated in sickle cell disease (SCD) patients with severe acute chest syndrome. However, fully matched RBC units may not be available for patients with multiple RBC antibodies. Intravenous immunoglobulin (IVIG) and steroids were reported for preventing potential delayed hemolytic transfusion reaction (HTR) in simple transfusion of antigen-positive RBCs. We investigated the efficacy and safety of IVIG and steroids in two SCD patients presented with acute chest syndrome receiving RBC exchange with multiple incompatible units. The first patient had multiple historical alloantibodies, including anti-Jsb, although none of them were reactive. IVIG (1 g/kg) was given before and after RBC exchange with methylprednisolone (500 mg IV) one hour before exchange. Her sickle hemoglobin (HbS) was reduced from 89.4% to 17.4% after the exchange with five Jsb-positive units. The patient improved clinically without acute or delayed hemolysis. The second patient had reactive anti-Jsb on two different admissions 18 months apart. Only one of the sixteen units used in the exchanges was Jsb negative. He received the same IVIG regimen during both admissions but 100 mg IV hydrocortisone instead of methylprednisolone. His HbS was reduced from 63.4% to 22.4% after the first exchange. Significant clinical improvements were achieved after both exchanges. No delayed HTR was observed. Our experience of these two patients suggested that IVIG and steroids may be used in preventing potential delayed HTR in some SCD patients with rare antibodies receiving large amounts of antigen-positive RBC products.

Is less more? Intravenous immunoglobulin for pediatric immune thrombocytopenia.

Treatment of pediatric immune thrombocytopenia (ITP) is guided by the risk of bleeding. Intravenous immunoglobulin (IVIg) is one of the first-line therapy options for new-onset pediatric ITP. However, the exact optimal dose of IVIg has not been determined. This retrospective cohort study included all hospitalized children with newly diagnosed ITP receiving IVIg as first-line therapy during 2010-2020. We compared the safety and efficacy of two common IVIg dose regimens, 1 and 2 g/kg. Outcomes were short and long-term treatment responses and adverse events to the different doses. A total of 168 children were included in our cohort. Eighty-two children were treated with 1 g/kg of IVIg and 86 with 2 g/kg. There was no difference in sustained response (platelet count > 20 × 109, > 14 days) between the groups (74.3% vs 76.7%, respectively, p = 0.72) and maximal platelet counts following treatment (p = 0.44). No difference was found regarding the percentage of chronic ITP between the two groups (24.4% in the 1 g/kg group as compared to 17.4% in the 2 g/kg group; p = 0.34). Logistic regression analysis demonstrated there was no effect of the IVIg dose on treatment failure and development of chronic ITP. As anticipated, 47.7% of adverse events were in the 2 g/kg group and 32.9% in the 1 g/kg group, with borderline statistical significance (p = 0.06). The initial treatment of newly diagnosed pediatric ITP using a 1 g/kg IVIg regimen may give comparable results to the double dose of 2 g/kg in attaining a prolonged safe hemostatic threshold, without impacting the incidence of chronic disease.

Hemophagocytic lymphohistiocytosis secondary to virus infection and followed by lupus nephritis recurrence in a renal transplantation pediatric recipient: a case report

BackgroundHemophagocytic lymphohistiocytosis (HLH) is a rare and life-threatening disorder characterized by systemic inflammation and organ failure as a result of dysregulated immune cell activation. HLH can be induced by a variety of factors including infection, tumours and autoimmune disease and can also occur in patients following solid organ transplantation. Occurrence of HLH and lupus nephritis (LN) successively within a short period of time after renal transplantation is uncommon.Case presentationWe described an 11-year-old female post-transplant patient who presented with hemocytopenia, fever, elevated serum ferritin, splenomegaly, hyperlipidemia, and hypofibrinemia, and was clinically diagnosed with HLH. After comprehensive treatment with corticosteroids, intravenous immunoglobulin (IVIG), and reducing immunosuppressants, her condition improved, but then hematuria ensued. The transplant kidney biopsy showed LN. She was treated with hydroxychloroquine and methylprednisolone while intensive immunosuppressive agents were given. She has remained in remission for two years until now.ConclusionsThe main inducing factors of HLH should be identified as early as possible, and accurate treatment plans should be taken. The long-course IVIG regimen may be one of the effective treatments for virus-induced HLH. After remission of HLH, we need to be alert to the recurrence of autoimmune diseases in patients with underlying diseases, and timely increase immunosuppressants.

Guillain-Barre Syndrome linked to SARS-CoV-2 infection – meta-analysis and literature review

Background
 The novel coronavirus disease-2019 (COVID-19), which is caused by Severe Acute Respiratory Distress Syndrome coronavirus 2 (SARS-CoV2), was originally detected in Wuhan, China in December 2019. In this meta-analysis and literature review, we compared and summarized the clinical presentation, cerebrospinal fluid (CSF) and electromyography (EMG) findings and outcomes in SARS-CoV-2 patients with Guillain-Barre Syndrome (GBS) and its variants.
 Methods
 We conducted a literature review in February 2023 searching for terms “Guillain-Barre Syndrome and COVID-19", “SARS neurology”, “COVID-19 complications”. We used PubMed and Google Scholar databases inquiring case reports or series of cases published between April 1, 2020, and September 14, 2023.
 Results
 Of the 52 GBS cases 61,5% (n=32) were male and 39,5% (n=20) were female. The mean age was 57 years old. A total of 75% (n=33) patients presented acute inflammatory demyelinating polyneuropathy (AIDP) variant, 6,8% (n=3) presented acute motor axonal neuropathy (AMAN) variant, 15,9% (n=7) presented acute motor-sensory axonal neuropathy (AMSAN) variant. A total of 85,7% (n=42) of patients were diagnosed with albuminocytological dissociation. During the hospitalization, a total of 30,8% (n=16) required mechanical ventilation. A total of 61,5% (n=32) of patients were treated with a 5-day regimen of intravenous immunoglobulin (IVIG) in dose 0.4 g/kg/day. There were 46,1% (n=24) complete recoveries from GBS, 32,7% (n=17) partial recoveries and 9,6% (n=5) of patients did not respond to treatment. A total of 11,5% (n=6) of patients died.
 Conclusion
 It is crucial to follow patients with COVID-19 and GBS over time to estimate properly the efficacy of treatment and evaluate the real percentage of recovery and complications.

Outcomes of ABO-incompatible liver transplantation in end-stage liver disease patients co-infected with hepatitis B and human immunodeficiency virus

Human immunodeficiency virus (HIV)-positive patients coinfected with hepatitis B virus (HBV) are eligible for liver transplantation (LT) in Africa and Southeast Asia, particularly China. However, the outcome of HIV-HBV coinfected patients referred for ABO-incompatible LT (ABOi-LT) is unknown. To clarify the outcome of ABOi-LT for HIV-HBV coinfected patients with end-stage liver disease (ESLD). We report on two Chinese HIV-HBV coinfected patients with ESLD who underwent A to O brain-dead donor LT and reviewed the literature on HIV-HBV coinfected patients treated with ABO-compatible LT. The pretransplantation HIV viral load was undetectable, with no active opportunistic infections. Induction therapy consisted of two sessions of plasmapheresis and a single dose of rituximab in two split doses, followed by an intraoperative regimen of intravenous immunoglobulin, methylprednisolone, and basiliximab. Post-transplant maintenance immunosuppressive agents consisted of tacrolimus and mycophenolate mofetil, and prednisone. At the intermediate-term follow-up, patients showed undetectable HIV viral load, CD4(+) T cell counts greater than 150 cells/μL, no HBV recurrence, and stable liver function. A liver allograft biopsy showed no evidence of acute cellular rejection. Both patients survived at 36-42 mo of follow-up. This is the first report of ABOi-LT in HIV-HBV recipients with good intermediate-term outcomes, suggesting that ABOi-LT may be feasible and safe for HIV-HBV coinfected patients with ESLD.

Disease profile of Guillain-Barré syndrome among Indian patients receiving intravenous immunoglobulin: a real-world observational study

<p class="abstract"><strong>Background:</strong> Objectives of the study were to evaluate the real-world usage pattern of intravenous immunoglobulin (IVIg) for the treatment of patients with Guillain‐Barré syndrome (GBS), and to understand the disease characteristics and demographic patterns of these patients.</p><p class="abstract"><strong>Methods:</strong> This real-world, retrospective, analysis included data of patients with GBS who received IVIg treatment at various centers across India. The study data was collected between April 2021 and March 2022. </p><p class="abstract"><strong>Results:</strong> A total of 3064 patients with GBS who received IVIg treatment were included. The mean (SD) age of the patients was 48.97 (14.97) years, and majority of the patients were men (68.8%). Acute inflammatory demyelinating polyneuropathy (AIDP) was the most common subtype of GBS (60.5%), followed by acute motor axonal neuropathy (AMAN; 11.6%), and acute motor sensory axonal neuropathy (AMSAN; 3%). The majority (>94%) of patients received IVIg therapy as first-line treatment. A large proportion of the patients (n=2402, 78.4%) were given the standard dose of 2 g/kg bodyweight (given over 5 days) and 97.1% (n=2974) of the total study population received IVIg regimen over the standard protocol of 5 days.</p><p class="abstract"><strong>Conclusions:</strong> In the clinical spectrum of GBS, AIDP was the most common subtype. IVIg was used at generally recommended dose and duration in patients with GBS.</p>

Exome sequencing contributes to identify comorbidities in a rare case of infant ARDS induced by the CD40LG mutation

BackgroundAcute respiratory distress syndrome (ARDS) causes significant mortality in young children with certain diseases. Early diagnosis and treatment can reduce infant mortality. Here, we report a rare case of exome sequencing in the early diagnosis of immunodeficiency in an infant.Case presentationA four-month-old full-term male infant presented with severe shortness of breath, hypoxemia, and unexplained parenchymal lung lesions. A series of examinations were performed to search for potential culprit viruses but negative results were obtained with the only exception being the rhinovirus that tested positive. The child’s family history revealed he had a brother who died of severe infection at the age of two years. We performed an exome sequencing analysis and a mutation analysis of CD40LG to obtain genetic data on the patient. Besides, we used flow cytometry to measure the CD40LG expression levels of activated T cells. A retrospective review of all the CD40LG mutant-induced X-linked hyper IgM syndromes (XHIGM) had been conducted to assess the differences between clinical and genetic molecular features. Finally, a regular intravenous immunoglobulin (IVIG) regimen led to steady breathing, the correction of hypoxemia, and a progressive improvement of lung CT scans. During follow-up, the patient received an IVIG regimen and his CT images improved. Moreover, his parents took advantage of pre-implantation genetic testing with in vitro fertilization to have a healthy twin offspring who did not carry such a mutation according to the early exome sequencing for the proband. Compared with other CD40LG mutant cases in our center, this proband displayed a normal plasma immunoglobulin level and he should be the youngest infant to have a molecular diagnosis of XHIGM.ConclusionUsually, XHIGM would not be suspected with a normal plasma immunoglobulin concentration. However, as we could not identify a potential comorbidity or risk factor, exome sequencing helps target this patient's real facts. Thus, this case report calls for exome sequencing to be performed in the case of unexplained infections when immunodeficiency is suspected after general immunological tests, especially for cases with a contributive family history among infants as the maternal transfused immunoglobulin might mask immune deficiency.

Challenges of Treating Late Onset Severe Antibody Mediated Rejection Post Orthotopic Heart Transplant

<h3>Introduction</h3> Outcomes of severe antibody mediated rejection (AMR) post orthotopic heart transplant (OHT) are often poor & management a challenge. <h3>Case Report</h3> A 50-year-old multiparous female with stable donor specific antibodies (DSAs) presented with cardiogenic shock (CS) & severe left ventricular (LV) dysfunction 4 years post OHT. Maintenance immunosuppressants (IS) included tacrolimus, mycophenolate & prednisolone with good adherence. Urgent endomyocardial biopsy (EMB), coronary angiogram & right heart catheterisation were performed. Progression to SCAI Stage D shock necessitated venoarterial extracorporeal membrane oxygenation (VA ECMO) within 24 hrs. Based on clinical presentation, the patient was treated with methylprednisolone, intravenous immunoglobulin (IVIG) & IV thymoglobulin alongside hemodynamic & other organ support (dialysis, ventilation). EMB confirmed pAMR3 fulfilling criteria for severe AMR together with CS & raised DSAs. Within days the patient's cardiac output improved allowing removal of VA ECMO. Following MDT consensus given persistent graft dysfunction & DSAs an AMR specific regimen of plasmapheresis, IVIG & rituximab were administered resulting in significant improvement in LV function & DSAs. A serious consequence of high dose IS was severe CMV reactivation (CMV peak 18,000 copies/ml). Successful treatment with IV ganciclovir caused leukopenia requiring mycophenolate cessation. Fortunately our patient survived to discharge day 152 post admission with graft recovery & excellent functional capacity. Ongoing management remains a challenge due to severe CAV & balancing risks associated with high dose IS with AMR recurrence. <h3>Summary</h3> This case highlights the importance of urgent evaluation including EMB, hemodynamic support & targeted anti-rejection therapy in patients post OHT with severe allograft dysfunction. It exemplifies challenges when treating severe AMR illustrating the importance of a collaborative team approach for successful outcomes.

Guillain-Barr\xe9 syndrome as the first presentation of human immunodeficiency virus infection

AimAntiretroviral therapy (ART) development has reduced the severity of neurological complications of the human immunodeficiency virus (HIV), but they remain prevalent and need prompt recognition. Acute inflammatory demyelinating polyneuropathy (AIDP) is a rare complication of human immunodeficiency virus (HIV) infection that may appear at any stage of the disease. In this case, AIDP represents a late presentation of HIV infection.MethodsDescriptive study. Patient data were collected from their medical records and by health assessment interviews.ResultsWe report a case of a 52-year-old male with acute lower limb weakness. Given the suggestive clinical presentation of AIDP and a positive HIV test, intravenous immunoglobulin (IVIG) was administered along with antiretroviral therapy. Progressive weakness to the upper limbs, autonomic dysfunction, and pain was observed. The second regimen of IVIG plus corticosteroids was administered. Muscle strength improved after three weeks.ConclusionsScreening for HIV in a patient with AIDP may provide a better outcome because of the early start of ART with good central nervous system penetration in HIV-infected patients.

Randomized trial of different initial intravenous immunoglobulin regimens in Kawasaki disease.

BackgroundWe aimed to assess the efficacy of different initial intravenous immunoglobulin (IVIG) regimens in Kawasaki disease (KD) patients to find more cost‐effective therapy options.MethodsA multicenter, open‐label, blind‐endpoint randomized controlled trial was conducted from January 2014 to December 2015. Patients with KD, within 10 days of illness, were randomly assigned to receive different IVIG regimens (Group A, 2 g/kg once; Group B, 1 g/kg for 2 consecutive days; Group C, 1 g/kg once) and aspirin 30mg/kg/d. Primary outcomes included hours to defervescence and development of coronary artery lesions during the study period. Major secondary outcomes included total fever days, total dose of IVIG, changes of laboratory data, length of stay, and hospitalization expenses. (ClinicalTrials.gov: NCT02439996).ResultsA total of 404 patients underwent randomization. No difference was found in the outcomes of defervescence among three groups at 6, 12, 24, and 36 hours after completion of initial IVIG infusion. There were no differences in the incidence of coronary artery lesions during the study period (at week 2, month 1, month 3, and month 6 of illness), changes of laboratory data, total fever days, and length of stay. Group C patients had the lowest total dose of IVIG (mean: 1.2 vs 2.2 vs 2.1 g/kg; P < 0.001) and hospitalization expenses (mean: 8443.8 vs 10798.4 vs 11011.4 Chinese Yuan; P < 0.001) than other two groups.ConclusionsA single dose of 1g/kg IVIG is a low‐cost treatment with the same efficacy as 2 g/kg IVIG and can be an option for the initial therapy of KD patients.

High-dose polyclonal intravenous immunoglobulin therapy for refractory viral infections including viremia after allogeneic hematopoietic cell transplantation

Refractory viremia/viral disease is a major life-threatening complication that may arise among patients undergoing allogeneic hematopoietic cell transplantation (allo-HCT). This study aimed to clarify the therapeutic effect of high-dose polyclonal intravenous immunoglobulin (IVIG) against viremia/viral diseases after allo-HCT. We conducted a pilot study to investigate the therapeutic effect of 400 mg/kg of IVIG given for 5 consecutive days against refractory viremia/viral disease after allo-HCT. Overall, 7 patients were drug-resistant and the other 7 had not previously received any drug for their viremia/viral disease. All patients completed the 5-day therapy regimen of IVIG. A complete response at Day 56 was observed for 8 of 14 patients (57.1%). Additionally, 10 of 14 patients (71.4%) were alive at Day 56, although only one death occurred due to the viremia/viral disease. Remarkably, all 3 cases who developed exogenous viremia/viral diseases including respiratory syncytial virus pneumonia/bronchitis and human parvovirus B19 viremia achieved a complete response, suggesting that high-dose polyclonal IVIG may be more effective against exogenous viruses rather than endogenous ones. Congestive heart failure was observed in 1 patient. High-dose polyclonal IVIG could be an effective and feasible therapy for refractory viremia/viral disease after allo-HCT.

Retrospective analysis of a single-center clinical experience toward development of curative treatment of 123 pemphigus patients with a long-term follow-up: efficacy and safety of the multidrug protocol combining intravenous immunoglobulin with the cytotoxic immunosuppressor and mitochondrion-protecting drugs.

Pemphigus vulgaris (PV) is a life-long IgG autoantibody-mediated blistering disease affecting the mucosal surfaces lined by the stratified epithelium (oral, nasal, genital) and sometimes also the skin. While corticosteroid treatment is life saving, the high dose and prolonged courses required for disease control are associated with significant adverse effects, including death. Although introduction of rituximab (RTX) provided for a favorable outcome, the high relapse rate, that is, up to 80%, precludes successful use of RTX as a monotherapy. Intravenous immunoglobulin (IVIg) is being increasingly utilized as off-label therapy for a variety of autoimmune and inflammatory diseases, including PV and pemphigus foliaceus (PF). The goal of pemphigus research is to develop an effective treatment modality that would allow patients to achieve and maintain a stable clinical remission without the need for additional treatments, or cure. This article summarizes clinical outcome of 123 pemphigus patients treated with a combination of IVIg, an immunosuppressive cytotoxic drug (ICD) and mitochondrion-protecting drugs in the Blistering Disease Clinic at the University of California, Irvine from 2007 to 2017. The mean time to disease control was 0.2 months and time to complete remission - 1.7 months. Duration of complete remission on drugs until relapse or end of treatment was 19.3 months. The mean duration of complete remission off drugs until relapse was 15.8 months. That until end of follow up was 48.4 months, with a minimum of 14 and a maximum of 91 months. The overall complete remission rate off all drugs was 100%, with 12% overall relapse rate. Most relapses, 8.1 vs. 3.3%, occurred during the time of treatment, compared to posttreatment. No patients had more than a single relapse. The duration of the posttreatment follow-up ranged from 9 to 97 months with a mean of 64.8 months, or 5.4 years. The total number of IVIg cycles ranged from 26 in patients without a relapse to 37 in patients with a relapse. The clinical outcome in patients that received IVIg with RTX or another ICD were found to be very similar. Thus, the multidrug IVIg regimen allowed to achieve three principal treatment objectives: (i) rapid control of pemphigus symptoms; (ii) stable disease remission; and (iii) overall safety of treatment. While the individualized therapeutic approaches to eradicate the autoreactive B cell clones causing disease in each particular PV or PF patient are being developed, all pemphigus patients can benefit from the treatment protocol described in this study.

Maintenance IV immunoglobulin treatment in chronic inflammatory demyelinating polyradiculoneuropathy.

Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) patients treated with intravenous immunoglobulin (IVIg) usually start with a standard dosage of 2 g/kg bodyweight. Only a minority of patients has a sustained improvement, and most require ongoing maintenance treatment. Preferred IVIg regimens, however, vary considerably between doctors and at present it is unknown which is optimal. As there are also large differences in IVIg dosage and interval requirements between patients, optimal IVIg maintenance treatment of CIDP is even more complex. The lack of evidence-based guidelines on how IVIg maintenance treatment should be administered may potentially lead to under- or overtreatment of this expensive therapy. We provide an overview of published practical IVIg maintenance treatment regimens, IVIg maintenance schedules used in randomized controlled trials and one based upon our own long-term experience on how this treatment could be given in CIDP.

Chronic persistent parvovirus B19 bone marrow infection resulting in transfusion-dependent pure red cell aplasia in multiple myeloma after allogeneic haematopoietic stem cell transplantation and severe graft versus host disease

Introduction: We report a chronic persistent Parvovirus B19 (PVB19) infection despite long-term immunoglobulin substitution intravenous immunoglobulin (IVIG) and tapering of immune-suppressive therapy in a 41-year-old patient after allogeneic haematopoietic stem cell transplantation (alloHSCT) and long-term immune-suppressive therapy due to a steroid-refractory graft versus host disease (GvHD).Clinical course: More than 18 month after alloHSCT the patient acquired a de novo transfusion-dependent pure red cell aplasia (PRCA) due to a PVB19 infection. Despite prompt tapering of GvHD-directed therapy and application of various IVIG regimens, transfusion-dependent anaemia (fourerythrocyte concentrates a month) persisted, and a high PVB19 replication is still evident for more than 3.5 years. Virological analysis at different time points showed a very high PVB19 load in the blood (range: 6.79E9–1.56E11), as well as highly elevated PVB19-IgG (range: 1.95–3.34) and -IgM (range: 1.97–9.74) levels in serology testing. Other virological parameters were not significantly elevated. After 30 months, a bone marrow (BM) examination still revealed a highly dysplastic erythropoiesis without any cellular maturation, and a high-grade expression of PVB19 within the dysplastic erythropoietic progenitor cells, consistent with a PRCA due to a PVB19 infection of the BM. We suggest that PRCA was most probably caused by a primary PVB19 infection of unknown source following alloHSCT with a PVB19-negative donor.Conclusion: PRCA due a PVB19 infection of the BM may persist over a long-time, despite prolonged administration of various IVIG regimen and tapering of GvHD-directed therapy. The case emphasizes the importance of PVB19 monitoring in heavily pre-treated haematological patients. Currently, PVB19-directed treatment options are extremely limited and optimized therapeutic strategies are urgently needed.

Lower Dosages of Intravenous Immunoglobulin (IVIg) in Treating Immune Thrombocytopenia with Long-Term Follow-up of Three Years

▪This study compared the effects of different dosages of intravenous immunoglobulin (IVIg) against immune thrombocytopenia. A total of 167 patients, 91 adults and 76 children, with ITP, followed-up for 3 years in the case-control study, were each divided into three subgroups according to the dosages of IVIg administered: group A (0.2g/kg/day), group B (0.3g/kg/day), group C (0.4g/kg/day). The therapeutic response in 91 adult patients did not differ significantly among the three groups of IVIg dosages (P=0.459). The response rate of IVIg treatment in the three adult groups was 97.1% for group A, and 97.2% for group B, 100% for group C. The mean time for raising platelets to 30 ×109/L in group A was 2.5 days, group B 3.2 days, group C 2.9 days (P=0.324). The median IVIg consumption in group A was 0.83 g/kg, group B 1.22 g/kg, and group C 1.64 g/kg (P<0.01). Similar results were shown in the children groups. The follow-up results showed no significant difference of clinical outcome between groups A, B and C. In conclusion, low-dose IVIg treatment is shown to be as effective as high-dose regimen without increasing the risk of developing the patients into chronic ITP conditions, suggesting that ITP patients could be treated more cost-effectively by lower than conventional dosage of IVIg regimen. DisclosuresNo relevant conflicts of interest to declare.

Different dosages of intravenous immunoglobulin (IVIg) in treating immune thrombocytopenia with long-term follow-up of three years: Results of a prospective study including 167 cases

This study compared the effects of different dosages of intravenous immunoglobulin (IVIg) against immune thrombocytopenia (ITP). A total of 167 patients, 91 adults and 76 children, with ITP, followed-up for three years in the case-control study, were divided into three subgroups according to the dosages of IVIg administered: group A (0.2 g/kg/day), group B (0.3 g/kg/day) and group C (0.4 g/kg/day). The therapeutic response in 91 adult patients did not differ significantly among the three groups of IVIg dosages (p = 0.459). The response rate of IVIg treatment in the three adult groups was 97.1% for group A, 97.2% for group B and 100% for group C. The mean time for raising platelets to 30 × 109/L in group A was 2.5 days, group B 3.2 days and group C 2.9 days (p = 0.324). The median IVIg consumption in group A was 0.83 g/kg, group B 1.22 g/kg and group C 1.64 g/kg (p < 0.01). Similar results were shown in the children groups. The follow-up results showed no significant difference of clinical outcome between groups A, B and C. In conclusion, low-dose IVIg treatment is shown to be as effective as high-dose regimen without increasing the risk of developing the patients into chronic ITP conditions, suggesting that ITP patients could be treated more cost-effectively by lower conventional dosage of IVIg regimen.

Immunoglobulin G Fc N-glycosylation in Guillain-Barré syndrome treated with intravenous immunoglobulin.

Immunoglobulin G Fc N-glycosylation in Guillain-Barré syndrome treated with intravenous immunoglobulin.

Efficacy of immunoglobulin plus prednisolone for prevention of coronary artery abnormalities in severe Kawasaki disease (RAISE study): a randomised, open-label, blinded-endpoints trial

Evidence indicates that corticosteroid therapy might be beneficial for the primary treatment of severe Kawasaki disease. We assessed whether addition of prednisolone to intravenous immunoglobulin with aspirin would reduce the incidence of coronary artery abnormalities in patients with severe Kawasaki disease. We did a multicentre, prospective, randomised, open-label, blinded-endpoints trial at 74 hospitals in Japan between Sept 29, 2008, and Dec 2, 2010. Patients with severe Kawasaki disease were randomly assigned by a minimisation method to receive either intravenous immunoglobulin (2 g/kg for 24 h and aspirin 30 mg/kg per day) or intravenous immunoglobulin plus prednisolone (the same intravenous immunoglobulin regimen as the intravenous immunoglobulin group plus prednisolone 2 mg/kg per day given over 15 days after concentrations of C-reactive protein normalised). Patients and treating physicians were unmasked to group allocation. The primary endpoint was incidence of coronary artery abnormalities during the study period. Analysis was by intention to treat. This trial is registered with the University Hospital Medical Information Network clinical trials registry, number UMIN000000940. We randomly assigned 125 patients to the intravenous immunoglobulin plus prednisolone group and 123 to the intravenous immunoglobulin group. Incidence of coronary artery abnormalities was significantly lower in the intravenous immunoglobulin plus prednisolone group than in the intravenous immunoglobulin group during the study period (four patients [3%] vs 28 patients [23%]; risk difference 0·20, 95% CI 0·12-0·28, p<0·0001). Serious adverse events were similar between both groups: two patients had high total cholesterol and one neutropenia in the intravenous immunoglobulin plus prednisolone group, and one had high total cholesterol and another non-occlusive thrombus in the intravenous immunoglobulin group. Addition of prednisolone to the standard regimen of intravenous immunoglobulin improves coronary artery outcomes in patients with severe Kawasaki disease in Japan. Further study of intensified primary treatment for this disease in a mixed ethnic population is warranted. Japanese Ministry of Health, Labour and Welfare.

An Evaluation of the Impact of a Single-Dose Intravenous Immunoglobulin Regimen in the Treatment of Clostridium difficile Infections

An Evaluation of the Impact of a Single-Dose Intravenous Immunoglobulin Regimen in the Treatment of <i>Clostridium difficile</i> Infections