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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.

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 Evidence suggests that seasonal variation in the onset of Kawasaki disease (KD) exists worldwide. Whether a seasonal component to successful i.v. immunoglobulin (IVIG) therapy exists in KD-positive children, however, is unknown. We addressed this question by focusing on patients with primary onset KD who were non-responsive to IVIG treatment, in the large nationwide Japanese KD survey datasets from 2009 to 2016. METHODS In these datasets, the IVIG therapy non-responders were defined as patients whose fever persisted ≥24 h or recurred ≤24 h after the end of the initial IVIG treatment (dosage, 2,000 mg/kg). Those who successfully responded to this treatment were defined as IVIG responders. The consecutive monthly trend of the proportion of IVIG non-responders was analyzed throughout the study period to investigate seasonal periodicity on Fourier analysis, and the monthly distributions of non-responders and responders were compared. RESULTS From a total of 113 691 KD-positive patients, 15.7% were IVIG non-responders, and 61% were male. The proportion of non-responders increased across each calendar year with fluctuation, and Fourier analysis indicated seasonal periodicity. The seasonality effect differed between responders and non-responders, with the proportion of responders tending to increase in autumn through winter, while the non-responders showed a decreasing trend in autumn. The seasonality effect tended to differ by sex. CONCLUSIONS The results indicate that the currently unknown etiological agents of KD might differ between IVIG responders and non-responders. In addition, immune reactivity against such agents possibly differs by sex in the IVIG non-responders.

Three weeks after single-lung transplantation for pulmonary fibrosis, a patient with high serum levels of de novo donor-specific antibodies received high-dose intravenous immunoglobulin (IVIG) infusion (scheduled dose: 2 g/kg on 2 days) to prevent antibody-mediated rejection. Within the first hours after completion of infusions, he experienced acute lung injury involving the transplanted lung. Given the clinical evolution and the absence of an alternative diagnosis, transfusion-related acute lung injury (TRALI) was diagnosed. The IVIG administered on each day was from the same batch. At day 110, because of an increase in the serum titers of donor-specific antibodies, IVIG therapy was reintroduced but from a different batch, with excellent clinical tolerance. The lung injury was explored biologically, but no mechanism was revealed. Given the increasing use of IVIG in solid-organ recipients, clinicians should be aware of possible TRALI after IVIG infusion.

Citation Winger EE, Reed JL, Ashoush S, El-Toukhy T, Ahuja S, Taranissi M. Elevated preconception CD56+16+ and/or Th1:Th2 levels predict benefit from IVIG therapy in subfertile women undergoing IVF. Am J Reprod Immunol 2011; 66: 394–403 Problem We sought to answer two questions: First, is there a group of patients who benefit from intravenous immunoglobulin (IVIG) in IVF? Second can this group of patients be identified by preconception blood testing? Method of study A total of 202 IVF cycles in subfertile women were divided into four groups. Group I: 62 cycles with preconception Th1:Th2 ratio and/or % CD56+ cell elevation using IVIG; Group II: 27 cycles with similar Th1:Th2 and/or % CD56+ cell elevation not using IVIG; Group III: 71 cycles with normal Th1:Th2 and/or % CD56+ cell levels using IVIG; Group IV: 42 cycles with normal Th1:Th2 and % CD56+ levels not using IVIG. These groups were similar with regard to patient age, diagnosis, and past failure history. Results The implantation rate (number of gestational sacs per embryo transferred, with an average of two embryos transferred per cycle) was 45% (55/123), 22% (12/54), 54% (75/139), and 48% (40/84) for Groups I–IV, respectively. The clinical pregnancy rate (fetal heart activity per IVF cycle started) was 61% (38/62), 26% (7/27), 69% (49/71), and 71% (30/42), respectively. The live birth rate was 58% (36/62), 22% (6/27), 61% (43/71), and 71% (30/42), respectively, and the live birth per embryo transferred was 40% (49/123), 13% (7/24), 43% (60/139), and 48% (40/84), respectively. There was a significant improvement in implantation, clinical pregnancy, live birth rate and live birth rate per embryo transferred for Group I versus Group II (P = 0.0032, 0.0021, 0.0017, and 0.0002, respectively) and for Group II versus Group IV (P = 0.0021, 0.0002

Intravenous application of immunoglobulins (IVIg) is an effective and usually well tolerated yet costly therapeutic regimen in chronic inflammatory demyelinating polyneuropathy (CIDP). We report two CIDP patients treated with subcutaneous infusion of immunoglobulins (SCIg) after IVIg therapy was shown to be effective. Application of SCIg was well tolerated, easy to manage, and led to stabilization of the disease course. SCIg may represent an effective new therapeutic option in CIDP and is associated with a cost reduction of at least 50% compared to IVIg therapy.

To compare the effects of intravenous immunoglobulin (IVIg) therapy and plasmapheresis for the IgG anti-GM1-positive subtype of Guillain-Barre syndrome (GBS), clinical and electrophysiological recoveries were analyzed in 24 patients treated with IVIg (n = 10) or plasmapheresis (n = 14). At entry, there were no significant differences between the two patient groups in age, sex, clinical severity (Hughes grade), sum scores of distally evoked amplitudes of compound muscle action potentials (CMAPs), and frequency of Campylobacter jejuni infection. The patients treated with IVIg had significantly lower Hughes grade scores 1, 3, and 6 months after onset (P = 0.03), and a higher probability to regain independent locomotion at 6 months [P(logrank) = 0.044]. In the IVIg group, markedly rapid recovery (improvement by two or more Hughes grade scores within 4 weeks) was more frequent (6 of 10 vs. 3 of 14, P = 0. 03), and delayed recovery (unable to walk independently at 6 months) was less frequent (0 of 10 vs. 4 of 14, P = 0.06). CMAP sum score at 6 months tended to be greater for the IVIg group (P = 0.07). For the IgG anti-GM1-positive subgroup of GBS patients, IVIg therapy may be a more efficacious treatment than plasmapheresis.

Clinical data about the course of long-term IVIG treatment in chronic inflammatory demyelinating polyneuropathy (CIDP) and in multifocal motor neuropathy (MMN) are sparse. Twenty patients with CIDP or MMN were investigated during up to 65 months of high-dose intravenous immunoglobulin (IVIG) therapy in an open and prospective study. In all therapy-responders (18/20), amelioration of symptoms was evident within the first 3 weeks of treatment. Dosage was reduced slowly according to the clinical symptoms. All 18 patients now show very few or no symptoms. The mean value of the Rankin disability scale changed from 2.5 to 1.1 during therapy. Ten patients are still regularly given IVIG. In eight patients no further therapy has been necessary for 7–63 months. Treatment could even be stopped in one patient after 46 months of therapy. No marked clinical side-effects were observed during 529 months of IVIG therapy. IVIG therapy is safe, and remains effective in the long-term treatment of CIDP and MMN.

Intravenous immunoglobulin (IVIG) is considered an efficacious but expensive treatment in dysimmune polyneuropathies. Nevertheless, to date, no studies have been conducted on the cost of this therapy. We retrospectively studied 21 patients affected by chronic inflammatory demyelinating polyradiculoneuropathy (idiopathic-CIDP and CIDP-MGUS), comparing the clinical response and costs of IVIG therapy with those of plasma exchange (PE). Patients were treated either with IVIG (400 mg/Kg/die for five days) or PE (6 exchanges in 3 weeks). Responders continued the same therapy, which was progressively tapered down to one IVIG infusion or one PE monthly. IVIG nonresponders received PE and vice versa. Patients were followed for a period ranging from 3 to 67 months (median: 29 months). The modified Rankin disability scale was used to determine functional impairment at baseline and during treatment. Eleven of 21 patients (52%) improved with the first choice of treatment. Of the remaining 10 patients, 3 benefited from the alternative treatment; 2 patients who did not respond to the alternative treatment were again treated with the first choice therapy and subsequently improved; 2 patients on IVIG abandoned the therapy. One nonresponder to IVIG is currently receiving PE. The overall response rate to the two therapies was similar. The mean cost per patient was 1,128,000 lire/session for PE therapy (excluding equipment and medical staff costs) and 1,204,000 lire/dose for IVIG therapy. However, the responders to PE required a longer treatment than responders to IVIG.