IVIG Treatment Research Articles

Cryptogenic cirrhosis and Common variable immunodeficiency: an unrecognized relationship

Objective: This study investigates the prevalence of Common Variable Immunodeficiency (CVID) in patients with cryptogenic cirrhosis. It aims to highlight CVID's role in liver involvement and raise awareness of its potential as an underlying cause of cryptogenic cirrhosis. Methods: This retrospective cohort study, approved by the ethics committee, included patients diagnosed with cryptogenic cirrhosis at a university hospital. Comprehensive evaluations were performed to exclude other causes of cirrhosis, and patients were screened for CVID based on the European Society for Immunodeficiencies (ESID) criteria. Results: This study included 30 patients with cryptogenic cirrhosis, among whom 6.7% (n=2) were diagnosed with CVID. Both CVID patients had low immunoglobulin levels and abnormal immune cell profiles, leading to recurrent infections in one case and suspicion due to low total protein levels in the other. IVIG treatment was initiated for both, and liver biopsy findings in one patient suggested CVID-related liver involvement. Conclusions: Determining the underlying causes of chronic liver diseases is crucial for guiding treatment and follow-up, potentially preventing cirrhosis progression and influencing liver transplantation eligibility. CVID screening in patients with cryptogenic cirrhosis should be considered, with appropriate treatments initiated as needed.

Chronic inflammatory demyelinating polyneuropathy associated with podocytopathy: a clinical case with literature review

Chronic imflammatory demyelinating polyneuropathy (CIPD) is a heterogenous group of disimmune polyneuropathies, characterised by progressive or relapsing-remitting disease course, with electrophysiologically and pathomorphologically detected peripheral nerve demyelination. Immunotherapy is considered reasonable in CIDP. CIDP associated with antibodies against nodo-paranodal proteins, namely, NF155, CNTN1, Contactin associated protein has been described amongst the rare atypical CIDP subtypes. The detection of antibodies against nodo-paranodal proteins might have a predictive value for a more severe disease course with resistance to corticosteroid and/or IVIG treatment. Herein we report a case of severe relapsing-remitting CIDP, characterized by respiratory muscle weakness requiring assisted ventilation, with a previous history of proteinuria, later also diagnosed with Minimal change disease (MCD).

The long-term risk of immune-related conditions in survivors of diffuse large B-cell lymphoma: A Danish nationwide registry study.

There is limited knowledge of the long-term effects on the immune system after treatment for diffuse large B-cell lymphoma (DLBCL). This study included DLBCL patients from the Danish Lymphoma Registry who obtained complete remission (CR) after (R)-CHOP (cyclophosphamide, doxorubicin, vincristine, prednisolone)-like immunochemotherapy. Each R+ CHOP-like treated patient was matched to five comparators from the Danish background population and furthermore compared to R- CHOP-like treated patients. Incidence rate ratios (IRRs) and risk differences (RDs) were calculated for a wide range of infections, autoimmune conditions, and immune deficiencies (AC-IDs) combined and by subtypes. R+ CHOP-like treated patients had a higher risk of infections overall (IRR 1.5, 95% confidence interval [CI] 1.4-1.7: 10-year RD 5.0%, 95% CI 2.2%-7.8%) and for a majority of the subtypes than matched comparators. Likewise, they had a higher risk of AC-IDs overall (IRR 1.4, 95% CI 1.1-1.7; RD 0.8%, 95% CI 0.7%-2.2%) than matched comparators, however only of clinical relevance for three subtypes; autoimmune diseases of the endocrine system, sarcoidosis and immune deficiencies. The addition of rituximab to CHOP-like therapy did not alter the incidence rates (IR) of infections overall (IRR 1.1, 95% CI 0.9-1.3) or AC-IDs overall (IRR 0.8, 95% CI 0.5-1.3) compared to CHOP-like therapy alone, although the IR for respiratory infections was significantly elevated (IRR 1.5, 95% CI 1.1-2.1). However, an increased use of IVIG treatment was observed among R+ CHOP survivors. R-CHOP-like treated patients face an increased risk of infections and AC-IDs overall compared with the background population. The risk of infections and AC-IDs did not change overall after the addition of rituximab to CHOP, however, an increased risk of respiratory infections is notable. These findings could highlight the need for expanded vigilance and prophylaxis strategies.

Beneficial effects of IVIG treatment on experimental-induced osteoporosis

Beneficial effects of IVIG treatment on experimental-induced osteoporosis

Intravenous immunoglobulin therapy: usage patterns and response to treatment in Qatar over ten years.

IVIg is a blood-derived antibody product initially designed as a replacement therapy in inborn errors of immunity (IEIs). However, over the last 50 years, IVIg has been used to treat a growing range of autoimmune, autoinflammatory, and secondary immunodeficiency disorders. The US FDA has licensed IVIg for use in the treatment of nine clinical indications; although, IVIg global usage extends to off-label indications with variable treatment responses. Data from Qatar on the use of IVIg is scarce; thus, hampering the formulation of local policies. This study aimed to examine the utilization patterns, clinical indications, and safety profile of IVIg usage in Qatar; a nation with a predominantly young population, and to investigate the response rates to short- and long-term IVIg treatment, as well as explore associations between age at first IVIg dose, clinical indication, and treatment response. A retrospective chart review was conducted of patients who received IVIg between March 2009, and March 2019, in Hamad General Hospital, Qatar. Demographics, immediate adverse effects of IVIg, and treatment response were collected. IVIg clinical indications were categorized into FDA- and/or EMA-approved, those supported by international guidelines; those approved as second-line therapy, and those with low or no supportive evidence. IVIg was used for 63 indications during the 10-years. The age of patients skewed towards a younger demographic (median (IQR) 24 (44-6) years); however, no significant differences in response to short- and long-term treatment between age groups were observed. Of the 841 patients, 62% received IVIg in concordance with international recommendations, while 14% bestowed the treatment for indications with low or no supportive evidence. Immediate IVIg adverse effects were documented in 4% of patients in all of the infusions received, with headaches being the most prevalent (1.8%). Variable treatment responses were observed, with the highest recovery reported in immune thrombocytopenic purpura (35%), followed by transverse myelitis (28%). This study provided crucial insights into IVIg utilization, safety, and treatment outcomes in Qatar's young population. Despite variability in treatment responses and off-label use, adherence to international recommendations remained eminent. Further research is warranted to inform local guidelines and optimize IVIg therapy outcomes.

Utilizing T-Lymphocyte Activation-Related Cytokines to Predict Non-Responsiveness to Treatment in Pediatric Kawasaki Disease.

To investigate the predictive value of T-lymphocyte activation-related cytokines in non-responsive Kawasaki disease. Eighty-two children with Kawasaki disease, hospitalized from June 2022 to December 2023, were divided into two groups based on treatment response: the sensitive Kawasaki disease group (n=71) and the non-responsive Kawasaki disease group (n=11). Serum levels of T-lymph activation-related cytokines, including interleukin-2, 6, 7, 12, 15, 17, and tumor necrosis factor alpha, were measured before and after IVIG treatment in both groups. The differences in cytokine levels between the two groups were compared pre- and post-treatment. The ability of these cytokines to discriminate non-responsive Kawasaki disease was evaluated using ROC curves to determine the cut-off value. Before initial treatment, IL-2, IL-6, IL-7, IL-12, IL-15, IL-17, and tumor necrosis factor-α values were significantly higher in the non-responsive Kawasaki disease group compared to the sensitive Kawasaki disease group. Comparisons before and after initial treatment showed significant decreases in IL-6 and 17 in the sensitive Kawasaki disease group and significant decreases in IL-6 and 7 in the non-responsive Kawasaki disease group. IL-6 and 17 significantly increased in the sensitive group compared to the non-responsive group after initial treatment. The ROC curves indicated that IL-6 predicted the area under the curve (AUC) for non-responsive Kawasaki disease to be 0.859 before treatment and 0.920 after treatment. Similarly, IL-17had AUC values of 0.699 before treatment and 0.884 after treatment. Reassessing IL-6 and IL-17 following the initial treatment for Kawasaki disease may improve early warning signals for unresponsive Kawasaki disease.

Role of leukocyte-associated Ig-like receptor-1 in the pathogenesis of Kawasaki disease and coronary artery aneurysms

ObjectiveTo elucidate the relationship between leukocyte-associated Ig-like receptor-1 (LAIR-1) and the pathogenesis of Kawasaki disease (KD) and coronary artery aneurysms(CAA). MethodsThe study cohort comprises children who were diagnosed with KD and were categorized into two groups: KD patients with CAA (KD-CAA) and KD without CAA (KD-NCAA), with healthy children serving as control group (HC). LAIR-1 on leukocytes was examined via flow cytometry, while serum LAIR-1 (sLAIR-1) was quantified using ELISA. IL-1β, IL-2, IL-4, IL-5, IL-6, IL-8, IL-10, IL-12p70, IL-17A, IFN-α, IFN-γ and TNF-α were examined by Immunofluorescence assay. ResultssLAIR-1 levels were elevated in the KD and KD-CAA groups compared with those in the HC and KD-NCAA groups (P < 0.05). sLAIR-1 exhibited an area under the curve value of 0.858 for predicting KD (P < 0.001) and 0.628 for predicting CAA (P = 0.055, borderline significance). LAIR-1 was increased on the neutrophils in KD group, whereas it was lower in KD-CAA than that in KD-NCAA, and decreased in KD after IVIG treatment. In contrast, LAIR-1 was reduced on CD4+ and CD8+ T lymphocyte in KD group, and increased in KD after IVIG treatment (all P < 0.05). LAIR-1 on neutrophils showed a positive correlation with IL-5, while on CD4+ T cells, it was negatively correlated with IL-2, IL-6, IL-10, IFN-γ, and IL-8. On CD8+ T cells, LAIR-1 was negatively correlated with IL-2 and IFN-γ. ConclusionsLAIR-1 may serve as a potential biomarker for KD and CAAs, while LAIR-1 might be implicated in KD pathogenesis and CAA.

Severity of Acute Kawasaki Disease Can Be Predicted by Evaluating the Body Temperature at the Completion of an Initial Immunoglobulin Treatment.

Objective: We aimed to determine whether the severity of acute Kawasaki disease (KD) can be predicted based on whether a patient remains febrile or becomes afebrile immediately after the completion of initial immunoglobulin treatment (IVIG). Methods: This retrospective cohort study at a single institution involved 306 patients with KD. They were categorized into four groups according to their fever status at two specific time points (end of the initial IVIG treatment and 24-36 h later): Group F-F, patients who remained febrile at both time points; Group F-AF, patients who were febrile at the end of the initial IVIG treatment but became afebrile 24-36 h later; Group AF-F, patients who were afebrile at the end of the initial IVIG treatment but became febrile 24-36 h later; and Group AF-AF, patients who remained afebrile at both time points. The clinical characteristics of the groups were compared. Results: Group F-F (n = 38) showed a significantly higher incidence of CAAs compared to Group AF-F (n = 37), 55.3% vs. 0.0% (p < 0.0001), although both groups were classified as resistant to the initial IVIG. Conclusions: In Japanese patients with acute KD, the presence or absence of fever at the completion of initial IVIG treatment may serve as an early predictor of the occurrence of CAAs. An earlier secondary treatment may be warranted for patients who are in a febrile state immediately after the completion of the initial IVIG treatment. Future research should include a prospective cohort study with a larger number of KD cases across multiple institutions to analyze the effects of other contributing factors related to CAL formation.

Abstract 4136984: Low Occurence of Fetal Extranodal Findings and AV Interval Prolongation in Prospectively Followed High Titer Anti-Ro Pregnancies

. Background: Third-degree atrioventricular block (AVB) is the most common manifestation of Anti-Ro antibody associated fetal cardiac disease. Extranodal findings of isolated cardiac echogenicity, valvulitis with insufficiency and AV interval prolongation have been reported but not described in large prospective series. Aims: To report the occurrence and outcomes of extranodal findings in subjects followed prospectively since 2020 in STOP BLOQ (Surveillance and Treatment to Prevent Fetal AV block Likely to Occur Quickly) and the Registry for Neonatal Lupus (RNL). Methods: We reviewed fetal echo reports from pregnant STOP BLOQ and RNL Anti-Ro antibody subjects. Pregnancies with high (&gt;1000 EU for anti-Ro52 or 60) antibody titers measured in a core research lab underwent surveillance with home fetal heart rate monitoring (FHRM) 3x/day and weekly or bi-weekly fetal echos from 17- 26 gestational weeks. Low titer subjects underwent echo or no surveillance based on local site protocol. We evaluated cardiac function, effusions, cardiac echogenicity and its location, any tricuspid (TV) or mitral (MV) insufficiency trivial or &gt;trivial) and AV conduction times (&lt;150, 150-170 or &gt;170 ms) Results: Echo reports were available in 622 prospectively followed pregnancies (376 high (40/376 with a previously affected child) and 246 low-titer pregnancies. All had subjectively normal ventricular function and none had effusions. Seven fetuses, 2 low and 5 high titer at 19 (range 16.7-21.7) weeks demonstrated cardiac echogenicity (n=6), AV or semilunar valve insufficiency (n=2) or AV interval 150-170 ms (n=2) (Table). Subjects 1 and 2, both high titer and treated with prophylactic Plaquenil (400 mg/d before 10 gestational weeks), had prior fetal AVB. Subject #1 had normal AV conduction but MV and TV echogenicity and moderate insufficiency of both valves which resolved in days after IVIG and dexamethasone (dex) treatment, but 3° AVB developed at 19 weeks after dex wean. Subjects 2-7 received no treatment and extranodal findings did not progress to cardiac dysfunction or AVB. During the same time period, 10 high titer subjects developed 2° or 3° AVB. Conclusion: Anti-Ro associated extranodal findings are rare, occurring in 1% of pregnancies overall and in 5% of pregnancies with a previously affected child. Unlike AVB, extranodal findings can occur in low titer pregnancies. The role of treatment for isolated extranodal findings in the absence of cardiac dysfunction is unclear.

Identifying Hemolytic Disease of the Fetus and Newborn within a Large Integrated Health Care System.

This study aims to identify hemolytic disease of the fetus and newborn (HDFN) pregnancies using electronic health records (EHRs) from a large integrated health care system. A retrospective cohort study was performed among pregnant patients receiving obstetrical care at Kaiser Permanente Southern California health care system between January 1, 2008, and June 30, 2022. Using structured (diagnostic/procedural codes, medication, and laboratory records) and unstructured (clinical notes analyzed via natural language processing) data abstracted from EHRs, we extracted HDFN-specific "indicators" (maternal positive antibody test and abnormal antibody titer, maternal/infant HDFN diagnosis and blood transfusion, hydrops fetalis, infant intravenous immunoglobulin [IVIG] treatment, jaundice/phototherapy, and first administrated Rho[D] Immune Globulin) to identify potential HDFN pregnancies. Chart reviews and adjudication were then performed on select combinations of indicators for case ascertainment. HDFN due to ABO alloimmunization alone was excluded. The HDFN frequency and proportion of each combination were fully analyzed. Among the 464,711 eligible pregnancies, a total of 136 pregnancies were confirmed as HDFN pregnancies. The percentage of the HDFN-specific indicators ranged from 0.02% (infant IVIG treatment) to 34.53% (infant jaundice/phototherapy) among the eligible pregnancies, and 32.35% (infant IVIG treatment) to 100% (maternal positive antibody test) among the 136 confirmed HDFN pregnancies. Four combination groups of four indicators, four combination groups of five indicators, and the unique combination of six indicators showed 100% of HDFN pregnancies, while 80.88% of confirmed HDFN pregnancies had the indicator combination of maternal positive antibody test, maternal/infant HDFN diagnosis, and infant jaundice/phototherapy. We successfully identified HDFN pregnancies by leveraging a combination of medical indicators extracted from structured and unstructured data that may be used in future pharmacoepidemiologic studies. Traditional indicators (positive antibody test results, high titers, and clinical diagnosis codes) alone did not accurately identify HDFN pregnancies, highlighting an unmet need for improved practices in HDFN coding. · A case ascertainment method was developed to identify HDFN from structured and unstructured data.. · The method used in this study may be used in future pharmacoepidemiologic studies.. · The study highlighted an unmet need for improved practices in HDFN coding..

Isolated peripheral neuroleukemiosis mimicking Guillain‐Barré syndrome in an adolescent with relapsed B‐lymphoblastic leukemia

AbstractBackgroundNeuroleukemiosis, leukemic infiltration of the peripheral nervous system (PNS), is rare. Very few cases of isolated neuroleukemiosis, PNS leukemic infiltration without leukemia blasts in the blood or bone marrow, have been reported in pediatrics. Most cases have occurred in adults with acute myelogenous leukemia (AML) in remission who presented with peripheral neuropathy. We describe a pediatric patient presenting with isolated neuroleukemiosis mimicking Guillain‐Barré syndrome.Patient descriptionA 15‐year‐old boy presented 1 month after IVIG treatment for Guillain‐Barre syndrome with worsening ataxia and weakness. He had a past medical history of B‐cell acute lymphoblastic leukemia (B‐ALL) in remission for 8 years. Examination revealed distal greater than proximal weakness of the bilateral lower extremities, areflexia at the Achilles tendon, and 1+ patellar and upper extremity reflexes. Initial magnetic resonance imaging (MRI) of the brain and spine were normal, and lumbar puncture revealed increased protein with uninterpretable white count due to excessive red blood cells. Repeat MRI brain and spinal cord showed extensive enlargement of all nerve roots and enhancement of the cauda equina and portions of cranial nerve VII bilaterally. Repeat lumbar puncture with cytology revealed leukemic blast cells. Blasts were not detected in peripheral blood smear or by flow cytometry. Bone marrow biopsy was also free of blast cells, confirming the diagnosis of relapsed B‐ALL restricted to the nervous system.ConclusionsNeuroleukemiosis is a rare entity with typical clinical features of mono‐ or polyneuropathy. It most often occurs in adults in remission from AML. However, neuroleukemiosis should also be on the differential for pediatric patients in remission from ALL presenting with neuropathy.

Combination of low-dose, long-term immunoglobulin and mirtazapine is effective in progressive multifocal leukoencephalopathy caused by JC virus infection

BackgroundProgressive multifocal leukoencephalopathy (PML) is an often fatal disease of the central nervous system caused by opportunistic infection of John Cunningham Polyomavirus (JCV). There’s still no antiviral therapeutic strategy which was generally recognized as effective. The prognosis may differ in patients with different pathological mechanisms and treatments. We aim to report the effectiveness of combined treatment of low-dose, long-term immunoglobulin and mirtazapine in a pathologically proved PML case.Case presentationA patient presented with progressive acalculia, right-left confusion and visual neglection was recorded. She received 10-year immunosuppressive therapy for dermatomyositis. White matter lesions located in bilateral parietal lobe and callosum area symmetrically in MR scanning. JC virus analysis and brain biopsy in left parietal lobe were performed. The number of JCV copies was 2595 in CSF and 282,809 in brain specimen. Abundant foamy macrophages and the lymphatic cells were obvious in immunohistochemistry staining. Few SV-40 positive JC infected cell and more CD4 + and CD68 + cells were predominant. Immunosuppressive drugs were terminated after being diagnosed as PML for positive JCV and pathological characteristics. In addition, immunoglobulin (5 g/day) and mirtazapine (45 mg/day) were used. JC virus in CSF decreased to 0 after treatment for 4 months and was still negative in June 2023. The clinical symptoms improved, and white matter lesions recovered significantly.ConclusionsWe demonstrated that the combination treatment of IVIG and mirtazapine was effective in PML. Low-dose, long-term immunoglobulin might regulate the immune status in our case with controlled inflammatory reaction instead of destructive virus spreading. The therapy may be a prospective option for PML.

Paternal HLA-Derived Epitopes and Live Birth in SecondaryRecurrent Pregnancy Loss: New Insights FromaClinical Trial.

Recurrent pregnancy loss (RPL), defined as two or more pregnancy losses before the 24th week of gestation, affects 1%-3% of women worldwide. Approximately, 40% of RPL cases are secondary RPL (sRPL), where women have given birth before facing pregnancy losses. The underlying causes of RPL remain unclear, but immune-related factors may play a role. Previously, a randomised controlled trial using immunoglobulin (IVIG) in sRPL women with a history of four pregnancy losses performed in our RPL unit did not show significant effects of IVIG treatment overall. Yet, some evidence suggests potential benefits for a subset of sRPL patients. In the cohort used for the randomised controlled trial, we examined the role of maternal HLA class II-presented fetal HLA-derived epitopes in sRPL using the predicted indirectly recognisable HLA epitopes (PIRCHE-II) algorithm. In the placebo group, sRPL mothers with an anti-HLA antibody response had higher PIRCHE-II scores when having a live birth compared with sRPL women who experienced another pregnancy loss. This difference was not observed in the IVIG-treated group. Furthermore, as a proxy for T-cell memory, the number of overlapping peptides between the two paternal haplotypes in couples having live births without treatment displayed a larger number of overlapping peptides. This effect was primarily driven by class II-derived peptides. These results suggest that specific combinations of sRPL mothers and fathers, particularly those with an anti-HLA antibody response, may generate higher PIRCHE-II scores, which could contribute to successful live births. Understanding these immune interactions may provide insights for personalised diagnostic and therapeutic strategies in sRPL.

Health Care Utilisation in a Cohort of Patients with Primary and Secondary Antibody Deficiency in the United Kingdom

IntroductionThis study investigates the frequency of hospital attendances, emergency care attendances and geographical influences on service interaction in cohorts of patients with primary and secondary antibody deficiency, to inform future service planning and delivery.MethodsThe COVID-19 in Antibody Deficiency (COV-AD) study was a United Kingdom study that enrolled 525 participants between April 2021 and September 2022. Data on health care utilisation was extracted from a screening cohort of participants at one participating site (Birmingham, UK). Hospital attendance (i.e. all outpatient and inpatient care episodes, including hospital-based IVIG treatment) and emergency care attendance patterns were analysed. Geographical differences in travel times to hospitals and associated costs were considered for all participants at all recruiting sites.ResultsIndividuals with antibody deficiency had a median of 7 hospital attendances per year. A diagnosis of secondary antibody deficiency, and antibody deficiency severe enough to require treatment with immunoglobulin replacement were associated with an increased frequency of hospital attendance. 12.7% of the cohort attended the Emergency Department at least once in the preceding twelve months. Individuals with secondary antibody deficiency were at greater risk of requiring emergency care over the preceding one-year and five-year periods. Individuals receiving subcutaneous immunoglobulin lived further from their local immunology centre and were more likely to engage with the COV-AD research study remotely, via dried blood spots sampling.ConclusionThis study highlights the utilisation of emergency and secondary care usage amongst patient with immunodeficiency and may inform service adaptation and development to better accommodate patient needs and circumstances.

Rational use of immunoglobulins (IVIgs and SCIgs) in secondary antibody deficiencies.

Immunoglobulins for intravenous use (IVIgs) and subcutaneous use (SCIgs) can prevent recurrent and severe infections in patients with secondary antibody deficiencies that are frequently linked to haematological/oncological malignancies as well as other clinical conditions and their respective treatments. Even so, as IVIgs and SCIgs are costly and their supply is limited, their clinical use must be optimised. The aim of this position paper is to provide structured practical guidance on the optimal use of IVIgs and SCIgs in secondary antibody deficiencies, particularly in haematological and oncological practice. The authors agree that the occurrence of severe infections is a prerequisite for the use of IVIgs. Serum IgG levels in general as well as IgG subclass levels can be additional indicators of whether a patient could benefit from IVIgs. Responsiveness to vaccines can help to identify immunodeficiency. Patients with chronic lymphocytic leukaemia or multiple myeloma who are receiving respective treatment, especially B-cell depletion therapy, but also some patients with autoimmune diseases are prone to antibody deficiencies and need IVIgs. For the optimal use of IVIgs and to maximise their potential benefit, the indication must be individually assessed for each patient. As a primary treatment goal, the authors define a sufficient prophylaxis of severe infections, which can be supported by normalising IgG levels. If the initiated treatment is insufficient or linked to intolerable adverse reactions, switching the product within the class of IVIgs or changing to a different batch of the same product can be considered. Pausing treatment can also be considered if there are no infections, which happens more frequently in summer, but treatment needs to be resumed once infections return. These structured recommendations for IVIg treatment in patients with secondary antibody deficiency may provide guidance for clinical practice and therefore help to allocate IVIgs to those who will benefit the most, without overusing valuable resources.

Serological indication of chronic inflammatory demyelinating polyneuropathy as an extrahepatic manifestation of hepatitis E virus infection

Guillain–Barré syndrome and neuralgic amyotrophy have been associated with hepatitis E virus (HEV) genotype 3 infections, while myasthenia gravis (MG) has been associated with HEV genotype 4 infections. However, whether chronic inflammatory demyelinating polyneuropathy (CIDP) is associated with HEV infections has not been conclusively clarified yet. 102 CIDP patients, 102 age- and sex-matched blood donors, 61 peripheral neuropathy patients (non-CIDP patients), and 26 MG patients were tested for HEV and anti-HEV IgM and IgG. Sixty-five of the 102 (64%) CIDP patients tested positive for anti-HEV IgG and one (1%) for anti-HEV IgM. No other patient tested positive for ati-HEV IgM. In the subgroup of CIDP patients with initial diagnosis (without previous IVIG treatment), 30/54 (56%) tested positive for anti-HEV IgG. Anti-HEV rates were significantly lower in blood donors (28%), non-CIDP peripheral neuropathy patients (20%), and MG patients (12%). No subject tested positive for HEV viremia. CSF tested negative for in 61 CIDP patients (54 patients with primary diagnosis). The development of CIDP but not non-CIDP polyneuropathy may be triggered by HEV exposure in an HEV genotype 3 endemic region. The increased anti-HEV seroprevalence in CIDP patients is not a consequence of IVIG therapy.

IVIg Treatment Ameliorates Inflammation in Patients with Sleep-related Electrical Status Epilepticus (ESES) (P5-14.003)

IVIg Treatment Ameliorates Inflammation in Patients with Sleep-related Electrical Status Epilepticus (ESES) (P5-14.003)

Impact of aspirin dosage on prognosis and coronary artery complications in pediatric Kawasaki disease patients

Kawasaki disease is a common systemic vasculitis in children, and early treatment is critical to prevent coronary artery complications. This study aimed to evaluate the impact of different aspirin dosages on the prognosis of Kawasaki disease. A total of 161 children diagnosed with Kawasaki disease were enrolled and divided into four groups based on the dosage of aspirin: 30–34 mg/kg, 35–39 mg/kg, 40–44 mg/kg, and 45–49 mg/kg. Demographic characteristics, pre-treatment laboratory indicators, post-treatment laboratory indicators, the incidence of coronary artery complications, and the rate of IVIG resistance were compared across the groups. No significant differences were found in baseline characteristics such as age, gender, weight, IVIG treatment choice, IVIG treatment duration, or the proportion of patients diagnosed before the age of 1 year. Laboratory indicators before and after treatment were also similar between groups. However, at 3-4 weeks after the onset of illness, the incidence of coronary artery complications was significantly higher in the high-dose aspirin groups compared to the low-dose aspirin group. Additionally, the high-dose aspirin group had a longer duration of fever and a higher proportion of recurrent fever compared to the low-dose group. The results suggest that although aspirin dosage did not appear to have a clear impact on the overall prognosis of Kawasaki disease, high-dose aspirin administered in the 3-4 weeks following disease onset may increase the risk of coronary artery complications and is associated with a higher incidence of recurrent fever. Caution is therefore advised in selecting aspirin dosages to minimize unnecessary risks.

1092 Postponing early intrauterine transfusion with IVIG treatment in severe hemolytic disease: Individual patient data meta-analysis

1092 Postponing early intrauterine transfusion with IVIG treatment in severe hemolytic disease: Individual patient data meta-analysis

Cutaneous Crohn Disease Presenting as "Knife-Edged" Ulcers: A Case Report

Background. CCD presents as non-caseating granulomas within the skin at a site distant from the GI tract. CCD is a debilitating extraintestinal sequela of CD that can sometimes precede its GI manifestations. In the absence of GI symptoms, the histopathologic and clinical features of CCD can present as a variety of inflammatory skin conditions that can range from ruptured follicle-associated granulomas to cutaneous ulcerations. While a variety of therapeutic options for patients with CCD and concurrent luminal CD have been described in the literature, there is no standard treatment algorithm for the management of refractory CCD with limited or covert GI involvement. Case Report. The authors discuss the case of a 33-year-old female who presented to the wound care clinic with multiple "knife-edged" cutaneous ulcerations involving the intertriginous spaces, found to be consistent with CCD. Her original cutaneous symptoms and diagnosis manifested with minimal GI involvement and responded to IVIG treatment. Conclusions. This case supports the inclusion of CCD in the differential diagnosis in patients with knife-edged granulomatous skin lesions in intertriginous locations. This clinical condition may present in the setting of no or limited GI symptoms. The management of CCD and a proposed treatment algorithm are also presented.