IVIG Infusion Research Articles

12392 Decoding LADA: A Closer Look At A Suspected Case Influenced By Prior IVIG Therapy

Abstract Disclosure: A. Gondhi: None. S. Antharam: None. S.S. Bantu: None. B. Abdulhadi: None. Introduction: LADA(Latent Autoimmune Diabetes of Adults) is defined as a new diagnosis of diabetes after the age of 35, presenting with features of T2DM but with the presence of T1DM associated autoantibodies. Correct diagnosis of LADA is important since prognosis and therapy differs from T2DM. We report an interesting case of transient elevation of Glutamic Acid decarboxylase (GAD-65) antibodies due to IVIG therapy leading to a misdiagnosis of LADA. Case presentation:A 46-year-old women with hypothyroidism was referred for management of LADA. A few months prior to the referral, she was hospitalized for acute bacterial meningitis and developed flaccid quadriparesis concerning for Guillain Barre syndrome (GBS). She received multiple IVIG infusions, steroids with resultant hyperglycemia requiring insulin and was transferred to a rehabilitation facility. During her stay in rehab, her initial dose of glargine 20 units twice daily was titrated down rapidly once steroids are tapered. Blood work revealed HbA1C 6.1%, blood glucose 116 mg/dL, C-peptide levels 4.1 ng/ml (0.9 -7 ng/ml), and GAD-65 antibodies >250 (N<5 IU/ml). She was diagnosed with LADA and discharged on 2 units of glargine. Her primary care physician advised to stay on the 2 units of glargine since her GAD-65 antibodies were significantly elevated and then referred to the endocrine clinic. Repeat bloodwork in our clinic revealed HbA1c of 5.8%, GAD-65 antibodies were repeated twice and were found to be undetectable <5. She was taken off insulin and has been doing well since. Discussion:IVIG is derived from plasma pooled from donors, used to treat a variety of diseases. Autoantibodies contained in IVIG may result in false positive serum tests if levels are checked soon after the infusion. This phenomenon has been documented in previous cases involving treponemal, H. pylori, hepatitis C as well as ANA antibodies. Increases in anti-GAD levels post-IVIG therapy have been previously reported. We saw a similar scenario in our patient, who was initially diagnosed with LADA and started on insulin due to an observed elevation in GAD 65 levels. However, this elevation was transient with negative repeat levels a few months later. Conclusion: Our case highlights the importance of recognizing that IVIG infusions can lead to the transfer of and to transient elevations in autoantibodies including GAD-65, which may lead to misdiagnosis and unnecessary therapies.

Identifying optimal serum 1,3-β-D-Glucan cut-off for diagnosing Pneumocystis Jirovecii Pneumonia in non-HIV patients in the intensive care unit

BackgroundSerum (1,3)-β-D-glucan (BDG) detection for diagnosis of Pneumocystis jirovecii pneumonia (PJP) in non-human immunodeficiency virus (HIV) immunocompromised patients lacks intensive care unit (ICU)-specific data. We aimed to assess its performance and determine the optimal cutoff for PJP in ICU population.MethodsThis retrospective study included critically ill non-HIV immunocompromised patients admitted to a medical ICU with suspected pneumonia, undergoing simultaneous microbiological testing for P. jirovecii on lower respiratory tract specimens and serum BDG. Confounders affecting BDG positivity were explored by multivariable logistic regression. Optimal cut-offs were derived from Youden’s index for the entire cohort and subgroups stratified by confounders. Diagnostic performance of serum BDG was estimated at different cutoffs.ResultsOf 400 patients included, 42% were diagnosed with PJP and 58.3% had positive serum BDG. Serum BDG’s area under the receiver operating characteristic curve was 0.90 (0.87–0.93). At manufacturer’s 150 pg/ml cut-off, serum BDG had high sensitivity and negative predictive value (94%), but low specificity and positive predictive value (67%). Confounders associated with a positive serum BDG in PJP diagnosis included IVIG infusion within 3 days (odds ratio [OR] 9.24; 95% confidence interval [CI] 4.09–20.88, p < 0.001), other invasive fungal infections (OR 4.46; 95% CI 2.10–9.49, p < 0.001) and gram-negative bacteremia (OR 29.02; 95% CI 9.03–93.23, p < 0.001). The application of optimal BDG cut-off values determined by Youden’s index (252 pg/ml, 390 pg/ml, and 202 pg/ml) specific for all patients and subgroups with or without confounders improved the specificity (79%, 74%, and 88%) and corresponding PPV (75%, 65%, and 85%), while maintaining reasonable sensitivity and NPV.ConclusionsTailoring serum BDG cutoff specific to PJP and incorporating consideration of confounders could enhance serum BDG’s diagnostic performance in the ICU settings.

Positive Outcome in a Pregnancy with Anti-kell Alloimmunization Treated with Intravenous Immunglobulin and Therapeutic Plasma Exchange Despite Persistence of High Titre Antibody: A Case Report

Background: Few reports have been published about the current clinical management of anti-Kell alloimmunization in pregnancy; its low frequency of occurrence means that the few long series published have covered an extensive time period in which different treatment approaches have overlapped. The objective of the present paper is to present our experience in the clinical management of a pregnant woman who was positive for the anti-Kell antibody. Materials and Methods: The laboratory follow-up included the weekly measurement of the antibody titre, and identification of the paternal and fetal genotype. The clinical management included TPE and IVIG administration. Obstetric monitoring included ultrasonographic monitoring of the fetus. Case Description: We report a case of anti-Kell alloimmunization with high antibody titre at first observation. Testing for fetal DNA circulating in maternal blood confirmed positivity for KEL1 gene. The patient underwent fifteen sessions of TPE after 18 weeks of gestational age, followed by weekly IVIG infusion, which continued until 27 weeks of pregnancy. Anti-Kell titres were measured before and after each TPE session. The patient had no need for IUBT and delivered by cesarean section at 34 weeks of gestational age. Pregnancy resulted in a live birth with mild HDFN. Discussion: HDFN is a potentially lethal complication of alloimmunization, and IUBT is the standard treatment for severe fetal anemia. TPE and IVIG are two alternative treatment modalities described in the literature to avoid or postpone the need for IUBT transfusion. In the case reported, despite any substantial change in antibody titre, pregnancy resulted in a live birth. This result suggests that the use of TPE and IVIG in alloimmunization during pregnancy could be an effective treatment strategy.

Hypovitaminosis D and Leukocytosis to Predict Cardiovascular Abnormalities in Children with Kawasaki Disease: Insights from a Single-Center Retrospective Observational Cohort Study.

Introduction: An aberrant immune response involving yet unidentified environmental and genetic factors plays a crucial role in triggering Kawasaki disease (KD). Aims: The aim of this study was to assess general and laboratory data at the onset of KD in a single-center cohort of children managed between 2003 and 2023 and retrospectively evaluate any potential relationship with the development of KD-related cardiovascular abnormalities (CVAs). Patients and methods: We took into account a total of 65 consecutive children with KD (42 males, median age: 22 months, age range: 2-88 months) followed at the Department of Life Sciences and Public Health in our University; demographic data, clinical signs, and laboratory variables at disease onset, before IVIG infusion, including C-reactive protein, hemoglobin, white blood cell (WBC) count, neutrophil count, platelet count, aminotransferases, natremia, albumin, total bilirubin, and 25-hydroxyvitamin D were evaluated. Results: Twenty-one children (32.3% of the whole cohort) were found to have echocardiographic evidence of CVAs. Univariate analysis showed that diagnosis of KD at <1 year or >5 years was associated with CVAs (p = 0.001 and p = 0.01, respectively); patients with CVAs had a longer fever duration and mostly presented atypical or incomplete presentations. Interestingly, all patients with CVAs had lower levels of vitamin D (less than 30 mg/dL, p = 0.0001) and both higher WBC and higher neutrophil counts than those without CVAs (p = 0.0001 and p = 0.01, respectively). Moreover, blood levels of albumin were significantly lower in KD patients with CVAs compared to those without (11/21, 52% versus 13/44, 30%, p = 0.02). Multiple logistic regression with correction for sex showed that serum vitamin D < 30 ng/mL, WBC count > 20.000/mm3, and age > 60 months at KD onset were the only independent factors statistically associated with CVAs. Conclusions: Hypovitaminosis D, WBC count over 20.000/mm3, and age above 5 years at KD onset emerged as independent factors statistically associated with the occurrence of CVAs.

Atypical Kawasaki Disease

The child was dull-looking, irritable and dehydrated. She had tachycardia and tachypnoea but no chest findings. The cardiac exam was normal. Initial diagnosis of Acute Gastroenteritis with some dehydration was made. Relevant investigations were sent, which showed high CRP, hence, Inj. Ceftriaxone were added and dehydration was corrected along with supportive measures. In view of irritability and high CRP, Lumbar Puncture was advised, but the parents refused. wStool analysis was normal and negative for reducing sugar and rotavirus antigen. With the above measures, the child initially showed improvement in lab parameters. Blood culture reported sterile, but fever spikes persisted and again on day 6, increased CRP, with a persistent dull appearance; antibiotics were upgraded to Inj. Meropenem and Amikacin. After 48 hours of upgradation of antibiotics, fever and irritability were persistent. A Neurology opinion was taken, an MRI of the Brain and EEG and a CSF examination was done, which showed no sequelae of Meningoencephalitis. As fever spikes continued, autoimmune causes of fever were considered and inflammatory markers (LDH and Ferritin), within normal range, were sent. 2D ECHO (Figure-1) was done on day 8 of illness, which showed dilated coronaries suggestive of Kawasaki Disease Z scores LM- 5.5, LAD- 6 RCA-5.2. IVIG 2gm/kg over 24 hrs along with Aspirin as per the dose recommended by the Paediatric Cardiologist was given. As the Fever persisted, despite IVIG infusion at 48 hrs, repeat CBC and CRP were done, which showed reduced CRP and improved TLC. 2D ECHO was repeated on day 10 of illness, which showed a further increase in Z-score in dilatation of coronaries (Z scores- LM +6.46. LAD- +10, RCA-+9.1). So, a repeat dose of IVIG along with IV steroids and LMWH was given and the child was shifted to a dedicated cardiac monitoring centre under Paediatric Rheumatologist. Gradually, fever spikes settled on repeat 2D ECHO, Z-score was further increased LM- 5.28, LAD-11.98, RCA-9.2, so started on cyclosporine and steroid continued. On follow-up, a week later, a gradual decrease in LMCA 2.5, LAD- 3.2 and RCA 3.9 in coronary dilatation was seen.

Nystagmus and Imbalance as the Presenting Symptoms of AntiGad Antibody Syndrome in A 67-Year-Old Female

Autoimmune encephalitis is a serious condition that causes brain inflammation, usually mediated by antibodies to various neuronal antigens [1]. One of the many antigens that may generate an immune response and cause this type of inflammation is the glutamic acid decarboxylase enzyme (GAD). AntiGAD (glutamic acid decarboxylase) antibodies are targeted against the GAD enzyme that converts glutamate to GABA, which is the nervous system’s primary inhibitory neurotransmitter [2]. With the body’s primary inhibitory neurotransmitter compromised, anti-GAD antibody syndrome is a rare type of autoimmune encephalitis that results in various symptoms related to prolonged excitation, such as seizures or nystagmus [3]. We present a rare case of autoimmune encephalitis caused by significantly elevated Anti-GAD antibody, with concomitant rheumatoid arthritis in the hands/wrists; symptoms of downbeat nystagmus, diplopia, headaches, and joint pain are improved with rituximab and IVIG infusion, following an unsuccessful treatment with oral prednisone.

Guillain-barre syndrome: a paediatric case scenario in a tertiary care hospital at southern India

Background: During hospital ward rounds which was conducted as a part of Doctor of Pharmacy curriculum at Caritas Hospital, Thellakom, the first author got a chance to deal with a case of GBS. Case:12 year old female patient admitted with complaints of weakness on both legs; later diagnosed by the neurologist with demyelinating poly neuropathy and Guillain-Barre Syndrome. Aim: The aim of this particular study was to study more about Guillain-Barre Syndrome which is an auto immune disorder involving peripheral nervous system which usually occurring very rarely, affects 1.55 people per 100,000 in the world’s population, and is characterised by flaccid paralysis of limbs. It typically happens as a result of an earlier illness or other triggering events that excite the immune system of the body and result in the development of various antibodies. Materials and Methods: Analyse various case reports published in various scientific databases like PubMed, Medline, CrossRef etc. Results: This particular case was treated as follows: The treatment involves intravenous infusion of IVIg at a dose which varies according to the weight and severity of condition in each individual. Supplementation with Methyl cobalamin, Alpha lipoic acid, Levo-carnitine, and Vitamin E can improve the neural health and hasten the healing of nerve injuries. Conclusion: Data till now tells us that out of all GBS diagnosed population, 80% have recovered completely, 15% of patients have shown some neurological disabilities, and 5% have succumb to death. Here, the therapy was successful and the patient got discharged from hospital.

Successful Treatment of Acute Enteric Myocarditis with IVIG Infusion: An Unusual Complication of Enteric Fever.

Myocarditis, the inflammation of myocardium can be caused by a variety of etiological agents with clinical manifestations ranging from asymptomatic to cardiogenic shock. Enteric fever is a very common illness in children and can have a wide range of complications both enteric and non-enteric. The reported incidence of myocarditis in enteric fever ranges from about 1-5%. Paediatricians must be aware of the rare complications that might make the diagnosis challenging. We present one such case of a 6-year-old boy who came to us with a history of fever, diarrhoea and hypotension. On initial evaluation, the child was in cardiogenic shock and was started on IV antibiotics and inotropes. Laboratory evaluation confirmed enteric fever, and Electrocardiogram and echocardiography revealed myocarditis with dilated cardiomyopathy. The child did not improve with initial supportive care. Administration of IVIG resulted in a dramatic improvement in LVEF. The child is now thriving well with normal cardiac function.

85 Incidence of Intravenous Immunoglobulins-related Adverse Events in Children

Abstract Background Intravenous immunoglobulins (IVIG) therapy is increasingly used in the treatment of various paediatric diseases. Data about frequency and underlying mechanisms of IVIG-related adverse events (IVIG-AEs) in children are limited. Objectives Objectives of this study were to document the incidence of IVIG-AEs in paediatric hospitalized patients and identify risk factors for IVIG-AEs. Design/Methods In this retrospective cohort study, we included patients aged &amp;lt; 18 years old who received IVIG therapy, for any indication, as inpatients at a Canadian paediatric tertiary care centre between 2016-2020. Patients and IVIG-perfusion characteristics were collected, as well as any AEs occurring during the perfusion or up to 10 days after. AEs were graded according to the Common Terminology Criteria for Adverse Events (CTCAE) version 5.0. Bivariate and multivariate logistic regressions were used to explore the associations between patients’ characteristics and the incidence of IVIG-AEs. Results We included 228 children in this study (mean age: 4 years), receiving 478 IVIG infusions. Indications for IVIG infusions included inflammatory (120/228, 52.6%), replacement (27/228, 11.8%) and auto-immune (81/228, 35.5%) disorders. A total of 211 IVIG-AEs were reported in 89/228 (39%) of patients, and 125/478 (26.2%) of infusions. Fever (65/478, 13.6%) was the most frequent AE, followed by headache (32/478, 6.7%) and tachycardia (31/478, 6.5%). Most IVIG-AEs were of mild (121/211, 57%) or moderate (66/211, 31%) severity, but 12% (24/211) were severe reactions (serious AEs or grade 3-4), and no fatal reactions (grade 5) were encountered. The highest proportion of IVIG-AEs occurred when administered for inflammatory conditions. Older age, dehydration, first infusion, higher IVIG dosage and concurrent allergies were significantly associated with IVIG-AEs, while treatment with a steroid was significantly associated with a reduced risk of AEs. Conclusion IVIG-AEs are frequent in children. While most of them are self-limited, more serious complications may occur. Adequate hydration, low concentrations of IVIG products and the administration of glucocorticoid prior to the infusion could minimize these AEs. Further research is necessary to prevent IVIG-AEs in high-risk children.

FcRN receptor antagonists in the management of myasthenia gravis.

Myasthenia gravis (MG) is an autoimmune disorder characterized by autoantibodies specifically directed against proteins located within the postsynaptic membrane of the neuromuscular junction. These pathogenic autoantibodies can be reduced by therapies such as plasma exchange, IVIG infusions and other immunosuppressive agents. However, there are significant side effects associated with most of these therapies. Since there is a better understanding of the molecular structure and the biological properties of the neonatal Fc receptors (FcRn), it possesses an attractive profile in treating myasthenia gravis. FcRn receptors prevent the catabolism of IgG by impeding their lysosomal degradation and facilitating their extracellular release at physiological pH, consequently extending the IgG half-life. Thus, the catabolism of IgG can be enhanced by blocking the FcRn, leading to outcomes similar to those achieved through plasma exchange with no significant safety concerns. The available studies suggest that FcRn holds promise as a versatile therapeutic intervention, capable of delivering beneficial outcomes in patients with distinct characteristics and varying degrees of MG severity. Efgartigimod is already approved for the treatment of generalized MG, rozanolixizumab is under review by health authorities, and phase 3 trials of nipocalimab and batoclimab are underway. Here, we will review the available data on FcRn therapeutic agents in the management of MG.

Abstract #1400160: Early Onset Osteoporosis Associated with Novel Mutation of WNT1 Gene

Canonical WNT signaling plays a significant role in bone development and maintenance of bone mass. WNT1 gene mutations have been associated with osteogenesis imperfecta type XV in homozygotes and early onset osteoporosis in heterozygotes. We present a case of early onset osteoporosis associated with a novel mutation of the WNT1 gene. A 56-year-old female presented for evaluation of early onset osteoporosis. History was significant for hypothyroidism as well as chronic inflammatory demyelinating polyneuropathy on IVIG infusions. She was diagnosed with low bone density in the setting of a vertebral compression fracture from a diving accident when she was 24. She had mild scoliosis, brittle teeth and 10 fractures as a child and young adult including rib fractures, pelvic fracture and compression fracture of the spine, some of which occurred in the setting of low impact trauma. Her family history was notable for possible osteoporosis in her grandmother. Initial evaluation demonstrated PTH 75 (18 - 80 PG/ML), Calcium 9.5 (8.5 - 10.5 MG/DL ), Albumin 4.1 (3.5 - 5.0 G/DL ), 25-hydroxy vitamin D 44.6 (30.0-100.0 ng/mL), Phosphorus 3.6 (2.4 - 4.8 MG/DL), creatinine 0.59 (0.44 - 1.03 MG/DL), TSH 1.24 (0.350 - 5.500 uIU/ML ), 24-hour urine calcium 133 (50 - 300 MG/24H), late night salivary cortisol 0.066 (less than 0.112 ug/dL), tryptase 6.1 (< 11.0 mcg/L), tissue transglutaminase IgA antibody 2.5 (0-20 units), hemoglobin A1c 4.1 (4.3 - 5.6 % ), IGF-1 112 (50 - 317 ng/mL), Alkaline Phosphorus 62 (34 - 104 IU/L). Dual-energy X-ray absorptiometry (DEXA) scan at the age of 50 showed bone mineral density (BMD) 0.828 g/cm2 with T score of -2.0 at lumbar spine, BMD of 0.593 g/cm2 with T score of -2.3 at left femoral neck and BMD of 0.707 g/cm2 with T score of -1.9. She sustained a low trauma spine fracture at L1 at the age of 55. Subsequent DEXA scan at age 55 showed significant decline of BMD with T score of -3.3 at lumbar spine and T score of -3.0 at the left femoral neck. Due to suspicion for osteogenesis imperfecta, a genetic panel was sent which showed a heterozygous variant in WNT1 gene, c.581T >G (p.L194R). She was treated with oral alendronate 70 mg once weekly for less than one year which was stopped due to gastrointestinal side effects. Follow-up is planned for discussion of additional therapeutic options. Heterozygous mutations in WNT1 have been described in the literature as a cause of early onset osteoporosis. The currently described variant mutation in the WNT1 gene has not been previously reported in literature. In silico analysis using SIFT and PolyPhen2 predict this variant to be deleterious/possibly damaging. Genetic testing and evaluation of bone density in other family members may help further support the clinical importance of this mutation.

Neuromyelitis Optica Spectrum Disorder Mimicking Pontine Stroke - A Cautionary Tale: Case Report and Systematic Literature Review (P13-5.014)

<h3>Objective:</h3> To report an unusual clinical pattern of Neuromyelitis Optica Spectrum Disorder (NMOSD) presenting as a pontine stroke mimic <h3>Background:</h3> A 58-year-old Hispanic woman presented to an outside hospital with right-sided numbness, blurry vision, and gait instability. Findings on brain MR imaging were thought to reflect acute pontine stroke; the patient commenced dual antiplatelet therapy. Three weeks later, she presented to our hospital with right-sided hemiparesis. Stroke workup was unrevealing for specific pathophysiology. She was readmitted about two weeks later with a new-onset right cranial nerve VI palsy with ipsilateral facial droop and tinnitus. Brain MRI demonstrated foci of diffusion restriction on DWI in the periventricular white matter and brainstem region suggesting rhombencephalitis of inflammatory or autoimmune origin. Despite IVIG infusion, the patient’s condition deteriorated and she was intubated for airway protection. Serological assays were positive for AQP4 and negative for all other autoantibodies, including anti-Gq1b. She was treated with methylprednisolone pulse dosing and plasmapheresis, followed by rituximab, with significant neurological improvement. <h3>Design/Methods:</h3> In addition to the case report, a systematic literature review was performed to identify NMOSD cases initially diagnosed as stroke. Publications were selected and curated in accordance with PRISMA guidelines. <h3>Results:</h3> Six NMOSD patients were initially thought to represent acute stroke. However, the persistence, progression, or recurrence of symptoms with additional history, follow-up neuroimaging, and further serological immune assays facilitated the accurate diagnosis of NMOSD. Notably, the age of onset in all cases was significantly more advanced than patients normally presenting with NMOSD (median age 32–41). <h3>Conclusions:</h3> NMOSD diagnosis should be considered in individuals presenting with atypical stroke-like symptoms, particularly in those greater than or equal to 41 years of age. This is important as early recognition and treatment with immune therapies can improve functional outcome. <b>Disclosure:</b> Dr. Huang has nothing to disclose. Dr. Arora has nothing to disclose. An immediate family member of Dr. Diamond has received personal compensation in the range of $500-$4,999 for serving as an Editor, Associate Editor, or Editorial Advisory Board Member for Consultant360. An immediate family member of Dr. Diamond has received publishing royalties from a publication relating to health care. Dr. McFarlane has nothing to disclose. Dr. Najjar has nothing to disclose.

A Case series with comparative analysis of children and young adults with Anti-N-Methyl-D-Aspartate Receptor Encephalitis (P5-5.004)

<h3>Objective:</h3> To compare clinical presentation of 5 patients of various age groups with Anti-N-Methyl-D-Aspartate (NMDA) Receptor Encephalitis <h3>Background:</h3> Anti-NMDA-receptor antibody encephalitis is a treatable neurological condition hypothesized to be secondary to immune response initiated by an ovarian tumor or a non-specific infectious etiology. The immune mediated trigger produces antibodies that cross the compromised blood brain barrier where they interact with NR1/NR2 subunits of NMDA receptor resulting in encephalitis. Clinical presentation can vary according to the age and can include psychiatric symptoms, cognitive impairment, seizures, autonomic instability, and movement disorders. <h3>Design/Methods:</h3> Medical chart review of patients seen in a university hospital with varied presentations and subsequent diagnosis of Anti-NMDA-receptor antibody encephalitis <h3>Results:</h3> We describe 5 cases of Anti-NMDA-receptor antibody encephalitis seen at a university hospital between 2016 and 2022. Three were children under 5 years of age (youngest is 2.5 months) with abnormal choreo-athetoid movements and two were young women with psychiatric symptoms. Two of the infants had a preceding infectious exposure while both the young women had ovarian cysts. Except one infant, all cases had seizures during their disease course. All cases had CSF antibody positivity except one. Management included high dose IV methyl prednisolone with good response, followed by IVIG and/or Rituximab infusions in resistant cases. <h3>Conclusions:</h3> Clinicians should have high suspicion for Anti-NMDA-receptor antibody encephalitis in patients presenting with abnormal movements or nonspecific sudden onset psychological symptoms in the presence or absence of a preceding infectious prodrome. Aggressive testing with serum and CSF NMDA antibody titers is warranted when EEG is not consistent with seizures as Anti-NMDA-receptor antibody encephalitis is a treatable neurological condition with high morbidity and mortality if left untreated. <b>Disclosure:</b> Dr. Vasireddy has nothing to disclose. Qutubuddin Khan has nothing to disclose. Dr. Guduru has nothing to disclose.

SARS-CoV-2 Antibody Semi-Quantitative Levels Is Important In The Management Of COVID-19 Vaccinations In Immunodeficiency Disorder Patients.

The efficacy and durability of SARS-CoV-2 immunological antibody response in Immunodeficiency Disorder (ID) patients (including Primary Immunodeficiency) can be utilized in their clinical management to avoid COVID-19 infection. We correlated SARS-CoV-2 antibody semi-quantitative test results with ID patients’ COVID-19 vaccinations, their timing, IVIG infusions, and clinical outcomes.

Characterization of IVIG infusion adverse reactions reported at a tertiary care immunology infusion center

IVIG is utilized in the treatment of various medical conditions including primary immunodeficiencies and autoimmune disorders. Anaphylaxis is rare and can be seen in IgA-deficient patients, although reactions rates and associated risks are poorly understood. We aim to characterize IVIG reaction severity and associated clinical factors in this cohort.

Follow-up and treatment of patients with Common Variable Immune Deficiency: A single-center experience

Objectives: Common Variable Immunodeficiency (CVID) is a primary immunodeficiency characterized by immunoglobulin production defect. Our study aimed to create awareness of primary immunodeficiency in adult patients, establish standard approaches for clinical follow-up of CVID patients, and reveal the clinical characteristics of CVID patients in our region.&#x0D; Method: The study was conducted in patients with diagnosed and newly diagnosed CVID. The demographic and clinical characteristics of the patients and their treatment data were analyzed retrospectively and prospectively.&#x0D; Results: Thirteen of our patients were female and 12 were male. The mean age at diagnosis of the patients was 30.32 (2-57) and the mean delay in diagnosis was 9.32 months (0-30). The most common clinical finding of our patients at the time of admission was an infection. Among the infections identified, 3 patients had URTI, 19 had LRTI, and 2 had gastroenteritis. In 16 of our patients, bronchiectasis was detected at the time of diagnosis, and in 1 during the follow-up period. In the examinations performed in terms of organomegaly, splenomegaly was found in 11 patients and hepatomegaly was found in 8 patients. When patients were screened for autoimmune disease, ITP and celiac were found in 2 patients at the beginning, while autoimmune thyroiditis was developed in 1 patient and SLE in 1 patient during follow-up. Our patients were given IVIG treatment at regular intervals. The number of reactions seen in a total of 421 IVIG infusions was two.&#x0D; Conclusion: Primary immunodeficiencies should definitely be considered in patients with recurrent infections and resistance to antibiotic therapy. Patients should be followed according to established follow-up and treatment protocols in order to reduce and diagnose complications.

Beau's Lines Associated With IVIG Infusions.

Beau's Lines Associated With IVIG Infusions.

Immunoglobulin replacement therapy in patients with immunodeficiencies: impact of infusion method on patient-reported outcomes

BackgroundUnderstanding the impact of different immunoglobulin (Ig) infusion methods (intravenous [IVIg] and subcutaneous [SCIg]) upon treatment experience can potentially facilitate optimization of patient outcomes. Here, the perspective of patients with primary and secondary immunodeficiency diseases (PID and SID, respectively) receiving IVIg and SCIg was evaluated, in terms of treatment satisfaction, accounting for treatment history, using Association des Patients Immunodéficients du Québec (APIQ) survey data.MethodsThe online APIQ survey (shared October 2020–March 2021) of patients with immunodeficiencies in Canada contained 101 questions on: Ig use, history, and detailed infusion characteristics; as well as structured patient-reported outcomes such as treatment satisfaction (via TSQM-9), symptom state (via PASS), general health perception (via GHP), and physical and mental function (via PROMIS). Adult respondents (≥ 18 years old) currently using Ig were compared by their current Ig infusion method (IVIg or SCIg cohort) overall, and in a sub-analysis, the IVIg cohort was compared with the SCIg cohort after stratification by respondents who started SCIg when naïve to Ig (‘SCIg naïve’) or with previous IVIg experience (‘SCIg switch’).ResultsIn total, 54 respondents currently used IVIg and 242 used SCIg. The average duration per infusion of a weekly SCIg infusion was significantly shorter compared with the average duration of a 3–4 weekly IVIg infusion (p < 0.001). The SCIg cohort was associated with significantly higher scores for the TSQM-9 effectiveness domain compared with the IVIg cohort. The scores for TSQM-9 convenience and global satisfaction domains were similar in the two cohorts. The SCIg cohort was also associated with a significantly higher proportion of respondents who were in an acceptable symptom state and a lower proportion who reported very poor or poor perception of health compared with the IVIg cohort. Further, the SCIg naïve subgroup was associated with significantly higher TSQM-9 effectiveness and convenience domain scores compared with the IVIg cohort, while there was no significant difference between the SCIg switch subgroup and the IVIg cohort in terms of convenience.ConclusionsA better understanding of how different IgRT administration methods impact treatment experience and satisfaction may assist with informed treatment decision making and ultimately further improvements in patient outcomes.

Opsoclonus Myoclonus Syndrome and Supraventricular Tachycardia in a Pediatric Patient: A Case Report and Literature Review

Objective NA. Background Opsoclonus myoclonus syndrome (OMS) is a rare movement disorder in children often associated with an underlying neuroblastoma. In other cases, it is believed that the tumor is occult or there is another immune-stimulating precipitating event. Diagnosis can be difficult, requiring 3 of 4 criteria: opsoclonus or ocular flutter, myoclonus or ataxia, behavioral or sleep disturbances, and neuroblastoma. Prompt treatment of OMS is crucial to preventing permanent neurologic sequelae. In order to better characterize the clinical profile of this syndrome and its associated conditions, we present a case report of a 9 month old male with OMAS without an associated neuroblastoma and with new onset supraventricular tachycardia (SVT) and review the associated literature. Design/Methods NA. Results A 9-month-old male with a past medical history of macrocephaly, hypotonia, and developmental delay presented with abnormal eye and body movements starting one month prior. Due to concern for OMS, the patient underwent an extensive initial workup including CT neck, chest, abdomen, and pelvis and VHA/HMA levels, which were all within normal limits. The patient's spinal fluid revealed no evidence of paraneoplastic, autoimmune, or infectious processes. Per neurology recommendations, the patient was started on IVIG and dexamethasone. After his first IVIG infusion, the patient's abnormal movements worsened, and his heart rate increased into the 300s. The IVIG was discontinued. However, the patient continued to have recurring bouts of SVT, which was eventually controlled with digoxin. After resuming treatment with IVIG and dexamethasone, the patient's opsoclonus myoclonus symptoms began to improve. All additional metabolic labs resulted normal and the patient was discharged. Conclusions Because OMS can cause permanent developmental delay, prompt recognition and treatment of this syndrome is necessary. This is a unique case of OMAS without neuroblastoma, associated with recurrent bouts of SVT. Recognizing rare complications of OMS is crucial to improving medical management of its sequelae.

NCMP-17. PLASMA EXCHANGE FOR IMMUNE CHECKPOINT INHIBITOR INDUCED ACUTE DEMYELINATING POLYNEUROPATHY REFRACTORY TO IVIG

Abstract The use of immune checkpoint inhibitors in the treatment of cancer has gained prominence due to their effectiveness. Unfortunately, neurological immune-related adverse events represent a growing problem in neuro-oncology practice. Many of these cases are rare and their diagnoses and management can be challenging. We present a case of a 69-year-old male with urothelial carcinoma status post left nephrectomy who developed bilateral lower extremity weakness that began following his first dose of Atezolizumab. After three doses, his lower extremity weakness worsened to the point where he could not walk. A NCV study was performed and was consistent with acute demyelinating polyneuropathy. A cervical MRI (Magnetic Resonance Imaging) showed an enhancing lesion at C6-C8. He was treated with three days of intravenous methylprednisolone every six hours for three days followed by three days of 0.4gm/kg IVIG. He regained his ability to walk after IVIG treatment. The enhancing lesion noted on MRI remained. Three weeks after the first IVIG treatment, he declined significantly, redeveloping symptoms where he could not ambulate and required assistance to use the toilet. He had no lower extremity reflexes and had loss of proprioception of his hands. He was scheduled for another IVIG infusion at .4gm/kg followed by a dose of IVIG .6gm/kg the next day, and then four more days of IVIG .25gm/kg. His lower extremity weakness and difficulty walking remained so he was arranged for plasmapheresis x five cycles. His weakness improved, reflexes returned, and he no longer had proprioceptive loss of his hands. A cervical MRI obtained one month after plasma exchange showed resolution of the C6-C8 enhancing lesion. He is now ambulating with a cane. This case highlights the effectiveness of plasma exchange in a case that was refractory to IVIG