intravenous immunoglobulin (IVIG) / Guys and St Thomas NHS Foundation Trust - LARVOL DELTA

P040 Clinical features, treatment and treatment response of 24 patients with epidermolysis bullosa acquisita. (PubMed, Br J Dermatol) - "All patients were treated initially with oral prednisolone and some patients required oral dapsone (n = 20), intravenous corticosteroids (n = 11), mycophenolate mofetil (n = 19) and azathioprine (n = 7). Overall, 63% (n = 15) of patients were treated with intravenous immunoglobulin (IVIG) and 63% (n = 15) with rituximab...In conclusion, the treatment of EBA can be challenging, and most patients in this series required combination treatment with corticosteroids, dapsone and an intravenous immunosuppressant. The treatment-refractory nature of the disease is also reflected by the high proportion of patients requiring rituximab, and 25% of patients having ongoing active disease despite maximal therapy."

Journal

Evaluation, Diagnosis, and Treatment of Sydenham Chorea: Consensus Guidelines. (PubMed, Pediatrics) - "In those with inadequate recovery, intravenous immunoglobulin or plasma exchange should be given. In SC relapse, repeat clinical assessments, etiological investigation, and antibiotics plus corticosteroid therapy should be considered. This consensus guideline will standardize the evaluation and management of patients with SC and direct future research to improve the lived experience and outcomes of patients and families."

Journal • Alzheimer's Disease • CNS Disorders • Cognitive Disorders • Movement Disorders • Pain • Pediatrics • Psychiatry

Observational Data on Parvovirus B19 Cases in Patients with Red Cell Disorders in the United Kingdom from 2023-2024 (ASH 2024) - "For the patients with sickle cell disorder, 35 out of the 84 patients were not on any disease modifying therapy, 44 were on hydroxycarbamide, 2 patients were on regular automated exchanges and the remaining patients were on either top up transfusions or a combination of voxelotor and hydroxycarbamide...2 patients were treated for fat embolism syndrome with a red cell exchange and 5 days of plasma exchange, one of these patients was also given intravenous immunoglobulin (IVIG) and the other was given cryoprecipitate and FFP transfusions to treat DIC, both were HbSC...The greater proportion of patients with HbSC (25%) requiring critical care vs HbSS(16.6%) could indicate that patients with HbSC are more vulnerable to developing a more severe disease course. The data collected over 1 year demonstrates that parvovirus B19 infection presents a significant risk to this cohort of patients."

Clinical • Observational data • Anemia • Autoimmune Hemolytic Anemia • Beta-Thalassemia • Cardiovascular • Genetic Disorders • Hematological Disorders • Immunology • Infectious Disease • Novel Coronavirus Disease • Sickle Cell Disease

FETAL AND NEONATAL ALLOIMMUNE THROMBOCYTOPENIA - SURVEY OF UK PRACTICE. (EHA 2025) - "The antenatal management of women who have had a previous pregnancy affected by FNAIT involves treatment with intravenous immunoglobulin (IVIg) at 0.5-2g/kg/week starting at 12-20 weeks and continuing until delivery... This is the first survey of UK practice in the antenatal management of patients with a history of FNAIT. A strength of this survey is the high response rates from fetal medicine centres, but is limited by looking only at expert clinicians in fetal medicine centres, when we aware that some patients are managed in smaller regional hospitals. The survey showed consistency over decisions about antenatal IVIg dosing but less consensus over when treatment should start."

Hematological Disorders • Immune Thrombocytopenic Purpura • Thrombocytopenia • Thrombocytopenic Purpura

The costs of treating bleeding episodes in patients with immune thrombocytopaenia in the United Kingdom. (PubMed, Hematology) - "The major cost driver for mild and serious events were intravenous immunoglobulin (IVIg) costs, amounting to £1,614 and £8,071 for the two severity categories, respectively...Real-world costs of managing bleeding in patients with ITP in the UK are substantial and greater than costs set only based on the UK NHS Tariff. Mitigating the risk of bleeding in patients with ITP is likely to yield not only clinical advantages for patients but also offer substantial cost savings to the health system."

Journal • Critical care • Immunology • Thrombocytopenia

Successful serial plasmapheresis for solar urticaria, a case report and literature review. (PubMed, J Dermatolog Treat) - "We reported a case of recalcitrant SU that had not responded to a combination of H1-antihistamines, immunosuppressants, omalizumab and intravenous immunoglobulin. No serious side effects from TPE were reported amongst the patients. This review underscores the efficacy of serial plasmapheresis procedures in treating refractory cases of SU, high3lighting the robust results observed."

Journal • Review • Dermatology • Immunology • Urticaria

IgNiTE: Investigating the Role of Early Intravenous Immunoglobulin Treatment for Children With Encephalitis (clinicaltrials.gov) - P3 | N=18 | Completed | Sponsor: University of Oxford | Unknown status ➔ Completed | N=308 ➔ 18

Enrollment change • Trial completion • CNS Disorders

Efficacy of rituximab in the treatment of epidermolysis bullosa acquisita (EADV 2023) - "All patients were on combination corticosteroid and immunosuppressant therapy, consisting of one or more of mycophenolate mofetil, intravenous immunoglobulin, azathioprine or dapsone. Conclusion Rituximab was well tolerated and resulted in clinical improvement in 71% of our patient cohort and enabled dose reduction of prednisolone and other immunomodulatory therapies in the majority of patients. This study represents the largest reported cohort of EBA patients treated with rituximab and supports it’s role in the management of this challenging disease."

Clinical

Desensitization and Immunomodulation in Sensitized Kidney Transplant Recipients: A Delphi Method Consensus from the European Guidelines for the Management of Graft Recipients (ENGAGE) Working Group of the European Society for Organ Transplantation (ESOT) [Board No. B171] (ATC 2023) - "* After two rounds of voting, 41/43 statements reached an overall agreement > 75% on the following themes: first line desensitization strategy with plasma exchange and intravenous immunoglobulin, lymphocyte depleting agents vs IL-2 receptor monoclonal antibodies, role of Rituximab to prevent Ab-mediated injury, role of Imlifidase as a desensitizing agent in recipients from deceased donors, maintenance immunosuppression with tacrolimus, mycophenolic acid and steroid vs minimization and use of mTOR-inhibitors. This methodology has allowed to reach a high degree of consensus among the experts in the management of desensitization and immunomodulation strategies of stratified immunological risk kidney transplant recipients"

Clinical • Immunomodulating • Antibody-mediated Rejection • Immune Modulation • Immunology • Nephrology • Solid Organ Transplantation • Transplantation • IL2

Multi-centre, randomised, open-label, blinded endpoint assessed, trial of corticosteroids plus intravenous immunoglobulin (IVIG) and aspirin, versus IVIG and aspirin for prevention of coronary artery aneurysms (CAA) in Kawasaki disease (KD): the KD CAA prevention (KD-CAAP) trial protocol. (PubMed, Trials) - P3 | "Several recent studies have indicated that coronary complications associated with KD across Europe are much higher than early trials of IVIG had initially suggested. KD-CAAP directly addresses this issue by exploring the therapeutic benefit of adjunctive corticosteroids in unselected KD cases. If we find that corticosteroids prevent CAA and are safe, this is a cheap and widely available intervention that could be implemented immediately for the benefit of children."

Journal • Cardiovascular • Vasculitis • CRP

Updated S2 K guidelines for the management of bullous pemphigoid initiated by the European Academy of Dermatology and Venereology (EADV). (PubMed, J Eur Acad Dermatol Venereol) - "The guidelines for the management of BP were updated. They summarize evidence- and expert-based recommendations useful in clinical practice."

Journal • Atopic Dermatitis • Bullous Pemphigoid • CNS Disorders • Dermatology • Dermatopathology • Immunology • Urticaria

Clinical features, investigations, and outcomes of pediatric limbic encephalitis: A multicenter study. (PubMed, Ann Clin Transl Neurol) - "A diagnosis of autoimmune LE was associated with significant morbidity and adverse outcomes in this pediatric cohort."

Clinical • Journal • Alzheimer's Disease • CNS Disorders • Critical care • Epilepsy • Immunology • Inflammation • Neuroblastoma • Oncology • Pediatrics • Solid Tumor

Use and Safety of Immunotherapeutic Management of N-Methyl-d-Aspartate Receptor Antibody Encephalitis: A Meta-analysis. (PubMed, JAMA Neurol) - "Factors at first event that were significantly associated with good functional outcome included adolescent age and first-line treatment with therapeutic apheresis, corticosteroids plus intravenous immunoglobulin (IVIG), or corticosteroids plus IVIG plus therapeutic apheresis...Factors significantly associated with nonrelapsing disease were rituximab use or maintenance IVIG use for 6 months or more...Modified NEOS score (including 4 of 5 original NEOS items) was associated with probability of poor functional status at 1 year (20.1% [40 of 199] for a score of 0 to 1 points; 43.8% [77 of 176] for a score of 3 to 4 points; P = .05). Factors influencing functional outcomes and relapse are different and need to be considered independently in development of evidence-based optimal management guidelines of patients with NMDARE."

Journal • Retrospective data • CNS Disorders • Critical care

[VIRTUAL] Toxic epidermal necrolysis in the setting of systemic lupus erythematosus: a case series (BAD 2021) - "Recent medication included prednisolone, esomeprazole and flucloxacillin...She had markers of active lupus and was treated with methylprednisolone, intravenous immunoglobulins (IVIG) and granulocyte colony-stimulating factor. She was discharged and started on belimumab for treatment-resistant SLE...A toothache was treated with co-codamol, amoxicillin and metronidazole, and she developed a macular erythema to her face, trunk and thighs. This recurred 2 months later and she was treated with penicillin V, flucloxacillin and metronidazole (for Clostridium difficile infection). She was found to have a high antinuclear antibody, double-stranded DNA and low complement, was diagnosed with SLE and treated with steroids, lansoprazole and hydroxychloroquine, and developed a widespread bullous eruption...The culprit drugs were thought to be clarithromycin or omeprazole...In the third patient only, immunofluorescence was consistent with lupus. These patients had a protracted ITU stay..."

Clinical • CNS Disorders • Epilepsy • Immunology • Infectious Disease • Inflammatory Arthritis • Lupus • Pneumonia • Respiratory Diseases • Septic Shock • Sjogren's Syndrome • Steven-Johnson Syndrome • Systemic Lupus Erythematosus

A HaemSTAR-led, UK-wide “Flash-Mob” audit of intravenous immunoglobulin use in immune thrombocytopenia (BSH 2019) - "347 (51.8%) of treatments involved the use of additional doses given in a manner not endorsed by the guidelines; 324 had IVIg over two consecutive days, 3 were dosed over five days and 20 received a different dosing regimen.Not only do these data suggest that we may be spending more money than we should be and exposing our patients to unnecessary risk by using significantly more IVIg than is recommended to treat ITP but they also show that it is possible to rapidly collect valuable health data utilising minimal resources by coordinating audit activity across the country with research networks such as HaemSTAR. We intend to repeat this national audit model with other important questions in haematology."