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Functional Profiling of Acetylcholine Receptor Antibodies Informs Therapeutic Outcome of FcRn Inhibitor Therapy in Myasthenia Gravis.

BACKGROUND AND OBJECTIVES: Myasthenia gravis (MG) is an autoimmune disorder of the neuromuscular junction most commonly associated with acetylcholine receptor (AChR) antibodies (AChR-Abs), detected in up to 80% of patients. The emergence of targeted therapies, such as neonatal Fc receptor (FcRn) inhibitors, highlights the need for standardized functional assays capable of dissecting AChR-Ab pathogenic mechanisms. By selectively reducing circulating IgG through FcRn blockade, these agents differ fundamentally from conventional immunosuppressants, emphasizing the need to characterize antibody-me…

Объединение весовых и глюкозных уровней с Myastenia Gravis: двухъярусное, двухвыборное Менделийское Рэндомирование и Ретроспективное тематическое исследование.

В предыдущих исследованиях указывалось на наличие связей между ожирением, регулированием глюкозы и аутоиммунными заболеваниями, однако связь уровней веса и глюкозы с миастенией грависа (МГ) остается неопределенной. Мы изучили эти ассоциации и потенциальный непрямой путь через уровни глюкозы. ЦЕЛЬ: Мы изучили связи уровней веса и глюкозы с риском MG с использованием менделийской случайности (MR) и ретроспективного исследования по контролю за конкретными случаями. МЕТОДЫ: Мы провели анализ ДР с использованием двух образцов с использованием обобщенных статистических данных по европейским группам

Совпадение в аннотации оригинала
BACKGROUND: Previous studies suggested links between obesity, glucose regulation, and autoimmune diseases, but the associations of weight and glucose levels with myasthenia gravis (MG) remain uncertain. We examined these associations and a potential indirect pathway through glucose levels. OBJECTIVE: We investigated the associations of weight and glucose le…

Efgartigimod in the treatment of immune checkpoint inhibitor-related myasthenia gravis -myositis overlap syndrome: a case report.

BACKGROUND: A subset of cancer patients receiving monoclonal antibody PD-1/PD-L1 inhibitors may develop immune checkpoint inhibitor (ICI)-related neurological complications, such as ICI-related myasthenia gravis(MG)-myositis overlap syndrome and ICI-related myocarditis. Standard management typically involves intravenous immunoglobulin (IVIG), plasma exchange (PE), and high-dose corticosteroids. The use of efgartigimod, a neonatal Fc receptor blocker, for the treatment of ICI-related MG-myositis overlap syndrome remains investigational, with only four relevant cases all representing overlap syn…

Efficacy of nipocalimab in patients with moderate-to-severe ocular symptoms of generalized myasthenia gravis in the phase 3 Vivacity-MG3 study.

BACKGROUND: In the global Vivacity-MG3 study, nipocalimab (30-mg/kg loading dose; 15-mg/kg every-2-weeks) demonstrated sustained disease control versus placebo plus standard-of-care for generalized myasthenia gravis (gMG); this post-hoc analysis evaluated nipocalimab efficacy for participants with moderate-to-severe ocular symptoms (baseline score of ≥2 points on either ocular-domain item of MG-activities-of-daily-living [MG-ADL]). RESEARCH DESIGN AND METHODS: Mean MG-ADL and Quantitative Myasthenia Gravis total scores change-from-baseline (CFB) through Week (W)24 were assessed using ANCOVA. …

[Research on Exoskeleton Human-Machine Interaction for Walking Assistance in Myasthenia].

To address the insufficient human-machine interaction adaptability in current lower-limb rehabilitation exoskeletons, this study proposes a lower-limb assistive exoskeleton featuring active actuation at the hip and knee joints and passive following at the ankle joint. A rigid self-adaptive human-machine interface is adopted to provide both effective assistive torque and structural compliance, aiming to deliver gait assistance for individuals with muscle weakness while improving wearing comfort. First, a bionic geometric structure of the exoskeleton was constructed based on human lower-limb bio

Fatal Myocarditis Relapse in a Patient with Myasthenia Gravis Treated with Efgartigimod Alfa: A Case Report.

Myasthenia gravis (MG) is an autoimmune neuromuscular disorder primarily caused by autoantibodies against acetylcholine receptors (AChR), leading to muscle weakness. In 2021, efgartigimod was FDA-approved as the first neonatal Fc receptor (FcRn) blocker for generalized MG, reducing pathogenic IgG autoantibodies and improving symptoms. We present a 39-year-old woman with AChR-positive generalized MG and a history of resolved inflammatory myocarditis. Owing to persistent weakness despite intravenous immunoglobulin, she transitioned to efgartigimod. Efgartigimod induced significant neuromuscular …

Immune checkpoint inhibitor-related myositis with hepatitis in HER2-positive metastatic breast cancer after trastuzumab deruxtecan plus sintilimab: a case report.

Immune checkpoint inhibitors (ICIs) have improved outcomes across multiple solid tumors, but they may trigger immune-related adverse events … n did not show a typical decremental response, left orbicularis oculi fatigue testing was negative, myasthenia gravis-related antibody testing was recommended but declined, and the response to cholinesterase inhibitor therapy was limited. Troponin testing, repeat echocardiography, and cardiac magnetic resonance imaging were not performed. Therefore, t …

Correction: Furlepa et al. Management of Triple M Syndrome: A Systematic Review with Narrative Synthesis of Immune Checkpoint Inhibitor-Induced Myasthenia Gravis, Myositis and Myocarditis. Cancers 2025, 17, 2063.

In the published publication [...].

Immune checkpoint inhibitor-related myositis, myocarditis, and myasthenia gravis overlap syndrome: a systematic review and pooled analysis of individual cases.

BACKGROUND: Immune checkpoint inhibitor (ICI)-related overlap syndrome involving myositis, myocarditis, and myasthenia gravis (3M overlap syndrome) is rare but potentially fatal. Current evidence is derived mainly from case reports and small case series, and individual case-level data on ancillary diagnostic findings, baseline comorbidities, treatment patterns, and short-term outcomes remain limited. METHODS: We performed a systematic review of published case reports and case series and a pooled analysis of individual case data on ICI-related 3M overlap syndrome, supplemented by three patient…

Myotoxicity in the era of cancer immunotherapy: from mechanisms to multidisciplinary management.

Myotoxicity, defined as immune-mediated injury to cardiac and/or skeletal muscle, has emerged as one of the most severe and lethal immune-related adverse events (irAE) in patients receiving immune checkpoint inhibitors (ICIs). Although myocarditis in oncology is not restricted to ICIs and may also occur after traditional cytotoxic chemotherapy, radiotherapy, or selected targeted therapies, ICI-associated cardiomyotoxicity is distinguished by its early onset, frequent overlap with myositis, and myasthenia gravis, and high case-fatality rate. This review outlines myotoxicity within the broader c…

The immune microenvironment and population heterogeneity in myasthenia gravis: implications for precision therapeutics.

Myasthenia gravis (MG) is a chronic autoimmune disorder of the neuromuscular junction characterized by fluctuating skeletal muscle weakness. Although the pathogenic roles of autoantibodies targeting the acetylcholine receptor (AChR), muscle-specific kinase (MuSK), and certain neuromuscular junction proteins have been well established, increasing evidence indicates that MG onset, progression, and therapeutic response can be shaped by the complex interactions within diverse immune microenvironments. Particularly, population heterogeneity, including differences in antibody subtype, age, sex, gene…

Microscopic Polyangiitis Following Kawasaki Disease in a Patient With Autoimmune Predisposition.

Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) is rare in children, and its pathogenesis remains incompletely unders … y, more than a decade before the onset of MPA. In addition, his mother had rheumatoid arthritis and myasthenia gravis, and his younger sister had immune thrombocytopenic purpura. This case raises the possibility that prior immune-mediated vascular inflammation, together with autoimmune genetic predisposition, may contribute to the later development of …

When gas travels: a case report of partial Horner's syndrome from subcutaneous emphysema post-laparoscopic hiatal hernia repair.

INTRODUCTION: Postoperative partial Horner's syndrome associated with subcutaneous emphysema after laparoscopic hiatal hernia repair has not … s examined and underwent a workup for brainstem infarct, carotid artery aneurysm or dissection, and myasthenia gravis. The examination revealed complete left-sided ptosis with equal, round, and reactive pupils. MRI of the brain and myasthenia gravis laboratory workup were unremarkable. CT angiography of the neck revealed subcutaneous emphysema adjacen …

Complications and Occurrence of Exacerbations in Patients with Myasthenia Gravis Treated with Oral Corticosteroids.

BACKGROUND: Myasthenia gravis is a rare disease characterized by autoantibody-driven impairment of neuromuscular junction activity. Standard-of-care therapy includes oral corticosteroids (OCS), despite associated complications. This study describes OCS-related complications and exacerbations in a real-world myasthenia gravis population. METHODS: This retrospective longitudinal cohort study leveraged US claims data (January 2016-March 2023; Optum's de-identified Clinformatics® Data Mart Database) to identify patients with myasthenia gravis (International Classification of Diseases, Tenth Revis…

Reversible complete atrioventricular block in immune checkpoint inhibitor-associated triple-M overlap syndrome: a case report.

BACKGROUND: Immune checkpoint inhibitors (ICIs) are increasingly used in the treatment of advanced malignancies but are associated with rare and potentially life-threatening immune-related adverse events. Overlap syndromes involving myocarditis, myositis, and myasthenia gravis-referred to as Triple-M overlap syndrome-have been reported; however, reversible high-grade atrioventricular block remains uncommon. CASE SUMMARY: A 74-year-old woman with advanced ovarian cancer treated with pembrolizumab presented with dyspnoea, myalgia, and right-sided ptosis 22 days after ICI therapy. Electrocardiog…

Medication use trajectories in myasthenia gravis pregnancies: a population-based drug utilization study.

OBJECTIVE: To examine medication use trajectories from 1 year before pregnancy to 6 months postpartum in a population-based birth cohort of patients with myasthenia gravis (MG). METHODS: We used harmonized and pooled national register data from Norway and Sweden (1994-2023) to identify MG pregnancies. Longitudinal dispensing records of symptomatic MG medications, oral corticosteroids (OCS), and non-steroidal immunosuppressant therapy (NSIST) were extracted from prescription registers, which were individually linked to hospital records in the national patient registers and demographic and clin…

Cell Death in Skeletal Muscle Diseases: Diverse Roles and Pathological Processes.

Skeletal muscle is vital for movement and metabolism, and its dysfunction underpins disorders like muscular dystrophy and sarcopenia, severe … acquired muscle disorders such as idiopathic inflammatory myopathy, sarcopenia, rhabdomyolysis, and myasthenia gravis (MG). We also explore the potential of cell death-related molecules as biomarkers and discuss emerging therapeutic strategies that target these pathways, aiming to provide new insights for diagnosis and treatment.

Neurologic Complications of Cancer Immunotherapy.

The use of oncologic-directed immunotherapies, including immune checkpoint inhibitors (ICIs) and chimeric antigen receptor T cell (CAR-T) th … reatening complications. ICI-related neurotoxicities may include peripheral neuropathies, myositis, myasthenia gravis, encephalitis, aseptic meningitis, myelitis, and demyelinating disorders. CAR-T therapies carry a comparatively higher risk of neurologic irAEs, most notably immune effector cell-associated neurotoxicity syndrome. In this review, neuro …

Efficacy and safety of low-dose rituximab in MuSK antibody-positive myasthenia gravis: a single-center retrospective study.

BACKGROUND: Rituximab (RTX) is increasingly used for refractory myasthenia gravis (MG), particularly muscle-specific kinase antibody-positive MG (MuSK-MG). However, the optimal dosing regimen remains to be standardized, and conventional high-dose protocols may increase treatment burden, cumulative exposure, and infection-related concerns. This study evaluated the clinical efficacy, safety, and immunological effects of a low-dose RTX regimen in patients with MuSK-MG. METHODS: We retrospectively analyzed 35 patients with MuSK-MG who received low-dose RTX at the First Affiliated Hospital of Guan…

Abatacept for steroid-refractory fulminant immune checkpoint inhibitor myocarditis with triple M overlap syndrome: a case report with serial endomyocardial biopsy assessment.

BACKGROUND: Triple M overlap syndrome is a rare, but often fatal, immune-related adverse event. Evidence on the effectiveness of immunosuppressive treatments, including abatacept, and disease monitoring in complex cases remains limited. CASE SUMMARY: A man in his 60s developed fulminant immune checkpoint inhibitor (ICI)-related myocarditis after nivolumab plus ipilimumab for recurrent lung adenocarcinoma, complicated by triple M overlap syndrome (myocarditis, myositis, and myasthenia gravis). Despite high-dose corticosteroids and intravenous immunoglobulin, cardiac biomarkers worsened, with p…