Uplizna (inebilizumab-cdon) / Tanabe Pharma, Jiangsu Hansoh Pharma, Amgen - LARVOL DELTA

Syncope in AQP4-IgG-positive neuromyelitis optica spectrum disorder: a case series and literature review. (PubMed, Front Neurosci) - "Both were treated with high-dose intravenous methylprednisolone followed by maintenance inebilizumab, leading to complete clinical remission, seroconversion to AQP4-IgG negativity, and an Expanded Disability Status Scale (EDSS) score of 0 at last follow-up. Syncope may be an underrecognized manifestation of AQP4-IgG-positive NMOSD. Patients presenting with APS should be screened for syncope attacks, which may require emergency pacemaker implantation."

Journal • CNS Disorders • Hypotension • Neuromyelitis Optica Spectrum Disorder • Rare Diseases

Systematic Review and Model-Based Meta-Analysis of Targeted Drugs for Systemic Sclerosis. (PubMed, Pharmaceutics) - "Guselkumab, an anti-IL-23 antibody, showed the greatest effect on mRSS, followed by tofacitinib, inebilizumab, and baricitinib. For FVC, B-cell-targeted therapies, with belimumab and rituximab, demonstrated the highest efficacy, while tocilizumab and nintedanib had more moderate effects. Time to 50% maximal response was approximately 27.5 weeks, indicating a 6.3-month period for half treatment response development. This model-based meta-analysis provides a broad comparison of targeted therapies in SSc, highlighting distinct efficacy patterns for skin versus lung involvement and offering hypothesis-generating insights that may support treatment selection and the design of future clinical trials."

Journal • Retrospective data • Review • Fibrosis • Immunology • Scleroderma • Systemic Sclerosis • IL23A

Long-term Inebilizumab Treatment Results in Mild Immunoglobulin Reduction but No Increase in Infection Risk (EULAR 2026) - No abstract available

Infectious Disease

Long-term Efficacy and Safety of Inebilizumab in IgG4-Related Disease: Primary Results from Year 1 of the Open Label Period of the Phase 3 MITIGATE Trial (EULAR 2026) - No abstract available

Clinical • P3 data • Inflammation

Immunogenicity And Impact of Anti-drug Antibodies on the Efficacy and Pharmacokinetics of Inebilizumab in MITIGATE, A Phase 3 Trial in IgG4-Related Disease (EULAR 2026) - No abstract available

Clinical • P3 data • PK/PD data • Inflammation

BEST-NMOSD: Rituximab Versus Ravulizumab, Inebilizumab, Satralizumab, and Eculizumab in NMOSD (clinicaltrials.gov) - P4 | N=540 | Not yet recruiting | Sponsor: Massachusetts General Hospital | N=160 ➔ 540

Enrollment change • CNS Disorders • Neuromyelitis Optica Spectrum Disorder • Rare Diseases

Idiopathic Hypertrophic Pachymeningitis: Diagnosis of Exclusion (AAN 2026) - "Additional immunosuppressive treatment with pulse steroids, methotrexate, and azathioprine were administered, but her condition progressed, causing complete left eye vision loss before initiation of rituximab. Hypertrophic pachymeningitis is a rare neurologic disorder that can be secondary to numerous primary rheumatologic conditions. Among these, IgG4-related disease remains a challenging diagnosis, though one of vital importance given recent FDA approval of inebilizumab for treatment of such. This case demonstrates the value of early dural biopsy and exhaustive evaluation prior to determining idiopathic etiology in patients with hypertrophic pachymeningitis given the potential for aggressive phenotype of disease; reconsideration of diagnosis should be had for cases refractory to typical first line immunosuppression given recent therapeutic advances."

CNS Disorders • Epilepsy • Infectious Disease • Inflammation

EVALUATING THE ECONOMIC VALUE OF MONOCLONAL ANTIBODIES FOR AQP4+ NMOSD IN THE US USING ATTACK-FREE SURVIVAL OVER A 5-YEAR HORIZON (ISPOR 2026) - "This comparative economic assessment provides payer-relevant evidence on the long-term value of monoclonal antibody therapies for AQP4+ NMOSD in the US and may help guide clinical and reimbursement decisions."

CNS Disorders • Neuromyelitis Optica Spectrum Disorder • Rare Diseases

IgG4-related sclerosing cholangitis: navigating diagnostic dilemmas and the challenge of relapse. (PubMed, Front Med (Lausanne)) - "Maintenance therapy with steroid-sparing immunomodulators (e.g., azathioprine, mycophenolate mofetil) or B-cell depleting agents such as rituximab is often required. The anti-CD19 monoclonal antibody inebilizumab has emerged as a potent new option for maintaining remission. Maintaining a high index of clinical suspicion for IgG4-SC is essential in patients with obstructive jaundice and biliary strictures. Future efforts should focus on validating specific biomarkers (e.g., circulating plasmablasts, autoantibody profiles) and developing evidence-based protocols for long-term management to prevent fibrotic complications and reduce the relapse rate."

Journal • Review • Biliary Cancer • Cholangiocarcinoma • Fibrosis • Hepatology • Immunology • Oncology • Pancreatitis • Solid Tumor • ANXA1 • ANXA11 • CD4

20240202: Open-label, Uncontrolled Study to Evaluate the Pharmacokinetics, Pharmacodynamics, Safety, and Tolerability of Inebilizumab in Pediatric Participants with Immunoglobulin G4-related Disease (IgG4-RD) (clinicaltrialsregister.eu) - P1/2 | N=2 | Not yet recruiting | Sponsor: Amgen Inc.

New P1/2 trial • Inflammation • Pediatrics

A Systematic Review of Erdheim-Chester Disease and IgG4-Related Disease: Building a Diagnostic Framework for the Rheumatologist. (PubMed, J Clin Rheumatol) - "This review provides a structured, multimodal approach to distinguish ECD from IgG4-RD, which improves diagnostic accuracy by integrating genetic testing, advanced imaging, neurological features, and treatment response. This allows clinicians to avoid misdiagnoses, implement targeted treatments, and improve patient outcomes."

Journal • Review • Fibrosis • Immunology • Inflammation • Langerhans Cell Histiocytosis • Metabolic Disorders • Pancreatitis • Rare Diseases • Rheumatology

Myasthenia Gravis Inebilizumab Trial (MINT): Efficacy, Pharmacodynamics, and Immunogenicity in AChR+ Cohort (Week 52) (AAN 2026) - P3 | "Inebilizumab leads to targeted depletion of B-cells and provides durable improvement in the AChR+ gMG subpopulation through Week-52."

Clinical • PK/PD data • CNS Disorders • Myasthenia Gravis • CD20

Inebilizumab treatment in Japanese patients with IgG4-related disease: a subgroup analysis of a randomized, double-blind, placebo-controlled, phase 3 trial (MITIGATE). (PubMed, Mod Rheumatol) - P3 | "Inebilizumab reduced flare risk and glucocorticoid exposure and was well tolerated in Japanese patients with IgG4-related disease, consistent with overall MITIGATE results."

Clinical • Journal • P3 data • Inflammation

Real-World Efficacy and Safety of Inebilizumab in Generalized Myasthenia Gravis (AAN 2026) - "All patients achieved a prednisone dose of ≤5 mg/day by month 15. Long-term B cell depletion with inebilizumab demonstrated sustained efficacy in reducing disease activity for generalized MG with controllable immune function impacts,making it a favorable option for gMG patients."

Clinical • Real-world • Real-world effectiveness • Real-world evidence • CNS Disorders • Herpes Zoster • Immunology • Infectious Disease • Myasthenia Gravis • Pneumonia • Respiratory Diseases • Varicella Zoster

Integrating B Cell Differentiation Model With Real-World Data Informs Determinants for Antibody-Secreting Cell Depletions in Systemic Sclerosis. (PubMed, CPT Pharmacometrics Syst Pharmacol) - "This study aimed to develop a systems model for B cell differentiation and tissue distribution to characterize the therapeutic responses to CD19+ (inebilizumab) and CD20+ cell depletion (rituximab) in patients with SSc. Overall, the systems model provided mechanistic insights into the contrasting responses of ASCs depending on the study drugs and identified potential predictors of treatment efficacy. By integrating RWD, our study provides a mechanistic framework to optimize dosing strategies and guide personalized treatment approaches to refine B-cell depletion therapies for SSc."

Journal • Real-world evidence • Fibrosis • Immunology • Scleroderma • Systemic Sclerosis • CD20

20240236: An Open-label Study to evaluate the Pharmacokinetics, Pharmacodynamics, Safety and Tolerability of Inebilizumab in children with gMG (clinicaltrialsregister.eu) - P1/2 | N=4 | Not yet recruiting | Sponsor: Amgen Inc.

New P1/2 trial • CNS Disorders • Myasthenia Gravis

Observational Study of Inebilizumab as a Salvage Therapy for Acute Optic Neuritis in Neuromyelitis Optica Spectrum Disorder (NMOSD-ON) (AAN 2026) - "Current first-line acute-phase therapies, such as intravenous methylprednisolone (IVMP), often result in suboptimal responses in a significant proportion of patients, thus highlighting the need for more effective acute-phase salvage therapies. Inebilizumab salvage therapy appears to be effective and well-tolerated in acute NMOSD-ON. Large-scale prospective randomized controlled trials are warranted to further validate these findings."

Clinical • Observational data • CNS Disorders • Herpes Zoster • Infectious Disease • Neuromyelitis Optica Spectrum Disorder • Ocular Inflammation • Ophthalmology • Optic Neuritis • Rare Diseases • Varicella Zoster

Missed or Delayed Infusions as Probable Culprits for Relapses in NMOSD Patients on Inebilizumab: A Real-world Study in a Predominantly Hispanic Cohort (AAN 2026) - "This Hispanic-predominant NMOSD cohort linked 43% of relapses to infusion delays or access failures. However, over half (57%) of relapses occurred despite full patient compliance. This indicates that both health-system barriers and treatment response affect real-world outcomes."

Clinical • Real-world • Real-world evidence • Neuromyelitis Optica Spectrum Disorder

Unexpected Pregnancy in a Patient with NMOSD after Treatment with Inebilizumab: A Case Report (AAN 2026) - "Following confirmation of pregnancy, her therapy was adjusted to low-dose methylprednisolone combined with azathioprine. This first reported case provides novel evidence that inebilizumab-induced B-cell depletion exerts a sustained effect, contributing to stable NMOSD control throughout pregnancy and the post-partum period. It also offers pioneering clinical insights into managing biological agents in special populations such as pregnant patients, highlighting that safe treatment adjustments and favorable outcomes can be achieved through multidisciplinary collaboration."

Case report • Clinical • CNS Disorders • Immunology • Infectious Disease • Neuromyelitis Optica Spectrum Disorder • Obstetrics • Rare Diseases

Efficacy and Safety of Inebilizumab in Treating AQP4-Positive NMOSD Patients with Comorbid Autoimmune Diseases (AAN 2026) - "Inebilizumab effectively prevents relapses and disability progression in NMOSD patients with comorbid autoimmune diseases. It may thus serve as a preferred therapeutic option for this patient population."

Clinical • Chronic Cough • CNS Disorders • Cough • Endocrine Disorders • Immunology • Infectious Disease • Inflammatory Arthritis • Lupus • Neuromyelitis Optica Spectrum Disorder • Rare Diseases • Respiratory Diseases • Rheumatoid Arthritis • Sjogren's Syndrome • Systemic Lupus Erythematosus

Severe Flare of Immunoglobulin G4-Related Lung Disease Following Transition From Prednisone to Inebilizumab (ATS 2026) - No abstract available

Pulmonary Disease • Respiratory Diseases

Rescue Treatment of Inebilizumab in Refractory Myasthenia Gravis: A Case Report (AAN 2026) - "He received sequential treatments including plasmapheresis, intravenous immunoglobulin (IVIG) pulse therapy, efgartigimod, and telitacicept, all with suboptimal responses...In June 2024, after two courses of eculizumab combined with telitacicept, he was successfully extubated and discharged... Inebilizumab demonstrates rapid and sustained clinical improvement in refractory myasthenia gravis with a favorable tolerability profile, worth further validation in larger cohorts."

Case report • Clinical • CNS Disorders • Infectious Disease • Myasthenia Gravis • Respiratory Diseases

Adherence and Safety of Intravenous Inebilizumab for NMOSD: Real-world Evidence from China (AAN 2026) - "In this Chinese single-center real-world cohort, intravenous inebilizumab substantially reduced relapse burden with an overall acceptable safety profile. Ongoing monitoring of infection risk and neutrophil levels and longer follow-up are warranted to further validate benefits and safety."

Adherence • Clinical • HEOR • Real-world • Real-world evidence • CNS Disorders • Infectious Disease • Nephrology • Neuromyelitis Optica Spectrum Disorder • Pneumonia • Rare Diseases • Respiratory Diseases

Neurological Manifestations of IgG4-related disease: To Wait Or To Mitigate? (AAN 2026) - "MITIGATE Trial studied Inebilizumab's (Uplizna) efficacy for treatment of IgG4-RD and was recently approved by the Food and Drug Administration (FDA).Design/ We managed three patients with histo-pathological confirmation of neurologically predominant IgG4-RD seen in the Neuroimmunology Clinic... Recognition of IgG4-RD requires high clinical suspicion and histopathologic confirmation. Corticosteroids remain the cornerstone of management but the emergence of Inebilizuamb poses a treatment dilemma in neurological-confined IgG4 since now there is an effective FDA approved drug available."

CNS Disorders • Endocrine Disorders • Infectious Disease • Inflammation • Metabolic Disorders

Efficacy and Safety of Rituximab Versus Inebilizumab in Patients With Neuromyelitis Optica Spectrum Disorder: A Dual-Center, Real-World Cohort Study. (PubMed, Eur J Neurol) - "In our real-world study of 276 patients, rituximab and inebilizumab demonstrated comparable efficacy in the medium term but differed in their safety profiles, with a significantly higher incidence of infusion-related reactions observed in the rituximab group."

Journal • Real-world evidence • CNS Disorders • Neuromyelitis Optica Spectrum Disorder • Rare Diseases