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

Inebilizumab for treatment of NMOSD in a Real-World Cohort: Analysis from the SPHERES Registry (ACTRIMS Forum 2026) - "Of second-line patients treated with inebilizumab, 14 had immediate prior exposure to rituximab, 3 to satralizumab, 2 to non-approved biologics, 3 to glucocorticoid steroids, 1 to immunosuppressant therapy and 1 to immunoglobulin therapy. This descriptive analysis of real-world NMOSD patients who were treated with inebilizumab offers important insights into clinical characteristics and treatment patterns. Additional analysis is being conducted to understand inebilizumab treatment persistence and long-term patient outcomes."

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

Treatment Pathways, Switching, and Barriers to Disease-Modifying Therapy in Hispanic Cohort of NMOSD (ACTRIMS Forum 2026) - "Disease-modifying therapy (DMT) exposures (rituximab, eculizumab, inebilizumab, ravulizumab, satralizumab, azathioprine, mycophenolate, interferons, glatiramer), reasons for discontinuation/switch, adherence/logistics, and EDSS trajectories were compiled. ResultsPatients cycled through a median of two DMTs (range: 1-4) before stabilization. In this largely Hispanic cohort, NMOSD treatment pathways were shaped by barriers as much as biology. Logistics failures, intolerance, and drug failure were the main reasons for switching therapies. Strikingly, nearly half of inebilizumab-treated patients experienced breakthrough relapses, diverging from trial outcomes and signaling a need for close monitoring and timely escalation."

CNS Disorders • Multiple Sclerosis • Neuromyelitis Optica Spectrum Disorder

Recognizing Very Late-Onset Seropositive NMOSD, A Case of Area Postrema Syndrome Followed by Bilateral Optic Neuritis in an Octogenarian (ACTRIMS Forum 2026) - "He had modest clinical improvement with five sessions of plasma exchange, five days of intravenous immunoglobulins (IVIG), and a five-day course of intravenous methylprednisolone followed by an oral prednisone taper. As his steroid taper continues, initiation of inebilizumab as a steroid-sparing maintenance therapy is planned for long-term management of seropositive NMOSD... The median age at onset of NMOSD disorder is approximately 40 years, but the disorder can affect individuals of any age, and up to 20-40% of cases occur in adults older than 50 years. Within this group, very late-onset (≥70 years) is rare, reported in about 11% of patients in one cohort. Importantly, patients with late-onset and very late-onset NMOSD have been observed to experience poorer recovery from acute attacks and more rapid disability accumulation, highlighting the importance of prompt recognition and timely initiation of effective therapy."

Clinical • Cardiovascular • CNS Disorders • Coronary Artery Disease • Dyslipidemia • Gastrointestinal Disorder • Hypertension • Infectious Disease • Neuromyelitis Optica Spectrum Disorder • Novel Coronavirus Disease • Ocular Inflammation • Ophthalmology • Optic Neuritis • Peripheral Arterial Disease • Pneumonia • Rare Diseases • Respiratory Diseases • Solid Tumor

Case Report: NMOSD Presenting as a Paraneoplastic Syndrome in Association with Leptomeningeal Enhancement (ACTRIMS Forum 2026) - "Her symptoms improved with pulse steroids, but she later developed mental status changes during the prednisone taper. Her clinical condition declined, and leptomeningeal enhancement progressed despite treatment with ravulizumab, inebilizumab and additional courses of steroids... Although leptomeningeal enhancement has been reported with AQP4+ NMOSD, it is a rare manifestation, and its presence warrants careful assessment for other etiologies, particularly if not responding as expected to NMOSD directed therapy and in individuals with a history of malignancy."

Case report • Clinical • Brain Cancer • Breast Cancer • CNS Disorders • HER2 Breast Cancer • Immunology • Inflammatory Arthritis • Lupus • Neuromyelitis Optica Spectrum Disorder • Ocular Inflammation • Ophthalmology • Optic Neuritis • Rare Diseases • Rheumatoid Arthritis • Rheumatology • Solid Tumor • Systemic Lupus Erythematosus • PGR

A Comparative Clinical Effectiveness Trial of Rituximab versus Ravulizumab, Inebilizumab, Satralizumab and Eculizumab to Prevent Relapses in Neuromyelitis Optica Spectrum Disorder (NMOSD) (ACTRIMS Forum 2026) - "BEST-NMOSD is the first comparative effectiveness trial comparing all approved NMOSD DMTs to rituximab. The primary endpoint combines efficacy and safety, allowing for a holistic comparison of treatments and reflecting real-world decisions. This study will deliver actionable results for patients, physicians, and regulatory authorities."

Clinical • CNS Disorders • Depression • Inflammation • Multiple Sclerosis • Neuromyelitis Optica Spectrum Disorder • Ophthalmology • Optic Neuritis • Rare Diseases

Outcomes and predictors of relapse and severe pneumonia in Chinese patients with AQP4-IgG-positive neuromyelitis optica spectrum disorder receiving inebilizumab: a prospective cohort study. (PubMed, Front Immunol) - "Patients with early-onset, long-standing, or highly active disease are at greater risk of breakthrough relapses, whereas those with high disability and hypogammaglobulinemia require vigilance for severe pneumonia. These findings advocate for a risk-stratified management approach to optimize treatment outcomes."

Journal • CNS Disorders • Infectious Disease • Neuromyelitis Optica Spectrum Disorder • Pneumonia • Rare Diseases • Respiratory Diseases

Unexpected pregnancy in a patient with AQP4-IgG + NMOSD after treatment with inebilizumab: a case report. (PubMed, BMC Neurol) - No abstract available

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

Industry Therapeutic Update from Amgen: Long Term Maintenance of NMOSD (AAN 2026) - "Learning Objectives Join us for a presentation on UPLIZNA where we will present our 4-year results demonstrating 98% reduction in attacks and a consistent safety profile. This session will examine how this 4+ year data might inform clinical decisions for both the bio-naive and switch patient scenarios."

Clinical • Neuromyelitis Optica Spectrum Disorder

Consensus Recommendations for the Diagnosis and Treatment of Neuromyelitis Optica Spectrum Disorders (NMOSD): The MENACTRIMS Guidelines. (PubMed, CNS Drugs) - "For acute treatment: initiate high-dose intravenous methylprednisolone promptly and use plasma exchange early for severe or steroid-refractory attacks. For long-term immunotherapy, monoclonal antibodies (rituximab, inebilizumab, eculizumab, ravulizumab, satralizumab, or tocilizumab) are recommended according to availability and patient factors; conventional immunosuppressants remain alternatives when biologics are inaccessible. Guidance is provided for pediatric patients and for pregnancy and breastfeeding, including planning after ≥ 12 months of disease stability and early postpartum treatment resumption. These MENACTRIMS guidelines aim to improve NMOSD outcomes across the region by promoting accurate diagnosis and timely, effective therapy."

Journal • Review • CNS Disorders • Immunology • Multiple Sclerosis • Neuromyelitis Optica Spectrum Disorder • Pediatrics • Rare Diseases • Solid Tumor

Product Sales Performance (PRNewswire) - "Repatha (evolocumab) sales increased 44% year-over-year to $870 million in the fourth quarter...UPLIZNA (inebilizumab-cdon) sales increased 131% year-over-year to $233 million in the fourth quarter and 73% for the full year, primarily driven by volume growth...TEZSPIRE (tezepelumab-ekko) sales increased 60% year-over-year to $474 million in the fourth quarter...Otezla (apremilast) sales were flat year-over-year at $625 million in the fourth quarter...Enbrel (etanercept) sales decreased 48% year-over-year to $532 million in the fourth quarter...PAVBLU (aflibercept-ayyh) generated $258 million of sales in the fourth quarter and $700 million for the full year....WEZLANA (ustekinumab-auub)/WEZENLA (ustekinumab) generated $44 million of sales in the fourth quarter and $273 million for the full year."

Sales • Ankylosing Spondylitis • Asthma • Cardiovascular • Chronic Rhinosinusitis With Nasal Polyps • Crohn's disease • Diabetic Macular Edema • Diabetic Retinopathy • Familial Hypercholesterolemia • Immunology • Myasthenia Gravis • Neuromyelitis Optica Spectrum Disorder • Psoriasis • Psoriatic Arthritis • Retinal Vein Occlusion • Rheumatoid Arthritis • Ulcerative Colitis • Wet Age-related Macular Degeneration

Changes in Blood Cells and Complements During Relapse Prevention Therapies for Aquaporin-4 Antibody-Positive Neuromyelitis Optica Spectrum Disorder. (PubMed, Int J Mol Sci) - "They were divided into the following treatment groups: glucocorticoids and/or immunosuppressants (GC/IS, n = 22), inebilizumab/rituximab (anti-CD19/20, n = 13), satralizumab (anti-IL-6R, n = 22), and eculizumab/ravulizumab (anti-C5, n = 13). It also showed that anti-C5 therapy strongly suppressed total complement activity but did not affect the C3 and C4 levels or blood counts. These findings may have implications for the mode of action of the drugs and the risk of adverse drug reactions, including infections."

Journal • CNS Disorders • Infectious Disease • Neuromyelitis Optica Spectrum Disorder • Rare Diseases

The evolution and current status of anti-B-cell therapies in autoimmune neurologic diseases. (PubMed, Handb Clin Neurol) - "With their increasing use, it is important to be aware of their side-effect profile, associated risk of infection, and their use in special populations. Anti-B-cell therapies have revolutionized our therapeutic approach to autoimmune disorders of the central nervous system and their high efficacy has led to an overall benefit in patients afflicted by these conditions."

Journal • Review • CNS Disorders • Immunology • Infectious Disease • Multiple Sclerosis • Neuromyelitis Optica Spectrum Disorder • Rare Diseases • CD4

Patient Preferences for Neuromyelitis Optica Spectrum Disorder (NMOSD) Treatments: Results from a Discrete Choice Experiment Study in the USA. (PubMed, Patient) - "Respondents with AQP4-Ab+ NMOSD placed the highest importance on reducing chance of relapse, placed a high importance on reduced administration frequency, and rated the safety attributes (avoiding treatments with a risk of meningococcal infection, risk of elevated liver enzymes, and risk of other infections) as comparable to each other but less influential than relapse prevention. Respondents were more likely to select a ravulizumab-like profile over comparators. These findings can inform shared decision-making in selecting treatments."

Journal • CNS Disorders • Infectious Disease • Meningococcal Infections • Neuromyelitis Optica Spectrum Disorder • Rare Diseases

Real-World Efficacy and Safety of Neuromyelitis Optica Spectrum Disorder Disease-Modifying Treatments. (PubMed, Neurol Neuroimmunol Neuroinflamm) - "Clinicians should consider using NMOSD-approved treatments given their favorable efficacy and safety profiles in the real-world setting. MMF and azathioprine should be avoided. We caution against rituximab as a default first-line given the cumulative risk of relapse, SIAEs, and TLAEs over time."

Journal • Real-world evidence • Retrospective data • CNS Disorders • Infectious Disease • Neuromyelitis Optica Spectrum Disorder • Ophthalmology • Rare Diseases

A rare case of IgG4-related sclerosing cholangitis followed by rapid subsequent diagnosis of cholangiocarcinoma. (PubMed, J Liver Cancer) - "Biliary tract cancer in the setting of IgG4 related disease has been previously described; however, this patient course is novel as it encompasses the spectrum of challenges in IgG4-SC management, including diagnostic uncertainty, risk of infection with immunosuppressive agents, and development of malignancy diagnosed shortly following IgG4-SC diagnosis. We review the literature of management, outcomes, and malignancy risk and furthermore, highlight a promising recent therapy in treatment of IgG4 related disease, inebilizumab."

Journal • Biliary Cancer • Biliary Tract Cancer • Cholangiocarcinoma • Immunology • Infectious Disease • Oncology • Solid Tumor

Cellular and humoral vaccination response under immunotherapies-German consensus on vaccination strategies in neurological autoimmune diseases. (PubMed, Ther Adv Neurol Disord) - "The specific humoral and cellular response to vaccination can be compromised under alemtuzumab, azathioprine, cladribine, cyclophosphamide, CD19/CD20 antibodies (inebilizumab, ocrelizumab, ofatumumab, rituximab, ublituximab), dimethyl fumarate/diroximel fumarate, FcRn inhibitors (efgartigimod, rozanolixizumab), complement C5 inhibitors (eculizumab, ravulizumab, zilucoplan), interleukin-6 receptor antibodies (tocilizumab, satralizumab), intravenous immunoglobulins, long-term steroid administration, methotrexate, mitoxantrone, mycophenolate mofetil, tacrolimus, teriflunomide, tumor necrosis factor-α blockers, and sphingosine-1-phosphate receptor modulators (fingolimod, ozanimod, ponesimod, siponimod), as well as after autologous stem cell transplantation...However, the humoral and cellular vaccination response may be impaired under immunotherapy necessitating close monitoring. Here, we provide applicable recommendations to optimize immunization for individuals receiving..."

Journal • CNS Disorders • Immunology • Infectious Disease • Oncology • Transplantation • IL6R

Inebilizumab: Pediatric Case Series in Anti-Aquaporin 4 Antibody Positive Neuromyelitis Optica Spectrum Disorder. (PubMed, Pediatr Neurol) - "This study adds to the literature because it shows that in our cohort inebilizumab was a safe medication for pediatric patients with NMOSD and prevented further relapses."

Journal • CNS Disorders • Immunology • Neuromyelitis Optica Spectrum Disorder • Pediatrics • Rare Diseases

On 11 December 2025, the Committee for Medicinal Products for Human Use (CHMP) adopted a positive opinion, recommending a change to the terms of the marketing authorisation for the medicinal product Uplizna. The marketing authorisation holder for this medicinal product is Amgen Europe B.V. (European Medicines Agency) - "The CHMP adopted a new indication as follows: Neuromyelitis optica spectrum disorders (NMOSD)...Uplizna is indicated for the treatment of adult patients with active IgG4 RD....Uplizna is indicated as an add-on to standard therapy for the treatment of gMG in adult patients who are anti-acetylcholine receptor (AChR) or anti-muscle-specific tyrosine kinase (MuSK) antibody positive."

CHMP • Immunology • Myasthenia Gravis • Neuromyelitis Optica Spectrum Disorder

A recalcitrant case of pemphigus treated with inebilizumab. (PubMed, JAAD Case Rep) - No abstract available

Journal • Dental Disorders • Dermatology • Immunology • Oncology • Oral Cancer • Pemphigus Vulgaris • Stomatitis

Amgen…announced that the U.S. Food and Drug Administration (FDA) has approved UPLIZNA (inebilizumab-cdon) for the treatment of generalized myasthenia gravis (gMG) in adults who are anti-acetylcholine receptor (AChR) and anti-muscle specific tyrosine kinase (MuSK) antibody positive. (Amgen Press Release) - "The approval of UPLIZNA for gMG is supported by data from the Myasthenia Gravis Inebilizumab Trial (MINT), the largest Phase 3 biologic study to include both AChR+ and MuSK+ patients, and the first to successfully incorporate a steroid taper into its protocol."

FDA approval • Myasthenia Gravis

Consensus recommendations for the diagnosis and management of neuromyelitis optica spectrum disorder: A Saudi expert panel review. (PubMed, Mult Scler Relat Disord) - "This expert consensus provides evidence-based guidance for the diagnosis and management of NMOSD in Saudi Arabia, striking a balance between clinical evidence and expert opinion where data gaps exist. It emphasises individualised care, antibody testing, and long-term immunosuppressive strategies while highlighting the need for further research on treatment duration and failure criteria."

Journal • Review • CNS Disorders • Immunology • Multiple Sclerosis • Neuromyelitis Optica Spectrum Disorder • Rare Diseases

What is in the Neuromuscular Junction Literature? (PubMed, J Clin Neuromuscul Dis) - "The role of thymectomy in older patients with MG is considered. The medical treatment section addresses corticosteroid regimens, intravenous immunoglobulin as maintenance therapy, a phase 3 study of the recently approved neonatal Fc receptor (FcRN) blocker nipocalimab, use of complement inhibitors and FcRN blockers in general, regimens for efgartigimod, and positive studies on the interleukin-16 receptor monoclonal antibody (Ab) satralizumab and the CD19 B-cell-depleting monoclonal Ab inebilizumab."

Journal • Review • CNS Disorders • Myasthenia Gravis • Ophthalmology • Thyroid Eye Disease • IL16

IgG4-Related disease: From diagnosis to remission. (PubMed, Best Pract Res Clin Rheumatol) - "Treatment strategies have evolved significantly with the introduction of B cell depletion therapy, particularly inebilizumab...Critical knowledge gaps persist regarding optimal biomarkers for disease activity, standardized remission criteria, and the precise etiopathogenesis of IgG4-RD. Despite therapeutic advances, challenges remain in balancing effective disease control with minimizing the harms of long-term immunosuppression, emphasizing the need for continued research into targeted therapies."

Journal • Review • Fibrosis • Immunology • Inflammation • Pancreatitis

Risk of hepatitis B and tuberculosis reactivation in patients with neuromyelitis optica spectrum disorder undergoing B cell depletion therapy. (PubMed, Mult Scler Relat Disord) - "Patients with NMOSD who were HBsAg-negative and anti-HBc-positive or had LTBI had no observed instances of HBVr/TBr while receiving B cell depletion therapy during the follow-up period, with or without prophylactic anti-HBV or anti-TB treatment."

Journal • CNS Disorders • Hepatitis B • Infectious Disease • Inflammation • Neuromyelitis Optica Spectrum Disorder • Pulmonary Disease • Rare Diseases • Respiratory Diseases • Tuberculosis

Inebilizuma treatment for acute serum AQP4 - IgG neuromyelitis optica: Case report. (PubMed, Medicine (Baltimore)) - "This case demonstrates that combination therapy with IVMP and inebilizumab during the acute phase significantly promoted visual recovery in this AQP4-IgG seropositive NMOSD patient. This finding enhances our understanding of NMOSD management. The case offers valuable clinical insights for therapeutic strategies in NMOSD. Future studies with larger cohorts are warranted to further evaluate the efficacy of the intravenous methylprednisolone pulse-inebilizumab combination in reducing relapse rates and improving long-term neurological outcomes in NMOSD."

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