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

B-cell centrality dictates therapeutic efficacy across autoimmune diseases: a systematic review. (PubMed, Front Immunol) - "We observed that autoantibody-mediated diseases pemphigus vulgaris and myasthenia gravis had the most superior and durable immune response to B-cell depletion by rituximab and inebilizumab, respectively...Combination therapy using belimumab and rituximab resulted in a significantly better clinical outcome than monotherapy in Sjögren's syndrome...The success of B-cell therapeutics appears to align with B-cell contribution, disease biology, and the depth of B-cell targeting in autoimmune conditions. Precision targeting is needed to effectively combat autoimmune diseases."

Journal • Review • CNS Disorders • Dermatology • Immunology • Inflammatory Arthritis • Myasthenia Gravis • Pemphigus Vulgaris • Rheumatoid Arthritis • Rheumatology • Scleroderma • Sjogren's Syndrome • Systemic Sclerosis

IgG4-Related Sclerosing Cholangitis Masquerading as PSC in Ulcerative Colitis (ACG 2026) - "Case Description/ A patient with ulcerative pancolitis (diagnosed in 2021, on Stelara) was referred in 2023 for persistent cholestatic liver enzyme elevation (AST 112, ALT 161, ALP 501, total bilirubin 1.5)...Ursodiol led to partial improvement...Prednisone 40 mg daily led to rapid biochemical and symptomatic improvement...Transition to inebilizumab is planned...PSC with elevated IgG4 remains a consideration, but the clinical course favors IgG4-RD. Accurate distinction is essential given differing treatment and prognosis."

Cholestasis • Fibrosis • Gastroenterology • Gastrointestinal Disorder • Hepatology • Immunology • Inflammation • Inflammatory Bowel Disease • Primary Sclerosing Cholangitis • Ulcerative Colitis

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

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

Immunogenicity and Impact of Anti-Drug Antibodies on the Efficacy and Pharmacokinetics of Inebilizumab in a Phase 3 Trial in IgG4-Related Disease (ACR Convergence 2026) - No abstract available

Clinical • P3 data • PK/PD data • Inflammation

Efficacy and Safety of Inebilizumab for the Treatment of IgG4-RD Across Age Subgroups: Analysis from the Open-label Period of the MITIGATE Trial (ACR Convergence 2026) - No abstract available

Clinical • Inflammation

Long-Term Inebilizumab Treatment Results in Mild Immunoglobulin Reduction but No Increase in Infection Risk (ACR Convergence 2026) - No abstract available

Infectious Disease

Efficacy and Safety of Inebilizumab in Newly Diagnosed and Recurrent IgG4-RD Patients: Results From the Open-Label Period of the MITIGATE Study (ACR Convergence 2026) - No abstract available

Clinical • Inflammation

A New ICD-10 Code Opens a Real-World Window on IgG4-Related Disease: Propensity-Matched Outcomes of Rituximab Versus Conventional Immunosuppression, With an Early Look at Inebilizumab (ACR Convergence 2026) - No abstract available

Clinical • Real-world • Real-world evidence • Inflammation

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

Clinical • P3 data • Inflammation

Autoimmune inflammatory diseases of the central nervous system: advances in clinical trials and immunotherapeutic strategies. (PubMed, Reumatologia) - "In AE, first-line immunotherapies (high-dose glucocorticoids, intravenous immunoglobulin, and plasma exchange) remain based mainly on observational data, while second-line therapies such as rituximab and cyclophosphamide are increasingly used despite limited comparative trial data. Recent systematic reviews and individual patient data meta-analyses have highlighted both the potential and the limitations of existing observational evidence, and several randomized phase II-III trials are now underway evaluating agents including bortezomib, satralizumab, and inebilizumab. In NMOSD, the therapeutic landscape has advanced more rapidly, with multiple pivotal randomized controlled trials of targeted biologics (e.g. complement inhibition with eculizumab, B-cell depletion with inebilizumab, interleukin-6 [IL-6] receptor blockade with satralizumab) demonstrating large reductions in relapse risk and leading to regulatory approvals...Emerging strategies include use of adaptive and..."

Journal • Review • CNS Disorders • Immunology • Inflammation • Multiple Sclerosis • Neuromyelitis Optica Spectrum Disorder • Pediatrics • Psychiatry • Rare Diseases • Solid Tumor • IL6

Tanabe Pharma Receives Approval in Japan for additional indication of generalized Myasthenia Gravis Anti-CD19 monoclonal antibody UPLIZNA for I.V. Infusion 100 mg (Mitsubishi Tanabe Press Release) - "The approval of UPLIZNA was based on data from the Myasthenia Gravis Inebilizumab Trial (MINT), which is being conducted in collaboration with Amgen Inc., the licensor of UPLIZNA."

Japan approval • Immunology • Myasthenia Gravis

Comparative effectiveness of inebilizumab versus conventional immunotherapies in AQP4-IgG-positive NMOSD: a prospective cohort study with time-varying treatment effects. (PubMed, J Neurol Neurosurg Psychiatry) - "Inebilizumab was associated with better relapse and disability outcomes than AZA or MMF. Compared with RTX, inebilizumab was associated with lower ARR, whereas no significant differences were detected in time to first relapse or disability progression."

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

IgG4-related disease - treatment with anti-CD20, anti-CD19 antibodies, and other new drugs. (PubMed, Klin Onkol) - "Anti-CD20 and anti-CD19 antibodies represent the treatment of choice for IgG4-RD. They achieve a high number of complete treatment responses; however, the treatment does not end there, as it is necessary to prevent disease relapses, which are common without maintenance therapy."

Journal • Review • Eosinophilia • Fibrosis • Immunology • Inflammation • Oncology • Renal Disease

Safety and Effectiveness of Inebilizumab in Patients with Neuromyelitis Optica Spectrum Disorder: Interim Analysis of a Post-marketing Surveillance in Japan. (PubMed, Neurol Ther) - P2/3 | "In this interim analysis, the safety profile of inebilizumab was consistent with prior evidence and no new safety concerns were identified. Inebilizumab reduced relapse rates and decreased concomitant use of oral glucocorticoids and immunosuppressants in patients with NMOSD."

Journal • P4 data • Cardiovascular • CNS Disorders • Infectious Disease • Interstitial Lung Disease • Nephrology • Neuromyelitis Optica Spectrum Disorder • Novel Coronavirus Disease • Pneumonia • Pulmonary Disease • Rare Diseases • Respiratory Diseases

Study to Evaluate the Pharmacokinetics, Pharmacodynamics, Safety, and Tolerability of Inebilizumab in Pediatric Participants With IgG4-RD (clinicaltrials.gov) - P2 | N=15 | Not yet recruiting | Sponsor: Amgen | Initiation date: Jul 2026 ➔ Oct 2026

Trial initiation date • Inflammation • Pediatrics

Inebilizumab in AQP4-Seropositive NMOSD: One-Year Follow-Up From a Multicenter, Real-World Study. (PubMed, Ann Clin Transl Neurol) - "Inebilizumab demonstrated real-world effectiveness and a manageable safety profile in a diverse population of patients with AQP4-IgG-seropositive NMOSD, supporting the findings of the pivotal N-MOmentum trial."

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

Inebilizumab Reduces Exacerbations, Rescue Therapy use in Generalized Myasthenia Gravis (NeurologyLive) - "The analysis...included 238 participants from MINT, an international, randomized, placebo-controlled phase 3 trial....By week 26, 16% of participants treated with inebilizumab experienced an exacerbation compared with 35% of those receiving placebo. In the AChR-positive subgroup, exacerbations occurred in 16.8% and 32.3% of participants, respectively, while rates in the MuSK-positive subgroup were 12.5% and 45.8%. Overall, inebilizumab treatment reduced the hazard of exacerbation by week 26 in the combined population (HR, 0.41; 95% CI, 0.24-0.70; P = .001). The treatment was also associated with a reduced hazard of exacerbation in the AChR-positive population through week 52 (HR, 0.39; 95% CI, 0.23-0.68; P < .001) and in the MuSK-positive population through week 26 (HR, 0.21; 95% CI, 0.06-0.79; P = .02)."

P3 data • Immunology • Myasthenia Gravis

BioDeformUNet: A Deep Learning Model for Biomechanically Informed Liver Image Registration. (PubMed, medRxiv) - "Our code is available at https://github.com/XinyueZhang831/BioDeformUNET...The inference time of BioDeformUNet was 0.6s per image pair, 0.2s for VoxelMorph, 0.3s for VFA, and 20.2s for Morfeus. BioDeformUNet achieved a similar performance to the biomechanical model-based algorithm but required fewer computational operations, resulting in a 34× speedup in DVF computation."

Journal • Oncology

"The Canadian Drug Expert Committee (CDEC) recommends that inebilizumab be reimbursed as add-on therapy for adult patients with gMG who are AChR or MuSK antibody positive and whose symptoms persist despite conventional therapy with AChEIs, corticosteroids, and/or NSISTs, only if the conditions…are met." (Ottawa (ON): Canadian Agency for Drugs and Technologies in Health)

Reimbursement • Myasthenia Gravis

Management of Exacerbations and Rescue Therapy: A Prespecified Analysis of the Phase 3 Myasthenia Gravis Inebilizumab Randomized Clinical Trial. (PubMed, JAMA Neurol) - P3 | "These findings further support the clinical benefits of inebilizumab in AChR+ and MuSK+ gMG. ClinicalTrials.gov Identifier: NCT04524273."

Clinical • Journal • P3 data • CNS Disorders • Myasthenia Gravis

Neuroimmunological overlap syndromes in optic neuritis, myelitis, and connective tissue diseases. (PubMed, Front Neurol) - "Four biologics inebilizumab (B-cell depletion), satralizumab (IL-6 blockade), and eculizumab/ravulizumab (complement inhibition) are approved for AQP4-IgG-positive NMOSD. Neuroimmunological overlap syndromes should be conceptualized as a unified neuroimmune continuum rather than discrete diseases. Priority research includes biomarker validation, head-to-head comparative cohorts, and randomized trials for MOGAD and CTD-associated neuroinflammation."

Journal • Review • CNS Disorders • Immunology • Inflammation • Neuromyelitis Optica Spectrum Disorder • Ocular Inflammation • Ophthalmology • Optic Neuritis • Rheumatology • GFAP • IFNG • IL17A • TNFA

A Phase 3 study of UPLIZNA in patients with chronic inflammatory demyelinating polyneuropathy (CIDP) will be initiated H2 2026 - H1 2027. (PRNewswire)

New P3 trial • Immunology • Inflammation

Product Sales Performance...Rare Disease (PRNewswire) - "TEPEZZA (teprotumumab-trbw) sales increased 14% year-over-year to $576 million in the second quarter, primarily driven by 6% higher net selling price and 6% volume growth...UPLIZNA (inebilizumab-cdon) sales increased 90% year-over-year to $335 million in the second quarter, primarily driven by volume growth; TAVNEOS (avacopan) sales increased 36% year-over-year to $150 million in the second quarter, driven by volume growth."

Sales • ANCA Vasculitis • Immunology • Myasthenia Gravis • Neuromyelitis Optica Spectrum Disorder • Thyroid Eye Disease • Vasculitis

Patient-reported outcomes in Chinese patients with AQP4-IgG-positive neuromyelitis optica spectrum disorder on inebilizumab therapy: a cross-sectional study. (PubMed, BMC Neurol) - "In this cross-sectional survey, Chinese patients with AQP4-IgG-positive NMOSD receiving inebilizumab reported high satisfaction, perceived multidimensional benefit, and strong treatment continuation willingness. These findings provide exploratory patient-reported evidence regarding the acceptability of inebilizumab in routine practice. However, the observed improvements are self-reported and associative rather than causal; interpretation should account for the cross-sectional, non-comparative design, potential selection and recall biases, and lack of objective outcome validation. Prospective controlled studies are required to confirm these preliminary findings."

Journal • Observational data • CNS Disorders • Neuromyelitis Optica Spectrum Disorder • Pain • Rare Diseases

B cell CD19 is transferred between immune cells in mice and humans. (PubMed, Nat Commun) - "In patients with chronic central nervous system (CNS) demyelination, CD19+ T cells display a pro-inflammatory phenotype and are concomitantly depleted by inebilizumab, an approved anti-CD19 antibody, which raises important considerations for the therapeutic use of monoclonal antibodies overall. Finally, we report that myeloid cells acquire CD19 and functional IgM after phagocytosis of apoptotic B cells and thereby gain functional B cell properties. These findings highlight the commonness of membrane and antigen-transfer between cells, resulting in transmission of cellular function."

Journal • Preclinical • CNS Disorders • Immunology • Multiple Sclerosis