The U.S. Food and Drug Administration (FDA) has approved Uplizna (inebilizumab-cdon) for the treatment of adults with immunoglobulin G4-related disease (IgG4-RD).
IgG4-RD
IgG4-RD is an immune-mediated condition that can affect multiple organ systems. Common features include IgG4-related autoimmune pancreatitis, swelling of or within an organ system , salivary gland disease (which can lead to enlargement of the salivary glands), swollen lymph nodes, skin manifestations, and symptoms consistent with allergies or asthma. The cause of IgG4-RD is unknown.
Inebilizumab
Inebilizumab is a humanized monoclonal antibody that targets the depletion of autoantibody-producing CD19+ B cells that contribute to the underlying disease. It is administered as two initial infusions, followed by one dose every six months.
Clinical Trial
The FDA approval follows positive data from the MITIGATE trial. This study was a randomized, double-blind, placebo-controlled, parallel-group, multicenter clinical trial evaluating the safety and efficacy of inebilizumab in reducing risk of flares in adults with IgG4-RD. The primary endpoint was time to first treated and adjudicated IgG4-RD flare. Secondary endpoints included annualized flare rate, flare- and treatment-free complete remission, and flare- and corticosteroid-free complete remission.
Key findings observed in this study included an 87% reduction in the risk of flare compared to placebo and a reduction in annualized flare rate for treated and adjudicated flare at 0.10 for patients receiving inebilizumab compared to 0.71 for patients receiving placebo. Additionally, 57.4% achieved flare-free, treatment-free, and complete remission at week 52 compared to 22.4% for the placebo group. Achievement of flare-free, corticosteroid-free, and complete remission at week 52 was at 58.8% in the inebilizumab group, and 22.4% in the placebo group.
The safety profile of inebilizumab was favorable. The most common adverse events in patients were urinary tract infection and lymphopenia.
This approval is the second indication for inebilizumab, which has previously been approved for the treatment of adult patients with AQP4-IgG+ neuromyelitis optica spectrum disorder (NMOSD). The treatment is also being evaluated for the treatment of generalized myasthenia gravis.
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To learn more about rare autoimmune disorders, visit https://checkrare.com/diseases/autoimmune-and-auto-inflammatory-disorders/
