Uplizna

Uplizna

Uplizna is an intravenous (IV) medication that targets CD19, a protein found on B-cells, leading to their depletion. This mechanism helps manage certain autoimmune conditions.

Conditions Treated 

Uplizna is approved for the treatment of:

Neuromyelitis Optica Spectrum Disorder (NMOSD) in adults who are anti-aquaporin-4 (AQP4) antibody-positive. It has been shown to significantly reduce the risk of relapse in these patients.

Immunoglobulin G4-related disease (IgG4-RD) in adults, where it has been effective in reducing the risk of disease flares.

Administration 

Uplizna is given as an intravenous (IV) infusion. The typical dosing schedule involves two initial infusions two weeks apart, followed by maintenance infusions every six months. To minimize the risk of infusion-related reactions, patients are usually premedicated with a corticosteroid, an antihistamine, and an antipyretic (such as acetaminophen and diphenhydramine) before each infusion. These reactions are most common with the first infusion but can occur at any time. The administration of Uplizna requires an experienced clinician and access to medical support for managing potential severe reactions.

Important Warnings and Side Effects

Infections

Before starting Uplizna, patients will be screened for hepatitis B virus and active or latent tuberculosis [9][15]. Treatment should be delayed if there is an active infection until it has resolved.

Vaccinations

Live or live-attenuated vaccines should be administered at least 4 weeks before starting Uplizna, and non-live vaccines at least 2 weeks prior. Live or live-attenuated vaccines should not be given during treatment or until B-cell counts have recovered after stopping the medication.

Hypogammaglobulinemia

Uplizna can cause a decrease in immunoglobulin levels (IgG and IgM) over time, which may increase the risk of recurrent or opportunistic infections. Immunoglobulin levels will be monitored, and in some cases, immunoglobulin replacement therapy may be considered.

Infusion-Related Reactions

These are uncommon but can be severe. Premedication helps reduce this risk.

Pregnancy

If a mother is treated with Uplizna during pregnancy, the infant's B-cells may be depleted, which could increase risks associated with live or live-attenuated vaccines for the infant.

Other Side Effects

Common side effects reported include back pain, joint pain (arthralgia), fever, and muscle pain (myalgia) [18]. Lymphopenia (low lymphocyte count) has also been observed.

Monitoring 

Before starting treatment, patients will undergo screening for hepatitis B and tuberculosis, and have their quantitative serum immunoglobulins tested. Liver function tests (ALT, AST, total bilirubin), complete blood count (CBC) with differential, and creatinine with eGFR are also part of the pretreatment investigations. Immunoglobulin G (IgG) levels will be monitored yearly during treatment.

This information is not a substitute for professional medical advice. Always consult with your healthcare provider for personalized advice and to discuss any concerns you may have about Uplizna.