Frequently Asked Questions

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What is neuromyelitis optica spectrum disorder (NMOSD)?

NMOSD (historically known as Devic’s disease) is a rare autoimmune disease involving axonal demyelination, which is characterized by repeat attacks and accumulating, often permanent disability. In the United States, it affects ~22,000 people. NMOSD is most prevalent in women and African and Asian populations.^1-3 Explore the demographics of NMOSD
What is the etiology of NMOSD?

The exact cause of NMOSD is still unknown. However, emerging evidence implicates deficits in B-cell tolerance as a potential trigger of autoimmunity in NMOSD, similar to other autoantibody-mediated diseases.^2,4 Explore clinical features of NMOSD
Do NMOSD attacks lead to disability?

Devastating attacks in NMOSD often lead to permanent disability, including blindness and paralysis. In one study of 175 patients with NMOSD, 67% of patients with optic neuritis and 83% of patients with transverse myelitis had little or no recovery.^5-7 See how NMOSD can cause permanent disability
How can I distinguish NMOSD from multiple sclerosis?

While NMOSD and multiple sclerosis (MS) do share certain clinical presentations and symptoms, NMOSD can be differentiated from MS through MRI distinction, certain symptomatology, and aquaporin-4 immunoglobulin (AQP4-IgG) testing.^5,7-13See similarities and differences between MS and NMOSD
What biomarker tests help diagnose NMOSD?

AQP4-IgG antibodies are present in up to 90% of patients with NMOSD. Among available tests, serum cell-based assays are the most sensitive method of detecting aquaporin-4 immunoglobulin (AQP4-IgG) antibodies.^7,11-13 Read more about cell-based assays in NMOSD
What role do B cells play in NMOSD?

B cells play multiple roles in the immunopathogenesis of NMOSD, including stimulating T cells and secreting cytokines. However, a pivotal role is played by plasmablasts and plasma B cells, which are largely responsible for the production of aquaporin-4 immunoglobulin (AQP4-IgG), a primary pathogenic driver of NMOSD.^14-16 Explore the role of B cells in NMOSD
What is the mechanism of action of UPLIZNA?

The precise mechanism by which UPLIZNA exerts its therapeutic effects in NMOSD is unknown. UPLIZNA is a B-cell depleter that binds to CD19. UPLIZNA is the only FDA-approved therapy for NMOSD that eliminates a primary cellular source of AQP4-IgG production by reducing CD19+ B cells, including plasmablasts and some plasma cells.^14-17 Learn more about the molecular attributes of UPLIZNA
What was the pivotal trial that studied UPLIZNA?

N-MOmentum is the largest clinical trial in NMOSD to date (N=230). N-MOmentum was a phase 2/3, multicenter, double-blind, randomized (3:1) placebo-controlled trial with an open-label extension period.^18-23 Learn more about the study design of N-MOmentum
How did the pivotal trial of UPLIZNA define an NMOSD attack?

The N-MOmentum trial used NMOSD-specific 18-point attack criteria to ensure investigators captured any sign or symptom of a new attack, from the most subtle to the most severe.^18,23 Explore the attack adjudication process
Does UPLIZNA reduce NMOSD attacks?

UPLIZNA delivered significant reduction in attacks through the first 28 weeks of treatment in the randomized controlled period (RCP) of N-MOmentum. In a post hoc analysis of patients who remained on UPLIZNA through the open-label period (OLP), attack rate reductions were sustained over time.^17,24 Learn more about the primary endpoint
Does UPLIZNA have long-term efficacy data?

The OLP of N-MOmentum demonstrated reduction in annualized attack rate over 2.5+ years.²⁴ Consider open-label treatment phase study limitations when interpreting results. The OLP was not blinded, not controlled, and included inherent self-selection bias for remaining in the trial. During the OLP, a total of 42 patients (19.4%) discontinued, of which 5 patients (2.3%) discontinued due to adverse events. Explore long-term attack-risk reduction
What additional efficacy data support UPLIZNA?

In addition to protecting patients against NMOSD attacks, UPLIZNA has demonstrated statistically significant reductions in lowering the risk of worsening disability (as measured by EDSS) and inpatient hospitalizations.^17,23 The EDSS analysis included visits during an attack, with EDSS progression driven by the expected increase in EDSS at the time of attack. Therefore, these results should not be interpreted as impacting disability independent of attack reduction. Additionally, subjects were not followed up for the same amount of time, resulting in missing data which may have introduced biases into the analysis. Subjects with missing data are imputed as worsening. Explore efficacy beyond attacks
What are the most common side effects with UPLIZNA?

Urinary tract infection and arthralgia were adverse events with an incidence of ≥10% and were seen more frequently with UPLIZNA than with placebo.¹⁷ Explore safety data
What is the risk for infusion-related reactions with UPLIZNA?

UPLIZNA can cause infusion reactions, which can include headache, nausea, somnolence, dyspnea, fever, myalgia, rash, or other symptoms. In the 28-week RCP, rates of infusion reactions with UPLIZNA were 9% vs 10% with placebo. All infusion-related reactions in patients receiving UPLIZNA were Grades 1 or 2. A corticosteroid, an antihistamine, and an antipyretic are given 30 to 60 minutes prior to administration to reduce infusion reactions.^17,18,23 Explore safety data
Are there head-to-head trials between UPLIZNA and rituximab?

No head-to-head trials have been conducted between UPLIZNA and rituximab or between UPLIZNA and any treatment that is currently approved for the treatment of NMOSD. The N-MOmentum trial did include 17 patients with prior rituximab experience, and a post hoc analysis was done to investigate outcomes in these patients.²⁵ Explore results in rituximab-experienced patients
What is the dosing schedule for UPLIZNA?

UPLIZNA is infused twice-yearly following 2 initial loading doses at Day 1 and Day 15 of treatment.¹⁷ Learn more about dosing
How is UPLIZNA administered?

UPLIZNA is administered as an intravenous infusion. The recommended dosage includes a 300-mg infusion followed 2 weeks later by a second 300-mg infusion. Subsequent doses (starting 6 months from the first infusion) are a single 300-mg infusion every 6 months for chronic usage. A corticosteroid, an antihistamine, and an antipyretic are given 30 to 60 minutes prior to administration to reduce infusion reactions.^17,18 Learn more about administration
What support is available for patients taking UPLIZNA?

Amgen By Your Side is a support program for patients prescribed UPLIZNA. Our dedicated team is your patient’s partner, committed to providing nonmedical support so they can start and continue on treatment as you prescribe. Learn more about Amgen By Your Side

Enroll Patient

Download the form, complete it with your patient, including HCP signature, and submit it by fax or email.

Contact the UPLIZNA Team

Connect with an UPLIZNA representative via email or phone to learn more about treatment information, patient support, and more.

IMPORTANT SAFETY INFORMATION AND INDICATIONS

CONTRAINDICATIONS

UPLIZNA^® (inebilizumab-cdon) is contraindicated in patients with a history of a life-threatening infusion reaction to UPLIZNA, active hepatitis B infection, or active or untreated latent tuberculosis.

WARNINGS AND PRECAUTIONS

Infusion Reactions: Can cause infusion reactions, including anaphylaxis. Symptoms can include headache, nausea, somnolence, dyspnea, fever, myalgia, rash, or palpitations. During the randomized clinical trial period (RCP), infusion reactions were observed with the first course of UPLIZNA in 9.3% of NMOSD patients. Infusion reactions of UPLIZNA were observed in 7.4% of IgG4-RD patients during the RCP. Infusion reactions were most common with the first infusion but were also observed during subsequent infusions.

Administer pre-medication with a corticosteroid, an antihistamine, and an antipyretic. For life-threatening infusion reactions, immediately and permanently stop UPLIZNA and administer appropriate supportive treatment. For less severe infusion reactions, management may involve temporarily stopping the infusion, reducing the infusion rate, and/or administering symptomatic treatment.
Infections: An increased risk of infections has been observed with other B-cell depleting therapies. The most common infections reported by UPLIZNA-treated patients in the NMOSD RCP and open-label clinical trial periods were urinary tract infection (20%), nasopharyngitis (13%), upper respiratory tract infection (8%), and influenza (7%). In the IgG4-RD RCP and open-label period, the most common infections reported by UPLIZNA-treated patients were upper respiratory tract infection (11%), nasopharyngitis (10%), urinary tract infection (9%), and influenza (6%). Delay UPLIZNA administration in patients with an active infection until the infection is resolved.

Possible Increased Risk of Immunosuppressant Effects with Other Immunosuppressants: UPLIZNA has not been studied in combination with other immunosuppressants. If combining UPLIZNA with another immunosuppressive therapy, consider the potential for increased immunosuppressive effects.

Hepatitis B Virus (HBV) Reactivation: Risk of HBV reactivation has been observed with other B-cell depleting antibodies. There have been no cases of HBV reactivation in patients treated with UPLIZNA, but patients with chronic HBV infection were excluded from clinical trials. Perform HBV screening in all patients before initiation of treatment. Do not administer to patients with active hepatitis. For patients who are chronic carriers of HBV [HBsAg+], consult liver disease experts before starting and during treatment.

Progressive Multifocal Leukoencephalopathy (PML): Although no confirmed cases of PML were identified in UPLIZNA clinical trials, JC virus infection resulting in PML has been observed in patients treated with other B-cell depleting antibodies and other therapies that affect immune competence. In UPLIZNA clinical trials one subject died following the development of new brain lesions for which a definitive diagnosis could not be established, though the differential diagnosis included an atypical NMOSD relapse, PML, or acute disseminated encephalomyelitis. At the first sign or symptom suggestive of PML, withhold UPLIZNA and perform an appropriate diagnostic evaluation. MRI findings may be apparent before clinical signs or symptoms. Typical symptoms associated with PML are diverse, progress over days to weeks, and include progressive weakness on one side of the body or clumsiness of limbs, disturbance of vision, and changes in thinking, memory, and orientation leading to confusion and personality changes.

Tuberculosis
Patients should be evaluated for tuberculosis risk factors and tested for latent infection prior to initiating UPLIZNA. Consider anti-tuberculosis therapy prior to initiation of UPLIZNA in patients with a history of latent active tuberculosis in whom an adequate course of treatment cannot be confirmed, and for patients with a negative test for latent tuberculosis but having risk factors for tuberculosis infection. Consult infectious disease experts regarding whether initiating anti-tuberculosis therapy is appropriate before starting treatment.

Vaccinations
Administer all immunizations according to immunization guidelines at least 4 weeks prior to initiation of UPLIZNA. The safety of immunization with live or live-attenuated vaccines following UPLIZNA therapy has not been studied, and vaccination with live-attenuated or live vaccines is not recommended during treatment and until B-cell repletion.
Vaccination of Infants Born to Mothers Treated with UPLIZNA During Pregnancy
In infants of mothers exposed to UPLIZNA during pregnancy, do not administer live or live-attenuated vaccines before confirming recovery of B-cell counts in the infant. Depletion of B-cells in these exposed infants may increase the risks from live or live-attenuated vaccines. Non-live vaccines, as indicated, may be administered prior to recovery from B-cell and immunoglobulin level depletion, but consultation with a qualified specialist should be considered to assess whether a protective immune response was mounted.
Reductions in Immunoglobulins: There may be a progressive and prolonged hypogammaglobulinemia or decline in the levels of total and individual immunoglobulins such as immunoglobulins G and M (IgG and IgM) with continued UPLIZNA treatment. Monitor the levels of quantitative serum immunoglobulins during treatment with UPLIZNA, especially in patients with opportunistic or recurrent infections, and until B-cell repletion after discontinuation of therapy. Consider discontinuing UPLIZNA therapy if a patient with low immunoglobulin G or M develops a serious opportunistic infection or recurrent infections, or if prolonged hypogammaglobulinemia requires treatment with intravenous immunoglobulins.
Fetal Risk: Based on animal data, UPLIZNA can cause fetal harm due to B-cell lymphopenia and reduce antibody response in offspring exposed to UPLIZNA even after B-cell repletion. Transient peripheral B-cell depletion and lymphocytopenia have been reported in infants born to mothers exposed to other B-cell depleting antibodies during pregnancy. Advise females of reproductive potential to use effective contraception while receiving UPLIZNA and for at least 6 months after the last dose.

ADVERSE REACTIONS

The most common adverse reactions in NMOSD (at least 10% of patients treated with UPLIZNA and greater than placebo) were urinary tract infection and arthralgia.
The most common adverse reactions in IgG4-RD (at least 10% of patients treated with UPLIZNA and greater than placebo) were urinary tract infections and lymphopenia.

INDICATIONS

UPLIZNA^® (inebilizumab-cdon) is indicated for the treatment of neuromyelitis optica spectrum disorder (NMOSD) in adult patients who are anti-aquaporin-4 (AQP4) antibody positive.

UPLIZNA^® is indicated for the treatment of Immunoglobulin G4-related disease (IgG4-RD) in adult patients.

Please see UPLIZNA Full Prescribing Information.