Frequently Asked Questions

Frequently Asked Questions
Open allClose all

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 ~16,000 to ~17,000 people. NMOSD is most prevalent in women and African and Asian populations.1-6 Explore the demographics of NMOSD

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.3,4,6 See how NMOSD can cause permanent disability

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.3,6-12 See similarities and differences between MS and 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.6,10-12 Read more about cell-based assays 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.13-15 Explore the role of B cells in NMOSD

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 eliminates plasmablasts and some plasma cells, a primary driver of NMOSD immunopathogenesis.13-16 Learn more about the molecular attributes of 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.16-20 Learn more about the study design of N-MOmentum

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.17,21 Explore the attack adjudication process

UPLIZNA delivered significant reduction in attacks through the first 28 weeks of treatment in the randomized controlled period of N-MOmentum. In a post hoc analysis of patients who remained on UPLIZNA through the open-label period, annualized attack rate continued to decline over time. Learn more about the primary endpoint

In a post hoc analysis of the open-label period of N-MOmentum, UPLIZNA demonstrated reduction in annualized attack rate over 2.5+ years.22 Explore long-term attack-risk reduction

In addition to protecting patients against NMOSD attacks, UPLIZNA has demonstrated statistically significant reductions in lowering the risk of worsening disability and inpatient hospitalizations.16,21 Explore efficacy beyond attacks

Urinary tract infection and arthralgia were the only adverse events with an incidence of ≥ 10% and were seen more frequently with UPLIZNA than with placebo.16 Explore safety data

UPLIZNA can cause infusion reactions, which can include headache, nausea, somnolence, dyspnea, fever, myalgia, rash, or other symptoms. Rates of infusion-related reactions with UPLIZNA treatment were low. 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.16,17,21 Explore safety data

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.2,3 Explore results in rituximab-experienced patients

UPLIZNA is infused twice-yearly following two initial loading doses at Day 1 and Day 15 of treatment.16 Learn more about dosing

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.16,17 Learn more about administration

Horizon By Your Side is a patient support program for patients prescribed UPLIZNA. Patient Access Liaisons offer a wide array of patient-focused services to help with the logistics of starting treatment. Learn more about Horizon By Your Side

Access and Support

Learn about Horizon By Your Side,
a personalized support program for your patients.

Find Support For Your Patients

Stay Up to Date

Get the latest UPLIZNA information and resources
as soon as they are available.

Sign Up Now

Tx Recommendations

INDICATION AND IMPORTANT SAFETY INFORMATION

INDICATION

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.

IMPORTANT SAFETY INFORMATION

UPLIZNA is contraindicated in patients with:

  • A history of life-threatening infusion reaction to UPLIZNA
  • Active hepatitis B infection
  • Active or untreated latent tuberculosis

WARNINGS AND PRECAUTIONS

Infusion Reactions: UPLIZNA can cause infusion reactions, which can include headache, nausea, somnolence, dyspnea, fever, myalgia, rash, or other symptoms. 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 anti-pyretic.

Infections: The most common infections reported by UPLIZNA-treated patients in the randomized and open-label periods included urinary tract infection (20%), nasopharyngitis (13%), upper respiratory tract infection (8%), and influenza (7%). Delay UPLIZNA administration in patients with an active infection until the infection is resolved.

Increased immunosuppressive effects are possible if combining UPLIZNA with another immunosuppressive therapy.

The risk of Hepatitis B Virus (HBV) reactivation has been observed with other B-cell-depleting antibodies. Perform HBV screening in all patients before initiation of treatment with UPLIZNA. Do not administer to patients with active hepatitis.

Although no confirmed cases of Progressive Multifocal Leukoencephalopathy (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. At the first sign or symptom suggestive of PML, withhold UPLIZNA and perform an appropriate diagnostic evaluation.

Patients should be evaluated for tuberculosis risk factors and tested for latent infection prior to initiating UPLIZNA.

Vaccination with live-attenuated or live vaccines is not recommended during treatment and after discontinuation, until B-cell repletion.

Reduction 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 level of immunoglobulins at the beginning, during, and after discontinuation of treatment with UPLIZNA until B-cell repletion especially in patients with opportunistic or recurrent infections.

Fetal Risk: May cause fetal harm based on animal data. Advise females of reproductive potential of the potential risk to a fetus and to use an effective method of contraception during treatment and for 6 months after stopping UPLIZNA.

Adverse Reactions: The most common adverse reactions (at least 10% of patients treated with UPLIZNA and greater than placebo) were urinary tract infection and arthralgia.

INDICATION AND IMPORTANT SAFETY INFORMATION

INDICATION

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.

IMPORTANT SAFETY INFORMATION

UPLIZNA is contraindicated in patients with:

  • A history of life-threatening infusion reaction to UPLIZNA
  • Active hepatitis B infection
  • Active or untreated latent tuberculosis

WARNINGS AND PRECAUTIONS

Infusion Reactions: UPLIZNA can cause infusion reactions, which can include headache, nausea, somnolence, dyspnea, fever, myalgia, rash, or other symptoms. 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 anti-pyretic.

Infections: The most common infections reported by UPLIZNA-treated patients in the randomized and open-label periods included urinary tract infection (20%), nasopharyngitis (13%), upper respiratory tract infection (8%), and influenza (7%). Delay UPLIZNA administration in patients with an active infection until the infection is resolved.

Increased immunosuppressive effects are possible if combining UPLIZNA with another immunosuppressive therapy.

The risk of Hepatitis B Virus (HBV) reactivation has been observed with other B-cell-depleting antibodies. Perform HBV screening in all patients before initiation of treatment with UPLIZNA. Do not administer to patients with active hepatitis.

Although no confirmed cases of Progressive Multifocal Leukoencephalopathy (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. At the first sign or symptom suggestive of PML, withhold UPLIZNA and perform an appropriate diagnostic evaluation.

Patients should be evaluated for tuberculosis risk factors and tested for latent infection prior to initiating UPLIZNA.

Vaccination with live-attenuated or live vaccines is not recommended during treatment and after discontinuation, until B-cell repletion.

Reduction 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 level of immunoglobulins at the beginning, during, and after discontinuation of treatment with UPLIZNA until B-cell repletion especially in patients with opportunistic or recurrent infections.

Fetal Risk: May cause fetal harm based on animal data. Advise females of reproductive potential of the potential risk to a fetus and to use an effective method of contraception during treatment and for 6 months after stopping UPLIZNA.

Adverse Reactions: The most common adverse reactions (at least 10% of patients treated with UPLIZNA and greater than placebo) were urinary tract infection and arthralgia.

  1. Wingerchuk DM, Hogancamp WF, O’Brien PC, Weinshenker BG. The clinical course of neuromyelitis optica (Devic’s syndrome). Neurology. 1999;53:1107-1114.
  2. Flanagan EP, Cabre P, Weinshenker BG, et al. Epidemiology of aquaporin-4 autoimmunity and neuromyelitis optica spectrum. Ann Neurol. 2016;79(5):775-783. doi:10.1002/ana.2461
  3. Borisow N, Mori M, Kuwabara S, Scheel M, Paul F. Diagnosis and treatment of NMO spectrum disorder and MOG-encephalomyelitis. Front Neurol. 2018;9(888):1-15. doi:10.3389/fneur.2018.00888
  4. Ajmera MR, Boscoe A, Mauskopf J, Candrilli SD, Levy M. Evaluation of comorbidities and health care resource use among patients with highly active neuromyelitis optica. J Neurol Sci. 2018;384:96-103. doi:10.1016/jns.2017.11.022
  5. Bukhari W, Prain KM, Waters P, et al. Incidence and prevalence of NMOSD in Australia and New Zealand. J Neurol Neurosurg Psychiatry. 2017;88(8):632-638. doi:10.1136/jnnp-2016-314839
  6. Jarius S, Ruprecht K, Wildemann B, et al. Contrasting disease patterns in seropositive and seronegative neuromyelitis optica: a multicentre study of 175 patients. J Neuroinflammation. 2012;9(14):1-17. doi:10.1186/1742-2094-9-14
  7. Dutra BG, da Rocha AJ, Nunes RH, Maia ACM Jr. Neuromyelitis optica spectrum disorders: spectrum of MR imaging findings and their differential diagnosis. Radiographics. 2018;38(1):169-193. doi:10.1148/rg.2018170141
  8. MS signs & symptoms. National Multiple Sclerosis Society. Accessed August 8, 2022. https://www.nationalmssociety.org/Symptoms-Diagnosis/MS-Symptoms
  9. National Organization for Rare Disorders. Neuromyelitis optica spectrum disorder. NORD. Updated 2018. Accessed August 8, 2022. https://rarediseases.org/rare-diseases/neuromyelitis-optica/
  10. Hamid SHM, Whittam D, Mutch K, et al. What proportion of AQP4-IgG-negative NMO spectrum disorder patients are MOG-IgG positive? A cross sectional study of 132 patients. J Neurol. 2017;264(10):2088-2094. doi:10.1007/s00415-017-8596-7
  11. Jiao Y, Fryer JP, Lennon VA, et al. Updated estimate of AQP4-IgG serostatus and disability outcome in neuromyelitis optica. Neurology. 2013;81(14):1197-1204. doi:10.1212/WNL.0b013e3182a6cb5c
  12. Wingerchuk DM, Banwell B, Bennett JL, et al; International Panel for NMO Diagnosis. International consensus diagnostic criteria for neuromyelitis optica spectrum disorders. Neurology. 2015;85(2):177-189. doi:0.1212/WNL.0000000000001729
  13. Forsthuber TG, Cimbora DM, Ratchford JN, Katz E, Stüve O. B cell-based therapies in CNS autoimmunity: differentiating CD19 and CD20 as therapeutic targets. Ther Adv Neurol Disord. 2018;11:1-13. doi:10.1177/1756286418761697
  14. Bennett JL, O’Connor KC, Bar-Or A, et al. B lymphocytes in neuromyelitis optica. Neurol Neuroimmunol Neuroinflamm. 2015;2(3):e104. doi:10.1212/NXI.0000000000000104
  15. Baker D, Marta M, Pryce G, et al. Memory B cells are major targets for effective immunotherapy in relapsing multiple sclerosis. EBioMedicine. 2017;16:41-50. doi:10.1016/j.ebiom.2017.01.042
  16. UPLIZNA (inebilizumab) [prescribing information] Horizon.
  17. Cree BAC, Bennett JL, Kim HJ, et al. N-MOmentum study investigators. Inebilizumab for the treatment of neuromyelitis optica spectrum disorder (N-MOmentum): a double-blind, randomised, placebo-controlled phase 2/3 trial. Lancet. 2019;394(10206):1352-1363. doi:10.1016/S0140-6736(19)31817-3
  18. Rensel M, Zabeti A, Mealy MA, et al. Long-term efficacy and safety of inebilizumab in neuromyelitis optica spectrum disorder: Analysis of aquaporin-4–immunoglobulin G-seropositive participants taking inebilizumab for ≥ 4 years in the N-MOmentum trial. Mult Scler. 2021;28(6):925-932. doi:10.1177/13524585211047223
  19. ENSPRYNG® (satralizumab-mwge) [prescribing information]. Genentech, Inc; 2022.
  20. SOLIRIS® (eculizumab) [prescribing information]. Alexion Pharmaceuticals, Inc; 2020.
  21. Data on File. Horizon; June 2020.
  22. Bennett JL, Aktas O, Rees WA, et al. Association between B-cell depletion and attack risk in neuromyelitis optica spectrum disorder: an exploratory analysis from N-MOmentum, a double-blind, randomised, placebo-controlled, multicentre phase 2/3 trial. Lancet. 2022;86(104321):1-19. doi:10.1016/j.ebiom.2022.104321
  23. Flanagan EP, Levy M, Katz E, et al. Inebilizumab for treatment of neuromyelitis optica spectrum disorder in patients with prior rituximab use from the N-MOmentum study. Mult Scler Relat Disord. 2022 Jan;57:103352. doi:10.1016/j.msard.2021.103352