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.

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Additional consensus statements from the international panel of NMOSD experts included:

  • Treatment with monotherapy is preferable to reduce IST-related side effects10
  • Patient preference should be a consideration when initiating or switching treatment10
  • Current clinical disease activity, serious treatment-related adverse events, and patient preference for another therapy should warrant switching treatment10
  • Vaccinations should be kept up to date, with specific guidance concerning meningococcal vaccination when prescribing eculizumab10

*The NMOSD Delphi Panel was convened to develop validated recommendations on the treatment of AQP4-IgG+ patients with NMOSD. This international panel of clinical experts drafted consensus statements regarding therapy initiation, monotherapy vs combination therapy, switching therapies, patient populations, safety, use of biomarkers and patient-reported outcomes, and gaps in research. Of 18 panelists, 14 agreed on the same initiation criteria for eculizumab, and 17 for satralizumab. Each therapy met the panel’s predefined consensus threshold (67% or greater).

AQP4-IgG+, aquaporin-4-immunoglobulin G positive; IST, immunosuppressive therapy; NMOSD, neuromyelitis optica spectrum disorder.

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AQP4-IgG Testing

AQP4-IgG antibody testing is essential for diagnosing NMOSD1-3

Esther

UPLIZNA patient since 2021. Individual results may vary.
Hear Esther’s story.

Early diagnosis through cell-based testing is crucial to beginning treatment1-3

  • AQP4-IgG is an NMOSD autoantibody that is not present in MS2
  • A cell-based assay is the most reliable method to detect AQP4-IgG autoantibodies, which are present in ~75% of NMOSD cases1,3-5

To receive the most accurate confirmation of your suspected NMOSD diagnosis, order a cell-based assay from the labs listed here:
ARUP
Laboratories6		 Athena
Diagnostics7		 Labcorp8 Mayo Clinic
Laboratories9		 Quest
Diagnostics10
Website Aruplab.com Athenadiagnostics.com Labcorp.com Mayocliniclabs.com Questdiagnostics.com
Method IFA IFA IFA FACS IFA
Lab Test Code 2013320 1282 505295 43638-6 (can be ordered with form online) 38321
CPT Code 86052 (if reflexed, add 86256) 86052 86052 86053 86052
Specimen Requirements Collect 1 mL (0.15 mL minimum) serum using a serum separator or a red-top tube then transfer to an ARUP Standard Transport Tube, refrigerated Collect 2 mL (0.15 mL minimum) serum using a serum separator or a red-top tube, refrigerated Collect 2 mL (1 mL minimum) serum using a red-top tube, room temperature Collect 3 mL (2 mL minimum) serum using a red-top tube, refrigerated Collect 2 mL (0.15 mL minimum) serum using a serum in a red-top tube then transfer to a transport tube, refrigerated
Turnaround
Time		 1 to 6 days 3 to 7 days 1 to 2 days 5 to 8 days Varies by region
Reference
Values		 <1:10 titer See laboratory report See laboratory report Negative Negative or <1:10 titer
Website ARUP
Laboratories6		 Aruplab.com Athena
Diagnostics7		 Athenadiagnostics.com Labcorp8 Labcorp.com Mayo Clinic
Laboratories9		 Mayocliniclabs.com Quest
Diagnostics10		 Questdiagnostics.com
Method ARUP
Laboratories6		 IFA Athena
Diagnostics7		 IFA Labcorp8 IFA Mayo Clinic
Laboratories9		 FACS Quest
Diagnostics10		 IFA
Lab Test Code ARUP
Laboratories6		 2013320 Athena
Diagnostics7		 1282 Labcorp8 505295 Mayo Clinic
Laboratories9		 43638-6 (can be ordered with form online) Quest
Diagnostics10		 38321
CPT Code ARUP
Laboratories6		 86052 (if reflexed, add 86256) Athena
Diagnostics7		 86052 Labcorp8 86052 Mayo Clinic
Laboratories9		 86053 Quest
Diagnostics10		 86052
Specimen Requirements ARUP
Laboratories6		 Collect 1 mL
(0.15 mL minimum)
serum using a serum
separator or a red-top
tube then transfer to
an ARUP Standard
Transport Tube,
refrigerated		 Athena
Diagnostics7		 Collect 2 mL
(0.15 mL minimum)
serum using a
serum separator or
a red-top tube,
refrigerated		 Labcorp8 Collect 2 mL (1 mL minimum)
serum using a
red-top tube,
room temperature		 Mayo Clinic
Laboratories9		 Collect 3 mL
(2 mL minimum)
serum using a red-top
tube, refrigerated		 Quest
Diagnostics10		 Collect 2 mL
(0.15 mL minimum)
serum using a serum
in a red-top tube then
transfer to a transport
tube, refrigerated
Turnaround Time ARUP
Laboratories6		 1 to 6 days Athena
Diagnostics7		 3 to 7 days Labcorp8 1 to 2 days Mayo Clinic
Laboratories9		 5 to 8 days Quest
Diagnostics10		 Varies by region
Reference Values ARUP
Laboratories6		 <1:10 titer Athena
Diagnostics7		 See laboratory report Labcorp8 See laboratory report Mayo Clinic
Laboratories9		 Negative Quest
Diagnostics10		 Negative or <1:10 titer
Cell-based assays are strongly recommended for their higher sensitivity and specificity—the likelihood of a false-negative result with an ELISA assay is almost 5x greater. False negatives are also more likely to occur during the recovery period following an attack or when taking an immunosuppressant. Consider retesting an AQP4-IgG– patient within 3 to 6 months.1,11,12
AQP4-IgG+ patient? Explore how UPLIZNA works

AQP4-IgG, aquaporin-4 immunoglobulin G; CBA, cell-based assay; CPT, current procedural terminology; ELISA, enzyme-linked immunosorbent assay; FACS, fluorescence-activated cell sorting; IFA, immunofluorescence assay; MS, multiple sclerosis; NMOSD, neuromyelitis optica spectrum disorder.

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.

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: BBased 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.

  • REFERENCES:
    1. 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
    2. 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
    3. 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
    4. 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
    5. 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
    6. Aquaporin-4 Receptor Antibody, IgG by IFA with Reflex to Titer, Serum. ARUP Laboratories. Accessed May 15, 2024. https://ltd.aruplab.com/Tests/Pub/2013320
    7. Aquaporin-4 (AQP4) (NMO-IgG) Antibody, CBA with Reflex to Titer. Athena Diagnostics. Accessed May 15, 2024. https://www.athenadiagnostics.com/view-full-catalog/aquaporin-4-aqp4-nmo-igg-antibody-cba-with-reflex-to-titer1
    8. Anti-Aquaporin 4 (AQP4), Serum. Labcorp, Accessed May 15, 2024. https://www.labcorp.com/tests/505295/anti-aquaporin-4-aqp4-serum
    9. Test ID: NMOFS, Neuromyelitis Optica (NMO)/Aquaporin-4-IgG Fluorescence-Activated Cell Sorting (FACS) Assay, Serum. Mayo Clinic Laboratories. Accessed May 15, 2024. https://www.mayocliniclabs.com/test-catalog/overview/38324
    10. Aquaporin-4 (AQP4) (NMO-IgG) Antibody with Reflex to Titer, Serum. Quest Diagnostics. Accessed May 15, 2024. https://testdirectory.questdiagnostics.com/test/test-detail/38321/aquaporin-4-aqp4-nmo-iggantibody-with-reflex-to-titer-serum?q=38321&cc=MASTER
    11. Prain K, Woodhall M, Vincent A, et al; Australian and New Zealand NMO Collaboration. AQP4 antibody assay sensitivity comparison in the era of the 2015 diagnostic criteria for NMOSD. Front Neurol. 2019;10(1028):1-7. doi:10.3389/fneur.2019.01028
    12. Waters PJ, Pittock SJ, Bennett JL, Jarius S, Weinshenker BG, Wingerchuk DM. Evaluation of aquaporin-4 antibody assays. Clin Exp Neuroimmunol. 2014;5(3):290-303. doi:10.1111.cen3.12107