AQP4-IgG Testing

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

Early diagnosis through cell-based testing is crucial to beginning treatment and preventing potentially irreversible damage1-3

  • 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		 Mayo Clinic
Laboratories8		 Quest
Diagnostics9
Website Aruplab.com Athenadiagnostics.com Mayocliniclabs.com Questdiagnostics.com
Method IFA IFA FACS IFA
Lab Test Code 2013320 1282 43638-6 (can be ordered with form online) 38321
CPT Code 86052 (if reflexed, add 86256) 86052 86053 86052
Specimen Requirements Collect 1 mL (0.15 mL minumum) 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 minumum) serum using a serum separator or a red-top tube, refrigerated Collect 3 mL (2 mL minumum) serum using a red-top tube, refrigerated Collect 2 mL (0.15 mL minumum) 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 5 to 8 days Varies by Region
Reference
Values		 < 1:10 titer See laboratory report Negative Negative or < 1:10 titer
Website ARUP
Laboratories6		 Aruplab.com Athena
Diagnostics7		 Athenadiagnostics.com Mayo Clinic
Laboratories8		 Mayocliniclabs.com Quest
Diagnostics9		 Questdiagnostics.com
Method ARUP
Laboratories6		 IFA Athena
Diagnostics7		 IFA Mayo Clinic
Laboratories8		 FACS Quest
Diagnostics9		 IFA
Lab Test Code ARUP
Laboratories6		 2013320 Athena-
Diagnostics7		 1282 Mayo Clinic
Laboratories8		 43638-6 (can be ordered with form online) Quest
Diagnostics9		 38321
CPT Code ARUP
Laboratories6		 86052 (if reflexed, add 86256) Athena
Diagnostics7		 86052 Mayo Clinic
Laboratories8		 86053 Quest
Diagnostics9		 86052
Specimen Requirements ARUP
Laboratories6		 Collect 1 mL
(0.15 mL minumum)
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 minumum)
serum using a
serum separator or
a red-top tube,
Refrigerated		 Mayo Clinic
Laboratories8		 Collect 3 mL
(2 mL minumum)
serum using a red-top
tube, refrigerated		 Quest
Diagnostics9		 Collect 2 mL
(0.15 mL minumum)
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 Mayo Clinic
Laboratories8		 5 to 8 days Quest
Diagnostics9		 Varies by Region
Reference Values ARUP
Laboratories6		 < 1:10 titer Athena
Diagnostics7		 See laboratory report Mayo Clinic
Laboratories8		 Negative Quest
Diagnostics9		 Negative or < 1:10 titer

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Explore the MOA
of UPLIZNA

AQP4-IgG, aquaporin-4 immunoglobulin G; FACS, fluorescence-activated cell sorting; IFA, immunofluorescence assay; MS, multiple sclerosis; NMOSD, neuromyelitis optica spectrum disorder.

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, 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 July 26, 2022. https://ltd.aruplab.com/Tests/Pub/2013320
  7. Aquaporin-4 (AQP4) (NMO-IgG) Antibody, CBA with Reflex to Titer. Athena Diagnostics. Accessed July 26, 2022. https://www.athenadiagnostics.com/view-full-catalog/a/aquaporin-4-(aqp4)-(nmo-igg)-antibody,-cba-with-re
  8. Test ID: NMOFS, Neuromyelitis Optica (NMO)/Aquaporin-4-IgG Fluorescence-Activated Cell Sorting (FACS) Assay, Serum. Mayo Clinic Laboratories. Accessed July 26, 2022. https://www.mayocliniclabs.com/testcatalog/Overview/38324
  9. Aquaporin-4 (AQP4) (NMO-IgG) Antibody with Reflex to Titer, Serum. Quest Diagnostics. Accessed July 26, 2022. https://testdirectory.questdiagnostics.com/test/test-detail/38321/aquaporin-4-aqp4-nmo-iggantibody-with-reflex-to-titer-serum?q=38321&cc=MASTER
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.