RISK OF RELAPSE
NMOSD is a relapsing autoimmune disease of the central nervous system
NMOSD relapses are unpredictable and tend to be severe and recurrent1-3
Relapses primarily target the optic nerves (optic neuritis) and spinal cord (transverse myelitis).
Over time, relapses have been shown to be inevitable for the majority of patients with NMOSD.1,2,4
The majority of patients with NMOSD are anti-AQP4 antibody positive5*
Up to 92.7% of patients with NMOSD who are anti-AQP4 antibody positive have relapsed, with relapses that may result in permanent disability.1,6,7†‡§
*A cross-sectional study of 132 patients with NMOSD over a period of 4 years.5
†Retrospective study from the German Neuromyelitis Optica Study Group database in patients with anti-AQP4 antibody-positive NMOSD (N=137) whose median disease duration was 60 months.1
‡Retrospective study from Mayo Clinic records in patients with anti-AQP4 antibody-positive NMOSD (N=140).6
§Population-based cohort study in patients with anti-AQP4 antibody-positive NMOSD from Olmsted County, Minnesota (N=5), and Martinique (N=31).7
Complement activation is an important cause of anti-AQP4 antibody-positive NMOSD pathophysiology
The complement cascade is a vital component of the body’s immune system8
- It is composed of a complex and tightly regulated group of more than 40 blood proteins known as complement proteins8
- The complement system acts quickly to detect, destroy, and eliminate microbes or cellular debris8,9
- It is responsible for MAC formation and bacterial lysis9
In anti-AQP4 antibody-positive NMOSD, complement activation can lead to inflammation and destruction of astrocytes10
Anti-AQP4 antibody binds to AQP4
Complement activation
Astrocyte and neuronal death
Abbreviations: AQP4, aquaporin-4; CNS, central nervous system; MAC, membrane attack complex.
WARNING: SERIOUS MENINGOCOCCAL INFECTIONS
Life-threatening and fatal meningococcal infections have occurred in patients treated with Soliris and may become rapidly life-threatening or fatal if not recognized and treated early.
- Comply with the most current Advisory Committee on Immunization Practices (ACIP) recommendations for meningococcal vaccination in patients with complement deficiencies.
- Immunize patients with meningococcal vaccines at least 2 weeks prior to administering the first dose of Soliris, unless the risks of delaying Soliris therapy outweigh the risk of developing a meningococcal infection. (See Serious Meningococcal Infections for additional guidance on the management of the risk of meningococcal infection).
- Vaccination reduces, but does not eliminate, the risk of meningococcal infections. Monitor patients for early signs of meningococcal infections and evaluate immediately if infection is suspected.
Soliris is available only through a restricted program under a Risk Evaluation and Mitigation Strategy (REMS). Under the Soliris REMS, prescribers must enroll in the program. Enrollment in the Soliris REMS program and additional information are available by telephone: 1-888-SOLIRIS (1-888-765-4747) or at www.solirisrems.com.
Contraindications
- Patients with unresolved serious Neisseria meningitidis infection
- Patients who are not currently vaccinated against Neisseria meningitidis, unless the risks of delaying Soliris treatment outweigh the risks of developing a meningococcal infection
Warnings and Precautions
Serious Meningococcal Infections
Risk and Prevention
The use of Soliris increases a patient’s susceptibility to serious meningococcal infections (septicemia and/or meningitis).
Vaccinate or revaccinate for meningococcal disease according to the most current ACIP recommendations for patients with complement deficiencies. Immunize patients without a history of meningococcal vaccination at least 2 weeks prior to receiving the first dose of Soliris. If Soliris must be initiated immediately in an unvaccinated patient, administer meningococcal vaccine(s) as soon as possible and provide 2 weeks of antibacterial drug prophylaxis. Discontinue Soliris in patients who are undergoing treatment for serious meningococcal infections.
REMS
Prescribers must counsel patients about the risk of meningococcal infection, provide the patients with the REMS educational materials, and ensure patients are vaccinated with meningococcal vaccine(s).
Other Infections
Serious infections with Neisseria species (other than N. meningitidis), including disseminated gonococcal infections, have been reported.
Patients may have increased susceptibility to infections, especially with encapsulated bacteria. Additionally, Aspergillus infections have occurred in immunocompromised and neutropenic patients. Use caution when administering Soliris to patients with any systemic infection.
Infusion-Related Reactions
Administration of Soliris may result in infusion-related reactions, including anaphylaxis or other hypersensitivity reactions. Interrupt Soliris infusion and institute appropriate supportive measures if signs of cardiovascular instability or respiratory compromise occur.
Adverse Reactions
The most frequently reported adverse reactions in the NMOSD placebo-controlled trial (≥10%) are: upper respiratory infection, nasopharyngitis, diarrhea, back pain, dizziness, influenza, arthralgia, pharyngitis, and contusion.
INDICATION
Neuromyelitis Optica Spectrum Disorder (NMOSD)
Soliris is indicated for the treatment of neuromyelitis optica spectrum disorder (NMOSD) in adult patients who are anti-aquaporin-4 (AQP4) antibody positive.
Please see full Prescribing Information for SOLIRIS, including Boxed WARNING regarding serious meningococcal infections.
WARNING: SERIOUS MENINGOCOCCAL INFECTIONS
Life-threatening and fatal meningococcal infections have occurred in patients treated with Soliris and may become rapidly life-threatening or fatal if not recognized and treated early.
- Comply with the most current Advisory Committee on Immunization Practices (ACIP) recommendations for meningococcal vaccination in patients with complement deficiencies.
- Immunize patients with meningococcal vaccines at least 2 weeks prior to administering the first dose of Soliris, unless the risks of delaying Soliris therapy outweigh the risk of developing a meningococcal infection. (See Serious Meningococcal Infections for additional guidance on the management of the risk of meningococcal infection).
- Vaccination reduces, but does not eliminate, the risk of meningococcal infections. Monitor patients for early signs of meningococcal infections and evaluate immediately if infection is suspected.
Soliris is available only through a restricted program under a Risk Evaluation and Mitigation Strategy (REMS). Under the Soliris REMS, prescribers must enroll in the program. Enrollment in the Soliris REMS program and additional information are available by telephone: 1-888-SOLIRIS (1-888-765-4747) or at www.solirisrems.com.
Contraindications
- Patients with unresolved serious Neisseria meningitidis infection
- Patients who are not currently vaccinated against Neisseria meningitidis, unless the risks of delaying Soliris treatment outweigh the risks of developing a meningococcal infection
Warnings and Precautions
Serious Meningococcal Infections
Risk and Prevention
The use of Soliris increases a patient’s susceptibility to serious meningococcal infections (septicemia and/or meningitis).
Vaccinate or revaccinate for meningococcal disease according to the most current ACIP recommendations for patients with complement deficiencies. Immunize patients without a history of meningococcal vaccination at least 2 weeks prior to receiving the first dose of Soliris. If Soliris must be initiated immediately in an unvaccinated patient, administer meningococcal vaccine(s) as soon as possible and provide 2 weeks of antibacterial drug prophylaxis. Discontinue Soliris in patients who are undergoing treatment for serious meningococcal infections.
REMS
Prescribers must counsel patients about the risk of meningococcal infection, provide the patients with the REMS educational materials, and ensure patients are vaccinated with meningococcal vaccine(s).
Other Infections
Serious infections with Neisseria species (other than N. meningitidis), including disseminated gonococcal infections, have been reported.
Patients may have increased susceptibility to infections, especially with encapsulated bacteria. Additionally, Aspergillus infections have occurred in immunocompromised and neutropenic patients. Use caution when administering Soliris to patients with any systemic infection.
Infusion-Related Reactions
Administration of Soliris may result in infusion-related reactions, including anaphylaxis or other hypersensitivity reactions. Interrupt Soliris infusion and institute appropriate supportive measures if signs of cardiovascular instability or respiratory compromise occur.
Adverse Reactions
The most frequently reported adverse reactions in the NMOSD placebo-controlled trial (≥10%) are: upper respiratory infection, nasopharyngitis, diarrhea, back pain, dizziness, influenza, arthralgia, pharyngitis, and contusion.
INDICATION
Neuromyelitis Optica Spectrum Disorder (NMOSD)
Soliris is indicated for the treatment of neuromyelitis optica spectrum disorder (NMOSD) in adult patients who are anti-aquaporin-4 (AQP4) antibody positive.
Please see full Prescribing Information for SOLIRIS, including Boxed WARNING regarding serious meningococcal infections.
References
1. 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. doi:10.1186/1742-2094-9-14 2. Wingerchuk DM, Hogancamp WF, O’Brien PC, Weinshenker BG. The clinical course of neuromyelitis optica (Devic’s syndrome). Neurology. 1999;53(5):1107-1114. doi:10.1212/wnl.53.5.1107 3. Kitley J, Leite MI, Nakashima I, et al. Prognostic factors and disease course in aquaporin-4 antibody-positive patients with neuromyelitis optica spectrum disorder from the United Kingdom and Japan. Brain. 2012;135(pt 6):1834-1849. doi:10.1093/brain/aws109 4. Mealy MA, Wingerchuk DM, Greenberg BM, Levy M. Epidemiology of neuromyelitis optica in the United States: a multicenter analysis. Arch Neurol. 2012;69(9):1176-1180. doi:10.1001/archneurol.2012.314 5. 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 6. 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 7. 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.24617 8. Merle NS, Noe R, Halbwachs-Mecarelli L, Fremeaux-Bacchi V, Roumenina LT. Complement system part II: role in immunity. Front Immunol. 2015;6:257. doi:10.3389/fimmu.2015.00257 9. Dunkelberger JR, Song WC. Complement and its role in innate and adaptive immune responses. Cell Res. 2010;20(1):34-50. doi:10.1038/cr.2009.139 10. Wingerchuk DM. Neuromyelitis optica spectrum disorders: critical role of complement-dependent cytotoxicity. Neurol Rev. 2017;3(suppl):S1-S4. 11. Pittock SJ, Lucchinetti CF. Neuromyelitis optica and the evolving spectrum of autoimmune aquaporin-4 channelopathies: a decade later. Ann N Y Acad Sci. 2016;1366(1):20-39. doi:10.1111/nyas.12794 12. Dutra BG, da Rocha AJ, Nunes RH, Maia ACM. Neuromyelitis optica spectrum disorders: spectrum of MR imaging findings and their differential diagnosis. Radiographics. 2018;38(1):169-193. doi:10.1148/rg.2018170141 13. Papadopoulos MC, Verkman AS. Aquaporin 4 and neuromyelitis optica. Lancet Neurol. 2012;11(6):535-544. doi:10.1016/S1474-4422(12)70133-3