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Steroid-sparing maintenance immunotherapy for MOG-IgG associated disorder
John J Chen1, Eoin P Flanagan2, M Tariq Bhatti2
1From the Departments of Ophthalmology (J.J.C., M.T.B.), Neurology (J.J.C., E.P.F., M.T.B., J.J., D.D., A.S.L.C., B.G.W., A.M., J.-M.T., V.A.L., C.F.L., A.K., S.J.P.), Laboratory Medicine and Pathology (E.P.F., J.J., D.D, J.P.F., A.M., V.A.L., S.J.P.), and Immunology (V.A.L.) and Center for MS and Autoimmune Neurology (E.P.F., D.D., B.G.W., A.M., V.A.L., C.F.L., A.K., S.J.P.), Mayo Clinic, Rochester, MN; Department of Ophthalmology and Visual Neurosciences (C.M.M., M.S.L.), University of Minnesota, Minneapolis; Departments of Neurology and Ophthalmology (J.L.B., V.S.P.), University of Colorado Denver School of Medicine, Aurora; Departments of Ophthalmology and Visual Sciences and Neurology (G.V.S.), Washington University, St. Louis School of Medicine, MO; Departments of Ophthalmology and Visual Science and Neurology (O.-O.O.A.), McGovern Medical School, Houston, TX; Departments of Neurology, Neurosurgery, and Neuro-Ophthalmology (E.R.E.), Mayo Clinic, Jacksonville, FL; Departments of Ophthalmology (M.D.A.) and Neurology (D.M.W.), Mayo Clinic, Scottsdale, AZ; Bascom Palmer Eye Institute (B.L.L.), University of Miami, FL; Department of Neurology and Ophthalmology (H.M., S.B.), Stanford University, Palo Alto, CA; Neuro-Ophthalmology (A.L.G.), Kaiser Permanente, Northern California, Vallejo; Department of Ophthalmology (V.S.), Baylor College of Medicine/Texas Children's Hospital, Houston; Department of Ophthalmology (G.A., D.M.C.), Massachusetts Eye and Ear Infirmary/Harvard Medical School, Boston; Department of Ophthalmology (G.H.), Boston Children's Hospital, Harvard Medical School, MA; and Neuro-Ophthalmology Unit (H.S.-K.), Department of Ophthalmology, Rabin Medical Center, Sackler School of Medicine, Tel Aviv University, Israel. Chen.john@mayo.edu.
Long-term immunotherapy for Myelin oligodendrocyte glycoprotein-immunoglobulin G (MOG-IgG) associated disorder (MOGAD) reduces relapses. Intravenous immunoglobulin (IVIG) showed the lowest relapse rate, while multiple sclerosis (MS) agents were ineffective.
Area of Science:
- Neurology
- Immunology
- Demyelinating Diseases
Background:
- Myelin oligodendrocyte glycoprotein-immunoglobulin G (MOG-IgG) associated disorder (MOGAD) is characterized by recurrent central nervous system (CNS) demyelinating attacks.
- The optimal long-term treatment strategy to prevent relapses in MOGAD remains uncertain.
- Understanding treatment efficacy is crucial for managing this rare autoimmune condition.
Purpose of the Study:
- To evaluate the effectiveness of various long-term immunotherapies in reducing relapse rates in patients with MOGAD.
- To compare the relapse rates associated with different maintenance immunosuppressive treatments.
- To assess the impact of traditional multiple sclerosis (MS) disease-modifying agents in MOGAD.
Main Methods:
- A multicenter retrospective study involving seventy patients diagnosed with MOGAD.
- Inclusion criteria required a history of at least one CNS demyelinating attack, MOG-IgG seropositivity, and a minimum of six months of immunotherapy.
- Relapse rates were analyzed before and during maintenance immunotherapy, including mycophenolate mofetil, rituximab, azathioprine, and intravenous immunoglobulin (IVIG).
Main Results:
- The median annualized relapse rate (ARR) decreased from 1.6 before treatment to 0.3 on maintenance immunotherapy across all evaluated treatments.
- Intravenous immunoglobulin (IVIG) demonstrated the lowest relapse rate (20% relapse, ARR 0), followed by azathioprine (59% relapse, ARR 0.2), rituximab (61% relapse, ARR 0.59), and mycophenolate mofetil (74% relapse, ARR 0.67).
- Patients treated with traditional MS disease-modifying agents experienced breakthrough relapses, with an ARR of 1.5.
Conclusions:
- Maintenance immunotherapy significantly reduces recurrent CNS demyelinating attacks in MOGAD patients.
- Intravenous immunoglobulin (IVIG) therapy appears to be the most effective treatment for reducing relapses in MOGAD.
- Standard multiple sclerosis (MS) disease-modifying agents are not effective for managing MOGAD and further prospective studies are warranted.
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