Related Experiment Video
Updated: Jun 20, 2026
Induction and Clinical Scoring of Chronic-Relapsing Experimental Autoimmune Encephalomyelitis
Published on: July 4, 2007
"Lupus Myelitis" Revisited: A Retrospective Single-Center Study of Myelitis Associated With Rheumatologic Disease
Jonathan D Krett1, Angeliki G Filippatou1, Paula Barreras1
1From the Division of Neuroimmunology and Neurological Infections (J.D.K., A.G.F., P.B., C.A.P., E.S.S.), Department of Neurology, Johns Hopkins University School of Medicine, Baltimore, MD; Multiple Sclerosis and Neuroimmunology Center (P.B.), Department of Neurology and Neurosurgery, Cedars-Sinai Medical Center, University of California, Los Angeles; and Division of Rheumatology (A.C.G.), Department of Medicine, Johns Hopkins University School of Medicine, Baltimore, MD.
Nearly half of patients with rheumatologic disease and myelitis were diagnosed with aquaporin-4 (AQP4)-IgG seropositive neuromyelitis optica spectrum disorder (NMOSD). Myelin oligodendrocyte glycoprotein (MOG)-IgG-associated disease (MOGAD) was less common, and further research is needed for seronegative cases.
Area of Science:
- Neurology
- Rheumatology
- Immunology
Background:
- Myelitis in patients with rheumatologic disease may be misdiagnosed, potentially being unrecognized aquaporin-4 (AQP4)-IgG seropositive neuromyelitis optica spectrum disorder (NMOSD) or myelin oligodendrocyte glycoprotein (MOG)-IgG-associated disease (MOGAD).
- The availability of sensitive cell-based assays for AQP4-IgG and MOG-IgG necessitates re-evaluation of these patient cohorts.
Purpose of the Study:
- To clinicoradiologically and serologically characterize patients with myelitis and co-existing rheumatologic disease.
- To determine the prevalence of AQP4-IgG NMOSD and MOGAD in this population.
- To investigate the characteristics of myelitis in patients seronegative for both AQP4-IgG and MOG-IgG.
Main Methods:
- Retrospective cohort study of patients diagnosed with myelopathy and rheumatologic comorbidity between 2018-2023.
- Electronic medical record (EMR) query identified potential cases, with subsequent chart review for inclusion.
- Patients with myelitis unrelated to multiple sclerosis (MS) were analyzed for serological status (AQP4-IgG, MOG-IgG), lesion characteristics, and functional outcomes.
Main Results:
- Of 41 reviewed patients, 49% were diagnosed with AQP4-IgG seropositive NMOSD, 7% with MOGAD, and 44% had "double-seronegative" myelitis.
- Common rheumatologic diagnoses included systemic lupus erythematosus (SLE) and Sjögren syndrome (SS).
- AQP4-IgG seropositive NMOSD patients more frequently exhibited longitudinally extensive cord lesions compared to "double-seronegative" patients. Functional outcomes were similar across diagnoses.
Conclusions:
- Approximately half of myelitis cases in this rheumatologic disease cohort were attributed to AQP4-IgG seropositive NMOSD.
- MOGAD represented a smaller but clinically significant proportion.
- Further research is required to elucidate the etiology of myelitis in patients seronegative for both AQP4-IgG and MOG-IgG.
Related Concept Videos
01:22Rheumatic Heart Disease II: Clinical Manifestations and Diagnostic Studies
01:19Multiple Sclerosis l: Introduction