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Induction of Paralysis and Visual System Injury in Mice by T Cells Specific for Neuromyelitis Optica Autoantigen Aquaporin-4
Published on: August 21, 2017
Neuromyelitis optica and non organ-specific autoimmunity
Sean J Pittock1, Vanda A Lennon, Jerome de Seze
1Department of Neurology, Mayo Clinic, Rochester, Minnesota 55905, USA.
Archives of Neurology
|January 16, 2008
Summary
Neuromyelitis optica spectrum disorders (NMOSDs) often co-occur with autoimmune diseases like systemic lupus erythematosus (SLE) and Sjögren syndrome (SS). NMO-IgG positivity in NMOSD patients indicates coexisting NMO, not a complication of SLE or SS.
Area of Science:
- Neuroimmunology
- Autoimmune Diseases
- Clinical Neurology
Background:
- Neuromyelitis optica (NMO) and related NMO spectrum disorders (NMOSDs) frequently present with markers of non-organ-specific autoimmunity.
- Diagnostic confusion can arise from overlapping clinical and serological features with other autoimmune conditions such as systemic lupus erythematosus (SLE) and Sjögren syndrome (SS).
Purpose of the Study:
- To investigate the longitudinal relationship between NMOSDs (including NMO, longitudinally extensive transverse myelitis, and recurrent optic neuritis) and autoimmune diseases.
- To differentiate co-occurring NMO from complications of SLE and SS through serological analysis.
Main Methods:
- A retrospective, blinded serological survey was conducted across two cohorts: US patients with NMOSDs and controls with SS/SLE, and French patients with SS/SLE and/or NMOSDs.
- Serological markers including NMO-IgG, antinuclear antibodies (ANA), and Sjögren syndrome A (SSA) antibodies were analyzed.
Main Results:
- NMO-IgG was detected in 66.7% of US NMOSD patients. ANA and SSA antibodies were more prevalent in NMO-IgG-seropositive NMOSD patients.
- In the French cohort, NMO-IgG was found in 35.7% of NMOSD patients with SS/SLE and 50.0% of NMO patients without SS/SLE.
- NMO-IgG was exclusively detected in patients with NMOSDs, not in controls with SS/SLE but without neurological involvement.
Conclusions:
- Seropositivity for NMO-IgG in patients with NMOSDs co-occurring with SLE/SS suggests coexisting NMO.
- This finding differentiates NMO from a potential vasculopathic or other complication of SLE or SS.
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