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Sean J. Pittock, MD; Vanda A. Lennon, MD, PhD; Jerome de Seze, MD; Patrick Vermersch, MD; Henry A. Homburger, MD; Dean M. Wingerchuk, MD; Claudia F. Lucchinetti, MD; Hélène Zéphir, MD; Kevin Moder, MD; Brian G. Weinshenker, MD

Arch Neurol. 2008;65(1):78-83.

Background  Neuromyelitis optica (NMO) is often associated with other clinical or serological markers of non–organ-specific autoimmunity.

Objective  To evaluate the relationship between NMO spectrum disorders (NMOSDs), including NMO, longitudinally extensive transverse myelitis, and recurrent optic neuritis, and autoimmune disease. We concentrated on the association with systemic lupus erythematosus (SLE), Sjögren syndrome (SS), or serological evidence of these disorders, which commonly is a source of diagnostic confusion.

Design  Retrospective blinded serological survey.

Setting  Mayo Clinic College of Medicine, Rochester, and Centre Hospitalier Régional Universitaire de Lille.

Methods  Group 1 included 153 US patients with NMOSDs (78 with NMO and 75 with longitudinally extensive transverse myelitis) and 33 control subjects with SS/SLE. Group 2 included 30 French patients with SS/SLE, 14 with NMOSDs (6 with NMO, 6 with longitudinally extensive transverse myelitis, and 2 with recurrent optic neuritis), 16 without NMOSDs, and 4 with NMO without SS/SLE.

Results  For group 1, NMO-IgG was detected in 66.7%, antinuclear antibodies in 43.8%, and Sjögren syndrome A (SSA) antibodies in 15.7% of patients with NMO and longitudinally extensive transverse myelitis. Five NMO-IgG–seropositive patients with NMOSDs had coexisting SLE, SS, or both. Antinuclear antibodies and SSA antibodies were more frequent in NMO-IgG–seropositive patients than in NMO-IgG–seronegative patients (P = .001). For group 2, NMO-IgG was detected in 5 of 14 patients (35.7%) with NMOSDs and SS/SLE and in 2 of 4 patients (50.0%) with NMO without SS/SLE (P = .59). We detected NMO-IgG only in patients with NMOSDs and not in 49 controls with SS/SLE but without optic neuritis or myelitis from the 2 cohorts (P = .01).

Conclusion  Neuromyelitis optica spectrum disorders with seropositive findings for NMO-IgG occurring with SS/SLE or non–organ-specific autoantibodies is an indication of coexisting NMO rather than a vasculopathic or other complication of SS/SLE.

Author Affiliations: Departments of Neurology (Drs Pittock, Lennon, Lucchinetti, and Weinshenker), Laboratory Medicine and Pathology (Drs Pittock, Lennon, and Homburger), Immunology (Dr Lennon), and Rheumatology (Dr Moder), Mayo Clinic, Rochester, Minnesota; and Departments of Neurology, Hospitale Universitaire de Strasbourg, Strasbourg, France (Dr de Seze); Centre Hospitalier Régional Universitaire de Lille, Lille, France (Drs Vermersch and Zéphir); and Mayo Clinic, Scottsdale, Arizona (Dr Wingerchuk).

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