Neuromyelitis optica and non organ-specific autoimmunity.

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.

[1]  Ichiro Nakashima,et al.  A serum autoantibody marker of neuromyelitis optica: distinction from multiple sclerosis , 2004, The Lancet.

[2]  P. Piper.  Disseminated lupus erythematosus with involvement of the spinal cord. , 1953, Journal of the American Medical Association.

[3]  B. Weinshenker,et al.  Brain abnormalities in neuromyelitis optica. , 2006, Archives of neurology.

[4]  E. Alexander.  Neurologic disease in Sjögren's syndrome: mononuclear inflammatory vasculopathy affecting central/peripheral nervous system and muscle. A clinical review and update of immunopathogenesis. , 1993, Rheumatic diseases clinics of North America.

[5]  E. Waubant,et al.  An open label study of the effects of rituximab in neuromyelitis optica , 2005, Neurology.

[6]  J. Olascoaga,et al.  Anticuerpos IgG-NMO en la neuromielitis óptica: a propósito de 2 casos , 2006 .

[7]  R. DeHoratius,et al.  Transverse myelopathy in systemic lupus erythematosus: an analysis of 14 cases and review of the literature , 2000, Annals of the rheumatic diseases.

[8]  S. Vukusic,et al.  Neuromyelitis optica IgG predicts relapse after longitudinally extensive transverse myelitis , 2006, Annals of neurology.

[9]  J. Sharp,et al.  Transverse myelopathy in systemic lupus erythematosus. Report of three cases and review of the literature. , 1975, Annals of internal medicine.

[10]  R. Jonsson,et al.  Classification criteria for Sjögren's syndrome: a revised version of the European criteria proposed by the American-European Consensus Group , 2002, Annals of the rheumatic diseases.

[11]  J. Parisi,et al.  Pattern-specific loss of aquaporin-4 immunoreactivity distinguishes neuromyelitis optica from multiple sclerosis. , 2007, Brain : a journal of neurology.

[12]  Y. Itoyama,et al.  Loss of aquaporin-4 in active perivascular lesions in neuromyelitis optica: a case report. , 2006, The Tohoku journal of experimental medicine.

[13]  K. Moder.  Use and interpretation of rheumatologic tests: a guide for clinicians. , 1996, Mayo Clinic proceedings.

[14]  A. Mochizuki,et al.  Steroid-responsive Devic’s variant in Sjögren’s syndrome , 2000, Neurology.

[15]  L. Fox,et al.  Devic's syndrome and systemic lupus erythematosus: a case report with necropsy. , 1979, Archives of neurology.

[16]  J F Fries,et al.  The 1982 revised criteria for the classification of systemic lupus erythematosus. , 1982, Arthritis and rheumatism.

[17]  A. Perniok,et al.  Recurrent transverse myelitis associates with anti-Ro (SSA) autoantibodies , 2004, Neurology.

[18]  A. Verma.  Revised diagnostic criteria for neuromyelitis optica , 2007 .

[19]  R. Mandler,et al.  Devic's neuromyelitis optica: A prospective study of seven patients treated with prednisone and azathioprine , 1998, Neurology.

[20]  Alexander El.  Neurologic disease in Sjögren's syndrome: mononuclear inflammatory vasculopathy affecting central/peripheral nervous system and muscle. A clinical review and update of immunopathogenesis. , 1993 .

[21]  A. López de Munain,et al.  [NMO-IgG antibodies in neuromyelitis optica: a report of 2 cases]. , 2006, Neurologia.

[22]  P C O'Brien,et al.  The clinical course of neuromyelitis optica (Devic’s syndrome) , 1999, Neurology.

[23]  E. vanSonnenberg,et al.  A case of neuromyelitis optica (Devic's syndrome) in systemic lupus erythematosus , 1976, Neurology.

[24]  Hans Lassmann,et al.  A role for humoral mechanisms in the pathogenesis of Devic's neuromyelitis optica. , 2002, Brain : a journal of neurology.

[25]  A. Verkman,et al.  The Journal of Experimental Medicine CORRESPONDENCE , 2005 .