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Frequency and syndrome specificity of antibodies to aquaporin-4 in neurological patients with rheumatic disorders

Item Type:Article
Title:Frequency and syndrome specificity of antibodies to aquaporin-4 in neurological patients with rheumatic disorders
Creators Name:Jarius, S. and Jacobi, C. and de Seze, J. and Zephir, H. and Paul, F. and Franciotta, D. and Rommer, P. and Mader, S. and Kleiter, I. and Reindl, M. and Akman-Demir, G. and Seifert-Held, T. and Kristoferitsch, W. and Melms, A. and Wandinger, K.P. and Wildemann, B.
Abstract:BACKGROUND: A new autoantibody (termed NMO-IgG, or AQP4-Ab) has recently been described in patients with neuromyelitis optica (NMO) and its formes frustes, longitudinally extensive transverse myelitis (LETM) and recurrent optic neuritis (rON). However, AQP4-Ab has been found also in patients with co-existing rheumatic diseases such as systemic lupus erythematosus (SLE) or Sjogren's syndrome (SS), conditions which are characterized by broad, polyspecific B cell activation. OBJECTIVES: In this study, we aimed at evaluating the syndrome specificity and frequency of AQP4-Ab in patients with rheumatic diseases and neurological symptoms. METHODS: For this purpose, serum samples from 109 neurological patients with established connective tissue disorders (CTD) (n = 54), possible CTD (n = 42), or vasculitis (n = 13) were analysed for the presence of AQP4-Ab by a cell-based assay employing recombinant human AQP4. RESULTS: AQP4-Ab was detectable in 31/40 (78%) patients with CTD and NMO spectrum disorders (median titre, 1:1000) but in none of the samples obtained from patients with CTD or vasculitis and neurological disorders other than NMO, LETM, or rON (n = 69). CONCLUSION: The high syndrome specificity of the antibody for neuromyelitis optica spectrum disorders (NMOSDs) in patients with CTD supports the concept of AQP4-Ab being involved in the pathogenesis of these neurological conditions, and argues against AQP4-Ab simply being part of the polyclonal B cell activation generally associated with rheumatic diseases. Moreover, the finding that AQP4-Ab is present in patients with CTD and co-existing NMOSD with approximately the same frequency as in patients without CTD strengthens the case of CTD and AQP4-Ab positive NMOSD representing two co-existing yet distinct entities in the majority of patients.
Keywords:Antibody to Aquaporin-4, Connective Tissue Disorders, Diagnosis, Longitudinally Extensive Transverse Myelitis, Neuromyelitis Optica (Devic's Disease), Neuropsychiatric Lupus, NMO-IgG, Rheumatic Diseases, Scleroderma, Sjogren's Syndrome, Systemic Lupus Erythematosus, Vasculitis
Source:Multiple Sclerosis Journal
ISSN:1352-4585
Publisher:Sage Publications (U.K.)
Volume:17
Number:9
Page Range:1067-1073
Date:September 2011
Official Publication:https://doi.org/10.1177/1352458511403958
PubMed:View item in PubMed

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