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

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Item Type:	Article
Title:	Frequency and syndrome specificity of antibodies to aquaporin-4 in neurological patients with rheumatic disorders
Creators Name:	Jarius, S., Jacobi, C., de Seze, J., Zephir, H., Paul, F., Franciotta, D., Rommer, P., Mader, S., Kleiter, I., Reindl, M., Akman-Demir, G., Seifert-Held, T., Kristoferitsch, W., Melms, A., 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
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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