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SHR Neuro Cancer Cardio Lipid Metab Microb

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, KP; Wildemann, B.
Frequency and syndrome specificity of antibodies to aquaporin-4 in neurological patients with rheumatic disorders.
Mult Scler. 2011; 17(9): 1067-1073. Doi: 10.1177/1352458511403958
Web of Science PubMed FullText FullText_MUG

Co-authors Med Uni Graz: Seifert-Held Thomas
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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.
Find related publications in this database (using NLM MeSH Indexing): Adolescent -; Adult -; Aged -; Aged, 80 and over -; Aquaporin 4 - immunology; Autoantibodies - immunology; Connective Tissue Diseases - immunology; Female -; Humans -; Male -; Middle Aged -; Myelitis, Transverse - immunology; Neuromyelitis Optica - immunology; Optic Neuritis - immunology; Rheumatic Diseases - immunology
Find related publications in this database (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