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

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...

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Main Authors: Jarius, Sven (Author) , Jacobi, Christian (Author) , de Seze, Jerome (Author) , Zephir, Helene (Author) , Paul, Friedemann (Author) , Franciotta, Diego (Author) , Rommer, Paulus (Author) , Mader, Simone (Author) , Kleiter, Ingo (Author) , Reindl, Markus (Author) , Akman-Demir, Gulsen (Author) , Seifert-Held, Thomas (Author) , Kristoferitsch, Wolfgang (Author) , Melms, Arthur (Author) , Wandinger, Klaus-Peter (Author) , Wildemann, Brigitte (Author)
Format: Article (Journal)
Language:English
Published: May 4, 2011
In: Multiple sclerosis journal
Year: 2011, Volume: 17, Issue: 9, Pages: 1067-1073
ISSN:1477-0970
DOI:10.1177/1352458511403958
Online Access:Verlag, lizenzpflichtig, Volltext: https://doi.org/10.1177/1352458511403958
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Author Notes:Sven Jarius, Christian Jacobi, Jerome de Seze, Helene Zephir, Friedemann Paul, Diego Franciotta, Paulus Rommer, Simone Mader, Ingo Kleiter, Markus Reindl, Gulsen Akman-Demir, Thomas Seifert-Held, Wolfgang Kristoferitsch, Arthur Melms, Klaus-Peter Wandinger and Brigitte Wildemann
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Summary: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 Sjögren’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.
Item Description:Gesehen am 04.07.2022
Physical Description:Online Resource
ISSN:1477-0970
DOI:10.1177/1352458511403958

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