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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| Hauptverfasser: | , , , , , , , , , , , , , , , |
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| Dokumenttyp: | Article (Journal) |
| Sprache: | Englisch |
| Veröffentlicht: |
May 4, 2011
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| In: |
Multiple sclerosis journal
Year: 2011, Jahrgang: 17, Heft: 9, Pages: 1067-1073 |
| ISSN: | 1477-0970 |
| DOI: | 10.1177/1352458511403958 |
| Online-Zugang: | Verlag, lizenzpflichtig, Volltext: https://doi.org/10.1177/1352458511403958 |
| Verfasserangaben: | 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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| 245 | 1 | 0 | |a Frequency and syndrome specificity of antibodies to aquaporin-4 in neurological patients with rheumatic disorders |c 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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| 520 | |a 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. | ||
| 650 | 4 | |a antibody to aquaporin-4 | |
| 650 | 4 | |a connective tissue disorders | |
| 650 | 4 | |a diagnosis | |
| 650 | 4 | |a longitudinally extensive transverse myelitis | |
| 650 | 4 | |a neuromyelitis optica (Devic’s disease) | |
| 650 | 4 | |a neuropsychiatric lupus | |
| 650 | 4 | |a NMO-IgG | |
| 650 | 4 | |a rheumatic diseases | |
| 650 | 4 | |a scleroderma | |
| 650 | 4 | |a Sjögren’s syndrome | |
| 650 | 4 | |a systemic lupus erythematosus | |
| 650 | 4 | |a vasculitis | |
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