期刊
MULTIPLE SCLEROSIS JOURNAL
卷 17, 期 9, 页码 1067-1073出版社
SAGE PUBLICATIONS LTD
DOI: 10.1177/1352458511403958
关键词
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
资金
- European Committee for Treatment and Research in Multiple Sclerosis (ECTRIMS)
- Bayer Schering Pharma
- Merck Serono
- German Research Foundation (DFG) [257]
- Austrian multiple sclerosis research society
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.
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