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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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