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

Jarius, Sven and Jacobi, Christian and de Seze, Jerome and Zephir, Helene and Paul, Friedemann and Franciotta, Diego and Rommer, Paulus and Mader, Simone and Kleiter, Ingo and Reindl, Markus and Akman-Demir, Gulsen and Seifert-Held, Thomas and Kristoferitsch, Wolfgang and Melms, Arthur and Wandinger, Klaus-Peter and Wildemann, Brigitte (2011) Frequency and syndrome specificity of antibodies to aquaporin-4 in neurological patients with rheumatic disorders. MULTIPLE SCLEROSIS JOURNAL, 17 (9). pp. 1067-1073. ISSN 1352-4585,

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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.

Item Type: Article
Uncontrolled Keywords: SYSTEMIC-LUPUS-ERYTHEMATOSUS; EXTENSIVE TRANSVERSE MYELITIS; NEUROMYELITIS-OPTICA; ANTI-AQUAPORIN-4 ANTIBODY; MULTIPLE-SCLEROSIS; PLASMA-EXCHANGE; NMO-IGG; DIAGNOSTIC-CRITERIA; GLUTEN SENSITIVITY; MYASTHENIA-GRAVIS; 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
Subjects: 600 Technology > 610 Medical sciences Medicine
Divisions: Medicine > Lehrstuhl für Neurologie
Depositing User: Dr. Gernot Deinzer
Date Deposited: 02 Jun 2020 07:45
Last Modified: 02 Jun 2020 07:45
URI: https://pred.uni-regensburg.de/id/eprint/20356

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