TY - JOUR
T1 - Frequency and syndrome specificity of antibodies to aquaporin-4 in neurological patients with rheumatic disorders
AU - Jarius, Sven
AU - Jacobi, Christian
AU - De Seze, Jerome
AU - Zephir, Helene
AU - Paul, Friedemann
AU - Franciotta, Diego
AU - Rommer, Paulus
AU - Mader, Simone
AU - Kleiter, Ingo
AU - Reindl, Markus
AU - Akman-Demir, Gulsen
AU - Seifert-Held, Thomas
AU - Kristoferitsch, Wolfgang
AU - Melms, Arthur
AU - Wandinger, Klaus Peter
AU - Wildemann, Brigitte
PY - 2011
Y1 - 2011
N2 - 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.
AB - 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.
KW - antibody to aquaporin-4
KW - connective tissue disorders
KW - diagnosis
KW - longitudinally extensive transverse myelitis
KW - neuromyelitis optica (Devic's disease)
KW - neuropsychiatric lupus
KW - NMO-IgG
KW - rheumatic diseases
KW - scleroderma
KW - Sjögren's syndrome
KW - systemic lupus erythematosus
KW - vasculitis
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U2 - 10.1177/1352458511403958
DO - 10.1177/1352458511403958
M3 - Article
C2 - 21543553
AN - SCOPUS:84860389062
VL - 17
SP - 1067
EP - 1073
JO - Multiple Sclerosis
JF - Multiple Sclerosis
SN - 1352-4585
IS - 9
ER -