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.
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 humanAQP4. 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.
Authors: J Birnbaum; N M Atri; A N Baer; R Cimbro; J Montagne; L Casciola-Rosen Journal: Arthritis Care Res (Hoboken) Date: 2017-07 Impact factor: 4.794
Authors: Wajih Bukhari; Laura Clarke; Cullen O'Gorman; Elham Khalilidehkordi; Simon Arnett; Kerri M Prain; Mark Woodhall; Roger Silvestrini; Christine S Bundell; Sudarshini Ramanathan; David Abernethy; Sandeep Bhuta; Stefan Blum; Mike Boggild; Karyn Boundy; Bruce J Brew; Wallace Brownlee; Helmut Butzkueven; William M Carroll; Celia Chen; Alan Coulthard; Russell C Dale; Chandi Das; Keith Dear; Marzena J Fabis-Pedrini; David Fulcher; David Gillis; Simon Hawke; Robert Heard; Andrew P D Henderson; Saman Heshmat; Suzanne Hodgkinson; Sofia Jimenez-Sanchez; Trevor J Kilpatrick; John King; Chris Kneebone; Andrew J Kornberg; Jeannette Lechner-Scott; Ming-Wei Lin; Christopher Lynch; Richard A L Macdonnell; Deborah F Mason; Pamela A McCombe; Jennifer Pereira; John D Pollard; Stephen W Reddel; Cameron Shaw; Judith Spies; James Stankovich; Ian Sutton; Steve Vucic; Michael Walsh; Richard C Wong; Eppie M Yiu; Michael H Barnett; Allan G Kermode; Mark P Marriott; John Parratt; Mark Slee; Bruce V Taylor; Ernest Willoughby; Robert J Wilson; Fabienne Brilot; Angela Vincent; Patrick Waters; Simon A Broadley Journal: J Neurol Date: 2020-01-31 Impact factor: 4.849
Authors: M Ringelstein; O Aktas; J Harmel; D Prayer; S Jarius; B Wildemann; H-P Hartung; S Salhofer-Polanyi; F Leutmezer; P S Rommer Journal: Nervenarzt Date: 2014-10 Impact factor: 1.214