TY - JOUR
T1 - Anti-aquaporin-4 titer is not predictive of disease course in neuromyelitis optica spectrum disorder
T2 - A multicenter cohort study
AU - Kessler, Remi A.
AU - Mealy, Maureen A.
AU - Jimenez-Arango, Jorge Andres
AU - Quan, Chao
AU - Paul, Friedemann
AU - López, Reydmar
AU - Hopkins, Sarah
AU - Levy, Michael
N1 - Funding Information:
RAK and MAM have no disclosures to report. Dr. Levy receives research support from NIH, Guthy Jackson Charitable Foundation, Viropharma, Acorda, Sanofi, NeuralStem and Genentech, and serves as a consultant for Chugai Pharmaceuticals, GlaxoSmithKline and Medimmune. FP is supported by Bundesministerium für Bildung und Forschung (Competence Network Multiple Sclerosis) and Guthy Jackson Charitable Foundation.
Publisher Copyright:
© 2017
PY - 2017/10
Y1 - 2017/10
N2 - Background Neuromyelitis optica spectrum disorder (NMOSD) is a rare autoimmune disease associated with a serological antibody to aquaporin-4 (AQP4) detectable in up to 80% of patients. The enzyme-linked immunosorbent assay (ELISA) is one of the most popular methods of testing for anti-AQP4 antibodies that results with a titer in which < 3 Units/ml is negative, 3–5 is borderline and 5+ is positive. The value of the positive titer in predicting long term disease course is currently unknown. Methods This is a retrospective analysis of NMOSD patients from five centers around the world: Baltimore, USA, Philadelphia, USA, Shanghai, China, Berlin, Germany, and Medellin, Columbia, where ELISA titers on anti-AQP4 antibody testing is available. Inclusion criteria include a diagnosis of NMOSD and seropositive anti-AQP4 antibody test with titer = /> 3 Units/ml. Patients were stratified into three groups by titer: 3–30 Units/ml (low), 31–100 Units/ml (medium), and 101+ Units/ml (high). Demographic factors such as age at onset, race, and sex were collected along with clinical features such as annualized relapse rate, duration of disease, location of relapses, and treatment history. Results A total of 139 NMOSD patients met criteria for inclusion in this study, stratified into three groups by titer: 42 subjects with low titers of 3–30 Units/ml, 30 subjects with medium titers of 31–100 Units/ml and 67 subjects with high titers of 101 or greater ELISA Units/ml. The average age at onset, sex and race distribution were not significantly different among the groups. The number of patients untreated in each group was similar (< 25%) as was the average annualized relapse rate (0.591–0.821 relapses/year). With an average of 10 years follow up, the average disability level was not different among the three titer groups (EDSS range 3.03–3.48). The distribution of lesions, as well as their preventive treatment regimens did not differ significantly. Conclusion Beyond a positive/borderline/negative result, the titer of the anti-AQP4 antibody ELISA assay is not predictive in the disease course for patients with NMOSD. Low titer patients experience the same disease course as medium-titer and high-titer anti-AQP4 antibody patients with NMOSD.
AB - Background Neuromyelitis optica spectrum disorder (NMOSD) is a rare autoimmune disease associated with a serological antibody to aquaporin-4 (AQP4) detectable in up to 80% of patients. The enzyme-linked immunosorbent assay (ELISA) is one of the most popular methods of testing for anti-AQP4 antibodies that results with a titer in which < 3 Units/ml is negative, 3–5 is borderline and 5+ is positive. The value of the positive titer in predicting long term disease course is currently unknown. Methods This is a retrospective analysis of NMOSD patients from five centers around the world: Baltimore, USA, Philadelphia, USA, Shanghai, China, Berlin, Germany, and Medellin, Columbia, where ELISA titers on anti-AQP4 antibody testing is available. Inclusion criteria include a diagnosis of NMOSD and seropositive anti-AQP4 antibody test with titer = /> 3 Units/ml. Patients were stratified into three groups by titer: 3–30 Units/ml (low), 31–100 Units/ml (medium), and 101+ Units/ml (high). Demographic factors such as age at onset, race, and sex were collected along with clinical features such as annualized relapse rate, duration of disease, location of relapses, and treatment history. Results A total of 139 NMOSD patients met criteria for inclusion in this study, stratified into three groups by titer: 42 subjects with low titers of 3–30 Units/ml, 30 subjects with medium titers of 31–100 Units/ml and 67 subjects with high titers of 101 or greater ELISA Units/ml. The average age at onset, sex and race distribution were not significantly different among the groups. The number of patients untreated in each group was similar (< 25%) as was the average annualized relapse rate (0.591–0.821 relapses/year). With an average of 10 years follow up, the average disability level was not different among the three titer groups (EDSS range 3.03–3.48). The distribution of lesions, as well as their preventive treatment regimens did not differ significantly. Conclusion Beyond a positive/borderline/negative result, the titer of the anti-AQP4 antibody ELISA assay is not predictive in the disease course for patients with NMOSD. Low titer patients experience the same disease course as medium-titer and high-titer anti-AQP4 antibody patients with NMOSD.
KW - Aquaporin-4, NMO-IgG, anti-AQP4, titer
KW - Devic's disease
KW - Neuromyelitis optica
KW - Relapses
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U2 - 10.1016/j.msard.2017.08.005
DO - 10.1016/j.msard.2017.08.005
M3 - Article
C2 - 29055457
AN - SCOPUS:85027832394
SN - 2211-0348
VL - 17
SP - 198
EP - 201
JO - Multiple Sclerosis and Related Disorders
JF - Multiple Sclerosis and Related Disorders
ER -