Epidemiology of aquaporin-4 autoimmunity and neuromyelitis optica spectrum

Eoin P. Flanagan; Philippe Cabre; Brian G. Weinshenker; Jennifer St Sauver; Debra J. Jacobson; Masoud Majed; Vanda A. Lennon; Claudia F. Lucchinetti; Andrew McKeon; Marcelo Matiello; Nilifur Kale; Dean M. Wingerchuk; Jay Mandrekar; Jessica A. Sagen; James P. Fryer; Angala Borders Robinson; Sean J. Pittock

doi:10.1002/ana.24617

Epidemiology of aquaporin-4 autoimmunity and neuromyelitis optica spectrum

Eoin P. Flanagan, Philippe Cabre, Brian G. Weinshenker, Jennifer St Sauver, Debra J. Jacobson, Masoud Majed, Vanda A. Lennon, Claudia F. Lucchinetti, Andrew McKeon, Marcelo Matiello, Nilifur Kale, Dean M. Wingerchuk, Jay Mandrekar, Jessica A. Sagen, James P. Fryer, Angala Borders Robinson, Sean J. Pittock

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133 Scopus citations

Abstract

Objective Neuromyelitis optica (NMO) and its spectrum disorders (NMOSD) are inflammatory demyelinating diseases (IDDs) with a specific biomarker, aquaporin-4-immunoglobulin G (AQP4-IgG). Prior NMO/NMOSD epidemiological studies have been limited by lack of AQP4-IgG seroprevalence assessment, absence of population-based USA studies, and under-representation of blacks. To overcome these limitations, we sought to compare NMO/NMOSD seroepidemiology across 2 ethnically divergent populations. Methods We performed a population-based comparative study of the incidence (2003-2011) and prevalence (on December 31, 2011) of NMO/NMOSD and AQP4-IgG seroincidence and seroprevalence (sera collected in 80-84% of IDD cases) among patients with IDD diagnosis in Olmsted County, Minnesota (82% white [Caucasian]) and Martinique (90% black [Afro-Caribbean]). AQP4-IgG was measured by M1 isoform fluorescence-activated cell-sorting assays. Results The age- and sex-adjusted incidence (7.3 vs 0.7/1,000,000 person-years [p < 0.01]) and prevalence (10 vs 3.9/100,000 [p = 0.01]) in Martinique exceeded that in Olmsted County. The AQP4-IgG age- and sex-adjusted seroincidence (6.5 vs 0.7/1,000,000 person-years [p < 0.01]) and seroprevalence (7.9 vs 3.3/100,000 [p = 0.04]) were also higher in Martinique than Olmsted County. The ethnicity-specific prevalence was similar in Martinique and Olmsted County: 11.5 and 13/100,000 in blacks, and 6.1 and 4.0/100,000 in whites, respectively. NMO/NMOSD represented a higher proportion of IDD cases in Martinique than Olmsted County (16% vs 1.4%; p < 0.01). The onset age (median = 35-37 years) and female:male distribution (5-9:1) were similar across both populations; 60% of prevalent cases were either blind in 1 eye, dependent on a gait aid, or both. Interpretation This study reports the highest prevalence of NMO/NMOSD in any population (10/100,000 in Martinique), estimates it affects 16,000 to 17,000 in the USA (higher than previous predictions), and demonstrates it disproportionately affects blacks.

Original language	English (US)
Pages (from-to)	775-783
Number of pages	9
Journal	Annals of neurology
Volume	79
Issue number	5
DOIs	https://doi.org/10.1002/ana.24617
State	Published - May 1 2016

ASJC Scopus subject areas

Neurology
Clinical Neurology

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10.1002/ana.24617

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Flanagan, E. P., Cabre, P., Weinshenker, B. G., Sauver, J. S., Jacobson, D. J., Majed, M., Lennon, V. A., Lucchinetti, C. F., McKeon, A., Matiello, M., Kale, N., Wingerchuk, D. M., Mandrekar, J., Sagen, J. A., Fryer, J. P., Robinson, A. B., & Pittock, S. J. (2016). Epidemiology of aquaporin-4 autoimmunity and neuromyelitis optica spectrum. Annals of neurology, 79(5), 775-783. https://doi.org/10.1002/ana.24617

Flanagan, EP, Cabre, P, Weinshenker, BG, Sauver, JS, Jacobson, DJ, Majed, M, Lennon, VA , Lucchinetti, CF , McKeon, A, Matiello, M, Kale, N, Wingerchuk, DM , Mandrekar, J, Sagen, JA, Fryer, JP, Robinson, AB & Pittock, SJ 2016, 'Epidemiology of aquaporin-4 autoimmunity and neuromyelitis optica spectrum', Annals of neurology, vol. 79, no. 5, pp. 775-783. https://doi.org/10.1002/ana.24617

@article{f0b110f598e64c949bcbd7aeccfc5f30,

title = "Epidemiology of aquaporin-4 autoimmunity and neuromyelitis optica spectrum",

abstract = "Objective Neuromyelitis optica (NMO) and its spectrum disorders (NMOSD) are inflammatory demyelinating diseases (IDDs) with a specific biomarker, aquaporin-4-immunoglobulin G (AQP4-IgG). Prior NMO/NMOSD epidemiological studies have been limited by lack of AQP4-IgG seroprevalence assessment, absence of population-based USA studies, and under-representation of blacks. To overcome these limitations, we sought to compare NMO/NMOSD seroepidemiology across 2 ethnically divergent populations. Methods We performed a population-based comparative study of the incidence (2003-2011) and prevalence (on December 31, 2011) of NMO/NMOSD and AQP4-IgG seroincidence and seroprevalence (sera collected in 80-84% of IDD cases) among patients with IDD diagnosis in Olmsted County, Minnesota (82% white [Caucasian]) and Martinique (90% black [Afro-Caribbean]). AQP4-IgG was measured by M1 isoform fluorescence-activated cell-sorting assays. Results The age- and sex-adjusted incidence (7.3 vs 0.7/1,000,000 person-years [p < 0.01]) and prevalence (10 vs 3.9/100,000 [p = 0.01]) in Martinique exceeded that in Olmsted County. The AQP4-IgG age- and sex-adjusted seroincidence (6.5 vs 0.7/1,000,000 person-years [p < 0.01]) and seroprevalence (7.9 vs 3.3/100,000 [p = 0.04]) were also higher in Martinique than Olmsted County. The ethnicity-specific prevalence was similar in Martinique and Olmsted County: 11.5 and 13/100,000 in blacks, and 6.1 and 4.0/100,000 in whites, respectively. NMO/NMOSD represented a higher proportion of IDD cases in Martinique than Olmsted County (16% vs 1.4%; p < 0.01). The onset age (median = 35-37 years) and female:male distribution (5-9:1) were similar across both populations; 60% of prevalent cases were either blind in 1 eye, dependent on a gait aid, or both. Interpretation This study reports the highest prevalence of NMO/NMOSD in any population (10/100,000 in Martinique), estimates it affects 16,000 to 17,000 in the USA (higher than previous predictions), and demonstrates it disproportionately affects blacks.",

author = "Flanagan, {Eoin P.} and Philippe Cabre and Weinshenker, {Brian G.} and Sauver, {Jennifer St} and Jacobson, {Debra J.} and Masoud Majed and Lennon, {Vanda A.} and Lucchinetti, {Claudia F.} and Andrew McKeon and Marcelo Matiello and Nilifur Kale and Wingerchuk, {Dean M.} and Jay Mandrekar and Sagen, {Jessica A.} and Fryer, {James P.} and Robinson, {Angala Borders} and Pittock, {Sean J.}",

note = "Publisher Copyright: {\textcopyright} 2016 American Neurological Association.",

year = "2016",

month = may,

day = "1",

doi = "10.1002/ana.24617",

language = "English (US)",

volume = "79",

pages = "775--783",

journal = "Annals of neurology",

issn = "0364-5134",

publisher = "John Wiley and Sons Inc.",

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TY - JOUR

T1 - Epidemiology of aquaporin-4 autoimmunity and neuromyelitis optica spectrum

AU - Flanagan, Eoin P.

AU - Cabre, Philippe

AU - Weinshenker, Brian G.

AU - Sauver, Jennifer St

AU - Jacobson, Debra J.

AU - Majed, Masoud

AU - Lennon, Vanda A.

AU - Lucchinetti, Claudia F.

AU - McKeon, Andrew

AU - Matiello, Marcelo

AU - Kale, Nilifur

AU - Wingerchuk, Dean M.

AU - Mandrekar, Jay

AU - Sagen, Jessica A.

AU - Fryer, James P.

AU - Robinson, Angala Borders

AU - Pittock, Sean J.

PY - 2016/5/1

Y1 - 2016/5/1

N2 - Objective Neuromyelitis optica (NMO) and its spectrum disorders (NMOSD) are inflammatory demyelinating diseases (IDDs) with a specific biomarker, aquaporin-4-immunoglobulin G (AQP4-IgG). Prior NMO/NMOSD epidemiological studies have been limited by lack of AQP4-IgG seroprevalence assessment, absence of population-based USA studies, and under-representation of blacks. To overcome these limitations, we sought to compare NMO/NMOSD seroepidemiology across 2 ethnically divergent populations. Methods We performed a population-based comparative study of the incidence (2003-2011) and prevalence (on December 31, 2011) of NMO/NMOSD and AQP4-IgG seroincidence and seroprevalence (sera collected in 80-84% of IDD cases) among patients with IDD diagnosis in Olmsted County, Minnesota (82% white [Caucasian]) and Martinique (90% black [Afro-Caribbean]). AQP4-IgG was measured by M1 isoform fluorescence-activated cell-sorting assays. Results The age- and sex-adjusted incidence (7.3 vs 0.7/1,000,000 person-years [p < 0.01]) and prevalence (10 vs 3.9/100,000 [p = 0.01]) in Martinique exceeded that in Olmsted County. The AQP4-IgG age- and sex-adjusted seroincidence (6.5 vs 0.7/1,000,000 person-years [p < 0.01]) and seroprevalence (7.9 vs 3.3/100,000 [p = 0.04]) were also higher in Martinique than Olmsted County. The ethnicity-specific prevalence was similar in Martinique and Olmsted County: 11.5 and 13/100,000 in blacks, and 6.1 and 4.0/100,000 in whites, respectively. NMO/NMOSD represented a higher proportion of IDD cases in Martinique than Olmsted County (16% vs 1.4%; p < 0.01). The onset age (median = 35-37 years) and female:male distribution (5-9:1) were similar across both populations; 60% of prevalent cases were either blind in 1 eye, dependent on a gait aid, or both. Interpretation This study reports the highest prevalence of NMO/NMOSD in any population (10/100,000 in Martinique), estimates it affects 16,000 to 17,000 in the USA (higher than previous predictions), and demonstrates it disproportionately affects blacks.

AB - Objective Neuromyelitis optica (NMO) and its spectrum disorders (NMOSD) are inflammatory demyelinating diseases (IDDs) with a specific biomarker, aquaporin-4-immunoglobulin G (AQP4-IgG). Prior NMO/NMOSD epidemiological studies have been limited by lack of AQP4-IgG seroprevalence assessment, absence of population-based USA studies, and under-representation of blacks. To overcome these limitations, we sought to compare NMO/NMOSD seroepidemiology across 2 ethnically divergent populations. Methods We performed a population-based comparative study of the incidence (2003-2011) and prevalence (on December 31, 2011) of NMO/NMOSD and AQP4-IgG seroincidence and seroprevalence (sera collected in 80-84% of IDD cases) among patients with IDD diagnosis in Olmsted County, Minnesota (82% white [Caucasian]) and Martinique (90% black [Afro-Caribbean]). AQP4-IgG was measured by M1 isoform fluorescence-activated cell-sorting assays. Results The age- and sex-adjusted incidence (7.3 vs 0.7/1,000,000 person-years [p < 0.01]) and prevalence (10 vs 3.9/100,000 [p = 0.01]) in Martinique exceeded that in Olmsted County. The AQP4-IgG age- and sex-adjusted seroincidence (6.5 vs 0.7/1,000,000 person-years [p < 0.01]) and seroprevalence (7.9 vs 3.3/100,000 [p = 0.04]) were also higher in Martinique than Olmsted County. The ethnicity-specific prevalence was similar in Martinique and Olmsted County: 11.5 and 13/100,000 in blacks, and 6.1 and 4.0/100,000 in whites, respectively. NMO/NMOSD represented a higher proportion of IDD cases in Martinique than Olmsted County (16% vs 1.4%; p < 0.01). The onset age (median = 35-37 years) and female:male distribution (5-9:1) were similar across both populations; 60% of prevalent cases were either blind in 1 eye, dependent on a gait aid, or both. Interpretation This study reports the highest prevalence of NMO/NMOSD in any population (10/100,000 in Martinique), estimates it affects 16,000 to 17,000 in the USA (higher than previous predictions), and demonstrates it disproportionately affects blacks.

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Epidemiology of aquaporin-4 autoimmunity and neuromyelitis optica spectrum

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