Neuromyelitis optica spectrum disorders: comparison according to the phenotype and serostatus
OBJECTIVE: To (1) determine the value of the recently proposed criteria of neuromyelitis optica (NMO) spectrum disorder (NMOSD) that unify patients with NMO and those with limited forms (NMO/LF) with aquaporin-4 immunoglobulin G (AQP4-IgG) antibodies; and (2) investigate the clinical significance of...
| Authors: | ; ; |
|---|---|
| Format: | Article |
| Status: | Published version |
| Publication date: | 2016 |
| Country: | España |
| Institution: | Universitat Pompeu Fabra |
| Repository: | Repositorio Digital de la UPF |
| OAI Identifier: | oai:repositori.upf.edu:10230/27111 |
| Online Access: | http://hdl.handle.net/10230/27111 http://dx.doi.org/10.1212/NXI.0000000000000225 |
| Access Level: | Open access |
| Keyword: | Ulls -- Malalties i defectes |
| _version_ | 1878731964561752064 |
|---|---|
| author | Sepúlveda, Maria |
| author2 | Munteis Olivas, Elvira Saiz, Albert |
| author2_role | author author |
| author_browse | Munteis Olivas, Elvira Saiz, Albert Sepúlveda, Maria |
| author_facet | Sepúlveda, Maria Munteis Olivas, Elvira Saiz, Albert |
| author_role | author |
| collection | Repositorio Digital de la UPF |
| description | OBJECTIVE: To (1) determine the value of the recently proposed criteria of neuromyelitis optica (NMO) spectrum disorder (NMOSD) that unify patients with NMO and those with limited forms (NMO/LF) with aquaporin-4 immunoglobulin G (AQP4-IgG) antibodies; and (2) investigate the clinical significance of the serologic status in patients with NMO. METHODS: This was a retrospective, multicenter study of 181 patients fulfilling the 2006 NMO criteria (n = 127) or NMO/LF criteria with AQP4-IgG (n = 54). AQP4-IgG and myelin oligodendrocyte glycoprotein immunoglobulin G (MOG-IgG) antibodies were tested using cell-based assays. RESULTS: Patients were mainly white (86%) and female (ratio 6.5:1) with median age at onset 39 years (range 10-77). Compared to patients with NMO and AQP4-IgG (n = 94), those with NMO/LF presented more often with longitudinally extensive transverse myelitis (LETM) (p < 0.001), and had lower relapse rates (p = 0.015), but similar disability outcomes. Nonwhite ethnicity and optic neuritis presentation doubled the risk for developing NMO compared with white race (p = 0.008) or LETM presentation (p = 0.008). Nonwhite race (hazard ratio [HR] 4.3, 95% confidence interval [CI] 1.4-13.6) and older age at onset were associated with worse outcome (for every 10-year increase, HR 1.7, 95% CI 1.3-2.2). Patients with NMO and MOG-IgG (n = 9) had lower female:male ratio (0.8:1) and better disability outcome than AQP4-IgG-seropositive or double-seronegative patients (p < 0.001). CONCLUSIONS: In patients with AQP4-IgG, the similar outcomes regardless of the clinical phenotype support the unified term NMOSD; nonwhite ethnicity and older age at onset are associated with worse outcome. Double-seronegative and AQP4-IgG-seropositive NMO have a similar clinical outcome. The better prognosis of patients with MOG-IgG and NMO suggests that phenotypic and serologic classification is useful. |
| eu_rights_str_mv | openAccess |
| format | article |
| id | ES_3a3653eec2ff4be7d61ed1f792bbdbf2 |
| institution | Universitat Pompeu Fabra |
| instname_str | Universitat Pompeu Fabra |
| network_acronym_str | ES |
| network_name_str | España |
| oai_identifier_str | oai:repositori.upf.edu:10230/27111 |
| publishDate | 2016 |
| publishDateSort | 2016 |
| publisher | Wolters Kluwer (LWW) |
| publisherStr | Wolters Kluwer (LWW) |
| reponame_str | Repositorio Digital de la UPF |
| spelling | Neuromyelitis optica spectrum disorders: comparison according to the phenotype and serostatus Sepúlveda, Maria Munteis Olivas, Elvira Saiz, Albert Ulls -- Malalties i defectes OBJECTIVE: To (1) determine the value of the recently proposed criteria of neuromyelitis optica (NMO) spectrum disorder (NMOSD) that unify patients with NMO and those with limited forms (NMO/LF) with aquaporin-4 immunoglobulin G (AQP4-IgG) antibodies; and (2) investigate the clinical significance of the serologic status in patients with NMO. METHODS: This was a retrospective, multicenter study of 181 patients fulfilling the 2006 NMO criteria (n = 127) or NMO/LF criteria with AQP4-IgG (n = 54). AQP4-IgG and myelin oligodendrocyte glycoprotein immunoglobulin G (MOG-IgG) antibodies were tested using cell-based assays. RESULTS: Patients were mainly white (86%) and female (ratio 6.5:1) with median age at onset 39 years (range 10-77). Compared to patients with NMO and AQP4-IgG (n = 94), those with NMO/LF presented more often with longitudinally extensive transverse myelitis (LETM) (p < 0.001), and had lower relapse rates (p = 0.015), but similar disability outcomes. Nonwhite ethnicity and optic neuritis presentation doubled the risk for developing NMO compared with white race (p = 0.008) or LETM presentation (p = 0.008). Nonwhite race (hazard ratio [HR] 4.3, 95% confidence interval [CI] 1.4-13.6) and older age at onset were associated with worse outcome (for every 10-year increase, HR 1.7, 95% CI 1.3-2.2). Patients with NMO and MOG-IgG (n = 9) had lower female:male ratio (0.8:1) and better disability outcome than AQP4-IgG-seropositive or double-seronegative patients (p < 0.001). CONCLUSIONS: In patients with AQP4-IgG, the similar outcomes regardless of the clinical phenotype support the unified term NMOSD; nonwhite ethnicity and older age at onset are associated with worse outcome. Double-seronegative and AQP4-IgG-seropositive NMO have a similar clinical outcome. The better prognosis of patients with MOG-IgG and NMO suggests that phenotypic and serologic classification is useful. This study was supported in part by Red Española de Esclerosis Múltiple (REEM) Instituto de Salud Carlos III, Spain (RD07/0060/01, P.V.; RD12/0032/0002, A.S.; Marató de TV3 [20141830], F.G.) and Instituto de Salud Carlos III, Spain (CM14/00081; T.A.). Wolters Kluwer (LWW) http://hdl.handle.net/10230/27111 http://dx.doi.org/10.1212/NXI.0000000000000225 |
| spellingShingle | Neuromyelitis optica spectrum disorders: comparison according to the phenotype and serostatus Sepúlveda, Maria Ulls -- Malalties i defectes |
| status_str | publishedVersion |
| title | Neuromyelitis optica spectrum disorders: comparison according to the phenotype and serostatus |
| title_full | Neuromyelitis optica spectrum disorders: comparison according to the phenotype and serostatus |
| title_fullStr | Neuromyelitis optica spectrum disorders: comparison according to the phenotype and serostatus |
| title_full_unstemmed | Neuromyelitis optica spectrum disorders: comparison according to the phenotype and serostatus |
| title_short | Neuromyelitis optica spectrum disorders: comparison according to the phenotype and serostatus |
| title_sort | Neuromyelitis optica spectrum disorders: comparison according to the phenotype and serostatus |
| topic | Ulls -- Malalties i defectes |
| topic_facet | Ulls -- Malalties i defectes |
| url | http://hdl.handle.net/10230/27111 http://dx.doi.org/10.1212/NXI.0000000000000225 |