Neuromyelitis optica spectrum disorder as an initial presentation of primary Sjögren's syndrome.

Longitudinal myelitis in patients with Sjögren's syndrome (SS) is a rarely reported occurrence. Here, we present a patient with longitudinal myelitis who was found to have both primary SS and a positive antibody to aquaporin-4 (NMO-IgG). We review the recent literature concerning the overlap between primary SS-associated myelitis and the presence of NMO-IgG, suggestive of a neuromyelitis optica spectrum disorder (NMOSD). A patient with longitudinal myelitis, SS, and a positive NMO-IgG is presented. A review of the relevant English literature based on a PubMed and Embase search is then discussed. The keywords used were Sjögren's syndrome, myelitis (longitudinal and transverse), neuromyelitis optica, and Devic's disease. Our patient fulfills the criteria for both primary SS and NMOSD. Several small studies have shown that most SS patients with longitudinal myelitis are positive for the antibody to aquaporin-4. Additionally, minor salivary gland biopsies of patients with NMO or NMOSD have evidence of lymphocytic inflammation, suggesting that there may be an overlap in the mechanism of NMOSD and longitudinal myelitis secondary to primary SS. This overlap in mechanism may have important ramifications with regard to prognosis and treatment of SS-related myelitis. The presentation of longitudinal myelitis in a patient with SS should be considered a possible NMOSD. Checking for the presence antiaquaporin-4 antibodies provides important prognostic information and may help to guide treatment decisions.

Kahlenberg JM 《-》

[Neuromyelitis optica spectrum disorders as initial presentation of Sjögren's syndrome: A case report].

Alva Díaz C ，Andamayo Villalba L ，Mori N ，Ventura Chilón JJ ，Romero R ... -  《-》

Neuromyelitis optica and non organ-specific autoimmunity.

Pittock SJ ，Lennon VA ，de Seze J ，Vermersch P ，Homburger HA ，Wingerchuk DM ，Lucchinetti CF ，Zéphir H ，Moder K ，Weinshenker BG ... -  《archives of neurology》

Brain abnormalities in Sjogren syndrome with recurrent CNS manifestations: association with neuromyelitis optica.

Min JH ，Kim HJ ，Kim BJ ，Lee KW ，Sunwoo IN ，Kim SM ，Kim BJ ，Kim SH ，Park MS ，Waters P ，Vincent A ，Sung JJ ，Lee KH ... -  《-》

Presence of anti-Ro/SSA antibody may be associated with anti-aquaporin-4 antibody positivity in neuromyelitis optica spectrum disorder.

Neuromyelitis optica (NMO) is often associated with systemic autoimmune diseases or serological markers of non-organ-specific autoimmunity, and has been most frequently associated with Sjögren's syndrome and anti-Ro/SSA antibody (SSA-Ab) positivity in Asian populations. We evaluated the clinical significance of anti-Ro/SSA antibody positivity in patients with NMO spectrum disorder (NMOSD). We retrospectively collected data from 106 consecutive patients with NMOSD and reviewed clinical features and laboratory findings. All patients underwent tests for SSA-Ab and anti-aquaporin-4 antibody (AQP4-Ab) using cell-based indirect immunofluorescence assays. Among 106 patients, 20 (18.9%) were positive for SSA-Ab. Of 48 AQP4-Ab-positive patients, 18 (37.5%) had SSA-Ab. AQP4-Ab seropositivity was 90.0% in patients positive for SSA-Ab, and 32.6% in patients without SSA-Ab (p<0.001). Presence of SSA-Ab was associated with systemic autoimmune diseases, including Sjögren's syndrome (p<0.001) and systemic lupus erythematosus (p=0.003), and with the presence of non-organ-specific autoantibodies such as anti-nuclear antibody and anti-dsDNA antibody in patients with NMOSD, but was not associated with annualized relapse rate or final Expanded Disability Status Scale score independent of AQP4-Ab positivity. We found that the presence of SSA-Ab was highly associated with seropositivity for AQP4-Ab in patients with NMOSD.

Park JH ，Hwang J ，Min JH ，Kim BJ ，Kang ES ，Lee KH ... -  《-》