Comparisons of presentations and outcomes of neuromyelitis optica patients with and without Sjögren’s syndrome

Abstract

Patients with neuromyelitis optica (NMO) often have an accompanying autoimmune disease, most commonly, but not limited to Sjögren's syndrome (SS). The aim of this study was to compare clinical and laboratory features between NMO patients with and without SS and to investigate the prognosis of NMO in patients with and without SS. Twenty-three NMO patients with SS and 42 NMO patients without SS were included. Clinical and laboratory profiles were compared, including annual relapse rate and time from onset of NMO to Expanded Disability Status Scale (EDSS) scores of 4.0 and 6.0. More NMO patients with SS than those without SS had anti-nuclear antibody, anti-SS-A/Ro and anti-SS-B/La antibodies (91.3 vs. 35.7%, p < 0.001, 87.0 vs. 2.3%, p < 0.001, and 34.8 vs. 0.0%, p < 0.001, respectively). Serum immunoglobulins (IgA, IgM and IgG) were markedly increased in NMO patients with SS in comparison with those without SS. Annual relapse rate and the time from disease onset to an EDSS score of 4.0 and 6.0 were not significantly different between the two groups. No differences between the two groups were found for the other parameters, including AQP-4 antibody status, length of spinal cord lesion and brain lesions. These results imply that NMO in SS more likely represents coexistence with SS rather than representing the result of direct central nervous system involvement in SS. Autoimmune response appears to be more intense in the NMO group with SS, but did not cause a more severe prognosis in comparison with the group without SS, indicating that we should pay attention to the potential benefit of the antinuclear antibodies in NMO.

Devic syndrome and pregnancy: A case series

“…Other suggested hypotheses include changes in T-helper cell function in pregnancy, akin to diseases such as systemic lupus 10 or Sjogren's, both of which are actually more common in patients with NMO. 11 With regard to the effect that NMO has on pregnancy, we showed that 15% of the pregnancies studied resulted in miscarriage, which is comparable to the 13% spontaneous miscarriage rate seen in a retrospective cohort of AQP4 positive women from the National NMO Service (Oxford, UK) 5 and higher than expected given that the median age of our patients was 27 years old. Three of the 8 pregnancies were preterm live births occurring at 36, 34, and 33 weeks (mean gestational age 34.3 weeks, SD 1.25).…”

Comprehensive review of neuromyelitis optica and clinical characteristics of neuromyelitis optica patients in Puerto Rico

“…Sjogren's myelitis has longitudinal spinal cord involvement that can be diagnosed as NMO or is AQP4+; therefore, most CNS involvement is due to coexisting NMOSD rather than direct CNS involvement. [130178310] In multiple studies, these types of comorbidities amongst NMO patients are discussed as an indication that NMO could be a complication of multisystem rheumatologic disease. Furthermore, the coexistence of these autoimmune comorbidities also suggested that NMO is an indication of a genetic propensity toward humoral autoimmunity.…”

Serum Clusterin and Complement Factor H May Be Biomarkers Differentiate Primary Sjögren's Syndrome With and Without Neuromyelitis Optica Spectrum Disorder

“…Generally, the autoimmune response appears to be more intense in the NMO group with SS (3). The prognosis of NMOSD patients complicated with autoimmune diseases was considered to be more poor than NMOSD patients without (4–6).…”