Neuromyelitis Optica Spectrum Disorder Prevalent in Sjogren Syndrome, Meta-Analysis Shows


A recent analysis revealed a significant association between neuromyelitis optica spectrum disorder and connective tissue disease in patients diagnosed or suspected with Sjogren syndrome.

In a meta-analysis newly published in the Journal of Integrative Neuroscience, findings revealed a frequent coexistence of neuromyelitis optica spectrum disorder (NMOSD) and connective tissue disease (CTD) among patients with a diagnosis or suspicion of Sjogren syndrome (SjS).1 These results imply that considerations should be taken for clinicians in the clinical practice for earlier diagnosis of these conditions as it may influence the timing of immunosuppressive therapy and the prevention of systemic disabilities.

In the literature review search, results yielded 3176 publications from 4 databases. Among these publications, 29 of them were included in the current systematic review. In the analysis of studies that recruited unselected patients with systemic lupus erythematosus (SLE) and SjS, investigators reported that the pooled percentages of NMOSD overlapping were 0.6% (95% CI, 0.1%-1.4%), and 6.5% (95% CI, 4.7-8.6), respectively.

Top Clinical Takeaways

  • The meta-analysis underscored the importance of early identification and accurate clinical evaluation for patients with both Sjogren syndrome and NMOSD.
  • Studies indicated a higher frequency of NMOSD among patients with rheumatologic disorders, particularly those with Sjogren syndrome and neurological manifestations.
  • Despite limitations in the analysis, the study emphasizes the need for large prospective studies to validate and enhance the accuracy of findings.

“Among all the studies, it was evident that patients with these conditions require early identification of both diseases and an accurate clinical evaluation to tailor effective therapy,” senior author Ennio Polilli, MSc, researcher in the department of clinical pathology at Pescara General Hospital in Italy, and colleagues wrote.1 “Identifying the coexistence of both diseases may reduce the risk of recurrence and clinical progression.”

READ MORE: Susceptibility-Weighted Imaging Features Show Promise in Distinguishing AQP4-Negative-NMOSD From MS

In this study, investigators aimed to estimate the association between CTD and MNOSD to help plan future effective strategies for optimal benefit. Authors conducted a systematic review of the literature published up until February 2023, among the included databases (PubMed, n = 272; Web of Science, n = 880; EmBase, n = 634; OVID, n = 1390). Then, researchers performed a random-effects proportional meta-analysis and evaluated the risk of bias of the included studies using the Joanna Briggs Institute checklist.2

Additional findings showed that studies that enrolled patients with rheumatologic disorders with a presence of nervous system symptoms involvement reported a higher rate of NMOSD, particularly women. For instance, the pooled percentage of NMOSD was 26.5% (95% CI, 5.5-54.6%) among patients with SjS. Similarly, in the recruitment of patients with NMOSD in the studies, investigators observed a pooled percentages of SjS or SLE of 7.0% and 3.5%, respectively.

“From a diagnostic standpoint, rheumatologists and neurologists may consider starting a work up for the diagnosis of coexisting conditions in their patients in high risk cases. For these cases, clinicians have to consider that patients should be monitored by a multidisciplinary team in order to prevent further relapses,” Polilli et al noted.1

All told, limitations of this analysis included it the small collection of studies and the low number of patients in the studies. The criteria for recruitment among the included literature were different, which researchers noted might have caused significantly differing results among studies. Authors also included studies with a retrospective design, of which only 1 was a cross-sectional design. Thus, investigators concluded that large prospective studies are needed to increase the accuracy of the data and confirm the implications of these results.

“From the point of view of rheumatologists, taking into account our data and those reported in the literature, it is reasonable to suggest that screening asymptomatic patients for NMOSD should not be recommended and that rheumatologic patients should begin a work up for NMOSD, especially in the presence of ON, NMO, longitudinal myelitis, or specific clinical signs of involvement of the following areas of the nervous system: brain, area postrema, diencephalon, or brainstem,” Polilli et al noted.1

1. Esposito JE, Annoni G, D'Amato M, et al. Systemic Connective Tissue Disease and Neuromyelitis Optica Spectrum Disorder Coexistence: A Systematic Review and Meta-Analysis. J Integr Neurosci. 2024;23(2):35. doi:10.31083/j.jin2302035
2. Munn Z, Moola S, Lisy K, Riitano D, Tufanaru C. Methodological guidance for systematic reviews of observational epidemiological studies reporting prevalence and cumulative incidence data. Int J Evid Based Healthc. 2015;13(3):147-153. doi:10.1097/XEB.0000000000000054
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