Prevalence of autoimmune comorbidities in aquaporin 4-immunoglobulin G positive neuromyelitis optica spectrum disorder: a systematic review and meta-analysis.
Ninglu Weng, Aikjot Randhawa, Karen Sayfee et al.
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In 30 seconds
This systematic review and meta-analysis estimated the prevalence of autoimmune comorbidities among people with aquaporin-4-immunoglobulin G-positive neuromyelitis optica spectrum disorder. Across 70 studies and 7843 participants, the pooled prevalence of at least one autoimmune comorbidity was 22.2% (95% CI, 19.3-25.3; I 2 = 78%). Sjögren's syndrome was the most frequently reported comorbidity, followed by systemic lupus erythematosus and autoimmune thyroid disease. Estimates varied across regions and income groups, and the authors advise caution because ascertainment methods were heterogeneous and validated diagnostic criteria were infrequently reported.
Key findings
- At least one autoimmune comorbidity had a pooled prevalence of 22.2% (95% CI, 19.3-25.3; I 2 = 78%).
- Sjögren's syndrome was most frequent (4.6%), followed by systemic lupus erythematosus (3.0%) and autoimmune thyroid disease (2.6%).
- Prevalence ranged from 31.1% in Europe to 16.8% in Asia; estimates were 25.1% in high-income and 7.0% in lower-middle-income settings.
Why it matters
Autoimmune comorbidities were documented in roughly one in five people with AQP4-IgG-positive NMOSD in the pooled evidence. Awareness of the commonly reported coexisting conditions may help clinicians interpret the broader clinical context, while substantial variation between studies and settings cautions against treating pooled estimates as universal.
What to keep in mind
Ascertainment methods were heterogeneous, and validated diagnostic criteria were infrequently reported. Prevalence estimates also varied substantially across studies (I 2 = 78%).