Pediatric Neuromyelitis Optica Spectrum Disorder and Sjögren Syndrome: More Common Than Previously Thought?

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Table 1.

Cases of pediatric NMO spectrum disorder and confirmed or probable SS.

Case	SS	Race	Age, Yrs	Sex	SSA, Units^*	SSB, Units^*	ANA Titer^†	NMO-IgG Serum	Salivary Biopsy Findings	Miscellaneous
#1	Confirmed	African American	11	F	108.7	35.5	1:160	Positive	Aggregates of minor salivary glands with several foci of periductal primarily lymphocytic inflammation consistent with SS.	Positive Schirmer test
#2	Confirmed	African American	13	F	108.9	76.7	≥ 1:1280	Positive	Multiple sections show regional mucous gland lobules exhibiting multiple discrete foci of lymphocytes and plasma cells consistent with SS.	Normal Schirmer test
#3	Probable	African American	15	M	109.6	68.5	≥ 1:1280	Positive	Deferred given history of autism and need for general anesthesia.	Anti-RNP antibody–positive, 113.9 units^*
#4	Confirmed	White	11	M	158.3	Negative	1:160	Positive	Multiple sections show regional mucous gland lobules exhibiting duct ectasia, acinar degeneration, stromal fibrosis, and an infiltrate of lymphocytes and plasma cells, and focus score > 0 foci/4 mm².	Reduced salivary pooling. Salivary gland cysts visualized on ultrasound.

↵* Normal range for anti-SSA, anti-SSB, and anti-RNP antibodies based on ELISA testing: 0–19 units.
↵† ANA titer reference range by immunofluorescence testing: 1:20. NMO: neuromyelitis optica; SS: Sjögren syndrome; ANA: antinuclear antibody; IgG: immunoglobulin G.