Table 2.
Demographic, clinical and immunological characteristics in Sjögren’s Syndrome (SS) Patients
| SS NMOSD (N=11) |
SS non- NMOSD demyelinating (N=8) |
SS non- demyelinating (N=90) |
p-values (SS NMOSD vs. SS non- demyelinating) |
p-values (NMOSD vs. non-NMOSD demyelinating) |
|
|---|---|---|---|---|---|
| Demographic Features | |||||
| Age at onset of sicca symptoms, years, Median (range) |
49.5 (25–63) | 41.5 (30–60) | 46 (14–72) | 0.28 | 0.37 |
| Gender, Female % (N) | 90.9 (10) | 87.5 (7) | 88.9 (80) | 1.00 | 1.00 |
| Ethnicity, non-Caucasian % (N) | 54.5 (6) | 50 (4) | 13.3 (12) | 0.65 | < 0.01 |
| African-American % (N) | 27.3 (3) | 50 (4) | 7.8 (7) | ||
| Hispanic % (N) | 9.1 (1) | 0 (0) | 0.0 (0) | ||
| Asian or Pacific Islander% (N) | 18.2 (2) | 0 (0) | 3.3 (3) | ||
| Other % (N) | 0.0 (0) | 0 (0) | 2.2 (2) | ||
|
Clinical, Antibody, and Immunological Features |
|||||
| Dry eyes % (N) | 90.9 (10) | 87.5 (7) | 92.2 (83) | 1.00 | 1.00 |
| Dry mouth % (N) | 100 (11) | 75 (6) | 96.7 (87) | 1.00 | 0.16 |
| Anti-nuclear antibodies, % (N) | 81.8 (9) | 50 (4) | 70.0 (63) | 0.51 | 0.32 |
| Anti-Ro52 antibodies % (N) | 72.7 (8) | 75 (6) | 63.3 (57) | 0.74 | 1.00 |
| Anti-Ro60 antibodies % (N) | 90.9 (10) | 75 (6) | 62.2 (56) | 0.09 | 0.55 |
| Anti-La/SS-B antibodies % (N) | 45.5 (5) | 25 (2) | 40.0 (36) | 0.73 | 0.63 |
| Rheumatoid Factor % (N) | 20.0 (2/10) | 25 (2) | 45.6 (41) | 0.18 | 1.00 |
| Polyclonal Gammopathy % (#/N) | 40.0 (4/10) | 50 (4/8) | 36.0 (31/86) | 1.00 | 1.00 |
| Other Neurological Syndromes | |||||
| PNS Syndromes % (N) | 0 (0) | 0 (0) | 8 (9) | N/Aa | N/Aa |
| Headache % (N) | 0 (0) | 50 (4) | 23 (21) | 0.06 | 0.02 |
| Cognitive Complaints % (N) | 0 (0) | 13 (1) | 11 (10) | 0.60 | 0.42 |
| Depression % (N) | 27 (3) | 13 (1) | 17 (15) | 0.45 | 0.60 |
| Stroke % (N) | 0 (0) | 0 (0) | 6 (5) | 1.00 | 1.00 |
| Seizure % (N) | 0 (0) | 0 (0) | 3 (3) | 1.00 | 1.00 |
Legend: FACS = Fluorescent Activated Cell Sorting; NMOSD = Neuromyelitis Optica Spectrum Disorder;
a
The frequency of PNS syndromes could not be determined in an unselected cohort of SS patients, given that such PNS syndromes constituted a reason for referral to our SS Center. Subtypes of PNS syndromes includes six patients with axonal sensorimotor polyneuropathies, three patients with sensory neuronopathies, and one patient with small-fiber neuropathy.