Table 3.
Diagnostic tests, immunological markers and systemic activity at the time of diagnosis
| Variable | Patients diagnosed with childhood-onset pSS (n = 39) |
|---|---|
| DIAGNOSTIC TESTS | |
| Abnormal ocular tests (any) | |
| Schirmer's test | NE |
| Rose bengal score/other ocular dye score | 9 (23.1) |
| Abnormal oral diagnostic tests (any) | |
| Unstimulated whole salivary flow | NE |
| Parotid sialography | NE |
| Salivary scintigraphy | 22 (81.5)a |
| Positive minor salivary gland biopsy | 38 (100)b |
| IMMUNOLOGICAL PROFILE | |
| Positive anti-SSA/SSB antibodies | 39 (100) |
| Anti-SSA antibodies | 39 (100) |
| Anti-SSB antibodies | 18 (46.1) |
| ANA | 37 (94.9) |
| Anti-dsDNA antibody | 0 |
| Low C3 levels | 4 (10.3) |
| Low C4 levels | 2 (5.1) |
| RF-positive | 17 (43.6) |
| Elevated ESR | 27 (69.2) |
| Elevated IgG | 35 (89.7) |
| SYSTEMIC ACTIVITY | |
| ESSDAI score/ Median(range) | 8 (2, 31) |
| ESSDAI domains (score > = 1) | |
| Constitutional | 11 (28.2) |
| Lymphadenopathy | 9 (23.1) |
| Glandular | 8 (20.5) |
| Articular | 4 (10.3) |
| Cutaneous | 14 (35.9) |
| Pulmonary | 3 (7.7) |
| Renal | 5 (12.8) |
| Muscular | 0 |
| PNS | 1 (2.6) |
| CNS | 0 |
| Hematological | 11 (28.2) |
| Biological | 31 (79.5) |
a Twelve patients didn’t complete Salivary scintigraphy
b One patient didn’t complete minor salivary gland biopsy
SS Sjögren syndrome, ESSDAI EULAR Sjögren's syndrome disease activity index, ESR Erythrocyte sedimentation rate, IgG Immunoglobulin G, PNS Peripheral nervous system, CNS Central nervous system, NE Not evaluated