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) |