Table 4 Summary of characteristics of childhood onset SS
Authors | Tomiita, M et al. [17] | Cimaz, R et al. [16] | Yokogowa, N et al. [14] | Hammenfors, D.S et al. [13] | Ramos-Casals, M et al. [5] | Basiaga, M. L et al. [12] | Our data |
|---|---|---|---|---|---|---|---|
Data of country | Japan nationwide | Multi-center | USA | Multi-center | Multi-center | International | China |
Published year (Study year) | 1997 (1994.7–1995.4) | 2003 (1998–2000) | 2016 | 2020 | 2021 | 2021 | Present study |
No. cases | 42 | 40 | 26 | 67 | 158 | 300 | 39 |
Female: male ratio | / | 7:1 | 12:1 | 6.4:1 | 6.2:1 | 5:1 | 3.9:1 |
Age of onset (yrs)a | 10.7 (3, 15) | 10.7 | / | 10.2 (1, 17) | 13.2 | / | 9.5 (4.7, 14.5) |
Age of diagnosis (yrs) a | / | 12.4 | 12.3 (4,17.8) | 12.1 (4, 18) | 14.2 | 12(1, 17.8) | 10.8 (6.3, 15.0) |
Time-interval from onset to diagnosis (mths)a | / | / | / | / | / | 4.4 (0.4, 72.9) | |
Recurrent parotitis | 35.7 | 72.5 | 65.3 | 63.8 | 35.8 | 46.7 | 25.6 |
Dry eyes | 6b | 12.5 | 65.3 | 62.6 | 79.7 | 48.0 | 10.4 |
Dry mouth | / | 12.5 | 65.4 | 80.3 | 70.2 | 52.0 | 17.9 |
Extra-glandular manifestation | |||||||
Fever | 52.3 | 10.0 | 23.0 | / | 11.9 | 11.3 | 30.8 |
Skin | 26.1 | / | 15.4 | 28.8 | 9.3 | 9.0 | 51.3 |
Arthralgia | 28.6 | 10.0 | 57.7 | 57.6 | 9.9 | 53.6 | 17.9 |
Hematological | / | / | 15.4 | 27.6 | 2.6 | 17.3 | 35.9 |
Hepatic | 2.4 | 10.0 | / | / | / | / | 33.3 |
Renal | 7.1 | 7.5 | 19.2 | 5.1 | 4.5 | 9.0 | 20.5 |
Pulmonary | 2.4 | / | / | 1.7 | 5.2 | 8.3 | 7.7 |
Neurological | 7.1 | 5.0 | 23.1 | 5.1 | 0.6 | 11.3 | 2.6 |
Lymphadenopathy | 26.2 | 7.5 | 42.3 | 59.3 | 25.2 | 18.0 | 23.1 |
Immunological profile | |||||||
anti-SSA or SSB | / | 73.6 | 84.6 | 74.6 | 88.5 | 97.3 | 100 |
anti-SSA | 61.9 | / | 84.6 | 74.6 | 82.7 | 97.3 | 100 |
anti-SSB | 31.0 | / | 65.4 | 40.3 | 61.9 | 97.0 | 46.1 |
Positive ANA | 81.0 | 85 | 96.2 | 92.5 | 90.3 | 99.7 | 94.9 |
Elevated IgG | 76.2 | 53.1 | / | / | / | 76.7 | 89.7 |
Positive RF | 57.1 | 75.0 | 72.7 | 45.3 | 67.6 | 85.7 | 43.6 |
Elevated ESR | / | 68.4 | / | / | / | 69.2 | |
Labial salivary gland biopsy (No. positive/No.performed) | 69.0% | 100% | 100% (15/15) | 79.3% (23/29) | 96.7% (118/122) | 53.4% (70/131) | 100% (38/38) |