Childhood-onset primary Sjögren’s syndrome in a tertiary center in China: clinical features and outcome
Authors' conclusion
The presentation of childhood-onset pSS is atypical, and extraglandular manifestations and systemic involvement are more common than in adult-onset pSS.
Study context
- Population
- Patients <18 years diagnosed with primary Sjögren's syndrome (pSS) by paediatric rheumatologists, tertiary center in China, 2013–2020
- Participants
- 39 patients
- Outcome
- Clinical features of childhood-onset pSS
Methodological detailssingle-centre, tertiary center, Electronic medical records from 2013 to 2020 collected and analysed
- Single-centre
- Tertiary center
- Electronic medical records from 2013 to 2020 collected and analysed
Main finding
As reported in the abstract
- Fulfilled AECG criteria
- 27 (69.2%)
- Fulfilled ACR/EULAR criteria
- 38 (97.4%)
- Fulfilled proposed juvenile pSS criteria
- 35 (89.7%)
- Female:male ratio
- 3.9:1
- Median age at first signs or symptoms, years
- 9.2 (4.7, 14.5)
- Median age at diagnosis, years
- 10.9 (6.3, 15.0)
- Rash or purpura
- 20, 51.3%
- Fever
- 12, 30.8%
- Glandular enlargement/recurrent parotitis
- 10, 25.6%
- Dry mouth and/or dry eyes
- 9, 23.1%
- Systemic damage
- 28 (56.4%)
- Haematological involvement
- 14, 35.9%
- Hepatic involvement
- 13, 33.3%
- Renal involvement
- 8, 20.5%
- Labial minor salivary gland biopsy; all exhibited focal lymphocytic sialadenitis
- 38 (97.4%)
- Global ESSDAI score ≥ 1 at diagnosis
- All patients
- Median total ESSDAI score at diagnosis
- 8 (2, 31)
Observational data: associations, not effects.
Further findings
Development of systemic lupus erythematosus (SLE) during follow-up · median follow-up 23.6 (7.9, 79.5) months
- Followed up
- 36 (92.3%)
- Developed SLE
- 3
- Follow-up times at SLE development, months
- 13.3, 38.8 and 63.8
Original publication
Childhood-onset primary Sjögren’s syndrome in a tertiary center in China: clinical features and outcome. Pediatr Rheumatol Online J. Published 27 January 2023. doi:10.
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