Clinical phenotypes, classification, and long-term outcomes of childhood-onset Sjögren's disease into adulthood: a single-centre cohort study
Authors' conclusion
This preliminary evaluation of long-term outcomes of childhood-onset Sjögren's disease in adulthood showed distinct patterns of disease and symptom trajectories and that a high proportion of children and young people develop damage in early adulthood.
Study context
- Population
- Individuals aged 13–36 years with childhood-onset SjD, single tertiary adolescent and young adult rheumatology service, University College London Hospital, UK28 (93%) female
- Participants
- 30 individuals
- Outcome
- Long-term disease trajectories (ESSDAI, ESSPRI, SSDDI) and complications of childhood-onset SjD
Methodological detailscombined retrospective and prospective, single-centre, long-term follow-up into adulthood
- Combined retrospective and prospective
- Single-centre
- Long-term follow-up into adulthood
Main finding
As reported in the abstract
High ESSDAI
3.9
mean · SD 2.2
Low ESSDAI
0.8
mean · SD 1.1
High EULAR Sjögren's Syndrome Patient Reported Index (ESSPRI)
5.6
mean · SD 2.7
Low ESSPRI
3.1
mean · SD 1.0
High ESSDAI vs Low ESSDAI: P < 0.0001 · High ESSPRI vs Low ESSPRI: P = 0.036
- Lymphoma
- Four (13%) of 30
- Accumulated damage (Sjögren's Syndrome Disease Damage Index (SSDDI) score ≥1)
- 17 (57%)
Observational data: associations, not effects.
Further findings
Damage accrual by activity and symptom trajectory
- Did not differ by activity trajectory
- P = 0.080
- Did not differ by symptom trajectory
- P = 1.0
Scores at last review
- Median ESSDAI (IQR)
- 2.0 (IQR 2.0-8.0)
- Median ESSPRI (IQR)
- 5.3 (3.0-7.0)
Dryness by diagnostic delay >3 years
- Delay >3 years vs ≤3 years
- nine [100%] of nine vs eight [38%] of 21; p=0.0014
Manifestations at onset
- Fatigue
- 22 [73%] of 30
- Arthralgia
- 21 [70%]
- Dryness
- 17 [57%]
- Glandular swelling
- 15 [50%]
- Skin rashes
- ten [30%]
- Mean age at onset, years (SD)
- 12.7 (SD 3.3)
Original publication
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