Sjögren's disease & overlap diseases

Juvenile Sjögren disease: time for age-specific classification criteria

Pediatr Rheumatol Online JPublished Retrospective cohort study

Authors' conclusion

Recurrent parotitis and scarce sicca manifestations are two distinct clinical features of juvenile versus adult SjD.

Study context

Population
Patients diagnosed with juvenile Sjögren’s disease (SjD) over the past five years, single centre
Participants
15 patients
Exposure
Primary SjD vs Secondary SjD
  • Primary SjDn = Ten
  • Secondary SjDn = five
Outcome
Clinical, laboratory and imaging manifestations of juvenile SjD
Methodological detailssingle-center, exploratory pilot
  • Single-center
  • Exploratory pilot

Main finding

As reported in the abstract

Median age of disease onset, years (range 8.5-17.5)
11.5
Median age at diagnosis, years (range 8.5-17.5)
12.5
Musculoskeletal manifestations at onset, n
9
Recurrent parotitis at onset, n
7
Mucocutaneous manifestations at onset, n
6
Cervical lymphadenopathy at onset, n
6
Raynaud's phenomenon or acrocyanosis at onset, n
3
Fever at onset, n
2
Dysphagia at onset, n
2
Respiratory symptoms at onset, n
2
Sicca manifestations at onset, n
2
Life-threatening macrophage activation syndrome (primary SjD), n
1
Anti-SSA antibodies positive
13/15
Abnormal large salivary gland US findings
All patients
US: heterogeneous echogenicity
14/15
US: gland enlargement
14/15
US: hyperemia
11/14
US: cystic changes
10/15
US: cervical lymphadenopathy
8/15
US: intraductal mucus plugging or calcifications
3/15
Small salivary gland biopsies negative for granulomas and malignancies, with lymphocytic sialoadenitis and focus score ≥1
13/13

Observational data: associations, not effects.

Original publication

Juvenile Sjögren disease: time for age-specific classification criteria. Pediatr Rheumatol Online J. Published 20 February 2026. doi:10.1186/s12969-026-01196-6

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