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Diagnosis of Sjögren's syndrome in children
A J Deprettere1, K J Van Acker, L S De Clerck
1Department of Pediatrics, University Hospital of Antwerp, Belgium.
Insights
Diagnosing Sjögren
Area of Science:
- Pediatric Rheumatology
- Ophthalmology
- Immunology
Background:
- Sjögren's syndrome (SS) is a chronic autoimmune disease primarily affecting exocrine glands.
- Diagnosis in children is challenging due to overlapping symptoms with other pediatric conditions.
Observation:
- Four children with suspected SS were treated and their cases reviewed alongside 23 previously reported pediatric cases.
- Key clinical and laboratory findings suggestive of SS in children were identified.
Findings:
- Proposed diagnostic criteria for pediatric SS, adapted from adult criteria, include: keratoconjunctivitis (Schirmer test, rose bengal test), xerostomia (reduced salivary flow), lymphocytic infiltration in salivary glands, and specific autoantibodies (RF, ANA, ENA).
- These criteria aim to standardize SS diagnosis in pediatric populations.
Implications:
- Applying adult diagnostic criteria to children may improve early identification and management of Sjögren's syndrome.
- Long-term follow-up is crucial for understanding the natural history of pediatric SS and for identifying children who may develop the condition over time.
Abstract:
We treated four children with clinical symptoms and laboratory findings suggestive of Sjögren's syndrome (SS). We also review the findings in 23 children with the diagnosis of SS whose cases were reported in the literature. We propose that the following criteria for the diagnosis of SS, which are mostly used in adults, should also be applied to children: (1) keratoconjunctivitis evidenced by a Schirmer test and a quantitative rose bengal test; (2) xerostomia shown by a decreased basal and stimulated salivary flow; (3) lymphocytic infiltration in a minor salivary gland biopsy specimen with at least two foci per 4 mm2; (4) laboratory evidence of a systemic autoimmune disorder on the basis of a rheumatoid factor of 1/160 or greater, antinuclear antibody of 1/160 or greater, or extractable nuclear antigen antibodies. Only close observation and long-term follow-up of these patients will allow a better insight in the natural history of SS in children. Those children who do not fulfill these diagnostic criteria also need close and prolonged follow-up study: one of the possibilities is that their conditions will ultimately evolve toward definite SS.