Related Experiment Video
Updated: Jun 5, 2025
Author Spotlight: A Focus on Standardized Salivary Gland Ultrasound Protocol in Connective Tissue Disease Research
Published on: October 13, 2023
Diagnosing a child presenting with symptoms suggesting Sjögren's disease: a tool for clinical practice
Sara M Stern1, Matthew L Basiaga2, Seunghee Cha3
1Department of Pediatrics, University of Utah, Salt Lake City, UT, USA.
Insights
A new clinical algorithm aids in diagnosing childhood Sjögren disease (cSjD). This tool shows good accuracy in an international cohort, offering a valuable diagnostic aid for cSjD-like symptoms in children.
Area of Science:
- Pediatric Rheumatology
- Autoimmune Diseases
- Clinical Diagnostics
Background:
- Childhood Sjögren disease (cSjD) is a rare autoimmune condition lacking established diagnostic or classification criteria.
- The absence of standardized criteria poses challenges in accurately identifying and managing cSjD in pediatric populations.
Purpose of the Study:
- To evaluate the accuracy of a newly developed clinical diagnostic algorithm for childhood Sjögren disease.
- To provide a validated tool for clinicians diagnosing cSjD in children.
Main Methods:
- Experts developed a cSjD diagnostic algorithm based on adult criteria, literature review, and expert consensus.
- The algorithm's performance was assessed using an international cohort of 300 clinician-diagnosed cSjD cases, with R statistical software employed for analysis.
Main Results:
- The algorithm features three pathways: parotitis, extraglandular manifestations, and sicca symptoms.
- Overall sensitivity was 75% in the analyzed subset (n=100).
- The sicca pathway demonstrated the highest sensitivity (82%), while the extraglandular pathway had the lowest (52%).
Conclusions:
- The developed algorithm serves as a practical clinical tool for evaluating children presenting with Sjögren disease-like symptoms.
- The algorithm demonstrated good performance in an international cohort, supporting its clinical adoption.
- Potential limitations include the low utilization of diagnostic testing within the studied population.
Objective:
Childhood SjD (cSjD) is a rare disease. There are no widely accepted diagnostic or classification criteria for cSjD. To fill this gap, members from the CARRA Sjögren Disease Workgroup and the International Childhood Sjögren Disease Workgroup created a clinical diagnostic algorithm. This study evaluated the accuracy of this algorithm using an international cohort of participants with clinician-diagnosed cSjD.
Methods:
First, experts developed a cSjD diagnostic algorithm through a series of virtual workgroup meetings. Using the adult classification criteria as a framework, experts modified the algorithm through opinion and literature review. The group discussed and finalized each algorithm step by achieving majority rule. Then, R statistical software was used to evaluate each participant's disease status in the diagnostic algorithm via an international cohort of 300 cSjD cases.
Results:
The diagnostic algorithm has three distinct clinical pathways representing the key clinical presentation in cSjD: parotitis, extraglandular manifestations, and sicca symptoms. The algorithm showed an overall sensitivity of 75% in the population that had enough data to complete at least one pathway of the algorithm (n = 100 filtered out of 300). The parotitis (70%) and sicca pathways (82%) had the highest sensitivity, and the extraglandular pathway (52%) had the lowest.
Conclusion:
As cSjD lacks a diagnostic strategy, this algorithm provides a clinical tool for evaluating children with cSjD-like symptoms. It performed well in an international cohort of cSjD, supporting the integration of this algorithm into clinical practice; however, its utility may be limited by low utilization of diagnostic testing in this population.
