Journal article
Evaluating a Child Presenting with Symptoms Concerning for Sjögren Disease: A Clinical Tool for Practice
Rheumatology (Oxford, England), Vol.64(5), pp.3039-3047
05/01/2025
DOI: 10.1093/rheumatology/keae640
PMCID: PMC12048086
PMID: 39673525
Abstract
Childhood Sjögren disease (cSjD) is a rare disease. There are no widely accepted diagnostic or classification criteria for cSjD. To fill this gap, members from CARRA Sjogren Workgroup and the International cSjD Workgroup created a clinical diagnostic algorithm. This study evaluates the accuracy of this algorithm using an international cohort of participants with clinician diagnosed cSjD.
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 evaluated each participant's disease status in the diagnostic algorithm via an international cohort of 300 cSjD cases.
The diagnostic algorithm has three distinct clinical pathways, which represents 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 1 pathway of the algorithm (n = 100 filtered out of 300). Parotitis (70%) and sicca pathways (82%) had the highest sensitivity and extraglandular pathways (52%) had the lowest.
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.
Details
- Title: Subtitle
- Evaluating a Child Presenting with Symptoms Concerning for Sjögren Disease: A Clinical Tool for Practice
- Creators
- S M Stern - University of UtahM L Basiaga - Mayo Clinic in FloridaS Cha - University of FloridaA Thatayatikom - AdventHealth for ChildrenE B Treemacki - University of UtahR L Randell - Duke UniversityB L P Dizon - National Institute of Arthritis and Musculoskeletal and Skin DiseasesS Appenzeller - Universidade Estadual de Campinas (UNICAMP)C Edens - University of Chicago Medical CenterJ E Orrock - The University of Texas at AustinS Tiger - Childhood Arthritis and Rheumatology Research AllianceJ Diianni - Childhood Arthritis and Rheumatology Research AllianceL Amoafo - University of UtahS M Lieberman - University of Iowa
- Resource Type
- Journal article
- Publication Details
- Rheumatology (Oxford, England), Vol.64(5), pp.3039-3047
- DOI
- 10.1093/rheumatology/keae640
- PMID
- 39673525
- PMCID
- PMC12048086
- NLM abbreviation
- Rheumatology (Oxford)
- ISSN
- 1462-0332
- eISSN
- 1462-0332
- Publisher
- OXFORD UNIV PRESS
- Grant note
- Sjoegren's Foundation
This work is supported by the Sjoegren's Foundation.
- Language
- English
- Electronic publication date
- 12/03/2024
- Date published
- 05/01/2025
- Academic Unit
- Stead Family Department of Pediatrics; Rheumatology, Allergy, and Immunology
- Record Identifier
- 9984758267302771
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