Clinical aspects
Differences in presentation between paediatric- and adult-onset primary Sjögren’s syndrome patients
G.E. Legger1, M.B. Erdtsieck2, L. De Wolff3, A.J. Stel4, L.I. Los5, G.M. Verstappen6, F.K. Spijkervet7, A. Vissink8, B. Van Der Vegt9, F.G. Kroese10, W. Armbrust11, S. Arends12, H. Bootsma13
- Department of Paediatric Rheumatology, University of Groningen, University Medical Center Groningen, The Netherlands. g.e.legger@umcg.nl
- Department of Paediatric Rheumatology, University of Groningen, University Medical Center Groningen, The Netherlands.
- Department of Rheumatology and Clinical Immunology, University of Groningen, University Medical Center Groningen, The Netherlands.
- Department of Rheumatology and Clinical Immunology, University of Groningen, University Medical Center Groningen, The Netherlands.
- Department of Ophthalmology, University of Groningen, University Medical Center Groningen, The Netherlands.
- Department of Rheumatology and Clinical Immunology, University of Groningen, University Medical Center Groningen, The Netherlands.
- Department of Oral and Maxillofacial Surgery, University of Groningen, University Medical Center Groningen, The Netherlands.
- Department of Oral and Maxillofacial Surgery, University of Groningen, University Medical Center Groningen, The Netherlands.
- Department of Pathology & Medical Biology, University of Groningen, University Medical Center Groningen, The Netherlands.
- Department of Rheumatology and Clinical Immunology, University of Groningen, University Medical Center Groningen, The Netherlands.
- Department of Paediatric Rheumatology, University of Groningen, University Medical Center Groningen, The Netherlands.
- Department of Rheumatology and Clinical Immunology, University of Groningen, University Medical Center Groningen, The Netherlands.
- Department of Rheumatology and Clinical Immunology, University of Groningen, University Medical Center Groningen, The Netherlands.
CER15011
2021 Vol.39, N°6 ,Suppl.133
PI 0085, PF 0092
Clinical aspects
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PMID: 34796855 [PubMed]
Received: 18/07/2021
Accepted : 13/09/2021
In Press: 10/11/2021
Published: 15/12/2021
Abstract
OBJECTIVES:
Primary Sjögren’s syndrome (pSS) is a rare disease in paediatric patients. Presenting symptoms differ from those in adult patients. The aim of this study was to evaluate presenting symptoms, classification criteria and clinical assessments, including salivary gland ultrasonography (SGUS), at disease onset in paediatric and adult patients with pSS.
METHODS:
Data of 23 paediatric- and 33 adult-onset patients with pSS were obtained from our standardised multidisciplinary REpSULT and RESULT cohorts, respectively. Clinical, patient-reported, serological, functional, biopsy and SGUS parameters were compared.
RESULTS:
In paediatric-onset pSS (pedSS) patients, recurrent parotid gland swelling (91% vs. 49%, p<0.001) and fever (30% vs. 3%, p=0.006) were more often present than in adult-onset patients. In contrast, sicca symptoms of mouth (52% vs. 79%, p=0.046) and eyes (26% vs. 73%, p<0.001) were less common in pedSS patients. In paediatric patients, the entry criteria of the ACR/EULAR classification were most often met due to activity in the glandular domain of the ESSDAI. When applying the ACR/EULAR classification criteria, only 78% of pedSS fulfilled these criteria compared to 100% of adult patients. Abnormal glandular function tests had a greater contribution to fulfilling the criteria in adults, while the biopsy had a greater contribution in paediatric patients. Anti-SSA/Ro serology had similar contribution for both cohorts. SGUS Hocevar score was significantly higher in paediatric compared to adult patients (median 25 vs. 18, p=0.004). Conclusion. PedSS has a different presentation than adult-onset pSS. Recurrent parotid gland swelling in paediatric patients should alert clinicians to the potential presence of pSS.
