Background/Purpose: Clinical presentation of Sjögren syndrome in children differs from adults: dryness symptoms are more common in adults, while parotitis is more common in children. Criteria developed for adult classification have demonstrated low sensitivity when applied to pediatric populations, and no child-specific criteria have been established. The latest adult classification criteria have not yet been evaluated for use in children. Our objective was to evaluate the applicability of these new criteria for use in children.

Methods: Retrospective chart reviews were conducted to collect individual patient level data for children diagnosed with Sjögren syndrome (based on clinical diagnosis at age <18 years). Data including clinical features, laboratory values, imaging studies, and test items in the 2016 ACR/EULAR criteria were collected, and de-identified data were entered into a REDCap database. This study was approved by the Institutional Review Boards or equivalent regulatory bodies at individual affiliate institutions.

Results: To date, 144 children with Sjögren syndrome were included from 11 institutions across 4 countries (data collection is ongoing). This constitutes the largest childhood Sjögren syndrome patient series to date. The majority of children (88%) were female with a mean age of 11.7 years at diagnosis (range 1–17.8 years). Twenty-three children (16%) also had another autoimmune disease (18 with SLE, 4 with uveitis, 1 with subacute cutaneous lupus). Frequency of clinical features were as follows: 54% with parotitis, 54% with dry eyes, 54% with dry mouth, 52% with arthralgias without arthritis, 29% with arthritis, 21% with lymphadenopathy, 18% with cytopenias, 14% with fevers, 13% with cutaneous vasculitis, 10% with weight loss, and <10% each with recurrent vaginitis, myositis, pulmonary, renal, or neurologic manifestations. Only 6 children had testing for all 5 items included in the 2016 ACR/EULAR criteria. Most children (93%) had testing for anti-SSA antibodies, but fewer underwent minor salivary gland (MSG) biopsy (46%), Schirmer testing (40%), ocular surface staining (OSS, 15%), or measurement of unstimulated whole saliva flow (UWSF, 13%). While most children studied (95.8%) were missing at least one data point, 38 of 144 children (26%) met the 2016 ACR/EULAR classification criteria for Sjögren syndrome. Of these 38 children: 35 (92%) had positive anti-SSA antibodies; 29 (76%) had positive Schirmer test; 22 (58%) had positive MSG biopsy; 4 (11%) had positive UWSF; and 1 (3%) had positive OSS. Of the 106 children not meeting criteria: 70 (66%) had positive anti-SSA antibodies; 10 (9%) had positive MSG biopsy; 8 (8%) had positive Schirmer test; and 4 (4%) had positive UWSF.

Conclusion:

Criteria items from the 2016 ACR/EULAR criteria are not routinely assessed in children diagnosed with Sjögren syndrome making formal retrospective assessment of criteria difficult. Prospective study of these criteria along with defining child-specific normal values and adding child-specific criteria items (such as recurrent parotitis) are warranted. Establishing criteria for childhood Sjögren syndrome is a key step toward better understanding and treating this condition.

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ACR Meeting Abstracts - https://acrabstracts.org/abstract/a-better-understanding-of-childhood-sjogren-syndrome-evaluation-of-the-2016-acr-eular-classification-criteria-for-use-in-diagnosing-sjogren-syndrome-in-children/