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. 2021 Jul 1;60(7):3144-3155.
doi: 10.1093/rheumatology/keaa757.

Childhood Sjögren syndrome: features of an international cohort and application of the 2016 ACR/EULAR classification criteria

Matthew L Basiaga  1 , Sara M Stern  2 , Jay J Mehta  3 , Cuoghi Edens  4 , Rachel L Randell  5 , Anna Pomorska  6 , Ninela Irga-Jaworska  6 , Maria F Ibarra  7 , Claudia Bracaglia  8 , Rebecca Nicolai  8 , Gordana Susic  9 , Alexis Boneparth  10 , Hemalatha Srinivasalu  11 , Brian Dizon  11   12 , Ankur A Kamdar  13 , Baruch Goldberg  14 , Sheila Knupp-Oliveira  15 , Jordi Antón  16   17 , Juan M Mosquera  16 , Simone Appenzeller  18 , Kathleen M O'Neil  19 , Stella A Protopapas  19 , Claudia Saad-Magalhães  20 , Jonathan D Akikusa  21 , Akaluck Thatayatikom  22 , Seunghee Cha  23 , Juan Carlos Nieto-González  24 , Mindy S Lo  25 , Erin Brennan Treemarcki  2 , Naoto Yokogawa  26 , Scott M Lieberman  27 ; Childhood Arthritis and Rheumatology Research Alliance and the International Childhood Sjögren Syndrome Workgroup
Affiliations

Childhood Sjögren syndrome: features of an international cohort and application of the 2016 ACR/EULAR classification criteria

Matthew L Basiaga et al. Rheumatology (Oxford). .

Abstract

Objective: Sjögren syndrome in children is a poorly understood autoimmune disease. We aimed to describe the clinical and diagnostic features of children diagnosed with Sjögren syndrome and explore how the 2016 ACR/EULAR classification criteria apply to this population.

Methods: An international workgroup retrospectively collected cases of Sjögren syndrome diagnosed under 18 years of age from 23 centres across eight nations. We analysed patterns of symptoms, diagnostic workup, and applied the 2016 ACR/EULAR classification criteria.

Results: We identified 300 children with Sjögren syndrome. The majority of patients n = 232 (77%) did not meet 2016 ACR/EULAR classification criteria, but n = 110 (37%) did not have sufficient testing done to even possibly achieve the score necessary to meet criteria. Even among those children with all criteria items tested, only 36% met criteria. The most common non-sicca symptoms were arthralgia [n = 161 (54%)] and parotitis [n = 140 (47%)] with parotitis inversely correlating with age.

Conclusion: Sjögren syndrome in children can present at any age. Recurrent or persistent parotitis and arthralgias are common symptoms that should prompt clinicians to consider the possibility of Sjögren syndrome. The majority of children diagnosed with Sjögren syndromes did not meet 2016 ACR/EULAR classification criteria. Comprehensive diagnostic testing from the 2016 ACR/EULAR criteria are not universally performed. This may lead to under-recognition and emphasizes a need for further research including creation of paediatric-specific classification criteria.

Keywords: Sjögren Syndrome; childhood Sjögren syndrome; pediatric rheumatology; recurrent parotitis.

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Figures

<sc>Fig</sc>. 1
Fig. 1
Distribution and proportion of children meeting ACR/EULAR criteria for Sjögren syndrome (A) Distribution by number of tests performed. (B) Proportion based on diagnostic testing. The 2016 ACR/EULAR Criteria includes a labial salivary gland biopsy with focal lymphocytic sialadenitis and focus score ≥1 (3 points), Ant-SSA/Ro (3 points), ocular staining ≥5 in at least one eye (1 point), Schirmer ≤ 5 mm/5 min on at least one eye (1 point), and unstimulated whole saliva flow rate ≤0.1 ml/min (1 point). A score of 4 or higher is required to meet Sjögren syndrome classification. The figure highlights the highest score they could have achieved based on which criteria where tested.
<sc>Fig</sc>. 2
Fig. 2
Prevalence of parotitis by age Percentage of patients reporting recurrent or persistent perotitis by age of Sjögren diagnosis.
See this image and copyright information in PMC

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