Sjögren’s Syndrome in Kids: Diagnostic Challenges & Treatment Options

Conclusion

In summary, Sjögren’s syndrome may develop during childhood, but presentation often differs from that in adults and diagnosis may be less straightforward due to the lack of well-established diagnostic biomarkers or diagnostic criteria. Inter­national collaborative studies are in progress to better define and understand the natural history of Sjögren’s syndrome in children, although this will take considerable time.

Of note, diagnosis in childhood may present a window of opportunity in which to intervene to prevent progression to the typical profound dryness experienced by many adults and will, hopefully, greatly alter the disease course, including a decrease in the risk of lymphoma.

Sara M. Stern, MD, is an assistant pro­fessor of pediatrics at the University of Utah School of Medicine, Salt Lake City.

Matthew L. Basiaga, DO, MSCE, is a senior associate consultant at the Mayo Clinic, Rochester, Minn.

Scott M. Lieberman, MD, PhD, is an associate professor of pediatrics at Carver College of Medicine, University of Iowa, Iowa City.

References

1. Shiboski CH, Shiboski SC, Seror R, et al. 2016 American College of Rheumatology/European League Against Rheumatism classification criteria for primary Sjögren’s syndrome: A consensus and data-driven methodology involving three international patient cohorts. Ann Rheum Dis. 2017 Jan;76(1):9–16.
2. Basiaga M, Stern SM, Mehta J, et al. A better understanding of childhood Sjögren syndrome: Evaluation of the 2016 ACR/EULAR classification criteria for the use of diagnosing Sjögren syndrome in children [abstract]. Arthritis Rheumatol. 2018;70(suppl 10).
3. Yokogawa N, Lieberman SM, Sherry DD, et al. Features of childhood Sjögren’s syndrome in comparison to adult Sjögren’s syndrome: Considerations in establishing child-specific diagnostic criteria. Clin Exp Rheumatol. 2016 Mar–Apr;34(2):343–351.
4. Hammenfors DS, Valim V, Bica BERG, et al. Juvenile Sjögren’s syndrome: Clinical characteristics with focus on salivary gland ultrasonography. Arthritis Care Res (Hoboken). 2019 Jan 30. doi: 10.1002/acr.23839. [Epub ahead of print]
5. Retamozo S, Acar-Denizli N, Rasmussen A, et al. Systemic manifestations of primary Sjögren’s syndrome out of the ESSDAI classification: Prevalence and clinical relevance in a large international, multi-ethnic cohort of patients. Clin Exp Rheumatol. 2019 May–Jun;
   37 Suppl 118(3):97–106.
6. Ramos-Casals M, Solans R, Rosas J, et al. Primary Sjögren syndrome in Spain: Clinical and immunologic expression in 1010 patients. Medicine (Baltimore). 2008 Jul;87(4):210–219.
7. Demarchi J, Papsidero S, Medina MA, et al. Primary Sjögren’s syndrome: Extraglandular manifestations and hydroxychloroquine therapy. Clin Rheumatol. 2017 Nov;36(11):2455–2460.
8. Tomiita M, Saito K, Kohno Y. The clinical features of Sjögren’s syndrome in Japanese children. Acta Paediatr Jpn. 1997 Apr;
   39(2):268–272.
9. Kornitzer JM, Kimura Y, Janow GL. Primary Sjögren syndrome in a child with neuromyelitis optica spectrum disorder. J Rheumatol. 2016 Jun;43(6):1260–1261.
10. Bogdanović R, Basta-Jovanović G, Putnik J, et al. Renal involvement in primary Sjogren syndrome of childhood: Case report and literature review. Mod Rheumatol. 2013 Jan;23(1):182–189.
11. Zhang X, Zeng X. Severe pulmonary hypertension in pediatric Sjögren syndrome: A case report. J Clin Rheumatol. 2007 Oct;
    13(5):276–277.
12. Cornec D, Devauchelle-Pensec V, Mariette X, et al. Severe health-related quality of life impairment in active primary Sjogren’s syndrome and patient-reported outcomes: Data from a large therapeutic trial. Arthritis Care Res (Hoboken). 2017 Apr;69(4):528–535.
13. Tesher MS, Esteban Y, Henderson TO, et al. Mucosal-associated lymphoid tissue (MALT) lymphoma in association with pediatric primary Sjogren Syndrome: 2 cases and review. J Pediatr Hematol Oncol. 2019 Jul;=41(5):413–416.
14. Collado P, Kelada A, Cámara M, et al. Extranodal marginal zone B cell lymphoma: An unexpected complication in children with Sjögren’s syndrome. Reumatol Clin. 2018 Jul–Aug;14(4):227–229.