Primary Sjögren's syndrome in children and adolescents: are proposed diagnostic criteria applicable?

OBJECTIVE: To compare the proposed criteria for the diagnosis of primary Sjögren's syndrome (pSS) in childhood to the validated American-European Consensus Group (AECG) classification criteria for pSS in adults.

METHODS: Charts of 7 children with pSS seen at British Columbia's Children's Hospital (BCCH) and data on 128 children identified through Medline in the English language literature between 1963 and 2003 were reviewed for pediatric and AECG criteria for pSS. The presence of > or = 4 criteria was required to satisfy the respective classification criteria. The expert clinical opinion of pediatric rheumatologists was considered the gold standard for diagnosis.

RESULTS: A total of 24/62 (39%) cases satisfied the AECG criteria; 47/62 (76%) satisfied the proposed pediatric criteria. Inclusion of recurrent parotitis increased the sensitivity of the pediatric clinical criteria. From the cases, 78/133 (59%) satisfied the pediatric oral symptom criteria; only 6/78 (8%) had xerostomia in the absence of recurrent parotitis. There was no reported case of recurrent conjunctivitis in the absence of keratoconjunctivitis sicca. We found 101/130 (78%) cases had at least one positive autoantibody test result [antinuclear antibodies (ANA), rheumatoid factor (RF), SSA, SSB]; 78/123 (63%) had autoantibodies to SSA or SSB.

CONCLUSION: The AECG adult criteria for pSS should not be applied to children as the sensitivity is unacceptably low. The inclusion of recurrent parotitis increases the sensitivity of the pediatric criteria, and recurrent parotitis should alert the clinician to the possibility of pSS. The inclusion of recurrent conjunctivitis did not improve the sensitivity over the AECG ocular criteria. The addition of ANA and RF to the AECG criteria did not change the number of patients satisfying the criteria for pediatric pSS.