Juvenile Sjögren disease: time for age-specific classification criteria.

objectiveThis study aims to report the clinical, laboratory, and imaging manifestations of juvenile Sjögren’s disease (SjD) and highlight the role of large salivary gland ultrasound and small salivary gland biopsy for the purpose of diagnosis and classification in this age group.

backgroundCurrently, there are no unified classification criteria for juvenile SjD, and pediatric-specific literature is scarce. The current literature lacks validated pediatric-specific classification and diagnostic tools. Many tests used in adult classification criteria are not routinely assessed in children because they can be difficult to perform on younger patients and lack age-specific thresholds for abnormal results. This study aims to help build the foundation for future proposals to specifically classify SjD in the pediatric age group.

methodsThis was a single-center exploratory pilot retrospective study of medical records of patients diagnosed with juvenile SjD over the past five years.

resultsFifteen patients with juvenile SjD were included. The median age of disease onset was 11.5 years (range 8.5–17.5 years), and the median age at diagnosis was 12.5 years (range 8.5–17.5 years). Ten patients had primary SjD, and five patients had SjD secondary to systemic lupus erythematosus and limited scleroderma. The clinical manifestations at onset included musculoskeletal manifestations (n = 9), recurrent parotitis (n = 7), mucocutaneous manifestations (n = 6), cervical lymphadenopathy (n = 6), Raynaud’s phenomenon or acrocyanosis (n = 3), fever (n = 2), dysphagia (n = 2), respiratory symptoms (n = 2), and sicca manifestations (n = 2). One patient with primary SjD presented with life-threatening macrophage activation syndrome. Sarcoidosis, IgG-4-related disease, and relevant infections were ruled out in all patients. Anti-SSA antibodies were positive in 13/15 patients. All patients had abnormal large salivary gland ultrasound findings, including heterogeneous echogenicity (14/15), gland enlargement (14/15), hyperemia (11/14), cystic changes (10/15), cervical lymphadenopathy (8/15), and intraductal mucus plugging or calcifications (3/15). Small salivary gland biopsies from 13/13 patients were negative for granulomas and malignancies and revealed lymphocytic sialoadenitis with a focus score of ≥1.

conclusionRecurrent parotitis and scarce sicca manifestations are two distinct clinical features of juvenile versus adult SjD. Large salivary gland ultrasound study is a promising practical tool that should be considered in suspected juvenile SjD patients. These clinical and imaging features should be considered in any proposed classification criteria for juvenile SjD.