Background/Purpose:  Juvenile Sjögren’sSyndrome (jSS) is a rare systemic autoimmune disease affecting predominantly salivary glands (SG) in children who do not meet criteria for SLE or MCTD. Currently, no specific criteria for diagnosis or study of the disease are available. Salivary gland ultrasonography (SGUS) in adults with primary Sjogren’s Syndrome (pSS) has proved to be a promising tool in detecting typical structural abnormalities for diagnosis and prognostic stratification of the disease; however, studies of its utility in jSS have been limited. SGUS is a non-invasive, inexpensive and non-irradiating procedure; therefore, further studies in children with jSS for potential usefulness of SGUS are needed.

Methods: Patients with jSS diagnosed between 2011-2016 at Shands Children’s Hospital, University of Florida, were studied. Clinical examination, diagnostic tests and SGUS were obtained during clinic visits. SGUS studies of major salivary glands using a Sonosite Edge with a linear high-frequency transducer 6-15 MHZ were performed by a RhMSUS certified pediatric rheumatologist. Based upon previous SGUS studies in pSS, the sonographic findings including parenchymal echogenicity, inhomogeneity, hypoechogenic areas, hyperechogenic reflections and borders were evaluated.

Results: Three patients with jSS were studied. The age at onset of symptoms was 7, 8 and 12 years, and the age at diagnosis of jSS was 11, 11 and 15 years respectively. All patients had active parotitis and constitutional symptoms with abnormal laboratory findings including highly elevated anti-SSA, anti-SSB Ab, rheumatoid factor and immunoglobulins (IgG>2,500) with negative anti-Smith and anti-DNA Ab. Abnormal findings on SGUS were found in all patients, however, two patients treated only with hydroxychloroquine showed more severe parenchymal changes (grade 3). After adding systemic corticosteroids, the severity of the parenchymal findings improved to grade 1-2 associated with clinical improvement.

Conclusion: The improvement of SGUS after steroid treatment in children with jSS has not been previously reported. SGUS findings may be a useful tool for monitoring disease activity and response to treatment. Further long-term investigations of SGUS including correlation of pathologic findings and development of a consensual scoring system in large jSS cohorts are warranted.