Background Juvenile Sjögren's syndrome (jSS) is a rare, poorly defined and possibly underdiagnosed condition. There is little information on the use of major salivary gland ultrasonography (SGUS) in this patient-group.
Objectives To characterize symptoms and clinical findings of jSS and to investigate SGUS as a diagnostic tool.
Methods Sixty-four patients were recruited from Brazil (n=40), Norway (n=11), the Netherlands (n=8) and Spain (n=5). All patients had disease onset at the age of 18 or younger. Clinical examination and sialometry was performed in 60/64 patients. Additional clinical information was obtained from the medical records and through patient interview. SGUS of the parotid and submandibular glands was performed in all patients using linear high-frequency transducers (6–15 MHz), by an expert in SGUS. Glandular homogeneity and presence of hypoechogenic areas were evaluated and glands characterized as normal or SS-like.
Results The female:male ratio was 6:1. Mean age at diagnosis was 12.1 years (range 4–18), with first symptoms occurring at 10.3 years (range 1–17). Time from onset of symptoms until diagnosis was 1.6 years (range -2–8 years). Subjective oral and ocular symptoms were reported in 70% and 64% patients, respectively. Reduced secretion of tears was detected in 41% patients, and hyposalivation in 31% patients. Minor salivary gland lip biopsy had been performed and focus score determined in 34 patients; 28 biopsies (82%) had focus score ≥1. Serologically, 92% were positive for ANA, 73% were anti-Ro/SSA+, 38% were anti-La/SSB+, and 41% were RF+. Salivary gland enlargement had been experienced by 53% of the patients; one patient had also experienced lacrimal gland enlargement. Systemic manifestations at some time-point, was registered in 66% of the patients. Systemic treatment at inclusion was registered in 67% of the patients; previous systemic treatment was registered in 83%. Diagnostic criteria for primary Sjögren's syndrome (pSS) was fulfilled by 34/64 patients (53%) and 39/64 patients (61%), AECG criteria and ACR/EULAR criteria, respectively. SGUS revealed SS-like changes in 37/64 patients (59%); interestingly, SS-like findings were observed in 22/23 patients in the European cohort, compared to 15/40 patients in the Brazilian cohort.
Conclusions Common symptoms and findings in jSS include dry mouth, systemic manifestations and salivary gland enlargement, followed by reduced tear secretion and hyposalivation.
Disclosure of Interest None declared