Session Type: ACR Poster Session B
Session Time: 9:00AM-11:00AM
Background/Purpose: Juvenile Sjögren’s syndrome (jSS) is a rare, poorly defined and possibly underdiagnosed condition. Mean age of diagnosis is approximately 10 years, with major salivary gland swelling as a common initial symptom. Extraglandular manifestations occur in approximately 50% of patients with jSS. Salivary gland ultrasonography (SGUS) is a non-invasive, non-irradiating imaging method. With regard to the late onset of sicca symptoms, the current lack of diagnostic criteria and the need for an alternative or supplement to the lip biopsy, a non-invasive diagnostic tool is especially important in the younger population. The aim of this study was to characterize symptoms and clinical findings of jSS and to investigate SGUS as a diagnostic method.
Methods: Patients were recruited from the Departments of Rheumatology at Haukeland University Hospital, Bergen, Norway, and Oslo University Hospital, Oslo, Norway. Patients were previously diagnosed with jSS by a specialist in rheumatology and had received the diagnosis before 18 years of age. Clinical examination, sialometry, Schirmers Itest and SGUS were performed in all patients, and information regarding autoantibodies and biopsy results obtained from the patients’ medical journal. The SGUS examination of the parotid and submandibular glands was performed using a GE LogiqE9 with a linear high-frequency transducer (6-15 MHz). Representative images were stored digitally and blindly evaluated by two investigators (DH and MVJ). Glandular homogeneity and presence of hypoechogenic areas were evaluated and glands characterized as normal or SS-like.
Results: Mean age at jSS diagnosis was 13.6 years (range 7-16), with first symptoms occurring at 11 years (range 6-17). Sicca symptoms were reported in 4/10 patients. Reduced secretion of tears (Schirmer I-test ≤ 5 mm/5 minutes) was detected in 3/10 patients, and hyposalivation (unstimulated whole saliva ≤ 1.5 ml/15 minutes) in 4/10 patients. Minor salivary gland lip biopsy had been performed and focus score determined in 8/10 patients; 7/8 biopsies had a focus score ≥ 1. All patients were positive for ANA and anti-Ro/SSA; 6/10 were also anti-La/SSB positive, and 6/10 were rheumatoid factor positive. Salivary gland enlargement had been experienced by 6/10 patients; 1/6 had also experienced lacrimal gland enlargement. Lymphadenopathy was noted in 3/10 patients, and articular involvement in 3/10 patients. Current treatment with hydroxychloroquine was noted in 7/10 patients, and previous treatment in two patients. Interestingly, SGUS revealed that all investigated patients (n=10) had pathological findings consistent with pSS in at least 1 out of 4 major salivary glands, whereas the American-European Consensus Group (AECG) criteria was fulfilled by only 6/10 patients.
Conclusion: The majority of patients in this jSS cohort present with autoantibodies and pathological SGUS findings. Major salivary gland swelling was commonly reported, whereas sicca symptoms were rare. Although further studies are warranted in larger cohorts, findings indicate SGUS as a suitable imaging method in the diagnosis of jSS, possibly enabling an early diagnosis of this rare clinical condition.
To cite this abstract in AMA style:Hammenfors DS, Valim V, Lilleby V, Bica B, Pasoto SG, Silva C, Nieto JC, Lieberman S, Thatayatikom A, Jonsson R, Brun JG, Jonsson MV. Ultrasonography of Major Salivary Glands in Juvenile SjöGren’s Syndrome − Preliminary Findings in a Multi-Center Study [abstract]. Arthritis Rheumatol. 2016; 68 (suppl 10). https://acrabstracts.org/abstract/ultrasonography-of-major-salivary-glands-in-juvenile-sjogrens-syndrome-%e2%88%92-preliminary-findings-in-a-multi-center-study/. Accessed February 28, 2020.
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ACR Meeting Abstracts - https://acrabstracts.org/abstract/ultrasonography-of-major-salivary-glands-in-juvenile-sjogrens-syndrome-%e2%88%92-preliminary-findings-in-a-multi-center-study/