青少年干燥综合征：以唾液腺超声为重点的临床特征。
Juvenile Sjögren's Syndrome: Clinical Characteristics With Focus on Salivary Gland Ultrasonography.
机构信息
University of Bergen and Haukeland University Hospital, Bergen, Norway.
Federal University of Espírito Santo, Vitória, Brazil.
出版信息
Arthritis Care Res (Hoboken). 2020 Jan;72(1):78-87. doi: 10.1002/acr.23839. Epub 2019 Dec 10.
OBJECTIVE
Juvenile Sjögren's syndrome (SS) is a rare, poorly defined, and possibly underdiagnosed condition affecting children and adolescents. The aim of this study was to characterize symptoms and clinical findings of juvenile SS and to explore the clinical application of major salivary gland ultrasonography (SGUS) in patients with juvenile SS.
METHODS
A cross-sectional multicenter study recruited patients with disease onset until age 18 years (n = 67). Disease characteristics were recorded, and unstimulated whole sialometry and SGUS examination of the parotid and submandibular salivary glands were performed.
RESULTS
The female:male ratio was 58:9. The mean age at first symptom was 10.2 years and 12.1 years at diagnosis. Ocular and oral symptoms were noted in 42 of 67 patients (63%) and 53 of 66 patients (80%), respectively. The American-European Consensus Group or American College of Rheumatology/European League Against Rheumatism classification criteria for primary SS were fulfilled by 42 of 67 patients (63%). Pathologic SGUS findings were observed in 41 of 67 patients (61%); 26 of 41 SGUS+ patients (63%) fulfilled primary SS criteria. Salivary gland enlargements/parotitis were noted in 37 of 58 patients and were nonsignificantly associated with SGUS+ status (P = 0.066). The mean levels of saliva were 5.6 ml/15 minutes in SGUS- patients compared to 3.3 ml/15 minutes in the SGUS+ patients (P = 0.049). A total of 36 of 41 SGUS+ patients (88%) were anti-Ro/La+ compared to 14 of 26 SGUS- patients (54%) (P = 0.001). In addition, 24 of 39 SGUS+ patients (62%) were positive for rheumatoid factor (RF), whereas only 5 of 25 SGUS- patients (20%) were RF+ (P = 0.001).
CONCLUSION
Juvenile SS is characterized by a large spectrum of clinical symptoms and findings. Several glandular and extraglandular parameters such as hyposalivation, swollen salivary glands, and autoantibodies are associated with pathologic SGUS findings.
目的
青少年干燥综合征（SS）是一种罕见、定义不明确且可能诊断不足的疾病，影响儿童和青少年。本研究旨在描述青少年 SS 的症状和临床特征，并探讨大唾液腺超声（SGUS）在青少年 SS 患者中的临床应用。
方法
一项横断面多中心研究招募了发病至 18 岁的患者（n=67）。记录疾病特征，进行非刺激全唾液量测定和腮腺及颌下腺的 SGUS 检查。
结果
女性与男性比例为 58:9。首发症状的平均年龄为 10.2 岁，诊断时为 12.1 岁。67 例患者中有 42 例（63%）出现眼部和口腔症状，66 例患者中有 53 例（80%）出现口腔症状。42 例患者（63%）符合原发性 SS 的美国-欧洲共识组或美国风湿病学会/欧洲抗风湿病联盟分类标准。67 例患者中有 41 例（61%）出现病理性 SGUS 结果；41 例 SGUS+患者中有 26 例（63%）符合原发性 SS 标准。37 例患者（58%）出现唾液腺肿大/腮腺炎，与 SGUS+状态无显著相关性（P=0.066）。SGUS-患者的平均唾液量为 5.6 ml/15 分钟，SGUS+患者为 3.3 ml/15 分钟（P=0.049）。41 例 SGUS+患者中有 36 例（88%）为抗 Ro/La+，而 26 例 SGUS-患者中有 14 例（54%）为抗 Ro/La+（P=0.001）。此外，39 例 SGUS+患者中有 24 例（62%）类风湿因子（RF）阳性，而 25 例 SGUS-患者中有 5 例（20%）RF 阳性（P=0.001）。
结论
青少年 SS 的临床症状和表现范围广泛。一些腺体和腺体外参数，如唾液分泌减少、唾液腺肿大和自身抗体，与病理性 SGUS 结果相关。
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