Juvenile Sjogren's Syndrome: Clinical Characteristics With Focus on Salivary Gland Ultrasonography
Juvenile Sjogren's Syndrome: Clinical Characteristics With Focus on Salivary Gland Ultrasonography
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DOI：
10.1002/acr.23839
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发表时间：
2020-01-01
影响因子：
4.7
通讯作者：
Jonsson, Malin, V
中科院分区：
文献类型：
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作者：
Hammenfors, Daniel S.;Valim, Valeria;Jonsson, Malin, V
目的青少年干燥综合征（SS）是一种罕见、定义不清且可能诊断不足的影响儿童和青少年的疾病。本研究的目的是描述青少年SS的症状和临床表现，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67例发病年龄在18岁以下的患者。记录疾病特征，并对腮腺和下颌下唾液腺进行无刺激全唾液腺测量和SGUS检查。结果男女比例为58：9。首次出现症状的平均年龄为10.2岁，诊断时为12.1岁。67例患者中有42例（63%）和66例患者中有53例（80%）出现眼部和口腔症状。67例患者中有42例（63%）符合美国-欧洲共识组或美国风湿病学会/欧洲抗风湿联盟的原发性SS分类标准。67例患者中有41例（61%）观察到病理性SGUS结果; 41例SGUS+患者中有26例（63%）符合主要SS标准。58例患者中有37例出现唾液腺增大/腮腺炎，与SGUS+状态无显著相关性（P = 0.066）。SGUS-患者的平均唾液水平为5.6 ml/15分钟，而SGUS+患者为3.3 ml/15分钟（P = 0.049）。41例SGUS+患者中共有36例（88%）为抗Ro/La+，而26例SGUS-患者中有14例（54%）（P = 0.001）。此外，39例SGUS+患者中有24例（62%）为类风湿因子（RF）阳性，而25例SGUS-患者中只有5例（20%）为RF+（P = 0.001）。结论青少年SS临床表现多样，临床症状多样。几个腺和腺外参数，如唾液分泌不足，肿胀的唾液腺，和自身抗体与病理SGUS的结果。
Objective Juvenile Sjogren'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.