Susić Gordana, Stojanović Roksanda, Milić Vera, Boricić Ivan, Mandić Bojan, Milenković Svetislav
Srp Arh Celok Lek. 2013 Mar-Apr;141(3-4):228-31. doi: 10.2298/sarh1304228s.
Sjögren's syndrome (SS) is an autoimmune disease of unknown etiology, clinically manifested by dry eyes (xerophthalmia) and dry mouth (xerostomia). In childhood SS is a rare disease, clinically atypically or asymptomatic and is often unrecognized. We report a girl with asymptomatic, juvenile form of primary Sjögren's syndrome (JSS).
A 13-year-old girl was initially observed for several months due to elevated sedimentation rate (ESR 75-90 mm/h) without signs of inflammation or other symptoms and disease signs. Subjective symptoms of dryness of the eyes and mouth were absent at the beginning. Ophthalmologic examination demonstrated hypolacrimia although the patients had no subjective signs of xerophthalmia. Ultrasonography (US) revealed mild enlargement and heterogeneity of large salivary glands parenchyma. Increased rheumatoid factor (RF), anti SS-A/Ro, anti SS-B/La antibodies were found in serum. Ophthalmologic examination demonstrated decreased lacrimation.JSS was confirmed on the basis of ophthalmologic examination, immunological tests, histological findings of biopsy of small and US of major salivary glands. During a 12-years follow-up period systemic or extraglandular manifestations of JSS and other autoimmune diseases were not observed.
Our experience suggests that in the differential diagnosis of unexplained elevated ESR the primary form of JSS should be also taken into consideration. Ultrasonographic changes of major salivary glands in the absence of symptoms of xerostomia point out that this noninvasive method has an important role in the diagnosis and management of patients with JSS.
干燥综合征（SS）是一种病因不明的自身免疫性疾病，临床表现为眼干（xerophthalmia）和口干（xerostomia）。儿童期SS是一种罕见疾病，临床表现不典型或无症状，常未被识别。我们报告一名患有无症状青少年型原发性干燥综合征（JSS）的女孩。
一名13岁女孩因血沉升高（ESR 75 - 90 mm/h）最初被观察了几个月，无炎症迹象或其他症状及疾病体征。起初无眼干和口干的主观症状。眼科检查显示泪液分泌减少，尽管患者无眼干的主观体征。超声检查（US）显示大唾液腺实质轻度肿大且不均匀。血清中类风湿因子（RF）、抗SS - A/Ro、抗SS - B/La抗体升高。眼科检查显示泪液分泌减少。基于眼科检查、免疫检测、小唾液腺活检的组织学结果及大唾液腺的超声检查确诊为JSS。在12年的随访期内，未观察到JSS的全身或腺外表现及其他自身免疫性疾病。
我们的经验表明，在不明原因血沉升高的鉴别诊断中，也应考虑原发性JSS。在无口干症状时大唾液腺的超声改变表明，这种非侵入性方法在JSS患者的诊断和管理中具有重要作用。
