Kaleda M I, Nikishina I P, Latypova A N
V.A. Nasonova Scientific and Research Institute of Rheumatology, Pediatric Department.
Ter Arkh. 2019 May 15;91(5):54-60. doi: 10.26442/00403660.2019.05.000189.
Aim to analyze demographic data, clinical features and results of laboratory and instrumental examinations in children with primary and secondary Sjögren syndrome (SS).
The study included all consequently patients, who hospitalized to the pediatric department of V.A. Nasonova Scientific and Research Institute of Rheumatology from January 2013 to December 2018, which verified the diagnosis of the SS.
The diagnosis of SS was established in 30 patients, among whom there were only 5 (16.7%) boys, the ratio of boys and girls was 1:5. According to the results of the examination, the following diagnoses were verified: 4 - primary SS, 9 - systemic lupus erythematosus with SS, 10 - juvenile rheumatoid arthritis with SS, 3 - mixed connective tissue disease, 3 - overlap syndrome, 1 - systemic sclerosis with SS. The median age of rheumatic disease onset was 10.4 (7.0; 13.75) years. The median of disease duration at the time of SS verification - 3.0 (0.85; 4.4) years. Recurrent parotitis were observed in 8 patients. 24 pts had isolated involvement of salivary glands, 6 - combined with lacrimal glands. Sicca syndrome was occurred in 8 patients. All patients had systemic manifestations: constitutional abnormalities - 50%, polyarthritis - 83.3%, lymphadenopathy - 73.3%, cutaneous involvement - 60%, pulmonary involvement - 23.3%. Of the hematological disorders, leuko/lymphopenia was more often recorded - in 30%, polyclonal hypergammaglobulinemia - in 26.7% of patients. ANA were detected in all cases, anti-Ro antibodies - 60%, a positive rheumatoid factor - 56.7% of patients. The most common combination of immunological disorders was the presence of ANA, RF and anti-Ro antibodies (40% of patients). The treatment for each patient was justified by the main manifestations and activity of rheumatic disease: 66.7% received nonsteroidal anti - inflammatory drugs, 80% - glucocorticoids, 46.7% - methotrexate, 20% - azathioprine, 43.3% - hydroxychloroquine, 10% - mycophenolatis mofetilum, 3.4% - cyclophosphamide. 66.7% of patients received the treatment of Biologics.
Early diagnosis of SS in children with rheumatic diseases significantly affects to the choice of treatment and prognosis. In children the SS has no clinical manifestations for a long time. Such symptoms as a hypergammaglobulinemia, positive RF without persistant arthritis, nonspecific skin lesions, recurrent parotid swelling may help to diagnosis of SS.
旨在分析原发性和继发性干燥综合征（SS）患儿的人口统计学数据、临床特征以及实验室和器械检查结果。
该研究纳入了2013年1月至2018年12月期间在弗拉基米尔·阿列克谢耶维奇·纳索诺夫风湿病科研所儿科住院并确诊为SS的所有患儿。
30例患儿确诊为SS，其中男孩仅5例（16.7%），男女比例为1:5。根据检查结果，确诊如下疾病：4例原发性SS，9例系统性红斑狼疮合并SS，10例幼年类风湿关节炎合并SS，3例混合性结缔组织病，3例重叠综合征，1例系统性硬化合并SS。风湿性疾病发病的中位年龄为10.4（7.0；13.75）岁。确诊SS时疾病持续时间的中位数为3.0（0.85；4.4）年。8例患儿出现复发性腮腺炎。24例患儿仅有唾液腺受累，6例合并泪腺受累。8例患儿发生干燥综合征。所有患儿均有全身表现：体质异常占50%，多关节炎占83.3%，淋巴结病占73.3%，皮肤受累占60%，肺部受累占23.3%。血液系统疾病中，白细胞/淋巴细胞减少更常见，占30%，多克隆高球蛋白血症占26.7%。所有病例均检测到抗核抗体（ANA），60%检测到抗Ro抗体，56.7%患儿类风湿因子呈阳性。最常见的免疫紊乱组合是ANA、类风湿因子和抗Ro抗体同时存在（占40%的患儿）。根据风湿性疾病的主要表现和活动情况，为每位患儿制定了合理的治疗方案：66.7%接受非甾体抗炎药治疗，80%接受糖皮质激素治疗，46.7%接受甲氨蝶呤治疗，20%接受硫唑嘌呤治疗，43.3%接受羟氯喹治疗，10%接受霉酚酸酯治疗，3.4%接受环磷酰胺治疗。66.7%的患儿接受了生物制剂治疗。
风湿性疾病患儿中SS的早期诊断对治疗选择和预后有显著影响。儿童SS在很长一段时间内可能没有临床表现。高球蛋白血症、无持续性关节炎的类风湿因子阳性、非特异性皮肤病变、复发性腮腺肿大等症状可能有助于SS的诊断。
