BACKGROUND Sjögren syndrome (SS) is a rare disease in pediatrics, and little attention has been paid to the clinical feature in these patients. To date, there are few cases concern about neurological and nephrological disorders in childhood Sjögren syndrome. We describe a case of Sjögren syndrome in a 12-year-old girl who developed neurological disorders and interstitial nephritis and review the literature currently available on this topic. CASE PRESENTATION A 12-year-old girl was admitted to our hospital for arthritis and glucosuria. She was required to do labial gland and renal biopsy, because the positive for anti-nuclear antibody and anti-Sjögren syndrome B (anti-SSB) antibody. Then the biopsy was performed revealing the lymphocytic infiltrate in the small area and renal tubular interstitial damage,thus the diagnosis of Sjögren syndrome with tubular interstitial damage was made. Three months later, she presented again with headache, fever, nausea, vomiting and was recovered without drug therapy. Based on the patient's medical history, laboratory and imaging examination, and treatment, we speculate that the disorders of the nervous system were caused by the Sjögren syndrome. The girl has stable renal function and no residual nervous system damage in the next 1.5 years, but she underwent low dose prednisone therapy because of persistent renal glucosuria. CONCLUSIONS Nephrological disorders and neurological involvement are rare manifestations of Sjögren syndrome in children, and rarely presented as the initial symptoms. It should be suspected in children presenting with unexplained renal diseases, neurological abnormalities, or unexplained fever. Although there is no guidelines on the diagnosis and treatment of children Sjögren syndrome are currently available, early recognition and the appropriate treatment of renal damage and neurologic involvement would improve prognosis and prevent complications.

中文翻译：

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小儿原发性干燥综合征（Sjogren syndrome）的肾病和神经系统疾病：一例病例报告并文献复习。

背景技术Sjögren综合征（SS）是儿科中一种罕见的疾病，很少关注这些患者的临床特征。迄今为止，很少有病例涉及儿童期Sjögren综合征的神经和肾脏疾病。我们描述了一名12岁少女Sjögren综合征的案例，该少女发展为神经系统疾病和间质性肾炎，并回顾了有关该主题的文献。病例介绍一名12岁女孩因关节炎和糖尿症入院。她被要求做唇腺和肾脏活检，因为抗核抗体和抗Sjögren综合征B（抗SSB）抗体呈阳性。然后进行活检，发现小区域有淋巴细胞浸润和肾小管间质损害，从而诊断出患有肾小管间质损害的干燥综合征。三个月后，她再次出现头痛，发烧，恶心，呕吐，未经药物治疗即康复。根据患者的病史，实验室检查和影像学检查以及治疗，我们推测神经系统疾病是由Sjögren综合征引起的。在接下来的1.5年中，该女孩的肾功能稳定并且没有残留的神经系统损害，但由于持续存在的肾糖尿症，她接受了低剂量泼尼松治疗。结论肾脏疾病和神经系统受累是儿童干燥综合征的罕见表现，很少表现为初始症状。患有无法解释的肾脏疾病，神经系统异常，或无法解释的发烧。尽管目前尚无有关儿童干燥综合征的诊断和治疗指南，但尽早识别肾损伤和神经系统受累并进行适当治疗可改善预后并预防并发症。