Long term follow-up is essential for these children due to the possibility of rapid progression. Acknowledgments We would like to thank Allen Cusack,PhD & MD for English language editing. Ethics approval and consent to participation Parental Ethotoin informed consent for publication was obtained. Abbreviations SSSj?gren syndromepSSprimary Sjogren syndromeEGMsextraglandular manifestationsESRerythrocyte sedimentation rateOGTToral glucose tolerance testHbA1cglycosylated hemoglobin A-1cRGrenal glucosuriaANAanti-nuclear antibodyanti-SSBanti-Sj?gren syndrome BP-ANCAperinuclear antineutrophil cytoplasmic antibodyanti-SSAanti-Sj?gren syndrome Aanti-dsDNAanti-double stranded DNAanti-RNPanti-ribonnucleoproteinanti-MPO-ANCAanti-myeloperoxidase antineutrophil cytoplasmic antibodyRFrheumatoid factoranti-CCPanti-cyclic citrullinated peptideHBVhepatitis B virusHCVhepatitis C virusHIVhuman immunodeficiency virusCSFcerebrospinal fluidPNSperipheral nervous systemCNScentral nervous systemTINtubulointerstitial nephritisRTArenal tubular acidosisGNglomerulonephritisMGNmembranous glomerulonephritisMPGNmembranoproliferative glomerulonephritis Authors contributions JZ wrote the first draft of the manuscript and contributed to patient management. lymphocytic Ethotoin infiltrate in the small area and renal tubular interstitial damage,thus the diagnosis of Sj?gren 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 patients medical history, laboratory and imaging examination, and treatment, we speculate that this disorders of the nervous system were caused by the Sj?gren 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 Sj?gren 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 around the diagnosis and treatment of children Sj? Ethotoin gren syndrome are currently available, early recognition and the appropriate treatment of renal damage and neurologic involvement would improve prognosis and prevent complications. and were also not found in the CSF. After these test results, she was diagnosed with aseptic meningoencephalitis but we could not exclude the possibility of viral meningitis. Therefore, the patient was treated with intravenous acyclovir. However,due to drug allergy,we stopped acyclovir treatment early. After 3 days, her headache and rash were significantly relieved. Based on the patients medical history, CSF examination, and treatment, we speculate that this disorders of the nervous system were more likely caused by the pSS. During the follow up of 1 1.5?years, her renal function was stable and no residual nervous system damage was apparent. She underwent low dose prednisone therapy (5-10?mg/d) for half a year because of persistent renal glucosuria. Open in a separate window Fig. 1 minor salivary gland biospy Open in a separate window Fig. 2 Kidney biopsy specimen Open in a separate window Fig. 3 T1-weighted and T2-weighted image showing normal signal intensity in the parenchymal and cerebellum. No abnormally was found in the shape, size and position of ventricle, cistern and sulci Discussion and conclusions We aimed to review all full-text, peer-review publications reporting childhood Sjogren syndrome with kidney or nerve damage. Records were identified from the PubMed, EMBASE databases. The search terms were primary Sjogren syndrome, child, children, and childhood. Results were limited to case reports written in English. The search date was December 23, 2019. The initial search yielded 511 articles, after excluding the duplicate articles and reading titles and abstracts, 61 papers were then read in detail. Finally, 20 case reports were included in the literature review after extracting and analyzing the data from the articles (Fig.?4). The information that was extracted from the papers were as follows: Rabbit polyclonal to PAX2 references and year, age and gender of patient, symptoms at onset, dry eyes or mouth, parotitis,neurologic manifestation, renal damage, elevated ANA, presence of anti-SSA and SSB antibodies, ESR, RF, hyperglobulinemic, schirmer test, CSF, renal and salivary gland biopsy and immunomodulatory therapy (Table?1) [2C21]. Open in a separate window Fig. 4 Study selection flow chart Table 1 neurological and nephrological manifestation in childhood Sjogren syndrome female; male; yes; no; months; weeks; not mention; unfavorable; positive; glucocorticoid; azathioprine; rituximab; hydroxychloroquine; cyclophosphamide; mycophenolate mofetil; methotrexate; cyclosporine A; tacrolimus Primary Sjogren syndrome is an autoimmune disorder that causes inflammation and injury to the exocrine glands [22], predominantly the lacrimal and salivary glands, resulting in dry eyes and mouth (sicca syndrome). There are few reports on childhood primary Sjogren syndrome, because SS is usually more common in adults than in children. The female to male ratio in adults is usually 9:1, and joint Ethotoin problems were present in 30C50%, while the incidence of kidney disease varies from 0.3% to up to 33.5%, depending on the study [23C27]. Other extraglandular diseases, such as cutaneous vasculitis, pulmonary manifestations, and peripheral nervous system manifestations occur in less than 10% [22]. In children, the sex ratio was 83C92.3% female [28, 29], and the most frequent symptom was parotid swelling,which was present in 42.3C53%, while central nervous system symptoms were present in 8.7%, Ethotoin and renal manifestations were present in.