Childhood primary Sjogren syndrome with central nervous system symptoms as the first manifestation: a case report and literature review

VernacularTitle:以中枢神经症状为首发表现的儿童原发性干燥综合征1例并文献复习
Author: Hua HUANG ¹ ; Youying MAO ; Yanliang JIN
Author Information

1. 上海交通大学医学院附属上海儿童医学中心上海 200127
Keywords: primary Sjogren syndrome; central nervous system; child
From: Journal of Clinical Pediatrics 2017;35(10):775-777
CountryChina
Language:Chinese
Abstract: Objective To explore the diagnosis and treatment of childhood primary Sjogren syndrome (pSS) with central nervous system symptoms as the first manifestation. Methods The clinical data of an 11-year-old male with pSS which had onset with fever and headache was retrospectively analyzed. The related literatures were reviewed. Results The subject was diagnosed with pSS by autoantibody detection, lip biopsy, and ophthalmologic examination. The symptoms were improved after immunosuppressive therapy. According to the literature, the incidence of childhood pSS was low, in which the incidence with involvment of the nervous system as primanry manifestation was even lower, and headache is the most common symptom of central nervous system. The application of corticosteroids and immunosuppressants may help improve the conditions. Conclusions Involvement of central nervous system in childhood pSS is not typical. Detection of autoantibodies and lip biopsy are helpful for diagnosis.