CAJ | 학술논문

结缔组织病（connective tissue disease，CTD）是一大类具有高度异质性、多系统累及的自身免疫性疾病，包括系统性红斑狼疮（systemic lupus erythematosus，SLE）、类风湿关节炎（rheumatoid arthritis，RA）、系统性硬化症（systemic sclerosis， SSc）、干燥综合征（primary sjogren's syndrome，pSS）等，其病理基础为血管和结缔组织的慢性炎症。 CTD 累及中枢神经系统（包括大脑、脑膜和脊髓）临床表现复杂多样，可以是 CTD 本身所致，亦可为感染、药物等因素继发，部分患者甚至以中枢神经系统症状为首发表现。 CTD 累及中枢神经系统，病情凶险，属于危急重症，是 CTD 患者死亡的主要原因之一。根据 CTD 中枢神经系统危急重症的临床特点，及时准确的诊断，积极合适的治疗是改善患者预后的关键。本文将阐述 CTD 累及中枢神经系统危急重症的临床特点及处理。
결체조직병（connective tissue disease，CTD）시일대류구유고도이질성、다계통루급적자신면역성질병，포괄계통성홍반랑창（systemic lupus erythematosus，SLE）、류풍습관절염（rheumatoid arthritis，RA）、계통성경화증（systemic sclerosis， SSc）、간조종합정（primary sjogren's syndrome，pSS）등，기병리기출위혈관화결체조직적만성염증。 CTD 루급중추신경계통（포괄대뇌、뇌막화척수）림상표현복잡다양，가이시 CTD 본신소치，역가위감염、약물등인소계발，부분환자심지이중추신경계통증상위수발표현。 CTD 루급중추신경계통，병정흉험，속우위급중증，시 CTD 환자사망적주요원인지일。근거 CTD 중추신경계통위급중증적림상특점，급시준학적진단，적겁합괄적치료시개선환자예후적관건。본문장천술 CTD 루급중추신경계통위급중증적림상특점급처리。
Connective tissue disease(CTD)is a group of highly heterogeneous autoimmune disease characterized by pathologic changes in systemic organs and chronic inflammation of connective tissue .These diseases include systemic lupus erythematosus (SLE), rheumatoid arthritis(RA),systemic sclerosis and Sj?gren syndrome.Its pathological basis is chronic inflammation of blood vessels and connective tissue.The clinical manifestations are complex and varied that can be caused by the primary disease or as a secondary conse -quence of the primary disease.Some cases show that the central nervous system symptoms are starting performance .The involvement of the central nervous system is the dangerous condition in clinic that may be one of the leading causes of death among CTD patients .Early recognition and diagnosis ,and active and efficient therapy are the vital importance for the prognosis .This review focuses on the clinical features and therapies of emergency and severe cases of CTD involved central nervous system .