CAJ | 학술논문

目的探讨危重症幼年特发性关节炎(JIA)的临床表现和对策.方法回顾分析13例幼年特发性关节炎合并严重并发症患儿的病史,及心电图、心脏腹部超声、肝肾功能、骨髓像等相关检查,总结危重症幼年特发性关节炎临床特点、治疗并且随诊.结果 13例患儿均诊断为全身型JIA.13例JIA中发生巨噬细胞活化综合征(MAS)9例,心功能衰竭6例,急性脑病5例,少尿、肾功能衰竭4例,肝脏衰竭2例,呼吸衰竭1例,休克9例,弥散性血管内凝血(DIC)4例,败血症合并深部巨大脓肿1例,胸腔积液4例,心包积液2例,肺炎7例.10例重要脏器功能衰竭在1～2周内恢复.严重的出血、贫血2周内恢复.1个月后复诊肝、肾功能均全部正常.抽搐、昏迷患儿智力正常.随诊中未发现继发性癫痫.随访中均未出现MAS.死亡3例.结论危重型JIA进展快,同时累及多个器官.充血性心力衰竭、急性肾功能衰竭、急性脑病最多见,常合并MAS.大剂量激素、综合对症治疗是关键.危重症JIA病死率高,但危重情况一旦纠正预后良好.
목적 탐토위중증유년특발성관절염(JIA)적림상표현화대책.방법 회고분석13례유년특발성관절염합병엄중병발증환인적병사,급심전도、심장복부초성、간신공능、골수상등상관검사,총결위중증유년특발성관절염림상특점、치료병차수진.결과 13례환인균진단위전신형JIA.13례JIA중발생거서세포활화종합정(MAS)9례,심공능쇠갈6례,급성뇌병5례,소뇨、신공능쇠갈4례,간장쇠갈2례,호흡쇠갈1례,휴극9례,미산성혈관내응혈(DIC)4례,패혈증합병심부거대농종1례,흉강적액4례,심포적액2례,폐염7례.10례중요장기공능쇠갈재1～2주내회복.엄중적출혈、빈혈2주내회복.1개월후복진간、신공능균전부정상.추휵、혼미환인지력정상.수진중미발현계발성전간.수방중균미출현MAS.사망3례.결론 위중형JIA진전쾌,동시루급다개기관.충혈성심력쇠갈、급성신공능쇠갈、급성뇌병최다견,상합병MAS.대제량격소、종합대증치료시관건.위중증JIA병사솔고,단위중정황일단규정예후량호.
Objective To discuss the clinical characteristic and treatment of severe juvenile rheumatoid arthritis.Methods Thirteen patients were diagnosed as juvenile rheumatoid arthritis complicated with fatal complications.Their medical history, physical examination and laboratory examination were recorded in detail. The clinical characteristic and treatment were analyzed. Long-term follow-up was carried out. Results Thirteen patients were JIA, 9 patients were diagnosed as MAS, 6 with heart failure, 5 with acute encephalopathy, 4 with oliguria or renal failure, 2 with hepar failure,2 with respiratory failure, 9 with shock, 4 with DIC, 1 with septicemia complicated huge deep abscess, 4 with pleural effusion, 2 with pericardial effusion, 7 with pneumonia. 10 patients who complicated with important organ failure recovered within 1～2 weeks. Patients who complicated with severe hemorrhage or anemia recovered within 2 weeks. Liver and renal function test were absolutely normal after one month. Patients who complicated with spasm or coma recovered completely.Secondary epilepsy was not found in follow-up, and no MAS.Three patients died. Conclusions Severe juvenile rheumatoid arthritis progresses rapidly, meanwhile involves many organs. Congestive heart failure, acute renal failure and acute encephalopathy axe common. Macrophage activation syndrome is common, large-dose hormone and integrate treatment are essential. The mortality rate of severe juvenile rheumatoid arthritis is high, but it may have a favorable prognosis if treated timely and appropriately.