全身型幼年特发性关节炎并发巨噬细胞活化综合征临床分析

目的 总结巨噬细胞活化综合征(MAS)的临床特征、治疗方法 ,以提高对本病的认识.方法 回顾性分析我院2006年10月至2007年9月收治的全身型幼年特发性关节炎并发MAS 3例患儿的临床资料.结果 3例患儿平均年龄为8.92岁,男2例,女1例.主要临床特征是:2例有前驱感染(1例为上呼吸道感染,1例为水痘病毒感染).3例患儿均有高热、中枢神经系统功能障碍表现,有白细胞、血小板减低,红细胞沉降率降低,高铁蛋白血症及凝血功能异常.2例肝脾和淋巴结肿大.虽经甲泼尼龙、地塞米松积极治疗,但3例患儿均因并发严重的中枢神经系统功能障碍而死亡.结论 MAS是全身型幼年特发性关节炎的一种严重而复杂的并发症,病死率高,感染可能是主要的触发因素.     

全身型幼年特发性关节炎并巨噬细胞活化综合征9例

目的探讨幼年特发性关节炎(SO-JIA)并巨噬细胞活化综合征(MAS)患儿的临床特点,为儿童SO-JIA并MAS的诊疗提供临床依据。方法回顾性分析2010年5月-2011年10月于上海交通大学医学院附属仁济医院儿科住院的9例SO-JIA并MAS患儿资料,总结其临床表现、实验室检查、治疗及预后等,并选择同期住院9例未并MAS的SO-JIA患儿作为对照。结果 SO-JIA并MAS患儿关节症状多以下肢关节为主,以膝、髋、踝关节多见,上肢关节少见;多为2个或2个以上的关节受累(7例),单关节累及较少(1例);关节以大关节受累为主,小关节受累较少(1例)。浅表淋巴结大易见,肝大较脾大多见。肺高分辨率CT显示肺间质性变6例,炎性渗出5例,胸腔积液1例。MAS发作时,ALT、LDH急剧上升,WBC、中性粒细胞、RBC、CRP、ESR、纤维蛋白原(FIB)下降明显,与发病前后比较差异有统计学意义。在疾病的极期阶段,并MAS的SO-JIA患儿较非MAS的SO-JIA患儿ALT、LDH、γ-GT、CK-MB、铁蛋白明显升高,WBC、中性粒细胞、RBC、CRP、ESR、FIB明显下降。经联合方案(激素联合环孢素及丙种球蛋白)治疗,好转7例,死亡2例。结论 MAS是SO-JIA的危重并发症,激素联合环孢素及丙种球蛋白治疗可有一定的临床缓解率,早期诊断和干预有利于控制病情、改善预后,检测一些敏感指标有利于MAS的早期发现。     

全身型幼年特发性关节炎发病机制研究进展

幼年特发性关节炎(JIA)是一组16岁以前起病,原因不明,以慢性关节炎为主要特征,可伴有其他组织、器官损害的慢性全身性疾病,并除外其他疾病所致关节炎.与其他类型JIA相比,全身型JIA(sJIA)常伴有特殊的临床表型,如弛张高热、皮疹、浆膜腔积液、肝脾淋巴结肿大及多器官损害,少数甚至伴发巨噬细胞活化综合征(MAS).此...     

全身型幼年特发性关节炎免疫发病机制研究进展

全身型幼年特发性关节炎(So-JIA)是幼年特发性关节炎(JIA)中的一种特殊类型,具有独特的临床表现,可合并巨噬细胞活化综合征(MAS)等严重并发症,伴明显的急性炎性反应指标.So-JIA的遗传背景可能更多涉及非组织相容性复合体(MHC)基因,如IL-6、IL-18、S100蛋白等基因多态性,在感染等触发因素刺激下激活吞噬细胞和内皮系统,产生大量炎性细胞因子,目前更多的证据表明So-JIA是一种自身炎症性疾病,So-JIA并发MAS可能具有更为复杂的遗传学基础.     

全身型幼年特发性关节炎并发巨噬细胞活化综合征临床分析．

目的总结巨噬细胞活化综合征（MAS）的临床特征、治疗方法,以提高对本病的认识。方法回顾性分析我院2006年10月至2007年9月收治的全身型幼年特发性关节炎并发MAS3例患儿的临床资料。结果3例患儿平均年龄为8．92岁,男2例,女1例。主要临床特征是：2例有前驱感染（1例为上呼吸道感染,1例为水痘病毒感染）。3例患儿均有高热、中枢神经系统功能障碍表现,有白细胞、血小板减低,红细胞沉降率降低,高铁蛋白血症及凝血功能异常。2例肝脾和淋巴结肿大。虽经甲泼尼龙、地塞米松积极治疗,但3例患儿均因并发严重的中枢神经系统功能障碍而死亡。结论MAS是全身型幼年特发性关节炎的一种严重而复杂的并发症,病死率高,感染可能是主要的触发因素。     

儿童寰枢椎半脱位的诊断与治疗

目的 探讨儿童寰枢椎半脱位的早期诊断与诒疗。方法 选择1992～2001年期问临床资料完整的32例寰枢椎半脱位患儿进行分析研究。结果 32例小儿经过Glission牵引、抗感染和对症处理，31例患儿症状体征完全消失，随访半年无复发。1例患儿经反复治疗，X线影像无明显改变。结论 寰枢椎半脱位是儿童期突发斜颈最常见的原因，早期诊断早期治疗预后良好。     

幼年特发性关节炎全身型合并巨噬细胞活化综合征4例报告并文献复习

目的 总结巨噬细胞活化综合征(macrophage activation syndrome,MAS)的临床特征及误诊原因,以提高对该病的认识.方法 回顾性分析54例幼年特发性关节炎全身型(systemic onset juvenile idiopathic arthritis,SO-JIA)合并MAS患儿的临床症状、体征、辅助检查及病情进展、诊断、治疗及预后.结果 54例SOJIA患儿中4例并发MAS(7.4%).临床特征有:持续高热、肝脾淋巴结增大、肝功能急剧恶化、皮肤黏膜易出血、外周血三系减少、中枢神经系统功能障碍、血沉进行性下降.结论 MAS是SOJIA的一个致死性并发症,起病突然,进展迅速,病死率高.在临床工作中需提高对其的认识,避免误诊.     

Macrophage activation syndrome in 13 children with systemic-onset juvenile idiopathic arthritis

Background  Macrophage activation syndrome (MAS) is a severe, potentially life-threatening condition induced by chronic rheumatic diseases, especially systemic-onset juvenile idiopathic arthritis (SoJIA) in childhood. This study aimed to analyze the clinical and laboratory characteristics of systemic-onset juvenile idiopathic arthritis (SoJIA) with macrophage activation syndrome (MAS) in 13 patients. Methods  Clinical and laboratory data of 13 SoJIA patients with MAS treated in our hospital from January 2003 to October 2007 were analyzed. Results  In the 13 patients, 9 were boys and 4 girls aged from 5 months to 12 years. Clinical manifestations were of no typical characteristics including persistent fever, anemia, arthritis, hepatosplenomegaly, lymph-adenopathy, dysfunction of the liver, abnormal fat metabolism, and hemophagocytic cells in the bone marrow. Two patients experienced acute respiratory distress syndrome, two had mutiorgan failure, and three died. The perforin A91V (NCBI:SNP rs35947132) gene in 6 patients was normal. Glucocorticoid and immunoimpressive therapy were effective in all patients and plasmapheresis used in one severe patient was also effective. Conclusions  MAS is a serious complication of JIA, especially systemic-onset juvenile idiopathic arthritis. It is essentially important to recognize and treat MAS earlier in order to lower the mortality.     

全身型幼年特发性关节炎合并巨噬细胞活化综合征诊断和治疗

巨噬细胞活化综合征(MAS)是继发于风湿免疫性疾病基础上的噬血细胞性淋巴组织细胞增多症(HLH),也是儿科急性危重症,其中,以全身型幼年特发性关节炎(sJIA)相关MAS(sJIA-MAS)最为常见,且具有较高的病死率,早期诊断、及时治疗是改善预后的关键。虽然sJIAMAS和HLH具有相似的临床和实验室特征,但由于二者的基础疾病不同,因此诊断标准也有所差异。糖皮质激素和环孢素A是MAS的一线治疗药物,但仍存在较大挑战,生物制剂靶向药物逐步被提出作为难治性MAS的治疗选择,甚至作为MAS的一线药物。该文重点阐述sJIA-MAS的临床和实验室特征、生物标志物、分类标准及治疗进展。     

FDG‐PET in macrophage activation syndrome associated with systemic juvenile idiopathic arthritis

We herein describe a case of systemic juvenile idiopathic arthritis (s‐JIA)‐associated macrophage activation syndrome (MAS) in which the ^18Ffluorodeoxyglucose positron emission tomography (^18FFDG‐PET) findings were characteristic. The pattern of greater ^18FFDG accumulation into the spleen compared with the liver was more remarkable in this patient compared with s‐JIA. This pattern, however, was also observed in cases of acute leukemia. In the present patient, serum interleukin (IL)‐18 was extremely elevated (255 000 pg/mL), whereas in leukemia patients it is mildly elevated (360–1480 pg/mL). ^18FFDG‐PET might be a useful indicator of s‐JIA and MAS in patients with fever of unknown origin. The pattern of ^18FFDG accumulation, however, can also be observed in acute leukemia. The combination of ^18FFDG‐PET and serum IL‐18 might be useful for the diagnosis of s‐JIA and MAS.     

幼年特发性关节炎的肾损害

幼年特发性关节炎(JIA)是影响多器官系统的全身性疾病,JIA并发肾损害丰要包括原发件肾损害、继发性肾淀粉样变性及药物性肾损害,常见的临床表现有蛋白尿、水肿、血尿、高血压及肾功能减退.治疗包括治疗原发病,抑制炎症反应,阻止肾淀粉样变,停用金制剂、青霉胺等肾损害药物及血液净化.幼年特发性关节炎全身型(SOJIA)合并巨噬细胞活化综合征(MAS)患儿出现严重肾脏损害,往往预后小良,早期、大剂量糖皮质激素单独或联合免疫抑制剂治疗,是决定预后的重要因素.     

Outcomes for juvenile idiopathic arthritis

Juvenile idiopathic arthritis (JIA) is the most common chronic rheumatological disease of childhood. Despite the current availability of potent disease-modifying anti-rheumatic medications, most children still experience a chronic course with prolonged periods of active disease. Goals for treatment should include disease remission with optimal physical functioning allowing children to lead normal lives without structural joint damage. However, recent studies demonstrate only moderate rates of remission, indicating that JIA is not as benign as previously thought. The probability of attaining remission within 5 years is approximately 50% across all JIA categories except for polyarthritis when the outlook is significantly worse. Longer term, about 50% of adults with JIA suffer from persistent inflammation and disability.There is a shift towards early aggressive treatment with the intention to switch off inflammation since there may be a ‘window of opportunity’ before the disease becomes chronic. There is clear evidence for improved outcome in adult patients treated with this approach (‘treat to target’) but limited paediatric evidence to date. The explosion in anticytokine agents for treatment of disease resistant to conventional therapy has expanded our armamentarium, improving short term clinical outcomes, but it is still unclear whether we have achieved significant improvements in outcome longer term. This review describes the disease and current and longer-term data on outcomes for this common chronic childhood condition.     

非甾体类消炎药联合甲氨蝶呤治疗全身型幼年特发性关节炎疗效观察

目的探讨非甾体类消炎药(NSAIDs)联合甲氨蝶呤(MTX)治疗全身型幼年特发性关节炎(SO-JIA)的疗效。方法分析比较32例SO-JIA患儿以NSAIDs联合MTX治疗后临床症状的变化,关节炎病情改善评估参照美国风湿病学会推荐的类风湿性关节炎改善标准。结果32例患儿治疗后,体温恢复正常27例,退热有效率为84.4%(27/32例),5例加用糖皮质激素后热退;31例关节炎症状改善,有效率为96.9%(31/32例),1例加用来氟米特后关节炎症状缓解。停药观察3例,复发1例。结论SO-JIA诊断确立后应尽早予NSAIDs联合MTX治疗,NSAIDs能较好地退热及减轻关节症状,MTX可有效持续改善关节炎症。糖皮质激素不作为首选退热用药。     

全身型幼年特发性关节炎合并肺间质病变概述

全身型幼年特发性关节炎是儿童常见的风湿性疾病之一,肺间质病变是指以肺间质和远端气隙重构为特征,进而导致异常气体交换的疾病.近年来,国内外对全身型幼年特发性关节炎合并肺间质病变的报道有增多趋势.该文总结了近年来报道中全身型幼年特发性关节炎儿童合并肺间质病变的情况,并对儿童肺间质病变的临床表现,影像学表现,诊断和治疗进行综...