Clinical features of macrophage activation syndrome as the onset manifestation of juvenile systemic lupus erythematosus.
Clinical features of macrophage activation syndrome as the onset manifestation of juvenile systemic lupus erythematosus.
复制标题
以巨噬细胞活化综合征为青少年系统性红斑狼疮发病表现的临床特征。
DOI：
10.1093/rap/rkz013
复制
发表时间：
2019
期刊：
影响因子：
--
通讯作者：
Kawano Y.
中科院分区：
文献类型：
--
作者：
45.Sato S;Uejima Y;Arakawa Y;Furuichi M;Suganuma E;Fujinaga S;Nakazawa A;Kawano Y.
目的巨噬细胞激活综合征（macrophage activation syndrome， MAS）是幼年系统性红斑狼疮（jSLE）的严重并发症。然而，对于这些疾病之间的关联知之甚少，特别是MAS作为jSLE的初始表现。本研究的目的是确定MAS作为jSLE初始表现的临床特征。方法回顾性分析2004 ~ 2016年诊断为MAS合并jSLE患者的临床特点。将这些患者的数据与没有MAS的jSLE患者组成的对照组的数据进行比较。结果46例患者中，有11例（23.9%）在jSLE的初始阶段被诊断为MAS。组间比较显示，合并MAS的jSLE患者发热、白细胞减少、高铁血症、天冬氨酸转氨酶升高的发生率高于对照组（P<0.01）。重要的是，MAS患者的神经系统症状明显比对照组更常见（P<0.01）， 11例MAS患者中有6例（54.6%）受到影响。治疗方面，所有11例同时患有jSLE和MAS的患者在诊断时均给予CSs， 7例接受免疫抑制剂治疗。本研究中没有患者死亡。结论mas可作为jSLE的首发表现发展。发热、高铁血症、细胞减少和肝功能紊乱的患者应怀疑MAS合并jSLE。此外，我们发现合并MAS的jSLE患者比没有MAS的jSLE患者有更多的神经系统症状。所有MAS患者均成功接受CSs治疗。早期诊断和强化治疗对改善临床结果至关重要。
ObjectivesMacrophage activation syndrome (MAS) is a severe complication of juvenile systemic lupus erythematosus (jSLE). However, little is known about the association between these conditions, especially in terms of MAS as the initial manifestation of jSLE. The aim of this study was to determine the clinical features of MAS as the initial manifestation of jSLE.MethodsWe carried out a retrospective review of the clinical features of MAS cases diagnosed concomitantly with jSLE from 2004 to 2016. Data from these patients were compared with those from a control group consisting of jSLE patients without MAS.ResultsEleven (23.9%) of the 46 patients recruited for this study were diagnosed with MAS during the initial stage of jSLE. The between-group comparisons demonstrated that fever, leucopenia, hyperferritinaemia and increased aspartate aminotransferase were more frequently observed in jSLE patients with MAS than in controls (P<0.01). Importantly, neurological symptoms were significantly more common in patients with MAS than in controls (P<0.01), with 6 (54.6%) of the 11 MAS patients affected. For treatment, all 11 patients with both jSLE and MAS were administered CSs upon diagnosis, and 7 received immunosuppressants. No patient involved in this study died.ConclusionMAS can develop as the initial manifestation of jSLE. MAS with jSLE should be suspected in patients with fever, hyperferritinaemia, cytopenia and liver disorder. In addition, we found that jSLE patients with MAS had more neurological symptoms than those without. All patients with MAS were successfully treated with CSs. Early diagnosis and intensive therapy are essential in improving clinical outcomes.