巨噬细胞活化综合征：362 例儿童发病与 1830 例成人发病系统性红斑狼疮患者中急性胰腺炎的一种严重且常见表现
Macrophage activation syndrome: A severe and frequent manifestation of acute pancreatitis in 362 childhood-onset compared to 1830 adult-onset systemic lupus erythematosus patients.
机构信息
Division of Rheumatology, Faculdade de Medicina da Universidade de São Paulo, Av. Dr. Arnaldo, no. 455, 3 andar, sala 3190-Cerqueira César, São Paulo 05403-010, Brazil; Pediatric Rheumatology Unit, Faculdade de Medicina da Universidade de São Paulo, São Paulo, Brazil.
Division of Rheumatology, Faculdade de Medicina da Universidade de São Paulo, Av. Dr. Arnaldo, no. 455, 3 andar, sala 3190-Cerqueira César, São Paulo 05403-010, Brazil.
出版信息
Semin Arthritis Rheum. 2016 Jun;45(6):706-10. doi: 10.1016/j.semarthrit.2015.10.015. Epub 2015 Nov 5.
OBJECTIVE
We previously reported a case series of acute pancreatitis (AP) and macrophage activation syndrome (MAS) in childhood (cSLE) patients; however, there are no data regarding the comparison of AP and MAS in large populations of cSLE and adult SLE (aSLE).
METHODS
A study included 362 cSLE and 1830 aSLE patients. MAS was diagnosed according to preliminary diagnostic guidelines and AP according to the presence of abdominal pain or vomiting associated to an increase of pancreatic enzymes and/or pancreatic radiological abnormalities. Demographic data, clinical features, SLEDAI-2K, SLICC/ACR-DI, and treatment were assessed.
RESULTS
Age in MAS patients was significantly lower compared with those without this complication [15 (8.8-55) vs. 33.5 (10.2-45.7) years, p = 0.007]. The frequencies of fever (94% vs. 37%, p = 0.001), leucopenia (82% vs. 19%, p = 0.0001), thrombocytopenia (65% vs. 19%, p = 0.013), hypertriglyceridemia (87% vs. 42%, p = 0.037), and hyperferritinemia (93% vs. 37%, p = 0.011) were also more frequently observed in AP patients with MAS compared in AP patients without MAS. Fever and hyperferritinemia concomitantly were more frequent in the former group (86% vs. 12%, p = 0.0015). Higher and significant frequency of AP in cSLE compared to aSLE patients [12/362 (3.3%) vs. 20/1830 (1.1%), p = 0.003], with similar AP duration [22 (6-60) vs. 15 (4-90) days, p = 0.534]. MAS (85% vs. 30%, p = 0.003) and death by MAS complication (31% vs. 0%, p = 0.017) were significantly higher in children compared with aSLE.
CONCLUSIONS
This study provides novel data demonstrating that MAS occur in the majority of cSLE with AP with a higher mortality compared to aSLE. In addition, we identified in AP patients, a cluster of MAS clinical and laboratorial parameters more associated with this complication.
目的
我们之前报道了一组儿童系统性红斑狼疮（cSLE）患者中出现的急性胰腺炎（AP）和巨噬细胞活化综合征（MAS）病例系列；然而，目前尚无关于 cSLE 和成人系统性红斑狼疮（aSLE）患者中 AP 和 MAS 大人群比较的数据。
方法
这项研究纳入了 362 例 cSLE 和 1830 例 aSLE 患者。根据初步诊断指南诊断 MAS，根据存在腹痛或呕吐、胰腺酶升高和/或胰腺影像学异常诊断 AP。评估人口统计学数据、临床特征、SLEDAI-2K、SLICC/ACR-DI 和治疗情况。
结果
MAS 患者的年龄明显低于无此并发症的患者[15（8.8-55）岁 vs. 33.5（10.2-45.7）岁，p=0.007]。AP 合并 MAS 患者的发热（94% vs. 37%，p=0.001）、白细胞减少（82% vs. 19%，p=0.0001）、血小板减少（65% vs. 19%，p=0.013）、高甘油三酯血症（87% vs. 42%，p=0.037）和高铁蛋白血症（93% vs. 37%，p=0.011）更为常见。发热和高铁蛋白血症同时在前者中更为常见（86% vs. 12%，p=0.0015）。与 aSLE 患者相比，cSLE 患者中 AP 的发生率更高[12/362（3.3%） vs. 20/1830（1.1%），p=0.003]，AP 持续时间相似[22（6-60）天 vs. 15（4-90）天，p=0.534]。与 aSLE 患者相比，儿童患者的 MAS（85% vs. 30%，p=0.003）和 MAS 并发症导致的死亡率（31% vs. 0%，p=0.017）更高。
结论
本研究提供了新的数据，表明 MAS 发生在大多数 AP 合并 cSLE 患者中，与 aSLE 相比，死亡率更高。此外，我们在 AP 患者中发现了一组与该并发症更相关的 MAS 临床和实验室参数簇。
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