Islam M I, Talukder M K, Islam M M, Laila K, Rahman S A
Dr Mohammad Imnul Islam, Associate Professor, Department of Paediatrics, Bangabandhu Sheikh Mujib Medical University (BSMMU), Dhaka, Bangladesh.
Mymensingh Med J. 2017 Apr;26(2):356-363.
Macrophage activation syndrome (MAS) is a potentially fatal complication of rheumatic disorders, which commonly occurs in systemic juvenile idiopathic arthritis (sJIA).This study was carried out with the aims of describing the clinical features, laboratory findings and outcomes of MAS associated with paediatric rheumatic diseases in the Department of Paediatrics, Bangabandhu Sheikh Mujib Medical University (BSMMU) and compare these results with previous studies on MAS. This retrospective study was conducted in the paediatric rheumatology wing of the Department of Paediatrics, Bangabandhu Sheikh Mujib Medical University (BSMMU), Dhaka, Bangladesh. Clinical and laboratory profile of all the diagnosed cases of MAS were analyzed from the medical records from January 2010 to July 2015. Among 10 MAS patients, 6 were female and 4 were male. Seven patients of systemic JIA, two patients of SLE and one patient with Kawasaki Disease developed MAS in their course of primary disease. Mean duration of primary disease prior to development of MAS was 2.9 years and mean age of onset was 9.1 years. High continued fever and new onset hepatosplenomegaly were the hallmark of the clinical presentation. White blood cell count and platelet count came down from the mean of 16.2 to 10.2×10⁹/L and 254 to 90×10⁹/L. Mean erythrocyte sedimentation rate was dropped from 56 to 29 mm/hr. Six patients had abnormal liver enzyme level (ALT) and 5 had evidence of coagulopathy (prolonged prothrombin time and APTT) at the onset of disease. Hyperferritinnemia were found in all the patients. Bone marrow study was done in 5 patients but features of hamophagocytosis were found only in 2 patients. All patients received intravenous steroid and 3 patients who did not respond to steroid received additional cyclosporine. Mortality rate was 30% in this series. Macrophage activation syndrome is a fatal complication of paediatric rheumatic diseases among which s-JIA was predominant. Early diagnosis and aggressive therapy is essential to reduce the morbidity and mortality of this illness.
巨噬细胞活化综合征（MAS）是风湿性疾病的一种潜在致命并发症，常见于全身型幼年特发性关节炎（sJIA）。本研究旨在描述孟加拉国班加班杜·谢赫·穆吉布医科大学（BSMMU）儿科中与儿童风湿性疾病相关的MAS的临床特征、实验室检查结果及预后，并将这些结果与以往关于MAS的研究进行比较。这项回顾性研究在孟加拉国达卡的班加班杜·谢赫·穆吉布医科大学（BSMMU）儿科的儿科风湿病科进行。对2010年1月至2015年7月病历中所有确诊的MAS病例的临床和实验室资料进行分析。10例MAS患者中，6例为女性，4例为男性。7例全身型JIA患者、2例系统性红斑狼疮（SLE）患者和1例川崎病患者在原发性疾病病程中发生了MAS。MAS发生前原发性疾病的平均病程为2.9年，平均发病年龄为9.1岁。持续高热和新发肝脾肿大是临床表现的标志。白细胞计数和血小板计数从平均16.2降至10.2×10⁹/L，从254降至90×10⁹/L。平均红细胞沉降率从56降至29mm/hr。6例患者发病时肝酶水平（ALT）异常，5例有凝血功能障碍证据（凝血酶原时间和活化部分凝血活酶时间延长）。所有患者均有高铁蛋白血症。5例患者进行了骨髓检查，但仅2例发现噬血细胞现象。所有患者均接受了静脉注射类固醇治疗，3例对类固醇无反应的患者接受了额外的环孢素治疗。本系列的死亡率为30%。巨噬细胞活化综合征是儿童风湿性疾病的一种致命并发症，其中以s-JIA为主。早期诊断和积极治疗对于降低该疾病的发病率和死亡率至关重要。
