Dilibe Arthur, Ugoala Onyinye S, Evbayekha Endurance O, Khalilullah Mohammad Z, Adabale Olanrewaju K, Poyser Tracy-Ann, Oriaifo Osejie F, Olori Ufuoma I, Aiwuyo Henry O
Internal Medicine, Brody School of Medicine at East Carolina University, Greenville, USA.
Internal Medicine, College of Medicine, University of Lagos, Lagos, NGA.
Cureus. 2023 Mar 1;15(3):e35670. doi: 10.7759/cureus.35670. eCollection 2023 Mar.
Hemophagocytic lymphohistiocytosis (HLH) is a rare and life-threatening syndrome of excessive inflammation and tissue destruction secondary to abnormal immune activation. The term macrophage activation syndrome (MAS) is used when HLH develops in the setting of systemic juvenile idiopathic arthritis (SJIA; formerly known as Still's disease), adult-onset Still's disease, or any other rheumatologic disorder. We present a case of a 21-year-old female with a known history of SJIA who presented to the hospital with fever, chills, myalgia, nausea, vomiting, and hypotension. Initial evaluation at the time of presentation suggested sepsis likely due to acute pyelonephritis, and the patient was started on antibiotics and intravenous fluid hydration. However, further workup suggested that her symptoms were non-infectious and were likely due to MAS, a rare complication of SJIA. We promptly diagnosed her, and she received a course of steroids and made an uneventful recovery.
噬血细胞性淋巴组织细胞增生症（HLH）是一种罕见且危及生命的综合征，继发于异常免疫激活，表现为过度炎症反应和组织破坏。当HLH在系统性幼年特发性关节炎（SJIA，以前称为斯蒂尔病）、成人斯蒂尔病或任何其他风湿性疾病背景下发生时，使用巨噬细胞活化综合征（MAS）这一术语。我们报告一例21岁女性患者，她有SJIA病史，因发热、寒战、肌痛、恶心、呕吐和低血压入院。就诊时的初步评估提示可能因急性肾盂肾炎导致脓毒症，患者开始接受抗生素治疗及静脉补液。然而，进一步检查表明她的症状并非感染性的，可能是由于MAS，这是SJIA的一种罕见并发症。我们迅速对她进行了诊断，她接受了一个疗程的类固醇治疗，康复过程顺利。
