Alowais Noura, Zamani Noura, Elbadawi Faisal, Ahmed Hassan, Dutta Pankhi, Sharif Aesha
Internal Medicine, Dubai Health, Dubai, United Arab Emirates.
Mohammed Bin Rashid University, Postgraduate Medical Education Division, Dubai, United Arab Emirates.
Eur J Case Rep Intern Med. 2024 Dec 3;11(12):005026. doi: 10.12890/2024_005026. eCollection 2024.
Hemophagocytic lymphohistiocytosis (HLH), is characterized by systemic uncontrolled inflammation resulting from immune dysregulation secondary to various triggers, including genetics, infections, autoimmune diseases, and malignancies. Macrophage activation syndrome (MAS) is an immune dysregulation phenomenon, in which an underlying rheumatological disease is present. We report a rare, interesting case of a middle-aged female, with a systemic lupus erythematosus (SLE) flare complicated by macrophage activation syndrome (MAS), in which tuberculous meningitis (TBM) was the identified trigger.
A 48-year-old female with an African ethnic background, diagnosed with SLE, presented with a 2-week history of high-grade fever, cough, and worsening arthralgia. She was initially admitted with suspected SLE flare secondary to community acquired pneumonia and was started on broad-spectrum antibiotics. Later during her hospital stay, she underwent extensive workup because of her clinical condition, as she had continuous spikes of fever, progressive cytopenia, accompanied with acute confusion state despite treatment. She was ultimately diagnosed with SLE flare complicated by MAS secondary to TBM in view of persistent fever, neuropsychiatric symptoms, hyperferritinemia and hypertriglyceridemia, supported by bone marrow aspiration findings, a tuberculosis (TB) positive contact and supporting cerebrospinal fluid analysis suggestive of TBM. She was immediately started on pulse doses of steroids and anti-tuberculosis medications in which significant clinical improvement was observed. She was discharged home, with continuity of care done at the outpatient department in addition to continuity of physical rehabilitation.
Given the wide range of clinical presentations and similarities between SLE and the life-threatening MAS, it is essential to maintain a high level of suspicion to establish the diagnosis and promptly start the necessary treatment to ensure better clinical and survival outcomes.
The wide range of clinical presentation of hemophagocytic lymphohistiocytosis/macrophage activation syndrome (HLH/MAS) makes it a challenging diagnosis. Hence, a high index of suspicion is necessary to establish the diagnosis and promptly start the necessary treatment.The mainstay of treatment is the early identification and management of the underlying etiology of MAS along with high-dose glucocorticoids and other immunosuppressive therapy targeting the immune dysregulation pathway.
噬血细胞性淋巴组织细胞增生症（HLH）的特征是由于各种触发因素（包括遗传、感染、自身免疫性疾病和恶性肿瘤）继发免疫失调而导致的全身性失控炎症。巨噬细胞活化综合征（MAS）是一种免疫失调现象，存在潜在的风湿性疾病。我们报告了一例罕见且有趣的中年女性病例，该患者系统性红斑狼疮（SLE）发作并伴有巨噬细胞活化综合征（MAS），其中结核性脑膜炎（TBM）是确定的触发因素。
一名48岁的非洲裔女性，被诊断为SLE，出现高热、咳嗽和关节痛加重2周的病史。她最初因疑似社区获得性肺炎继发SLE发作入院，并开始使用广谱抗生素。在住院后期，由于她持续发热、进行性血细胞减少，尽管接受了治疗仍伴有急性意识模糊状态，因此根据其临床情况进行了广泛的检查。鉴于持续发热、神经精神症状、高铁蛋白血症和高甘油三酯血症，结合骨髓穿刺结果、结核（TB）阳性接触史以及支持结核性脑膜炎的脑脊液分析结果，她最终被诊断为SLE发作并伴有TBM继发的MAS。她立即开始接受大剂量类固醇和抗结核药物治疗，随后观察到明显的临床改善。她出院回家，除了进行身体康复的连续性护理外，还在门诊进行连续性护理。
鉴于SLE和危及生命的MAS之间临床表现广泛且相似，必须保持高度怀疑以确立诊断并及时开始必要的治疗，以确保更好的临床和生存结果。
噬血细胞性淋巴组织细胞增生症/巨噬细胞活化综合征（HLH/MAS）临床表现广泛，使其诊断具有挑战性。因此，需要高度怀疑指数来确立诊断并及时开始必要的治疗。治疗的主要方法是早期识别和处理MAS的潜在病因，同时使用大剂量糖皮质激素和其他针对免疫失调途径的免疫抑制疗法。
