Yasmeen Juveriya, Salimova Dinara, Lohala Nita, Aloyan Tatevik, Siraw Bekure B
Internal Medicine, Ascension Saint Joseph Hospital, Chicago, USA.
Cureus. 2025 Jun 29;17(6):e86998. doi: 10.7759/cureus.86998. eCollection 2025 Jun.
Hemophagocytic lymphohistiocytosis (HLH) is a rare, potentially fatal hyperinflammatory syndrome marked by excessive immune activation, cytokine storm, multiorgan failure, and high mortality. Systemic lupus erythematosus (SLE) is considered to be one of the triggers. SLE-associated HLH is especially challenging to diagnose and manage, as it can mimic lupus flares. We describe a 30‑year‑old woman with SLE who developed secondary HLH. Initially thought to have an SLE flare, she rapidly deteriorated with multiorgan dysfunction. The diagnosis of HLH was confirmed using the HLH-2004 criteria and a significantly elevated H-score. Despite intensive treatment with corticosteroids and immunosuppressive agents, the patient ultimately died from HLH-related complications. This case highlights the critical need for early recognition and prompt differentiation of HLH from lupus flares in SLE patients. Timely, targeted therapy is essential to improve outcomes in this high-risk group.
噬血细胞性淋巴组织细胞增生症（HLH）是一种罕见的、潜在致命的高炎症综合征，其特征为过度免疫激活、细胞因子风暴、多器官功能衰竭和高死亡率。系统性红斑狼疮（SLE）被认为是触发因素之一。SLE相关的HLH在诊断和管理方面极具挑战性，因为它可能模仿狼疮发作。我们描述了一名30岁患有SLE的女性，她发展为继发性HLH。最初认为是SLE发作，她迅速出现多器官功能障碍而病情恶化。使用HLH-2004标准和显著升高的H评分确诊为HLH。尽管使用皮质类固醇和免疫抑制剂进行了强化治疗，患者最终仍死于HLH相关并发症。该病例凸显了在SLE患者中早期识别HLH并迅速将其与狼疮发作区分开来的迫切需求。及时、有针对性的治疗对于改善这一高危群体的预后至关重要。
