Abdelfattah Ahmed H, Macpherson Alexandra, Javed Faiza
Internal Medicine/Hospital Medicine, University of Kentucky College of Medicine, Lexington, USA.
Cureus. 2024 May 18;16(5):e60567. doi: 10.7759/cureus.60567. eCollection 2024 May.
Systemic lupus erythematosus (SLE) is an autoimmune disease characterized by the immune system erroneously attacking healthy tissues and organs. SLE has a wide variety of clinical presentations. The signs and symptoms of SLE are very well-known, though rare presentations could occur that require early clinical attention. Macrophage activation syndrome (MAS) is a severe and life-threatening condition in which the immune system becomes overactive, leading to the excessive stimulation and proliferation of immune cells. MAS can occur as a primary immune disorder, which is not very common. It can also happen secondary to a wide variety of pathological conditions, which include infections, malignancies, autoimmune, and rheumatologic disorders. In rare cases, SLE can present with overlapping features of MAS, further complicating the clinical picture, and may require specialized management. Early recognition and intervention of this overlap are essential for improving outcomes, as delayed diagnosis and treatment can lead to significant morbidity and mortality. Here, we present a case of a young adult female who was diagnosed with SLE with the initial presentation of MAS in the form of fever, splenomegaly, cytopenia, and hemophagocytosis.
系统性红斑狼疮（SLE）是一种自身免疫性疾病，其特征是免疫系统错误地攻击健康组织和器官。SLE有多种临床表现。SLE的体征和症状广为人知，不过也可能出现需要早期临床关注的罕见表现。巨噬细胞活化综合征（MAS）是一种严重的、危及生命的病症，其中免疫系统变得过度活跃，导致免疫细胞过度刺激和增殖。MAS可作为原发性免疫疾病出现，这种情况不太常见。它也可能继发于多种病理状况，包括感染、恶性肿瘤、自身免疫性疾病和风湿性疾病。在罕见情况下，SLE可表现出与MAS重叠的特征，使临床情况更加复杂，可能需要特殊管理。早期识别和干预这种重叠情况对于改善预后至关重要，因为延迟诊断和治疗可能导致严重的发病率和死亡率。在此，我们报告一例年轻成年女性病例，该患者最初以发热、脾肿大、血细胞减少和噬血细胞增多形式出现MAS，随后被诊断为SLE。
