Rakotonirina L N, Andrianiaina M O, Rakotonirainy O H, Ramanandafy H, Randriamifidy N H, Rapelanoro Rabenja F
Department of Rheumatology Joseph Raseta Befelatanana University Hospital Antananarivo Madagascar.
Department of Internal Medicine Joseph Raseta Befelatanana University Hospital Antananarivo Madagascar.
Clin Case Rep. 2025 Apr 10;13(4):e70419. doi: 10.1002/ccr3.70419. eCollection 2025 Apr.
Hemophagocytic lymphohistiocytosis (HLH) is a rare and potentially fatal clinical and histological entity. Macrophage Activation Syndrome (MAS) is an HLH secondary to rheumatic and autoimmune diseases. Secondary MAS in systemic lupus erythematosus (SLE) is underdiagnosed. Its treatment is not yet standardized. The treatment of MAS in the context of SLE primarily involves corticosteroids and immunosuppressants, with the potential addition of Intravenous Immunoglobulins (IVIG) and biological treatments for refractory cases. Early detection and prompt intervention are crucial to reduce associated mortality. The presence of infection worsens the patient's prognosis. The coexistence of SLE and hepatitis B during MAS is rarely described in the literature, and its management remains debated. We report the case of a 42-year-old Malagasy woman presenting with SLE complicated by MAS at the time of diagnosis, associated with viral hepatitis B.
噬血细胞性淋巴组织细胞增生症（HLH）是一种罕见且可能致命的临床和组织学病症。巨噬细胞活化综合征（MAS）是继发于风湿性和自身免疫性疾病的HLH。系统性红斑狼疮（SLE）中的继发性MAS诊断不足。其治疗尚未标准化。SLE背景下MAS的治疗主要涉及皮质类固醇和免疫抑制剂，难治性病例可能加用静脉注射免疫球蛋白（IVIG）和生物治疗。早期发现和及时干预对于降低相关死亡率至关重要。感染的存在会使患者预后恶化。MAS期间SLE与乙型肝炎并存的情况在文献中很少描述，其管理仍存在争议。我们报告了一例42岁马达加斯加女性的病例，该患者在诊断时患有SLE并伴有MAS，同时合并乙型病毒性肝炎。
