Kübler L, Bittmann I, Kuipers J G
Klinik für internistische Rheumatologie, Rotes Kreuz Krankenhaus, St.-Pauli-Deich 24, 28199, Bremen, Germany.
Pathologisches Institut, Agaplesion Diakonieklinikum, Rotenburg, Germany.
Z Rheumatol. 2020 Dec;79(10):1040-1045. doi: 10.1007/s00393-020-00836-w.
This article presents a case of fulminant macrophage activation syndrome (MAS) as a rare complication of active systemic lupus erythematosus in a 33-year-old female patient. Initial presentation showed severe lupus disease exacerbation with renal involvement, hemolytic anemia, and neuropsychiatric changes. Early therapy focused on broad immunosuppression (high-dose corticosteroids and cyclophosphamide); however, disease remission could not be achieved. After an additional inflammatory focus and underlying malignancy were excluded, the triplet of pancytopenia, fever, and high ferritin levels indicated MAS, a bone marrow biopsy confirmed secondary hemophagocytic histiocytosis. Treatment with an interleukin‑1 antagonist (anakinra) induced a fast, effective therapeutic success.
本文介绍了一例暴发性巨噬细胞活化综合征（MAS），这是一名33岁女性患者活动性系统性红斑狼疮的罕见并发症。初始表现为严重的狼疮病情加重，伴有肾脏受累、溶血性贫血和神经精神改变。早期治疗集中于广泛的免疫抑制（大剂量皮质类固醇和环磷酰胺）；然而，疾病缓解未能实现。在排除了额外的炎症病灶和潜在恶性肿瘤后，全血细胞减少、发热和高铁蛋白水平这三联征提示为MAS，骨髓活检证实为继发性噬血细胞性组织细胞增多症。使用白细胞介素-1拮抗剂（阿那白滞素）治疗取得了快速、有效的治疗成功。
