Tayer-Shifman Oshrat E, Ben-Chetrit Eldad
a Rheumatology Unit, Hadassah Hebrew University Medical Center , Jerusalem , Israel.
Mod Rheumatol. 2015;25(6):954-7. doi: 10.3109/14397595.2013.844403. Epub 2013 Oct 21.
A patient with antiphospholipid syndrome, SLE and refractory fever is described. The cause for the fever was macrophage activation syndrome (MAS). The diagnosis of MAS was made with the help of PET-CT. Since the syndrome was refractory to conventional therapy with high-dose steroids and cyclosporin, anakinra was administered with complete recovery of the patient. The present case illustrates the difficulties in diagnosing MAS when multiple bone marrow biopsies fail to show hemophagocytosis. It emphasizes the significance of PET-CT in the diagnosis and evaluation of treatment of MAS. Finally, it describes the important role of Anakinra in treating refractory cases of MAS.
本文描述了一名患有抗磷脂综合征、系统性红斑狼疮且发热难治的患者。发热原因是巨噬细胞活化综合征（MAS）。借助正电子发射断层扫描-计算机断层扫描（PET-CT）做出了MAS的诊断。由于该综合征对大剂量类固醇和环孢素的常规治疗无效，给予阿那白滞素治疗后患者完全康复。本病例说明了当多次骨髓活检未能显示噬血细胞现象时，诊断MAS的困难。它强调了PET-CT在MAS诊断和治疗评估中的重要性。最后，它描述了阿那白滞素在治疗难治性MAS病例中的重要作用。
