Egües Dubuc César Antonio, Uriarte Ecenarro Miren, Meneses Villalba Carlos, Aldasoro Cáceres Vicente, Hernando Rubio Iñaki, Belzunegui Otano Joaquín
Servicio de Reumatología, Hospital Universitario Donostia, San Sebastián, España.
Servicio de Reumatología, Hospital Universitario Donostia, San Sebastián, España.
Reumatol Clin. 2014 Sep-Oct;10(5):321-4. doi: 10.1016/j.reuma.2013.09.004. Epub 2013 Dec 7.
Hemophagocytic syndrome (HS) occurs in autoimmune diseases and belongs to the hemophagocytic lymphohistiocytosis group of diseases. This paper describes the features of 2 patients with systemic lupus erythematosus (SLE) who presented HS as the initial clinical manifestation.
Both patients had prolonged fever not associated to an infectious process and did not respond to broad-spectrum antibiotics.
The diagnosis of HS secondary to SLE is complicated, because it has some features in common, but HS is characterized by hyperferritinemia, hipofibrinogemia, hypertriglyceridemia and a decrease in the erythrocyte sedimentation rate, unlike SLE. HS treatment when associated to SLE is not well established, but steroids and/or immunoglobulins are effective as the initial treatment, and in refractory cases, cyclosporine or cyclophosphamide may be associated.
HS can be the initial manifestation of SLE and should be suspected in patients with organ enlargement, cytopenias, clotting disorders, liver disorders and prolonged fever unresponsive to antibiotics. Anakinra may be a treatment option in adult HS associated to SLE.
噬血细胞综合征（HS）发生于自身免疫性疾病，属于噬血细胞性淋巴组织细胞增生症疾病组。本文描述了2例以HS为首发临床表现的系统性红斑狼疮（SLE）患者的特征。
两名患者均有与感染过程无关的长期发热，且对广谱抗生素无反应。
继发于SLE的HS诊断较为复杂，因为它有一些共同特征，但与SLE不同的是，HS的特征为高铁蛋白血症、低纤维蛋白原血症、高甘油三酯血症以及红细胞沉降率降低。HS与SLE相关时的治疗方法尚未完全确立，但类固醇和/或免疫球蛋白作为初始治疗有效，在难治性病例中，可联合使用环孢素或环磷酰胺。
HS可能是SLE的首发表现，对于器官肿大、血细胞减少、凝血障碍、肝脏疾病以及对抗生素无反应的长期发热患者应怀疑HS。阿那白滞素可能是成人SLE相关HS的一种治疗选择。
