Costa Andreia, Pais Cármen, Cerqueira Sofia, Salvador Fernando
Serviço de Medicina Interna. Centro Hospitalar de Trás-os-Montes e Alto Douro. Vila Real. Portugal.
Acta Med Port. 2018 Oct 31;31(10):593-596. doi: 10.20344/amp.9028.
Systemic lupus erythematosus is a heterogeneous and unpredictable autoimmune disease which can be complicated to approach and treat. Hemophagocytic lymphohistiocytosis and diffuse alveolar hemorrhage are rare disease complications. The authors describe a clinical case of a 32-year-old woman with lupus and fever of unknown origin. From the investigations performed, the myelogram revealed hemophagocytosis and Leishmania parasites, therefore liposomal amphotericin B was then started. In addition to directed therapy, she maintained fever that evolved with diffuse alveolar hemorrhage. The myelogram was repeated and showed that she still had hemophagocytosis but now without parasites. Corticotherapy was increased and intravenous Immunoglobulin was started, with improvement. Rituximab was started as a result of macrophage activation syndrome and diffuse alveolar hemorrhage. Months after discharge, she began once again to have sustained fever and Leishmania parasites were found again, therefore liposomal amphotericin B was started once more associated with miltefosine. She continues being followed-up as she is asymptomatic and using steroidsin weaning scheme.
系统性红斑狼疮是一种异质性且难以预测的自身免疫性疾病，其诊治过程可能较为复杂。噬血细胞性淋巴组织细胞增生症和弥漫性肺泡出血是罕见的疾病并发症。作者描述了一名32岁狼疮伴不明原因发热女性的临床病例。从所进行的检查来看，骨髓检查显示存在噬血细胞现象和利什曼原虫，因此开始使用脂质体两性霉素B进行治疗。除了针对性治疗外，她仍持续发热，并出现了弥漫性肺泡出血。再次进行骨髓检查显示仍有噬血细胞现象，但此时没有寄生虫。增加了皮质激素治疗并开始静脉注射免疫球蛋白，病情有所改善。由于巨噬细胞活化综合征和弥漫性肺泡出血开始使用利妥昔单抗。出院数月后，她再次出现持续发热，再次发现利什曼原虫，因此再次开始使用脂质体两性霉素B并联合米替福新治疗。她目前仍在接受随访，无症状且正在按照减停激素方案使用激素。
