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An unexpected diagnostic course of systemic lupus erythematosus

机译:系统性红斑狼疮的意外诊断过程

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摘要

Thrombotic microangiopathy (TM), especially thrombotic thrombocytopenic purpura (TTP) is described in systemic lupus erythematosus (SLE) as a severe hematological involvement. However hemolytic uremic syndrome (HUS) is seen less frequently in SLE, particularly as an initial presentation. Here we present a 15-year old boy presenting with gross hematuria, decreased urinary output and petechial lesions. He was diagnosed as atypical HUS according to the classical triad of TM, along with observation of hypocomplementemia and negative stool cultures. In addition, his symptoms fulfilled the 2012 revised criteria for the classification of SLE. He was treated with plasma infusions and methylprednisolone/prednisone. At follow up his laboratory findings and general condition improved and no relapse was seen.
机译:血栓性微血管病(TM),特别是血栓性血小板减少性紫癜(TTP)在系统性红斑狼疮(SLE)中被描述为严重的血液学累及。然而,溶血性尿毒症综合征(HUS)在SLE中的出现频率较低,特别是作为初始表现。在这里,我们介绍了一个15岁的男孩,表现为肉眼血尿,尿量减少和皮疹。根据TM的经典三联征,他被诊断为非典型HUS,同时观察到补体不足和粪便培养阴性。此外,他的症状符合2012年修订的SLE分类标准。他接受血浆输注和甲基强的松龙/泼尼松治疗。随访时,他的实验室检查结果和总体状况有所改善,未见复发。

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