Shah Ami A, Higgins John P, Chakravarty Eliza F
Division of Rheumatology, Department of Medicine, at the Johns Hopkins University School of Medicine, Baltimore, MD, USA.
Nat Clin Pract Rheumatol. 2007 Jun;3(6):357-62. doi: 10.1038/ncprheum0511.
A 19-year-old woman with newly diagnosed systemic lupus erythematosus (SLE) presented with hemolytic anemia, thrombocytopenia, hypertension, tonic-clonic seizures, blurry vision, nephrotic syndrome and renal insufficiency.
At a general hospital, the investigations included brain MRI, echocardiography, laboratory tests including measurement of the amount of protein excreted daily, platelet count, levels of lactate dehydrogenase, creatinine and anticardiolipin antibodies, direct Coombs' test, peripheral blood smear, and measurement of blood pressure. At a tertiary institution the investigations included physical examination, electroencephalography, brain MRI, magnetic resonance angiography, repetition of laboratory tests plus measurement of von Willebrand factor-cleaving protease activity, measurement of levels of antibodies to double-stranded DNA and platelets, and renal biopsy.
Thrombotic microangiopathic hemolytic anemia with a possible underlying diagnosis of malignant hypertension, antiphospholipid antibody syndrome, catastrophic antiphospholipid antibody syndrome, thrombotic thrombocytopenic purpura, or active SLE.
At the general hospital, therapy included a single dose of intravenous cyclophosphamide 500 mg, eight daily plasma exchange treatments, three daily infusions of methylprednisolone 1 g followed by methylprednisolone 60 mg every 8 h, an infusion of rituximab 657 mg and ultrafiltration via hemodialysis. At the tertiary institution, therapy included an infusion of cyclophosphamide 650 mg, aspirin 81 mg daily, prednisone 40 mg daily, mycophenolate mofetil 750 mg twice daily, and aggressive management of hypertension.
一名新诊断为系统性红斑狼疮（SLE）的19岁女性，出现溶血性贫血、血小板减少、高血压、强直阵挛性发作、视力模糊、肾病综合征和肾功能不全。
在一家综合医院，检查包括脑部MRI、超声心动图、实验室检查，包括每日蛋白排泄量测定、血小板计数、乳酸脱氢酶、肌酐和抗心磷脂抗体水平、直接抗人球蛋白试验、外周血涂片以及血压测量。在一家三级医疗机构，检查包括体格检查、脑电图、脑部MRI、磁共振血管造影、重复实验室检查以及血管性血友病因子裂解蛋白酶活性测定、双链DNA抗体和血小板水平测定，以及肾活检。
血栓性微血管病性溶血性贫血，可能的潜在诊断为恶性高血压、抗磷脂抗体综合征、灾难性抗磷脂抗体综合征、血栓性血小板减少性紫癜或活动性SLE。
在综合医院，治疗包括单次静脉注射环磷酰胺500mg、每日8次血浆置换治疗、每日3次静脉注射甲泼尼龙1g，随后每8小时注射甲泼尼龙60mg、静脉注射利妥昔单抗657mg以及通过血液透析进行超滤。在三级医疗机构，治疗包括静脉注射环磷酰胺650mg、每日阿司匹林81mg、每日泼尼松40mg、每日两次霉酚酸酯750mg，以及积极控制高血压。
