Abudiab Muaz, Krause Megan L, Fidler Mary E, Nath Karl A, Norby Suzanne M
Clin Nephrol. 2013 Oct;80(4):293-7. doi: 10.5414/CN107465.
Thrombotic thrombocytopenic purpura (TTP), hemolytic uremic syndrome (HUS), and scleroderma renal crisis (SRC) all present with features of thrombotic microangiopathy. Distinguishing among these entities is critical, however, as treatments differ and may be mutually exclusive. We describe the case of a 25-year-old woman with an undefined mixed connective tissue disease who presented 6 weeks post-partum with fever, transient aphasia, thrombocytopenia, hemolytic anemia, and acute kidney injury eventually requiring initiation of hemodialysis. Renal biopsy revealed thrombotic microangiopathy. Renal function did not improve despite immediate initiation of plasma exchange, and an angiotensin-converting enzyme (ACE) inhibitor was initiated following discontinuation of plasma exchange. At last follow up, she remained dialysis dependent. Due to the myriad causes of thrombotic microangiopathy and potential for diagnostic uncertainty, the patient's response to therapy should be closely monitored and used to guide modification of therapy.
血栓性血小板减少性紫癜（TTP）、溶血性尿毒症综合征（HUS）和硬皮病肾危象（SRC）均表现为血栓性微血管病的特征。然而，区分这些疾病至关重要，因为治疗方法不同且可能相互排斥。我们描述了一名25岁患有未明确混合性结缔组织病的女性病例，她产后6周出现发热、短暂性失语、血小板减少、溶血性贫血和急性肾损伤，最终需要开始血液透析。肾活检显示血栓性微血管病。尽管立即开始血浆置换，但肾功能并未改善，在停止血浆置换后开始使用血管紧张素转换酶（ACE）抑制剂。在最后一次随访时，她仍依赖透析。由于血栓性微血管病的病因众多且存在诊断不确定性，应密切监测患者对治疗的反应并用于指导治疗调整。
