血漿交換術合併脈衝式環磷醯胺靜脈注射治療在全身性紅斑性狼瘡併血栓性小血管病變：一病例報告暨文獻回顧

Combination Therapy of Plasma Exchange and Intravenous Cyclophosphamide in a Case with Thrombotic Microangiopathy and Systemic Lupus Erythematosus: A Case Report and Literature Review

一位17歲全身性紅斑性狼瘡的（SLE）女性因關節炎、腎功能異常和嚴重貧血而被診斷疾病復發而住院。住院期間，病患對高劑量類固醇反應不佳。隨後，血液抹片檢查顯示小血管病變性溶血性貧血（microangiopathic hemolytic anemia）、血小板減少、腎功能不全，但並沒有神經學異常和發燒的情況。因此，在這些情況下被診斷為全身紅斑性狼瘡復發併血栓性小血管病變（thrombotic microangiopathy）。之後，病人接受血漿交換（plasma exchange）和脈衝式環磷醯胺（cycJophsphamide）靜脈注射治療，最後，病況穩定出院。血栓性小血管病變很少發生在全身性紅斑性狼瘡病人身上。不過，二者的臨床表現卻很相似而易誤診。此外，血栓性小血管病變對血漿交換治療效果不錯，但全身性紅斑性狼瘡則是以類固醇和免疫抑制藥物治療為主。在全身性紅斑性狼瘡合併血栓性小血管病變的病人，適當的治療尚未有定論，我們藉此提出這個成功接受血漿交換和免疫抑制劑治療的經驗。

A 17-year-old girl was admitted due to systemic lupus erythematosus (SLE) with disease flare with arthritis, renal dysfunction and abnormal hemogram. The patient responded poorly to high-dose steroid therapy. Although the patient did not have neurological symptoms or fever, laboratory tests identified microangiopathic hemolytic anemia, thrombocytopenia, and renal impairment. Therefore, SLE with disease flare complicated by thrombotic microangiopathy (TMA) was diagnosed. The patient later responded to therapy with aggressive plasma exchange and cytotoxic agents. TMA is a rare hematological life-threatening disorder that can coexist with SLE. Manifestations of TMA are similar to those in SLE and, consequently, TMA can be misdiagnosed as SLE flare. Additionally, TMA has good response to plasma exchange and SLE is typically well controlled with steroids or cytotoxic agents. There is no documented therapy for patients with SLE and TMA. Therefore this report describes a lupus patient with disease flare combined with TMA who was successfully treated with combination therapy of plasma exchange and intravenous cyclophosphamide.