全身性紅斑狼瘡併狼瘡腎炎及栓塞性腎小血管病變：一病例報告

Systemic Lupus Erythematosus with Lupus Nephritis and Renal Thrombotic Microangiopathy: A Case Report

栓塞性腎小血管病變在全身性紅斑狼瘡的併發症中相當罕見，加上全身性紅斑狼瘡的臨床症狀及病理變化和栓塞性腎小血管病變又很類似，診斷上具有相當的挑戰性。謹慎的判斷在栓塞性腎小血管病變的潛在病因，對治療的選擇及預後的評估有決定性的影響。這位五十二歲的亞洲女性是全身性紅斑狼瘡的患者，因高血壓惡化、腎功能異常及蛋白尿至本科求診。腎臟切片結果顯示第四型狼瘡腎炎及栓塞性腎小血管病變。這種臨床及病理表現在文獻上並不常見。這位病人對以類固醇及細胞毒殺藥物為主的脈衝治療加上抗凝血藥物使用反應良好。

Renal thrombotic microangiopathy (TMA) is an uncommon vascular complication of systemic lupus erythematosus (SLE). The diagnosis of TMA in a setting of SLE is challenging since both share common features of clinical appearance and pathology. Carefully tracing the cause of TMA is mandatory for the therapeutic plans and prognostic evaluation of this renal vasculopathy in SLE. We present a case of a 52 year-old Asian lady with SLE who developed poorly controlled hypertension, deteriorating renal function and proteinuria. The renal biopsy was compatible with lupus nephritis, class IVa, and TMA, which was rarely seen in the literature. She responded well to pulse therapy with methylprednisolone and cyclophosphamide and the administration of warfarin.