全身性紅斑狼瘡合併非栓塞性血小板低下紫斑症之血栓性微血管病變的臨床特徵、治療及預後：台灣單一中心的照護經驗

Clinical Characteristics and Treatment Outcome of non-Thrombotic Thrombocytopenic Purpura Thrombotic Microangiopathy in Systemic Lupus Erythematosus: a Single Medical Center Experience in Taiwan

目的：非栓塞性血小板低下紫斑症之血栓性微血管病變是全身性紅斑狼瘡患者罕見及致命的併發症，從單一醫療中心資料收集分析此類病人特徵、診斷、治療與預後。
方法：我們回顧2016年至2024年6月間來自台北榮民總醫院敏免疫風濕科之患者資料，收納10名確診為全身性紅斑狼瘡病人合併出現血栓性微血管病變且都排除栓塞性血小板低下紫斑症的病患，紀錄患者的於診斷血栓性微血管病變當時的臨床症狀與診斷相關（如微小血管性溶血性貧血、血小板低下及器官損傷等）檢驗數值，接受的治療與追蹤6個後的腎臟預後（與確定診斷時的血清肌酸酐比較改善≥25%）及血小板數及LDH的恢復狀況。
結果：所納入的10位全身性紅斑狼瘡併血栓性微血管病變患者病人皆出現急性腎衰竭。一旦符合血栓性微血管病變的診斷後皆接受血漿置換術（平均10.6次,範圍界於5-20次），皆使用中等（約7.5-30毫克prednisolone/天）至脈衝（≥250毫克prednisolone/天）類固醇治療，基於當下全身性紅斑狼瘡發作活性及感染調整使用免疫抑制劑治療。治療後追蹤6個月，有40%病人腎功能改善達診斷時的血清肌酸酐減少25%以上，有30%的病人血小板增加至正常數量（大於或等於15萬/mm3），30%的病人的LDH下降至正常範圍。總共有20%病人血小板及LDH皆回復正常，同時也達到腎功能改善肌酸酐減少25%以上。有20%的病人於治療後追蹤期間死亡。
結論：全身性紅斑狼瘡合併非栓塞性血小板低下紫斑症之血栓性微血管病變是罕見及致命的疾病，需要早期懷疑，盡早安排適當的檢查及治療。目前本土相關病例報告不多，我們收集10個此類病人，整理治療及預後，發現積極治療病人本身的免疫疾病及誘發因子（如經歷補體增強事件），小心控制感染是重要的照顧原則。

Objectives: Non-thrombotic thrombocytopenic purpura thrombotic microangiopathy (non-TTP TMA) is a rare and life-threatening complication of systemic lupus erythematosus (SLE). This study investigated the clinical features and treatment outcomes of non-TTP TMA secondary to SLE at a single medical center in Taiwan.
Methods: We retrospectively collected 10 patients with non-TTP TMA and SLE admitted to Taipei Veterans General Hospital between 2016 and 2024. Patient demographic information, TMA features at TMA diagnosis (microangiopathic hemolytic anemia, thrombocytopenia, and end-organ damage), treatment, and outcomes including renal remission (≥25% improvement in serum creatinine from baseline), hematologic remission (platelet count and lactate dehydrogenase (LDH) normalization), and mortality were analyzed.
Results: All ten TMA patients had acute renal injury and underwent plasma exchange (mean 10.6 times, range 5-20 times), medium dose corticosteroids or pulse therapy, and immunosuppressants. After treatment for 26 weeks, 40% of the patients achieved renal remission (≥25% improvement in serum creatinine from baseline), platelet count normalized in 30% , LDH levels normalized in 30%, and 20% achieved both LDH and platelet normalization. The overall mortality rate was 20%.
Conclusion: SLE-associated non-TTP TMA is rare, but potentially life-threatening. Early suspicion of the condition, investigation, and prompt treatment of fatal disease are critical. It is important to treat the underlying disease and precipitating factors (complement-amplifying conditions) to control the infection intensively and carefully.