系統性紅斑狼瘡併瀰漫性肺泡出血以血漿置換術作為救援性治療：一家醫學中心的28例病患經驗

Plasma exchange as the rescuing therapy for critical diffuse alveolar hemorrhage in systemic lupus erythematosus: experiences of 28 patients in one center

目的：探研究在瀰漫性肺泡出血的系統性紅斑狼瘡患者使用血漿置換的角色。方法：我們對於在1985年2月及2012年1月之間系統性紅斑性狼瘡合併瀰漫性肺泡出血之病患，因危急情況而接受救援性血漿置換術，進行回顧性的分析。結果：我們的研究共包括28例瀰漫性肺泡出血的系統性紅斑狼瘡患者，其中22位病患接受血漿置換術，另外6位病患未接受血漿置換術。血漿置換術總共進行189次，每個病患每次療程的中位數為七次（範圍為2-33）。期間共有四位患者於治療期間死亡：其中一位因敗血症而死亡，一位因瀰漫性肺泡出血併急性呼吸衰竭，其他兩位併發心臟血栓而造成循環衰竭。而其接受血漿置換術的病患5年存活率為較未接受者好（分別為64％及33%）。治療期間沒有病患產生過敏性休克。且部分患者同時接受類固醇脈衝治療（1500-6000毫克）及血漿置換術。在所有療程中，平均系統性紅斑性狼瘡的疾病活動指數分別為在接受救援性血漿置換術的24.9在及在接受完血漿置換術3週後的9.1。結論：我們的資料顯示比較起使用救援性血漿置換術及同時併用或未併用類固醇脈衝治療，在系統性紅斑性狼瘡併危急瀰漫性肺泡出血病患未使用血漿置換術有較高的死亡率。

Objective: To determine the role of plasma exchange (PE) in patients with systemic lupus erythematosus (SLE) with diffuse alveolar hemorrhage (DAH). Methods: We performed a retrospective analysis to evaluate patients in critical condition with SLE with DAH who underwent rescue plasma exchange (RPE) between February 1985 and January 2012. Results: Our study included 28 patients with SLE with DAH. Twenty-two patients received PE with or without pulse methylprednisolone therapy as rescue therapies and other six patients did not undergo the PE. Overall PEs were performed 189 sessions, with a median of seven sessions for each patient per course (range, 2-33). Four of these patients died at therapies: one of septicemia, one of DAH with acute respiratory failure, and two of cardiac thrombosis with circulation failure. The overall 5-year survival rate was better in patients receiving RPE (64% vs. 33%). No patient developed anaphylaxis. Patients received methylprednisolone pulse therapy (1500-6000 mg) simultaneously with or separately from RPE. In all courses, we evaluated the disease activity with the Systemic Lupus Erythematosus Disease Activity Index (SLEDAI) and the mean SLEDAI scores were 24.9 and 9.1 before and 3 weeks after RPE, respectively. Conclusions: Our data suggest that the patients with SLE in critical condition of DAH not receiving PE may have a higher immediate mortality rate in comparison with those receiving PE with or without concomitant pulse methylprednisolone therapy.