紅斑性狼瘡併發瀰慢性肺泡出血：一家醫學中心之回溯性研究

Diffuse alveolar hemorrhage in systemic lupus erythematosus: a retrospective study in a single center in Taiwan

目的：研究目的是探討紅斑性狼瘡病人併發瀰漫性肺泡出血之臨床表徵及治療結果。方法：此為一項回溯性研究，自1992年10月至2008年6月，收集紅斑性狼瘡併發瀰漫性肺泡出血的病人臨床資料，分析其臨床表徵、檢查結果、SLEDAI分數、治療種類及治療結果，並且探討影響預後之因素。結果：共19名紅斑性狼瘡病人總共併發24次瀰漫性肺泡出血，其中5名病人共發生兩次併發瀰漫性肺泡出血。喘是最常見的症狀（100%），其次為咳血症狀（84.2%），而狼瘡性腎炎為最常見的肺外症狀（84.2%）。發生發瀰漫性肺泡出血時之SLEDAI分數平均偏高（20.9分），死亡率高達79％。本研究未能指出具統計意義之預後因素，但是五位復發病人其中有四位合併感染，且五位病人均死亡。結論：紅斑性狼瘡病人併發瀰漫性肺泡出血具有極高的死亡率。針對併有狼瘡性腎炎或高SLEDAI分數的病人，需高度警覺並且給予及時積極的治療。

Objective: To investigate the clinical features and outcomes of systemic lupus erythematosus (SLE) patients with diffuse alveolar hemorrhage (DAH). Methods: We retrospectively obtained clinical data, investigated the clinical manifestations, laboratory data, SLE disease activity index(SLEDAI) score in SLE patients with DAH from October 1992 to June 2008. In addition, we examined the prognostic factors for survival of these patients. Results: A total of 24 episodes in 19 SLE patients with DAH were included. Among them, fi ve patients experienced 2 episodes of DAH. Dyspnea was the most common symptom (100%), and hemoptysis occurred in 16 patients (84.2%). Lupus nephritis was the most common extra-pulmonary manifestation (84.2%). High SLEDAI score was noted at the onset of DAH (mean SLEDAI = 20.9). Mortality rate was high (79%). No signifi cant prognostic factors could be demonstrated in this study. However, 4 out of 5 patients with recurrent DAH were associated with infection and all of them succumbed. Conclusions: Due to high mortality rate of DAH in SLE patients, high suspicion of this rare entity in patients with lupus nephritis or high SLEDAI is important, and a prompt and appropriate treatment is mandatory.