紅斑性狼瘡併發頑固性乳糜胸－－一病例報告

Refractory chylothorax in a patient with systemic lupus erythematosus: case report

我們報告一位系統性紅斑狼瘡引起兩側乳糜胸少見案例。病人21歲女性罹患狼瘡已有六年一直控制良好，此次在體檢中發現無症狀之兩側胸肋腔積水。物理檢查僅發現兩側呼吸音減少及輕微兩頰紅斑。胸部X光呈現兩側肺肋積水，肋穿刺抽出牛乳狀液體。分析成分細胞主要為淋巴細胞（左邊93%，右邊81%）、膽固醇、三酸甘油脂以及蛋白質。抽出液檢測ANA陽性，細菌暨抗酸菌陰性。肋膜切片無癌細胞及結核菌。淋巴攝影檢查在胸腔及腹腔無淋巴漏液。最後診斷為系統性紅斑狼瘡相關之乳糜胸。本案例以高劑量類固醇及賽得星治療皆無明顯效果，治療12月之後仍有明顯雙側乳糜胸但無任何臨床症狀。

We describe a rare case of SLE-related chylothorax. A 21 year-old woman with a six-year history of SLE suffered a silent bilateral pleural effusion. On physical examination, diminished breathing sounds over the bilateral lung fields and a malar rash were noted. Chest radiography revealed bilateral massive pleural effusion. Thoracocentesis was performed and an odorless milky fluid from each side was aspirated. These fluids contained predominantly mature lymphocytes (93% for the left pleural cavity, 81% for the right pleural cavity) and no evidence of malignancy. The major compositions of the left and right pleural fluid (respectively) were cholesterol, triglycerides, and total protein. Pleural fluid tested positive for ANAs. There was no evidence of microorganisms or mycobacteria. A pleural biopsy was negative for malignancy or tuberculosis. Bipedal lymphangiography showed no evidence of lymphatic leak in the thoracic or peritoneal cavities. This presentation is most compatible with a diagnosis of lupus-related chylous pleural effusion. Our case was resistant to hydrocortisone and cyclophosphamide treatment. The chylothorax did not resolve 12 months later after initial presentation.