以蛋白質流失腸病變為初始表現的全身性紅斑狼瘡－－病案報告及文獻回顧

Protein-Losing Enteropathy as an Initial Manifestation of a Lupus Patient-- a Case report and Review of Literatures

有許多疾病可造成蛋白質流失腸病變，膠原病即為其中之一。不過以此為初始表現的全身性紅斑狼瘡，國外的文獻報告不多，國內則未有發現。一位二十四歲的女孩因一年的全身水腫及低蛋白血症住進臺大醫院，經二十四小時糞便α 1-antitrypsin廓清試驗證實蛋白確由腸道流失，內視鏡生檢顯示有腸道淋巴管擴張。全身性紅斑狼瘡的一些臨床表徵，如毛髮脫落、皮膚紅疹、漿膜炎及雷諾氏現象，於隨後出現。住院中並發生腦梗塞，其ANA及anti-dsDNA昇高，白血球降低，但抗燐脂抗體為陰性。雖然經過類固醇治療一度好轉，病人仍因併發反覆性肺炎及氣胸而於四個月後死於呼吸衰竭。

A 24-year-old woman presented anasarca and low serum protein for 1 year. Protein-losing enteropathy was confirmed by increased clearance of fecal α 1-antitrypsin as well as normal renal and hepatic functions. Clinical Manifestations of systemic lupus erythematosus appeared later, including alopecia, skin rash, serositis, and Raynaud's phenomenon. Laboratory examinations showed high ANA and anti-dsDNA titers, leukopenia but negative anticardiolipin antibodies. In spite of corticosteroid treatment, she developed cerebral infarction and recurrent hydropneumothorax, and expired 4 months later after admission. The association of proteinlosing enteropathy and systemic lupus erythematosus is discussed with literature reviews.