南台灣兒童及青少年的系統性紅斑狼瘡在臨床表現與實驗室的特徵

Clinical and Laboratory Features of Juvenile-onset Systemic Lupus Erythematosus in Southern Taiwan

系統性紅斑狼瘡可能會有各種非專一性的症狀表現，在孩童時期的病徵常容易與其他疾病混淆，以至於易被誤診或延遲診斷。我們的研究收集高雄榮總過去十二年所遇到的新病例，包含在最初診斷時的臨床表現與實驗室數據。這份研究共包含了八十九名女性與十一名男性。女性與男性的比例為8.1比1。全體病人的平均年齡為十四點四歲，在我們的研究中，最常見兒童與青少年的臨床表徵為腎臟的侵犯（60%）。關節炎則在37%的病人上呈現，而皮膚炎及黏膜侵犯則可在48%的病人身上發現，中樞神經侵犯則有11%，另外有38%的病人一開始會以發燒來表現。實驗室數據的異常，包含在所有病人中有93.4%病人抗核抗體（ANA）呈陽性反應，而抗雙股去氧核糖核酸抗體則有81.9%為陽性。有25個病人有作抗磷脂質抗體，其中十二個病人為陽性（48%），此外在血液學表現中，貧血較為常見共有40%。我們的結果也顯示了，青少年開始的系統性紅斑狼瘡較成年才開始的症狀表現，有較多的腎臟侵犯而較少的面部紅班。

Systemic Lupus Erythematosus (SLE), which may exhibit different clinical manifestations, is easily misdiagnosed as other disease at first visit especially with children. We collected 100 SLE patients with disease onset under 20 years old and analyzed the clinical and laboratory features of these patients. Demographic data reveals that the female to male ratio is 8.1:1 (89 females and 11 males), with an average disease onset of 14.4+3.6 year-old. The percentage of different organ involvements in these patients is 60% of nephropathy, 37% of arthritis, 48% of mucocutaneous lesions, 11% of CNS involvements, and 38% of fever. Laboratory abnormalities include ANA 93.4%, anti-ds DNA antibody 81.9%, anti-cardiolipin antibody 48% and 40% of anemia. These results indicate that Juvenile-onset SLE have more renal involvement but less malar skin rashes than adult-onset.