狼瘡腎炎組織病理學特徵：臺灣單一醫學中心經驗

Histopathological Characteristics of Lupus Nephritis: A 10-year Single Center Experience in Taiwan

目的：狼瘡腎炎（LN）患者間的臨床表徵及腎臟病理結果變化甚大。在此我們回顧性分析臺灣單一醫學中心狼瘡腎炎患者之腎臟組織病理變化。方法：依據2003年ISN／RPS分類，我們分析了自2006年至2017年間接受腎臟切片的164位狼瘡腎炎患者之腎炎分型、急性活性指數（activity index, AI）以及慢性活性指數（chronicity index, CI），並比較各分型間臨床特徵。結果：164位狼瘡腎炎患者，年齡中位數為30.4歲。當中有138例（84.1%）為女性；91例（55.5%）為第四型，23例（14.0%）為第五型，19例（11.6%）為第三型，14例（8.5%）為第三型加第五型，13例（7.9%）為第四型加第五型，第二型有4例（2.4%）。細胞性新月體（cellular crescents）以及壞死性病灶（necrotizing lesions）在第四型LN最常見。第四型LN的AI最高，第四型加上第五型的狼瘡腎炎患者之蛋白尿值高過其餘分型。結論：第四型狼瘡腎炎不僅是最多見，亦是最嚴重的一個分型，需要即時的治療。

Objective: The clinical features and pathologic patterns of Lupus Nephritis (LN) vary widely. Accurate treatment, according to the pathologic findings of renal biopsies from patients with lupus nephritis is crucial. We have retrospectively reviewed renal biopsies from LN patients in a tertiary referral center. Methods: A retrospective observational study which enrolled 164 biopsy-proven LN patients between 2006 and 2017 was conducted. The distribution of histological classes according to the ISN/RPS classification, as well as the clinical features of each LN patient, were described. Results: The median (interquartile range, IQR) age of the enrolled subjects was 30.4 (24.1 - 39.0) years; with 138 (84.1%) being female. Ninety-one (55.5%) participants were categorized as Class IV LN, followed by 23 (14.0%) as Class V, 19 (11.6%) as Class III, 14 (8.5%) as Class III+V, 13 (7.9%) as Class IV+V and 4 (2.4%) as Class II LN. Cellular crescents and necrotizing lesions were most frequently observed amongst the Class IV LN patients; where 41 (46.6%) had cellular crescents, accounting for 87.2% of crescentic LN in this study. Patients with Class IV+V lupus nephritis had the highest levels of proteinuria when compared with their counterparts. Subjects with Class IV LN scored the highest activity index. Conclusions: We concluded that Class IV was the most common and severe form of LN. Patients with Class IV (with or without V) tended to have severer proteinuria, higher anti-double-stranded DNA levels, and lower levels of serum complements.