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目的:探讨新月体比例在Ⅳ型狼疮性肾炎(LN)患者中的临床意义。方法:回顾性分析南京军区南京总医院肾脏科2000年1月至2010年12月经肾活检证实新月体比例≥10%的Ⅳ型LN患者520例,根据光镜下肾小球中新月体形成的百分比将患者分为三组,组1:新月体比例≥10%且<25%(n=240);组2:新月体比例≥25%且<50%(n=160);组3:新月体比例≥50%(n=120),以100例无新月体形成的Ⅳ型LN患者为对照组比较四组间临床、实验室检查和病理特点。结果:520例患者中男性70例(13.4%),女性450例(86.6%),其平均年龄31.6±11.3岁,平均发病年龄28.8±11.1岁,SLE病程34.5±42.9月,LN病程20.7±34.1月。与对照组相比,新月体组患者病程更短,临床主要表现急进性肾炎综合征(21.8%)、肾病综合征(23.4%)、发作性肉眼血尿(26.7%)和慢性肾衰(7.9%),蛋白尿,镜下血尿和小管损伤指标更重,低蛋白血症,高胆固醇血症更常见,补体水平、ANA、ds-DNA滴度更低,狼疮抗凝物(LA)和抗中性粒细胞胞质抗体(ANCA)阳性率更高。新月体组患者,免疫荧光可见肾小球免疫复合物沉积较弱。光镜下球性废弃及袢坏死比例高,白金耳和毛细血管袢微血栓较少见,肾小管间质(急性/慢性病变)和血管病变重,间质广泛炎细胞浸润。肾外表现如贫血和高血压发生率高,而血小板减低、白细胞减少和肾外器官受累发生率低。新月体比例与浆膜炎发生率、高血压比例、贫血、肉眼血尿发生率、ANCA阳性率、袢坏死比例、尿素氮、肌酐水平、补体C3及C4水平、尿N-乙酰-β-D-氨基葡萄糖苷酶、视黄醇结合蛋白、活动指数(AI)和慢性指数(CI)评分成正相关,与年龄、LN病程、SLE病程和白金耳比例成负相关。结论:Ⅳ型LN合并新月体形成的患者多起病急、病程短,肾脏表现较重,肾外器官受累较轻,免疫学活动性较低,病理上肾小球毛细血管袢坏死、肾小管萎缩、间质炎细胞浸润、纤维化突出,肾小球免疫复合物沉积较少。
Objective: To investigate the clinical significance of crescent proportion in type Ⅳ lupus nephritis (LN). Methods: A total of 520 patients with type Ⅳ LN confirmed by renal biopsy from January 2000 to December 2010 in Nanjing General Hospital of Nanjing Military Command were retrospectively analyzed. According to the light microscopy, Percentage formed Patients were divided into three groups, Group 1: crescent proportions ≧ 10% and <25% (n = 240); Group 2: crescent proportions ≧ 25% and <50% (n = 160); Group 3: The proportion of crescent was ≥50% (n = 120). The clinical, laboratory and pathological features were compared among the four groups of type Ⅳ LN patients with no crescent formation. Results: Among 520 patients, 70 (13.4%) were male and 450 (86.6%) were female, with an average age of 31.6 ± 11.3 years and an average age of 28.8 ± 11.1 years. SLE duration was 34.5 ± 42.9 months and LN duration was 20.7 ± 34.1 month. Compared with the control group, the patients with crescentic disease had a shorter duration of disease. The clinical manifestations were mainly acute nephritic syndrome (21.8%), nephrotic syndrome (23.4%), fulminant gross hematuria (26.7%) and chronic renal failure %), Proteinuria, microscopic haematuria and tubule damage indicators were more severe, hypoproteinemia, hypercholesterolemia were more common, complement levels, ANA, ds-DNA titers lower, lupus anticoagulant (LA) Neutrophil cytoplasmic antibodies (ANCA) positive rate higher. Crescent-body patients, immunofluorescence showed glomerular immune complex deposition is weak. Light microscopic ball abandonment and necrosis a high proportion of platinum ear and capillaries microthrombus less common, tubulointerstitial (acute / chronic lesions) and vascular disease, interstitial inflammatory cell infiltration. Extrarenal manifestations such as anemia and high incidence of hypertension, and thrombocytopenia, leukopenia and low incidence of extra-renal organ involvement. Crescent ratio and serosal inflammation, the proportion of hypertension, anemia, gross hematuria, ANCA positive rate, necrosis rate, urea nitrogen, creatinine levels, complement C3 and C4 levels, urine N-acetyl-β-D - glucosaminidase, retinol binding protein, activity index (AI) and chronic index (CI) score was positively correlated with age, LN duration, SLE duration and platinum ear ratio was negatively correlated. CONCLUSION: Patients with type IV LN combined with crescent formation are characterized by acute onset, short course of disease, severe renal manifestations, mild extrarenal organ involvement, low immunological activity, necrosis of glomerular capillaries in pathology, Tubule atrophy, interstitial inflammatory cell infiltration, prominent fibrosis, glomerular immune complex deposition less.