新月体比例在Ⅳ型狼疮性肾炎患者中的临床意义

Significance of crescent formation in patients with diffuse proliferative lupus nephritis

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。

Objectlve：Crescentic nephritis is not rare in diffuse proliferative lupus nephritis （DPLN）. However, little is known about the clinicopathological features in DPLN with crescents worldwide. This study was undertaken to investigate the clinicopathological features and outcome of Chinese DPLN patients with different degree of crescents. Methodology：Five hundred and twenty DPLN patients with more than 10% histological crescents （cDPLN） were enrolled in this retrospective study. They were divided into three groups： group 1 （10% ≤ Crescent% 〈25, n = 240）, group 2 （25% ≤ Crescent%〈50, n = 160） , and group 3 （ Crescent% ≥ 50, n = 120）. Another 100 patients without histological crescents were regarded as a control group. Their clinicopathologieal features, treatment response and outcome were compared among four groups. Results：They were 450 （ 86. 6% ） females and 70 （ 13.4% ） males with an average age of 31.7± 11.4 ys. Compared with controls, cDPLN patients had shorter LN duration （20. 7±34. 1） vs （30. 4±48. 9） mths, higher prevalence of RPGN （ 21.8% ） , and gross hematuria （ 26. 7% ）. Laboratory findings indicated more severe hypo-proteinemia, hyperlipoidemia, and renal insufficiency, heavier proteinuria and microscopic hematuria, higher levels of tubular injury parameters and lower serological activity in crescentic groups. Histologically, cDPLN patients had severe glomerular and tubulointerstitial lesions, extensive leukocyte infiltration, together with a lesser degree of immune complex deposition. Crescent ratio correlated positively with the ratio of serositis, hypertension, anemia, gross hematuria, positive ANCA, cappilary necrosis, the levels of blood urea nitrogen, creatinine, complement C3, C4, urinary NAG enzyme, RBP enzyme and AI, CI score, while correlated negatively with age, LN duration, SLE duration and the proportion of histological platinum ear phenomenon. Conclusion： cDPLN patients with acute onset and short disease duration mostly showed severe renal manifestations, less extra-renal organ involvement, lower serological activity, serious capillary necrosis, severe tubulointerstitial inflammation, atrophy and fibrosis, prominent leukocyte infiltration and less glomerular immune complex deposition.