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目的 了解狼疮新月体肾炎 (CLN)患者临床和病理特征。方法 对 38例CLN的临床、血清学和病理改变进行分析归纳。并将肾功能异常和肾功能正常的CLN患者进行比较。结果 CLN患者均为女性。6 3 2 %临床表现为急进性肾炎综合症 ;5 2 %表现为慢性肾功能减退 ;31 6 %患者肾功能正常 ,临床表现为肾病综合征或慢性肾炎综合征。肾外表现主要为贫血、关节病变、皮疹和浆膜腔炎。病理上除新月体形成较多外 ,还有明显增殖性病变 (每个肾小球内细胞数 >15 0个者 ,81 6 % )、毛细血管袢坏死 (6 5 7% )、间质血管炎 (6 0 5 % )、间质大量炎细胞浸润 (92 1% )等明显活动性病变 ,平均活动指数高达 7 74± 2 6 8。结论 多数CLN患者临床表现较重为急进性肾炎综合症 ,但约有 1/ 3患者临床表现相对较轻 ,肾功能正常。两组CLN患者临床及血清学检查均具有较高的活动性 ,但肾功能正常患者病理上活动性病变更明显。从重复肾活检病例看 ,约半数CLN患者由其它类型转型所致。
Objective To understand the clinical and pathological features of lupus nephritis (CLN). Methods Clinical, serological and pathological changes of 38 cases of CLN were analyzed and summarized. The patients with abnormal renal function and normal renal function were compared. Results All patients with CLN were female. 62.3% of the patients had acute nephritic syndrome, 52% showed chronic renal failure, and 31.6% of patients had normal renal function. The clinical manifestations were nephrotic syndrome or chronic nephritic syndrome. Renal manifestations mainly anemia, joint disease, rash and serositis. In addition to the pathological appearance of more crescent forms, there were also significant proliferative lesions (> 15 0 cells per glomerular fraction, 81 6%), capillary necrosis (65.7%), interstitial Vasculitis (65.0%), interstitial inflammatory cell infiltration (92.1%) and other obvious active lesions, with an average activity index of up to 7 74 ± 26.8. Conclusions Most patients with CLN have severe clinical manifestations of acute nephritic syndrome. However, about one third of patients have relatively mild clinical manifestations and normal renal function. Clinical and serological tests of both CLN patients were highly active, but pathological active lesions were more obvious in patients with normal renal function. From repeated renal biopsy cases, about half of CLN patients are caused by other types of transformation.