成人过敏性紫癜性肾炎与IgA肾病临床病理和转归对比分析

目的:比较成人过敏性紫癜性肾炎(Henoch-Sch(o)nlein nephritis, HSPN)与IgA肾病(IgA nephropathy,IgAN)临床病理和转归.方法 :对肾活检证实的31例HSPN患者和62例IgAN患者临床、病理及转归进行同期对照分析.结果:对两组患者起病时高血压发生率、血肌酐及蛋白尿水平进行比较,差异无统计学意义38.7% vs 27.4%,(121±164 )vs( 106±43) μmol/L, (3.2±3.1) vs (2.8±2. 9) g/d,P＞0.05].在肾病理改变上,HSPN患者40.6%(13/31)出现内皮增生,IgAN仅为19.4%(12/62),差异有统计学意义(P=0.021).用肾病理半定量评分,肾小管间质慢性病变IgAN较HSPN明显(2 vs 1,P=0.009),但在新月体形成(包括袢坏死)及肾小球硬化等病理表现二者并无明显差别(1 vs 0,1 vs 1,P＞0.05).用免疫荧光检查,免疫球蛋白IgA除了在系膜区沉积外,71.0%(22/31)HSPN患者IgA还沉积在毛细血管袢,而IgAN患者为43.5%(27/62),二者比较差异有统计学意义(P=0.013).平均随访47个月,以肌酐倍增为随访终点,HSPN与IgAN累计肾生存率分别为87.1%和91.9%,二者并无明显差别(P=0.481).结论:成人HSPN与 IgAN在肾病理改变上存在差异,但二者在肾临床表现及远期预后上并无明显区别.

Comparative analysis of clinicopathological findings and outcome of Henoch-Sch(o)nlein nephritis and IgA nephropathy in adults

OBJECTIVE: To compare the clinicopathological findings and prognosis of Henoch-Schonlein nephritis (HSPN) and IgA nephropathy (IgAN) in adults. METHODS: We enrolled 31 patients with HSPN and 62 patients with IgAN in the present study. They were followed up for 47+/-23 months and 47+/-20 months respectively, and their clinical manifestations and renal pathological findings were collected. Renal pathological changes were semiquantitatively graded. RESULTS: The clinical manifestations, including hypertension, excretion of serum creatinine and urinary protein, were similar in patients with HSPN and IgAN 38.7% vs 27.4%, (121+/-164) vs (106+/-43) micromol/L, (3.2+/-3.1) vs (2.8+/-2.9) g/d, P>0.05]. Renal pathological investigation showed endothelial proliferation in 40.6% (13/31) of HSPN patients and 19.4% (12/62) of IgAN patients and the difference was significant (P=0.021). In patients with IgAN, the tubulointerstitial chronicity index was higher than that in HSPN (2 vs 1, P=0.009), but there were no statistically significant differences in crescent formation(including segmental glomerular necrosis) and glomerular sclerosis(1 vs 0, 1 vs 1, P>0.05). In patients with HSPN capillary wall staining for IgA was more frequently found than in IgAN (71.0% vs 43.5%, P=0.013). With creatinine level doubling as the end point, the follow-up data indicated that the renal survival was 87.1% in HSPN and 91.9% in IgAN and there was no statistically significant difference between HSPN and IgAN (P=0.481). CONCLUSION: Although significant pathological difference was found between HSPN and IgAN, the renal clinical manifestations and long term outcome were similar between the two diseases in adults.