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目的:比较新月体性过敏紫癜性肾炎(HSPCN)与新月体性IgA肾炎(IgACN)临床病理特征及预后的异同。方法:对1985至2004年在解放军肾脏病研究所住院肾活检确诊(新月体形成率?50%)的HSPCN和IgACN患者进行流行病学、临床表现、病理特征及预后的回顾性分析。结果:HSPCN和IgACN患者各24例,分别占同期294例新月体性肾炎患者的8·16%。HSPCN和IgACN组肾脏病程分别为(3·84±6·40)月和(5·91±9·75)月(无统计学差异)。两组患者临床多表现为肉眼血尿(HSPCNvsIgACN:62·5%vs70·8%)和大量蛋白尿(95·8%vs100%),HSPCN与IgACN组表现为急进性肾功能减退者分别为15例(62·5%)和22例(91·7%),但HSPCN组高血压及肾功能损害程度均较IgACN组轻,HSPCN组仅4例(16·7%)出现中重度肾功能减退(血清肌酐≥265μmol/L),IgACN组11例(45·8%)出现中重度肾功能减退。肾组织病理检查:两组袢坏死(58·3%vs54·2%)、节段性新月体(53·2%vs60·0%)、球囊壁粘连(91·7%vs62·5%)等节段性病变发生率均较高;肾小球硬化(平均球性硬化率3·32%vs17·6%)、间质纤维化(半定量评分0·38±0·58vs0·83±0·89)和肾小管萎缩(半定量评分0·71±0·86vs1·58±0·97)等慢性化病变程度HSPCN组轻于IgACN组。两组患者肾小球免疫病理均以IgA在系膜区沉积为主(伴或不伴血管袢沉积),其中伴C3沉积者HSPCN组显著少于IgACN组(75%vs100%)。IgG、IgM、C4和C1q沉积两组无显著差异。HSPCN肾组织炎细胞浸润程度更严重。HSPCN组患者平均随访时间(46·7±30·5)个月(13~120月,中位时间40个月),5年肾存活率91·7%;IgACN组患者平均随访时间(54·8±30·7)个月(17~144个月,中位随访时间49个月),5年肾存活率77·8%。结论:HSPCN与IgACN具有相似之处:肉眼血尿和大量蛋白尿发生率高,中重度肾功能减退发生率低,肾组织节段性病变多见,二者存在明显的不同之处:HSPCN较IgACN肾功能损害程度及肾组织慢性化病变程度轻,预后优于IgACN。
Objective: To compare the clinicopathological characteristics and prognosis between nephrotic nephritis (HSPCN) and crescentic IgA nephritis (IgACN). Methods: The retrospective analysis of the epidemiological, clinical manifestations, pathological features and prognosis of HSPCN and IgACN patients who were diagnosed by renal biopsy in the Institute of Nephrology of Chinese PLA from 1985 to 2004 (crescent formation rate was 50%) were retrospectively analyzed. Results: 24 cases of HSPCN and IgACN patients accounted for 8.16% of 294 cases of crescentic nephritis respectively. The renal course of HSPCN and IgACN groups was (3.84 ± 6 · 40) months and (5 · 91 ± 9 · 75) months, respectively (with no significant difference). The clinical manifestations in both groups were gross hematuria (HSPCNvsIgACN: 62.5% vs70.8%) and mass proteinuria (95.8% vs100%). In HSPCN and IgACN group, the patients with acute renal dysfunction were 15 (62.5%) and 22 cases (91.7%). However, the levels of hypertension and renal damage in HSPCN group were lower than those in IgACN group. Only 4 cases (16.7%) in HSPCN group had moderate or severe renal dysfunction Serum creatinine ≥265μmol / L), IgACN group of 11 patients (45.8%) with moderate to severe renal dysfunction. Renal histopathological examination revealed necrosis of the two groups (58.3% vs54.2%), segmental crescent (53.2% vs60.0%), balloon wall adhesion (91.7% vs62.5%), etc. The incidence of segmental lesions were high; glomerulosclerosis (mean ratio of global sclerosis: 3.32% vs 17.6%), interstitial fibrosis (semiquantitative score: 0.38 ± 0.58 vs. 0.83 ± 0. 89) and tubular atrophy (semiquantitative score 0.71 ± 0.86vs1.58 ± 0.97) were lower in HSPCN group than in IgACN group. Glomerular immunopathology in both groups were mainly IgA deposition in the mesangial area (with or without angiogenic deposition), of which HSPCN group with C3 deposition was significantly less than IgACN group (75% vs100%). IgG, IgM, C4 and C1q deposition of two groups no significant difference. HSPCN renal tissue inflammatory cell infiltration more serious. The mean follow-up time was (46.7 ± 30.5) months (13 to 120 months, median 40 months) in HSPCN group and 91.7% in 5 years. The average follow-up time in IgACN group was 54 · 8 ± 30 · 7) months (17 ~ 144 months, with a median follow-up time of 49 months). The 5-year renal survival rate was 77.8%. Conclusion: There are similarities between HSPCN and IgACN: high incidence of gross hematuria and massive proteinuria, low incidence of moderate and severe renal dysfunction, segmental lesions of renal tissue more common, there are significant differences between the two: HSPCN compared with IgACN The extent of renal dysfunction and chronic renal tissue lesions, the prognosis is better than IgACN.