Abstract：
BACKGROUND: Diffuse crescentic IgAN (CIgAN) is an uncommon phenotype of IgAN, which presents as rapidly progressive renal failure, similar to patients with pauci-immune crescentic glomerulonephritis(PCGN). There are limited data on outcomes comparisons between the two.
METHODS: In this single-center, retrospective cohort study, we compared the clinical features, pathological presentation, and renal outcomes of 52 patients with CIgAN and 42 patients with renal-limited PCGN from January 2007 to December 2019.
RESULTS: The CIgAN patients were younger (30.5 ± 13.8 years) than PCGN patients (46.1 ± 11.8 years) (P = 0.001). The CIgAN patients had a higher prevalence of hypertension (86.5% Vs. 41.3%, P = 0.001); and degree of proteinuria (4.2 ± 2.7 g/24 h Vs. 2.3 ± 1.16 g/24 h; P = 0.001) than PCGN patients. The chronicity in terms of global glomerulosclerosis, interstitial fibrosis, and tubular atrophy was higher in the CIgAN group than in the PCGN group. The remission rate with immunosuppression was significantly higher in the PCGN group than in the CIgAN group (P = 0.016). The end-stage renal disease (ESRD) or death within 1 year of diagnosis was significantly more in the CIgAN group (62.3% Vs. 39.1%) than PCGNgroup. For patients who were dialysis-dependent at presentation, the primary outcome of ESRD or death within one year was seen in 90.9% of patients of CIgAN and 44.1% in the PCGN group (P = 0.001). The long-term death non-censored renal survival is poor in the CIgAN group than in PCGN patients. However, patient survival is poor in PCGN patients.
CONCLUSIONS: CIgAN is a different form of RPGN compared to PCGN and carries a poor prognosis despite similar immunosuppressive therapy in the long term.
METHODS: In this single-center, retrospective cohort study, we compared the clinical features, pathological presentation, and renal outcomes of 52 patients with CIgAN and 42 patients with renal-limited PCGN from January 2007 to December 2019.
RESULTS: The CIgAN patients were younger (30.5 ± 13.8 years) than PCGN patients (46.1 ± 11.8 years) (P = 0.001). The CIgAN patients had a higher prevalence of hypertension (86.5% Vs. 41.3%, P = 0.001); and degree of proteinuria (4.2 ± 2.7 g/24 h Vs. 2.3 ± 1.16 g/24 h; P = 0.001) than PCGN patients. The chronicity in terms of global glomerulosclerosis, interstitial fibrosis, and tubular atrophy was higher in the CIgAN group than in the PCGN group. The remission rate with immunosuppression was significantly higher in the PCGN group than in the CIgAN group (P = 0.016). The end-stage renal disease (ESRD) or death within 1 year of diagnosis was significantly more in the CIgAN group (62.3% Vs. 39.1%) than PCGNgroup. For patients who were dialysis-dependent at presentation, the primary outcome of ESRD or death within one year was seen in 90.9% of patients of CIgAN and 44.1% in the PCGN group (P = 0.001). The long-term death non-censored renal survival is poor in the CIgAN group than in PCGN patients. However, patient survival is poor in PCGN patients.
CONCLUSIONS: CIgAN is a different form of RPGN compared to PCGN and carries a poor prognosis despite similar immunosuppressive therapy in the long term.
摘要：
背景：弥漫性新月体IgAN（CIGAN）是IgAN的一种罕见表型，表现为快速进行性肾衰竭，与免疫型新月体肾炎（PCGN）患者相似。关于两者之间结果比较的数据有限。
方法：在这个单中心，回顾性队列研究，我们比较了临床特征，病理表现,2007年1月至2019年12月，52例CIgAN患者和42例肾受限PCGN患者的肾脏结局。
结果：TheCIgAN患者（30.5±13.8岁）比PCGN患者（46.1±11.8岁）年轻（P=0.001）。CIgAN患者的高血压患病率较高（Vs为86.5％。41.3%,P=0.001）；和蛋白尿程度（4.2±2.7g/24hVs。2.3±1.16g/24h；P=0.001）比PCGN患者高。就全球肾小球硬化而言，慢性，间质纤维化,CIgAN组肾小管萎缩高于PCGN组。PCGN组的免疫抑制缓解率明显高于CIgAN组（P=0.016）。在CIgAN组中，终末期肾病（ESRD）或诊断后1年内死亡的发生率明显更高（62.3％Vs。39.1%)比PCGN组。对于在就诊时依赖透析的患者，主要结局为ESRD或一年内死亡的患者为90.9%,PCGN组为44.1%(P=0.001).与PCGN患者相比，CIgAN组的长期死亡非删失肾生存率较差。然而,PCGN患者的生存率较差。
结论：与PCGN相比，CIGAN是一种不同形式的RPGN，尽管长期免疫抑制治疗相似，但预后较差。
方法：在这个单中心，
结果：TheCIgAN患者（30.5±13.8岁）比PCGN患者（46.1±11.8岁）年轻（P=0.001）。
结论：与PCGN相比，CIGAN是一种不同形式的RPGN，尽管长期免疫抑制治疗相似，但预后较差。
