National Clinical Research Center of Kidney Diseases, Jinling Hospital, Nanjing University School of Medicine, Nanjing, People's Republic of China.
BMC Nephrol. 2018 Nov 22;19(1):334. doi: 10.1186/s12882-018-1117-9.
IgA vasculitis (IgAV, formerly Henoch-Schönlein purpura) is a type of systemic vasculitis. This study aimed to explore the clinicopathological features, treatment and renal outcomes of adult IgAV-related nephritis (Henoch-Schönlein purpura nephritis) patients with different degrees of crescent formation.
Adult patients with biopsy-proven IgAV-related nephritis in Nanjing Jinling Hospital were enrolled and divided into three groups as follows: control (no crescents, n = 257), group 1 (crescents < 25%, n = 381), and group 2 (crescents ≥25%, n = 60). The clinicopathological features, treatment and renal outcomes were compared among the three groups.
There were no significant differences in gender and age at biopsy among the three groups. Groups with more crescents had shorter renal durations and higher prevalence of macroscopic hematuria, proteinuria and nephrotic syndrome than the control group. The presence of renal insufficiency at biopsy was similar, whereas laboratory findings indicated that patients with ≥25% crescents had higher levels of serum creatinine and blood urea nitrogen than the control and group 1. Histologically, the incidence of glomeruli-Bowman's capsule adhesion and capillary necrosis were proportional to the degree of crescent formation. Patients with more crescents received more positive immunosuppressive therapies. During follow-up, the levels of proteinuria and hematuria were in remission after treatment, and patients without crescents had lower levels of proteinuria. At the last follow-up, the renal function had deteriorated in the control and group 1, whereas the levels of serum creatinine at biopsy and last follow-up were similar in group 2. There was a significant difference in renal survival from end-stage renal disease (ESRD) or 50% decline in renal function among the three groups (log-rank, P = 0.030). However, no association between crescent formation and renal outcomes was found after adjusting potential confounders.
Adult IgAV-related nephritis patients with more crescents had more-severe renal manifestations and worse treatment responses, whereas the proportions of crescents were not associated with higher risks for ESRD or 50% decline in renal function. A more suitable pathological classification standard is needed to predict renal prognosis.
IgA 血管炎（IgAV，以前称为过敏性紫癜）是一种系统性血管炎。本研究旨在探讨不同新月体形成程度的成人 IgAV 相关肾炎（过敏性紫癜肾炎）患者的临床病理特征、治疗和肾脏结局。
纳入南京金陵医院经活检证实的成人 IgAV 相关肾炎患者，分为三组：对照组（无新月体，n=257）、组 1（新月体<25%，n=381）和组 2（新月体≥25%，n=60）。比较三组患者的临床病理特征、治疗和肾脏结局。
三组患者的性别和活检时年龄无显著差异。新月体比例较高的组肾脏持续时间较短，肉眼血尿、蛋白尿和肾病综合征的发生率高于对照组。活检时肾功能不全的发生率相似，而实验室检查结果表明，新月体≥25%的患者血清肌酐和血尿素氮水平高于对照组和组 1。组织学上，肾小球-鲍曼囊粘连和毛细血管坏死的发生率与新月体形成程度成正比。新月体比例较高的患者接受更多的免疫抑制治疗。随访期间，治疗后蛋白尿和血尿水平缓解，无新月体的患者蛋白尿水平较低。最后一次随访时，对照组和组 1 的肾功能恶化，而组 2 的活检和最后一次随访时的血清肌酐水平相似。三组患者的终末期肾病（ESRD）或肾功能下降 50%的肾脏存活率存在显著差异（对数秩检验，P=0.030）。然而，在校正潜在混杂因素后，新月体形成与肾脏结局之间没有关联。
新月体比例较高的成人 IgAV 相关肾炎患者肾脏表现更严重，治疗反应更差，而新月体比例与发生 ESRD 或肾功能下降 50%的风险无关。需要更合适的病理分类标准来预测肾脏预后。
