Two Cases of ANCA-negative Pauci-immune Crescentic Glomerulonephritis in Pregnant or Maternal Women

Here two cases of renal failure occurring in pregnancy were reported,with biopsy-proven pauci-immune crescentic glomerulonephritis at postpartum.It is most rare that pauci-immune crescentic glomerulonephritis as the cause of renal failure in pregnancy.Their renal function could remain stable for some time due to timely and appropriate treatment.Therefore,the pregnant and maternal who have the performance of renal impairment should be closely tracked their blood pressure,urinary protein and renal function,if necessary,execute renal biopsy,for a clear and accurate pathological diagnosis is of great significance to grasp the opportunity of treatment.But the long-term prognosis is still poor.

Significance of anti neutrophil cytoplasmic antibodies in patients with pauci immune crescentic glomerulonephritis

Objective To evaluate the significance of serum anti neutrophil cytoplasmic antibodies (ANCAs) and the effects of immune suppressive treatments on its activity in patients with pauci immune crescentic glomerulonephritis (PICGN) Methods Serum ANCAs and myeloperoxidase (MPO) ANCA were detected by indirect immunofluorescence and enzyme linked immuno^sorbent assay (ELISA) methods respectively, and the renal tissues infiltrating cells including CD4 +, CD8 +, CD68 + and PCNA + cells were determined by four layer peroxidase antiperoxidase (PAP) method The clinical manifestations, pathologic features and immune pathologic changes in patients with positive ANCAs were compared with that in patients with negative ANCAs The effects of immune suppressive therapy on clinic and pathologic changes as well as ANCAs activity were also investigated in ANCAs positive and ANCAs negative patients Results Both of clinic active manifestations such as the degree of hematuria, rapidly progressive renal failure and pathologic active features including segmental capillary necrosis and vasculitis were much common in patients with positive ANCAs as compared with that in patients with negative ANCAs The number of infiltrating cells in renal tissue, especially CD4 + cells, was markedly higher in ANCAs positive patients than that in ANCAs negative patients The effects of immune suppressive therapy were also much better in patients with positive ANCAs than that in patients with negative ANCAs Conclusion Serum ANCAs is not only a marker for diagnosis of systemic vasculitis, but also a sensitive predictor for evaluation of diseases' activity and treatment in patients with PICGN The good effect of immune suppressive treatments on patients with PICGN is partially associated with the degree of ANCAs activity

ANCA相关性血管炎的免疫发病机制新进展

抗中性粒细胞胞浆抗体(anti-neutrophil cytoplasmic antibodies,ANCA)相关性系统性血管炎(ANCA-associatedsystemic vasculitis,AASV)是典型的自身免疫疾病,其发病机制包括天然免疫和获得性免疫的共同参与。大量临床及实验证据显示ANCA不仅是AASV的血清学标志,也在血管及组织损伤中起核心作用。本文就ANCA作用模式、感染与分子模拟、病原体相关分子模式识别、补体系统及三级淋巴组织参与免疫发病机制的最新进展作一综述。

ANCA阴性少免疫沉积型新月体性肾炎患者血清AECA与临床表现的关系研究

目的:探讨抗中性粒细胞胞浆抗体(ANCA)阴性少免疫沉积型新月体性肾炎患者血清抗内皮细胞抗体(AECA)表达情况,及其与临床表现的关系。方法:选取2010年至2015年在我院治疗的少免疫沉积型新月体性肾小球肾炎患者,其中ANCA阴性患者45例(观察组),ANCA阳性患者49例(对照组),采用Western blot检测各组患者血清AECA水平。结果:观察组平均年龄和伯明翰血管炎活动性评分(BVAS)分别为(41.08±9.43)岁和(15.03±3.82)分,明显低于对照组(P<0.05);观察组出现发热、关节痛的比例分别为26.67%和13.33%,明显低于对照组(P<0.05);观察组出现肾病综合征比例为48.89%,高于对照组(P<0.05);观察组血清AECA阳性率为46.67%,明显低于对照组的81.63%(P<0.05);观察组Ig G-AECA共识别7种蛋白,而对照组共识别11种蛋白,其中观察组抗90 k D抗体阳性率为13.33%(6/45),明显低于对照组的51.02%(25/49)(P<0.05);观察组抗76 k D抗体阳性患者出现皮疹的比例为100%,明显高于阴性患者(P<0.05),抗200 k D抗体阳性患者BVAS评分为(18.02±2.51)分,明显高于阴性患者(P<0.05)。结论:ANCA阴性少免疫沉积型新月体性肾炎患者血清存在不同的AECA,可能与某些临床表现有关;ANCA阴性和阳性患者AECA有所不同,两者临床表现的差异可能与AECA的不同有关,需待进一步研究。

双冲击疗法治疗寡免疫复合物型新月体肾炎

本文为国内首次报道运用甲基强的松龙及环磷酰胺双冲击疗法治疗15例寡免疫复合物型新月体肾炎,取得一定疗效,无明显副反应。治疗3个月,66.7%患者肾功能及尿检异常明显改善。长期随访的11例中(平均随访27.8个月),仅3例仍需透析治疗,4例肾功能保持正常,无一例死亡。双冲疗法对肾组织中浸润细胞均有明显抑制作用,治疗后 CD4^+、CD8^+、单核细胞(CD68^+)及增殖细胞核抗原(PCNA^+)细胞数均有明显下降;治疗前肾脏局部 CD4/CD8的比值、单核细胞及 PCNA^+细胞浸润的多少,可作为判断治疗反应的一项指标。

ANCA阴性寡免疫复合物新月体肾炎患者的临床病理特征

目的比较抗中性粒细胞胞浆抗体(ANCA)阴性(ANCA-)与ANCA阳性(ANCA+)寡免疫复合物新月体肾炎(PICGN)临床病理特征及预后的异同。方法对住院肾活检确诊的75例PICGN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①ANCA-组发病年龄小于ANCA+组[(51.7±13.7)岁vs.(44.3±14.1)岁](P<0.05);②ANCA-组肉眼血尿发生率显著高于ANCA+组(56.7%vs.31.1%)(P<0.05),ANCA-组尿蛋白定量(3.7g/dvs.2.4g/d)和达到肾病范围蛋白尿患者比例(40.0%vs.15.6%)均显著高于ANCA+组(P<0.05),ANCA-组血红蛋白水平较高(9.2g/dlvs.7.8g/dl)(P<0.01);③病理检查:ANCA-组新月体形成比例显著低于ANCA+组(63.5%vs.77.8%)(P<0.01);④ANCA-组中位随访时间49个月(7～144个月),3例失访,无死亡病例。ANCA+组中位随访时间40月(6～120个月),3例失访,12例死亡。Kaplan-Meier图预测ANCA-组5年存活率显著高于ANCA+组(100.0%vs.58.0%)(P<0.01)。结论与ANCA+的PICGN相比,ANCA-患者尿检异常较重,然而肾活检新月体比例较低,慢性化病变较轻,肾外器官受累也较少,预后相对较好。

抗中性粒细胞胞浆抗体阴性的少免疫沉积型新月体性肾小球肾炎15例分析

目的 了解抗中性粒细胞胞浆抗体 (ANCA)阴性的少免疫沉积型新月体肾炎的临床病理特点。方法 对近 10年来诊断为少免疫沉积型新月体肾炎患者进行ANCA检测 ,并对其中ANCA阴性患者进行临床病理分析。结果 33例少免疫沉积型新月体肾炎中 ,ANCA阴性患者 15例 ,占 4 5 % ,为同期所有新月体肾炎的15 %。其中 9例伴多系统受累 ,6例仅累及肾脏。 2例患者病理可见小血管炎。经积极治疗 ,1/13例临床痊愈 ,6 /13例临床缓解 ,6 /13例无效。结论 ANCA阴性少免疫沉积型新月体肾炎在临床、病理上与ANCA阳性病人相似 ,但好发年龄偏低 。