合并恶性高血压和血栓性微血管病的青年IgA肾病患者治疗及预后

Treatment and prognosis of malignant hypertension complicated with thrombotic microangiopathy for young IgAN patients

目的探讨青年IgA肾病(IgAN)合并恶性高血压(MHT)及血栓性微血管病(TMA)的临床病理特征及预后,及其与肾脏局部血管紧张素受体(AT1R,AT2R,MASR)、醛固酮受体(AldR)和肾素表达水平的相关性。方法分析13例青年IgAN同时并发MHT和TMA患者的临床病理数据,探讨影响预后的相关因素。应用免疫组织化学法观察AT1R、AT2R、MASR、AldR和肾素在肾组织的表达情况。结果10例患者进入主要终点,中位随访时间11个月。贫血、尿蛋白≥1.0 g/d、肾活检时eGFR降低、毛细血管内增殖性病变是影响患者预后的独立危险因素。试验组AT1R和AT2R各部位表达显著强于对照组(P<0.01),MASR在肾小球、肾脏血管表达以及AldR在肾小管间质、肾脏血管表达显著强于对照组(P<0.01)。毛细血管内增殖程度与AldR在肾小管的表达呈显著正相关;肾素表达与血肌酐水平呈正相关。结论青年IgAN伴有MHT及TMA患者预后较差,贫血、尿蛋白≥1.0 g/d、肾脏病理损伤重是疾病进展的主要因素。肾脏局部肾素-血管紧张素-醛固酮系统的激活在本病发生和进展中具有重要作用。

Objective To observe the treatment and prognosis of malignant hypertension(MHT)complicated with thrombotic microangiopathy(TMA)for young patients with IgA nephropathy(IgAN),and its relationship with the expression level of angiotensin receptors in the kidney(AT1R,AT2R,MASR),aldosterone receptors and renin.Methods The clinicopathological data of 13 cases of MHT complicated with TMA for young IgAN patients were analyzed,related factors affecting the prognosis were discussed,and the expression of AT1R,AT2R,MASR,AldR and renin in renal tissues were observed by immunohistochemistry(IHC).Results Ten patients entered the primary endpoint with a median follow-up of 11 months.Independent risk factors affecting prognosis for patients involved anemia,24-hour proteinuria≥1.0 g/d,decreased eGFR during renal biopsy and pathological capillary proliferative lesions.The expression of AT1R and AT2R in glomerulus,tubulointerstitium and renal vessels of the experimental group were significantly higher than those of the control group(P<0.01),and the expression of MASR in the glomerulus and renal vessels and the expression of AldR in the renal tubulointerstitium and renal vessels were significantly higher than those in the control group(P<0.01).There was significant positive correlation between the degree of proliferation in capillaries and the expression of AldR in renal tubules,and the expression of renin was positively correlated with the level of serum creatinine.Conclusion Young IgAN patients with MHT and TMA have poor prognosis,and those are all the main factors of disease progression,such as anemia,24-hour proteinuria≥1.0 g/d,and renal pathological severe damage.The activation of renin angiotensin aldosterone system plays an important role in the pathogenesis and progression of the disease.