背景:血栓性微血管病(TMA)是狼疮性肾炎(LN)预后的重要危险因素。LN合并TMA患者往往病情严重,病死率高,预后差。在本研究中,我们回顾性分析临床表现,实验室结果,肾脏病理表现,分析LN-TMA患儿发生终末期肾病(ESRD)的危险因素。
方法:选择74例LN和肾TMA(rTMA)患者,并与128例没有TMA(1:2比例)的LN对照进行比较,病理类型和治疗。
结果:收缩压的平均值,舒张压(DBP),乳酸脱氢酶(LDH),血尿素氮(BUN),尿蛋白定量(PRO),尿液红细胞,N-乙酰-β-D-葡萄糖苷酶(NAG),视黄醇结合蛋白,系统性红斑狼疮疾病活动评分(SLEDAI),TMA组的活动指数(AI)评分均高于非TMA组(p&lt;0.05和p&lt;0.01)。补体C3,血红蛋白,血小板,估计肾小球滤过率,TMA组的慢性指数(CI)评分均低于非TMA组(p&lt;0.05和p&lt;0.01)。肾小球新月形的病例数,纤维新月形,毛细血管内增殖,肾小管萎缩,间质纤维化,TMA组的C3和C1q沉积高于非TMA组(p&lt;0.05和p&lt;0.01)。TMA组的3年和5年肾脏生存率(88.93%vs.97.00%,p<0.05)和TMA组(61.41%vs.82.31%,p<0.05)显著低于非TMA组。多因素Cox回归分析显示,治疗前血肌酐(≥110μmol/L),TMA和间质纤维化是LN患儿发生ESRD的独立危险因素。
结论:TMA患儿的一般情况很危急,预后较差。及早发现,早期治疗和开发新的治疗方法是改善儿童LN-TMA结局的关键.
BACKGROUND: Thrombotic microangiopathy (TMA) is an important risk factor for the prognosis of lupus nephritis (LN). Patients with LN complicated with TMA tend to be critically ill with high mortality and poor prognosis. In the present study, we retrospectively analyzed the clinical manifestations, laboratory results, renal pathological manifestations, and prognosis of children with LN-TMA and analyzed the risk factors for end-stage renal disease (ESRD) in children with LN-TMA.
METHODS: Seventy-four patients with LN and renal TMA (rTMA) were selected and compared to 128 LN controls without TMA (1:2 ratio) matched according to demographics, pathological type and treatments.
RESULTS: The mean values of systolic blood pressure, diastolic blood pressure (DBP), lactate dehydrogenase (LDH), blood urea nitrogen (BUN), urinary protein quantitation (PRO), urine red blood cells, N-acetyl-β-D-glucosidase (NAG), retinol-binding protein, systemic lupus erythematosus disease activity score (SLEDAI), and activity index (AI) scores in the TMA group were all higher than those in the non-TMA group (p < 0.05 and p < 0.01). The mean values of complement C3, hemoglobin, platelets, estimated glomerular filtration rate, and chronic index (CI) score in the TMA group were all lower than those in the non-TMA group (p < 0.05 and p < 0.01). The number of cases of glomerular crescent, fibrous crescent, endocapillary proliferation, tubular atrophy, interstitial fibrosis, C3 and C1q deposition in the TMA group was higher than that in the non-TMA group (p < 0.05 and p < 0.01). The 3-year and 5-year renal survival rates in the TMA group (88.93% vs. 97.00%, p < 0.05) and TMA group (61.41% vs. 82.31%, p < 0.05) were significantly lower than those in the non-TMA group. Multivariate Cox regression analysis showed that serum creatinine before treatment (≥110 μmol/L), TMA and interstitial fibrosis were independent risk factors for the development of ESRD in LN children.
CONCLUSIONS: The general condition of children with TMA is critical, and the prognosis is poor. Early detection, early treatment and the development of new treatments are key to improving LN-TMA outcomes in children.