12例狼疮性肾炎合并血栓性微血管病变临床特点及预后分析

Clinical characteristics and prognosis of 12 cases of lupus nephritis complicated with thrombotic microangiopathy

摘要:

目的 分析狼疮性肾炎（lupus nephritis, LN）合并血栓性微血管病变 （thrombotic microangiopathy, TMA）患儿的临床特征、病理特点、治疗方案及预后，并寻找与非TMA的LN患儿临床及病理方面的差异。方法 回顾性收集2010年12月—2021年12月收住南京医科大学附属儿童医院肾脏科的12例合并TMA的LN患儿为TMA组，选择同期住院行肾活检的24例不合并TMA的LN患儿为非TMA组，分析比较两组临床表现、实验室检查和病理结果等情况。结果 12例LN合并TMA的患儿中，８例（67％）伴有高血压，3例（25%）发展为慢性肾脏病5期。与非TMA组比较，TMA组肾小管间质损害较重，发病时系统性红斑狼疮疾病活动指数评分更高，胆固醇水平更高（P<0.05）。两组在新月体比例、血红蛋白水平及血小板计数间差异无统计学意义（P>0.05）。结论 LN合并TMA患儿高血压比例高，肾小管间质损害较严重，系统性红斑狼疮疾病活动指数评分较高，有较高水平的胆固醇。

Abstract:

Objective To investigate the clinical characteristics, pathological features, treatment regimen, and prognosis of children with lupus nephritis (LN) and thrombotic microangiopathy (TMA), as well as the treatment outcome of these children and the clinical and pathological differences between LN children with TMA and those without TMA.Methods A retrospective analysis was conducted on 12 children with LN and TMA (TMA group) who were admitted to the Department of Nephrology, Children's Hospital of Nanjing Medical University, from December 2010 to December 2021. Twenty-four LN children without TMA who underwent renal biopsy during the same period were included as the non-TMA group. The two groups were compared in terms of clinical manifestations, laboratory examination results, and pathological results.Results Among the 12 children with TMA, 8 (67%) had hypertension and 3 (25%) progressed to stage 5 chronic kidney disease. Compared with the non-TMA group, the TMA group had more severe tubulointerstitial damage, a higher Systemic Lupus Erythematosus Disease Activity Index (SLEDAI) score at onset, and higher cholesterol levels (P<0.05). There were no significant differences between the two groups in the percentage of crescent bodies and the levels of hemoglobin and platelets (P>0.05).Conclusions There is a higher proportion of individuals with hypertension among the children with LN and TMA, as well as more severe tubulointerstitial damage. These children have a higher SLEDAI score and a higher cholesterol level.