表现为肾病综合征的局灶节段性肾小球硬化患儿肾组织免疫荧光沉积情况和糖皮质激素疗效的关系

Correlation between immunofluorescence deposition and corticosteroid effect in childhood focal segmental glomerulosclerosis manifesting with nephrotic syndrome

目的·分析表现为肾病综合征的局灶节段性肾小球硬化(FSGS)患儿肾组织免疫荧光沉积情况和糖皮质激素疗效的关系。方法·回顾性分析1990年1月至2015年12月在上海交通大学医学院附属新华医院小儿肾脏内科临床诊断为肾病综合征并行肾穿刺活检明确病理类型为FSGS患儿的肾组织免疫荧光沉积情况及临床随访资料。结果·47例表现为肾病综合征的FSGS患儿,免疫荧光类型分布:IgA为主型2例(占4.26%)、IgM为主型7例(占14.89%)、补体为主型4例(占8.51%)、IgG+A+M型1例(占2.13%)、IgG+A+M补体型5例(占10.64%)、IgA+补体型1例(占2.13%)、IgM+补体型12例(占25.53%)、无免疫复合物型15例(占31.91%)。免疫荧光沉积情况:IgA^+有9例,IgM^+有25例,IgG^+有8例,C3^+有23例,C4^+有3例,C1q^+有6例,FN^+有5例,免疫荧光沉积全阴性的有12例。口服泼尼松足量治疗4周后完全缓解34例(占72.34%),部分缓解7例(占14.89%),无缓解6例(占12.77%)。不同免疫荧光类型患者间的激素疗效差异无统计学意义(H=1.792,P=0.408);不同免疫荧光沉积情况中C1q^+与C1q-患者的激素疗效差异有统计学意义(χ~2=7.22,P=0.027),余组间差异均无统计学意义。结论·对于表现为肾病综合征的FSGS患儿,若肾组织有C1q沉积,则糖皮质激素对其的疗效相对较差。

Objective · To explore the correlation between immunofluorescence （IMF） deposition and corticosteroid effect in childhood focal segmental glomerulosclerosis （FSGS） manifesting with nephrotic syndrome （NS）. Methods · Renal IMF deposition and clinical data of the children clinically diagnosed with NS and pathologically diagnosed with FSGS in the Department of Pediatric Nephrology in Xinhua Hospital, Shanghai Jiao Tong University from January 1990 to December 2015 were reviewed and analyzed retrospectively. Results · The renal pathological types classified by IMF of 47 patients diagnosed with FSGS manifesting with NS showed that 2 cases （4.26%） were IgA type, 12 cases （14.89%） were IgM type, 4 cases （8.51%） were complement （C） type, 1 case （2.13%） was IgG＋A＋M type, 5 cases （10.64%） were IgG＋A＋M＋C type, 1 case （2.13%） was IgA＋C type, 12 cases （25.53%） were IgM＋C type, and there was no immune complexes present in 15 cases （31.91%）. The IMF deposition showed 9 cases with IgA＋, 25 cases with IgM＋, 8 cases with IgG＋, 23 cases with C3＋, 3 cases with C4＋, 6 cases with C1q＋, 5 cases with FN＋, 12 cases with all negative. After 4 weeks of treatment with oral prednisone at full dose, complete remission was presented in 34 cases （72.34%）, partial remission was presented in 7 cases （14.89%）, and no remission was presented in 6 cases （12.77%）. There was no statistically difference in the corticosteroid effect among the different types of IMF （H=1.792, P=0.408）. The corticosteroid effect had statistical differences between C1q＋ and C1q- patients （χ^2=7.22, P=0.027）, while it had no significant differences in other conditions. Conclusion · In childhood FSGS manifesting with NS, C1q＋ patients have relatively poor reaction to the corticosteroid therapy compared to C1q- ones.