Kemper M J, Meyer-Jark T, Lilova M, Müller-Wiefel D E
Pediatric Nephrology, University Children's Hospital, Hamburg, Germany.
Clin Nephrol. 2003 Oct;60(4):242-7. doi: 10.5414/cnp60242.
Growing evidence shows that steroid-sensitive nephrotic syndrome (SSNS) is the result of a primary T-cell disturbance and leads to secondary anatomical and functional, however, not to immunological glomerular changes. In addition, immunoglobulin abnormalities in SSNS indicate a role of B-cell involvement.
We therefore analyzed T- and B-cell activation markers in children with SSNS at different stages of the disease including different treatment regimens by measuring the soluble IL-2 receptor (sCD25) and the soluble low-affinity IgE receptor (sCD23), respectively. Seventy-five patients with SSNS (median age 8.0, range 2.5 - 18 years) were studied, 33 in relapse (RL) including 21 patients relapsing during alternate-day steroids (RL-SD). Forty-two patients were studied in remission (RM; 14 off treatment and 28 on alternate-day steroids (RM-AD)) and 22 age-matched children served as controls.
Serum concentrations of sCD25 were increased in RL (113.6 +/- 19.5 micromol/l) compared to RM (79.8 +/- 8 micromol/l, p < 0.02) and controls (74.8 +/- 0.9 micromol/l, p < 0.02). Patients with RL-SD did not have elevated sCD25. In relapse, sCD25 was inversely correlated with age (R = -0.36, p < 0.04) and positively correlated with total IgG (R = 0.37, p < 0.04). Urinary excretion of sCD25 was also significantly elevated in RL of SSNS compared to RM and controls (71.2 +/- 11.9 micromol/g creatinine vs. 39.1 +/- 4.8 and 32.0 +/- 4.2 micromol/g, p < 0.05). Serum levels of sCD 23 were significantly elevated in RL (6.22 +/- 0.65 microg/l) compared to RM (3.1 +/- 0.83 microg/l, p < 0.02) and to controls (3.4 +/- 0.93 microg/l). The highest values, however, were found in RL-SD (7.8 +/- 1.7 microg/l) vs. untreated RL (p < 0.007) and RM-AD (p < 0.002). In untreated RL there was a significant correlation of sCD23 and total IgE (R = 0.67, p < 0.02) and in RL-SD with total IgG (R = 0.45, p < 0.05). sCD23 and sCD25 were not correlated with each other.
These data document parallel abnormalities of both CD23-mediated B as well as CD25-mediated T-cell activation and suggest that SSNS is not solely a disorder of T-cell dysfunction.
越来越多的证据表明，类固醇敏感性肾病综合征（SSNS）是原发性T细胞紊乱的结果，会导致继发性解剖学和功能性改变，但不会引起免疫性肾小球变化。此外，SSNS中的免疫球蛋白异常表明B细胞参与其中。
因此，我们通过分别测量可溶性白细胞介素-2受体（sCD25）和可溶性低亲和力IgE受体（sCD23），分析了处于疾病不同阶段（包括不同治疗方案）的SSNS患儿的T细胞和B细胞活化标志物。研究了75例SSNS患者（中位年龄8.0岁，范围2.5 - 18岁），其中33例处于复发期（RL），包括21例在隔日服用类固醇期间复发的患者（RL-SD）。42例患者处于缓解期（RM；14例未接受治疗，28例隔日服用类固醇（RM-AD）），22例年龄匹配的儿童作为对照。
与RM（79.8±8微摩尔/升，p<0.02）和对照组（74.8±0.9微摩尔/升，p<0.02）相比，RL患者的血清sCD25浓度升高（113.6±19.5微摩尔/升）。RL-SD患者的sCD25未升高。在复发期，sCD25与年龄呈负相关（R = -0.36，p<0.04），与总IgG呈正相关（R = 0.37，p<0.04）。与RM和对照组相比，SSNS的RL期尿中sCD25排泄量也显著升高（71.2±11.9微摩尔/克肌酐 vs. 39.1±4.8和32.0±4.2微摩尔/克，p<0.05）。与RM（3.1±0.83微克/升，p<0.02）和对照组（3.4±0.93微克/升）相比，RL患者的血清sCD23水平显著升高（6.22±0.65微克/升）。然而，最高值出现在RL-SD（7.8±1.7微克/升），与未治疗的RL（p<0.007）和RM-AD（p<0.002）相比。在未治疗的RL中，sCD23与总IgE显著相关（R = 0.67，p<0.02），在RL-SD中与总IgG相关（R = 0.45，p<0.05）。sCD23和sCD25彼此不相关。
这些数据证明了CD23介导的B细胞以及CD25介导的T细胞活化存在平行异常，并表明SSNS并非仅仅是T细胞功能障碍性疾病。
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