摘要
目的:特发性局灶节段性肾小球硬化(FSGS)组织形态学病变多样,塌陷型和细胞型病变性质特殊,不同于其他类型,尤其塌陷型患者对治疗反应及预后差,本文将探讨塌陷型、细胞型FSGS的临床病理特点并进行比较。方法:回顾分析2000年1月至2008年12月间,经肾活检结合临床确诊的特发性FSGS患者共411例,依据哥伦比亚FSGS分型标准,比较塌陷型、细胞型FSGS患者的临床表现、组织形态学特点及对治疗的反应。结果:(1)411例FSGS患者中,塌陷型20例(4.9%)、细胞型35例(8.5%)、经典型152例(36.9%)、顶部型159例(38.7%)、门周型45例(11%)。(2)塌陷型和细胞型均以青少年男性多见,肾活检时年龄分别为(23.6±5.96)岁和(28.9±15.0)岁,病程分别为(22.2±61.0)月和(25.1±54.3)月。(3)塌陷型和细胞型FSGS患者肾活检时均表现为大量蛋白尿[(9.34±5.34)g/24h和(8.60±4.44)g/24h],塌陷型尿蛋白定量≥10 g/24h的发生率(55%)高于细胞型(37.1%),但尚未达统计学差异;细胞型患者血清白蛋白水平[(18.2±4.06)g/L]明显低于塌陷型患者[(22.3±8.95)g/L](P<0.05)。(4)塌陷型和细胞型患者的肾小管功能损伤明显,尿NAG、尿RBP异常升高百分率分别为95%、100%和73.7%、85.3%;二者血清肌酐水平[(119.3±54.8)μmol/L和(125.5±58.3)μmol/L]和肾功能不全发生率(35.0%和54.3%)均无明显差别,高血压发生率(分别为30%和22.9%)和镜下血尿发生率(50%和45.7%)相似。(5)塌陷型患者球性、节段硬化比率[(9.15±17.3)%、(18.2±13.8)%]高于细胞型患者[(3.04±11.7)%、(13.5±9.17)%],未达统计学差异。塌陷型患者肾小管间质慢性病变发生率及血管病变发生率明显高于细胞型[(55%vs25.7%),(65%vs37.1%)](P<0.05)。塌陷型患者中有9例(45.0%)合并有细胞型病变。(6)塌陷型、细胞型FSGS患者在经雷公藤多苷片或雷公藤多苷片联合激素治疗后,缓解率和肾功能恢复正常的比率分别为64.3%、68.2%和35.7%、40.9%。结论:在特发性FSGS中,塌陷型、细胞型FSGS分别占4.9%和8.5%,二者在临床表现、病理改变以及对治疗的反应上有一定的重叠和相似性。这两型患者多见于青少年男性,塌陷型和细胞型FSGS均表现大量蛋白尿,但塌陷型超大量蛋白尿发生率更高(达55%)。两型均伴有明显的肾小管功能损伤,塌陷型FSGS小管间质慢性病变更重。两型患者肾功能不全、高血压及镜下血尿发生率无差别。塌陷型和细胞型对激素和雷公藤多苷片均有较高的治疗缓解率。
Objective ： Idiopathic collapsing and cellular focal segmental glomerulosclerosis （FSGS） are actually different from other variants of FSGS due to their complicated pathogenesis. Collapsing FSGS has a more aggressive clinical course, with fewer remissions and worse prognosis. However, the relationship of collapsing and cellular FSGS in Chinese patients has not been investigated. Methodology：Four hundred eleven patients with biopsy-proven idiopathic FSGS were studied retrospectively. They were subdivided into five histological variants on the basis of the columbia FSGS classification： collapsing（ COLL）, cellular（ CELL）, classic（ NOS）, tip（GTL） and perihilar（PH）. In this study, we compared the clinical and histological characteristics of patients between COLL and CELL variants. Results： （ 1 ）411 cases consisted of 4.9% COLL, 8.5% CELL, 36.9% NOS, 38.7% GTL, and 11% PH. （2）COLL and CELL were predominately in youth. The median onset age of COLL and CELL patients was （23.6 ±5.96） and （28.9 ± 15.0） years old. Mean time to biopsy were （22. 2 ±61.0） and （25.1 ±54. 3） months respectively. （3）Both COLL and CELL cases had massive proteinuria [ （9. 34 ± 5.34）g/24h and （8.60 ± 4. 44）g/24h ], while a higher ratio of patients with proteinuria ≥ 10 g/24h was present in COLL group （55.5 % ） as compared with CELL group（ 37. 1% ） （but without significant differences）. The level of serum albumin [ （ 18.2 ± 4. 06）g/L ] in CELL was lower than that in COLL [ （22. 3 ± 8.95 ）g/L ] （P 〈 0. 01 ）. （4） Both the patients with COLL and CELL FSGS had severe damage of renal tubular function （ the proportion of patients with higher urine NAG and RBP level were 95% , 100% and 73.7% ,85.3% respectively）, had the same level of serum creatinine, the proportion of renal dysfunction, the same incidence of hypertension （30% and 22. 9% respectively） and microscopic hematuria （50% and 45.7% ）. （5）There was no difference in the ratio of global glomerulosclerosis and segmental glomerulosclerosis in the two groups. The patients with COLL variant had more chronic tubular interstitial lesion and vascular lesion than that CELL variant [ （55% vs 25.7% ）, （65% vs 37. 1% ） ] （P 〈0. 05）. There were 9 cases with COLL group （45%） accompanied with cellular lesion. （6）After received therapy by Tripterygium wilfordii （TW） or combined TW with steroid, the ratio of remission and renal function recovery with COLL and CELL were 64. 3% , 68. 2% and 35.7% , 40. 9% respectively. Conclusion：The incidence of COLL and CELL was 4. 9% and 8.5% in Chinese idiopathic FSGS. There were overlapping or common clinical and pathologic features between COLL and CELL variants. The therapy by TW or combined TW with steroid had higher ratio of remission in both FSGS subgroups.
出处
《肾脏病与透析肾移植杂志》
CAS
CSCD
北大核心
2009年第2期125-132,共8页
Chinese Journal of Nephrology,Dialysis & Transplantation
基金
全军“十一五”科技攻关课题(06G040)
江苏省自然科学基金重点项目(BK2007718)
关键词
局灶节段性肾小球硬化
塌陷型
细胞型
临床病理特征
focal segmental glomerulosclerosis collapsing cellular clinical-pathological features
