摘要
目的:分析原发性干燥综合征患者肾损害的临床表现、免疫学特点、肾脏病理改变和预后。方法:回顾性分析62例原发性干燥综合征[女60例,男2例,平均年龄(36.8±10.7)岁]伴肾损害患者的临床表现和肾脏病理特征及预后。结果:88.1%患者尿渗量减低,49例(79%)存在肾小管酸中毒(RTA),其中I型44例(89.8%),II型2例(4.1%),III型3例(6.1%)。47例(75.8%)存在低钾血症,9例(14.5%)存在肾性尿崩症,1例(1.6%)存在Fanconi综合征。43例(69.4%)有蛋白尿,尿蛋白0.42～7.51 g/d[平均(1.17±1.16)g/d],其中41例(95.3%)尿蛋白<2 g/d,均为小管性蛋白尿,2例(4.7%)尿蛋白>3.5 g/d,4例(6.5%)有镜下血尿,9例(14.5%)存在慢性肾功能不全。45例(72.6%)存在高球蛋白血症。血清自身抗体阳性率高低依次为:抗SSA(86.0%),ANA(77.4%),抗SSB(52.6%),RF(39.5%),抗心磷脂抗体(21.6%),抗RNP抗体(15.3%)。肾脏病理56例为间质性肾炎,其中6例伴系膜增生及IgA沉积,2例伴肾小球膜性病变,6例肾小管间质及小球基本正常。56例平均随访(37.5±38.0)个月(中位随访时间21个月),5年、10年人肾存活率均为100%。结论:原发性干燥综合征肾损害以尿浓缩功能减低和RTA伴低钾血症最为常见,肾脏病理主要为间质性肾炎,少部分患者可有系膜增生伴IgA沉积、肾小球膜性病变,远期预后较好。
Objective： To analyze clinical manifestations, immunological characteristics, pathological changes and prognosis in primary Sjogren＇s syndrome patients with renal involvement. Methodology ：Sixty-two primary Sjogren＇s syndrome patients with renal involment,female/male 60/2 eases and average age of（36. 8 ＋ 10. 7）years old,were enrolled in this retrospec- tive study. Their clinical manifestations,renal pathological features and prognosis were analyzed. Results：88. 1% cases showed a decrease in urine osmosis,49 cases （79%） renal tubular acidosis （RTA） ,including type 1 44 cases （89. 8% ） ,type II 2 cases （4. 1%） and type Ill 3 cases （6. 1%）. 47 cases （75.8%） presented with hypokalemia. 9 cases （14.5%） with nephrogenic diabetes insipidns and one case （ 1.6% ） with Fanconi syndrome. 43 cases （69. 4% ） had urinary protein excretion from 0. 42 ＋ 7.51 g/d,of which 41 （95.3%） had proteinuria less than 2 g/d,and 2 （4.7%） more than 3.5 g/d. 4 cases （6.5%） had microscopic hematuria,9 （ 14. 5% ） chronic renal insufi3ciency, and 45 （ 72. 6% ） had hyperglobulinaemia. The positive rate of serum autoantibody levels were as follows ： anti-SSA （86. 0% ） ,ANA （77. 4% ） ,anti-SSB （52. 6% ） ,RF（39. 5% ） ,anticardiolipin antibodies （ 21.6% ）, and anti-RNP antibody （ 15. 3% ）. In histological examinations, 56 cases （ 90. 3% ） presented with interstitial nephritis,6 combined with mesangial proliferation and IgA deposition,2 combined with membranous nephropathy,and 6 （9. 7% ） presented with normal. 56 cases were followed up （37. 5 ＋38. 0） months,all of 5 and 10 years renal and patient survival were 100%. Conclusion：Urine concentration ability decline and RTA with hypokalemia were most common in primary Sj6gren＇s syndrome patients with renal involvement. The interstitial nephritis was the major pathological changes and could be combined with mesangial proliferation and IgA deposition,membranous nephropathy. Their long-term prognosis was good.
出处
《肾脏病与透析肾移植杂志》
CAS
CSCD
北大核心
2010年第3期225-229,251,共6页
Chinese Journal of Nephrology,Dialysis & Transplantation
关键词
干燥综合征
肾活检
临床病理
Sjoegren＇s syndrome renal biopsy clinical pathology
