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目的分析原发性干燥综合征(pSS)合并肾脏损害的病理特点及其与临床的关系。方法采用半定量分析方法对比研究16例有肾脏损害pSS患者的肾活检病理,分析肾小管、间质、肾小球病理变化间相互关系及肾脏病理变化与临床特征、唇腺病理相关性。结果16例患者肾脏组织病理分类:间质性肾炎(IN)8例(50%),其中单纯IN 5例,合并IN 3例;弥漫系膜增殖性肾小球肾炎(MePGN)3例;轻度系膜增殖性肾小球肾炎3例;局灶节段硬化性肾小球肾炎(FSGS)2例;膜增殖性肾炎(MPGN)1例;膜型肾病(MN)2例。免疫荧光检查发现系膜区、肾小球、肾小管基底膜、间质可有IgG、IgA、IgM、C3、C1q沉积。85%以上病例存在间质局灶炎症且有弥漫分布的细胞浸润。临床存在肾小管酸中毒(RTA)患者11例(68.8%),其中8例有IN,肾功能不全2例患者中,1例为MN,1例同时有IN、FSGS。肾小球硬化率与血管壁增厚有关(r=0.77,P=0.001),与肾小管萎缩、间质纤维化无关。肌酐清除率与调整后慢性肾小管间质肾炎(CTIN)值有关(r=-0.52,P=0.04),肌酐清除率与肾小球硬化率有关(r=-0.53,P=0.04)。唇腺活检淋巴细胞灶数,与肾脏炎细胞灶数无相关(r=0.28,P=0.30)。结论pSS合并肾脏损害并非单一间质性肾炎,肾小球病变也不少见,且不完全继发于间质性肾炎,部分病例较重,及时活检有助合理治疗和判断预后。
Objective To analyze the pathological characteristics of primary sjogren syndrome (pSS) combined with renal damage and its relationship with clinical features. Methods The semi-quantitative analysis was used to compare the renal biopsy pathology of 16 patients with pSS with renal damage. The correlation between pathological changes of renal tubules, interstitium and glomeruli was analyzed. The pathological changes and clinical features of renal pathology were also analyzed. Results Sixteen patients had pathological classification of renal tissue: interstitial nephritis (IN) in 8 cases (50%), including 5 cases of IN alone, 3 cases of IN, 3 cases of diffuse mesangioproliferative glomerulonephritis (MePGN), 3 cases of mild 3 cases of mesangial proliferative glomerulonephritis; 2 cases of focal segmental glomerulonephritis (FSGS); 1 case of proliferative glomerulonephritis (MPGN); 2 cases of membranous nephropathy (MN). Immunofluorescence revealed mesangial area, glomerular, tubular basement membrane, interstitial may have IgG, IgA, IgM, C3, C1q deposition. More than 85% of cases exist interstitial inflammation and diffuse distribution of cell infiltration. Eleven patients (68.8%) had renal tubular acidosis (RTA), of whom 8 had IN, 2 had renal insufficiency, 1 had MN, and 1 had both IN and FSGS. The rate of glomerulosclerosis was related to the thickening of the vessel wall (r = 0.77, P = 0.001), which was not associated with tubular atrophy and interstitial fibrosis. Creatinine clearance was related to adjusted CTIN (r = -0.52, P = 0.04). Creatinine clearance was associated with glomerular sclerosis (r = -0.53, P = 0.04). Labial gland number of lymphatic foramina was not correlated with the number of nephritic foci (r = 0.28, P = 0.30). Conclusions The pSS combined with renal impairment is not single interstitial nephritis, glomerular lesions are not uncommon, and not completely secondary to interstitial nephritis, some cases of heavier, timely biopsy can help rational treatment and prognosis.