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目的:研究原发性干燥综合征合并肾脏损伤患者临床表现、免疫学特征、病理表现及疗效,以期对患者进行更好的诊断及治疗,改善预后。方法:收集2002年1月1日至2016年3月31日在我院肾脏内科就诊,并符合2002欧美共识小组(AECG)诊断标准的141例原发性干燥综合征肾脏损伤患者,回顾性分析其临床表现、免疫学特征、病理表现及疗效。结果:141例患者中抗干燥综合征抗原A(SSA)抗体(+)92例(65.3%),抗干燥综合征抗原B(SSB)抗体(+)62例(44.0%),血清免疫球蛋白G(Ig G)升高122例(86.5%),抗核抗体(ANA)(+)75例(53.2%),类风湿因子(RF)升高37例(26.2%)。确诊非霍奇金淋巴瘤1例[腮腺黏膜相关淋巴组织(MALT)淋巴瘤]。肾小管酸中毒(RTA)94例(66.7%),其中Ⅰ型(远端)RTA 82例(58.2%),以低钾乏力或麻痹为首发症状59例(41.8%)。蛋白尿93例(66.0%),其中24 h蛋白尿<1 g者69例(48.9%)。肾功能处于慢性肾脏病(CKD)1期38例(27.0%),2期44例(31.2%),3期41例(29.1%),4期16例(11.4%),5期2例(1.4%)。56例患者接受肾活检,其中肾小管间质性肾炎41例(73.2%),肾小球病变以Ig A肾病及局灶节段性肾小球硬化为最常见的2种类型。89例伴肾功能不全、高血清免疫球蛋白或肾脏病理提示炎细胞重度浸润的患者接受单纯激素或激素联合免疫抑制剂治疗,肌酐由(118.50±72.55)μmol/L降至(98.19±41.55)μmol/L(P=0.025),24 h尿蛋白定量由901.50(509.25,1 577.00)mg降至206.50(94.75,328.75)mg(P<0.001),血清Ig G水平由(24.54±9.92)g/L降至(16.81±4.93)g/L(P<0.001)。结论:远端RTA是干燥综合征肾累及患者常见临床表现,且常以低钾乏力或麻痹为首发症状。肾功能不全发生率也较高,在肾功能不全、高免疫球蛋白血症或肾脏病理提示炎细胞浸润较重的患者中进行适当的免疫抑制治疗可减少患者蛋白尿,改善其免疫学状况及肾功能。
Objective: To study the clinical manifestations, immunological characteristics, pathological findings and therapeutic effects of patients with primary Sjogren’s syndrome complicated with renal injury, in order to better diagnose and treat patients and improve the prognosis. Methods: A total of 141 patients with primary Sjögren’s syndrome who had kidney injury who were treated in our hospital from January 1, 2002 to March 31, 2016 and met the diagnostic criteria of 2002 European and American Consensus Group (AECG) were retrospectively analyzed Its clinical manifestations, immunological characteristics, pathology and efficacy. Results: Among the 141 patients, 92 (65.3%) were anti-SSR antibodies (+), 62 (44.0%) were anti-SSR antibodies (+), serum immunoglobulin 122 cases (86.5%) of G (Ig G) were elevated, 75 cases (53.2%) of antinuclear antibody (ANA) (+) and 37 cases (26.2%) of rheumatoid factor (RF). One case of non-Hodgkin’s lymphoma confirmed [MALT lymphoma of the parotid gland]. Ninety-four patients (66.7%) had renal tubular acidosis (RTA), of which 82 (58.2%) were type I (distal) RTAs and 59 (41.8%) had first symptoms of hypokalemia or paralysis. There were 93 cases (96.0%) of proteinuria, of which 69 cases (48.9%) had 24 hours proteinuria <1 g. Renal function was in CKD stage 1 38 cases (27.0%), stage 2 44 cases (31.2%), stage 3 41 cases (29.1%), stage 4 16 cases (11.4%), stage 2 1.4%). Fifty-six patients underwent renal biopsy, including 41 cases of tubulointerstitial nephritis (73.2%), the most common type of glomerulonephritis with IgA nephropathy and focal segmental glomerulosclerosis. 89 patients with renal insufficiency, high serum immunoglobulin or renal pathology prompted severe infiltration of inflammatory cells in patients with hormone or hormone therapy combined with immunosuppressive therapy, creatinine decreased from (118.50 ± 72.55) μmol / L to (98.19 ± 41.55) (24.54 ± 9.92) g / L, P <0.001). The levels of serum Ig G decreased from 901.50 (509.25, 1577.00) mg to 206.50 (94.75, L to (16.81 ± 4.93) g / L (P <0.001). Conclusion: Far-end RTA is a common clinical manifestation in patients with Sjogren’s syndrome and often presents with hypokalemia or paralysis as the first symptom. The incidence of renal insufficiency is also high. Appropriate immunosuppressive therapy in patients with renal insufficiency, hyper-immunoglobulinic acid, or renal pathology suggestive of severe infiltration of inflammatory cells can reduce proteinuria in patients and improve their immunological status and Kidney function.