抗肾小球基底膜病临床特点分析

发布时间：2018-02-24 01:29

本文关键词： 抗肾小球基底膜抗体肾脏病理抗中性粒细胞胞浆抗体贫血血浆置换　出处：《吉林大学》2014年硕士论文　论文类型：学位论文

【摘要】：目的探讨抗肾小球基底膜(GBM)病的特点，以加深对该组疾病的认识，为临床诊疗提供帮助，提高早期诊断率，早期治疗，降低死亡率，改善预后。方法对我科近3年来检测出的19例抗GBM抗体相关疾病患者的临床病理资料进行回顾性分析。结果 1、19例患者临床表现轻重不一，15例表现为急性肾炎综合征伴急进性肾衰竭，进行性贫血，无肾功能正常患者，3例伴有肺出血，1例住院期间死亡。 2、13/19例(68.4%)患者为单纯抗GBM抗体阳性，3/19例(15.8%)抗GBM抗体伴抗中性粒细胞胞浆抗体(ANCA)阳性，3/19例(15.8%)抗GBM抗体伴某种抗核抗体(ANA)阳性，其中1例同时伴抗SSa（++）、抗组蛋白抗体（++），1例抗52ka抗体（++）、抗组蛋白抗体（++），，1例抗组蛋白抗体（++）。伴ANCA阳性组ANCA抗体浓度较低，与其他两组相比，发病年龄较大，吸烟率高，其中一例为19例患者中病情最重（重度贫血、抗GBM抗体转阴时间最长50天、血浆置换次数最多24次，环磷酰胺冲击6次）。 3、进行激素联合环磷酰胺、血浆置换的18例患者中，4例初次就诊时肌酐＜500umol/L，14例初次就诊时肌酐＞500umol/L。 4、13例患者行肾活检，11/13例为新月体肾炎，1例新月体肾炎伴急性肾小管损伤，1例为轻度系膜增生性肾小球肾炎。 9例患者免疫荧光为典型的IgG、C3沿肾小球毛细血管壁线样沉积。3例免疫荧光沿系膜区及毛细血管壁颗粒样分布，1例仅存在系膜区免疫复合物沉积。 5、11例患者入院时存在肺部感染，并使用抗生素治疗；出院后，维持透析患者均为轻度贫血。结论 1.抗GBM抗体相关疾病的临床表现轻重不一。 2.肾脏免疫病理表现一部分为典型的IgG、C3沿肾小球毛细血管攀呈线样沉积，一部分表现为颗粒样沉积，另存在一定特殊的其他类型病理表现。 3.血浆置换，糖皮质激素、环磷酰胺联合免疫治疗对该组疾病效果较好，早期诊断，及时强化免疫抑制治疗对改善患者预后至关重要。 4.抗GBM病导致终末期肾病靠透析治疗的患者贫血程度较轻。 5.少数患者合并某种ANA抗体阳性，结合患者入我院前曾多处就诊，服用药物有关。
[Abstract]:Purpose. To explore the characteristics of anti-GBM disease in order to deepen the understanding of the disease, to provide help for clinical diagnosis and treatment, to improve the rate of early diagnosis, early treatment, reduce mortality and improve prognosis. The clinicopathological data of 19 patients with anti GBM antibody associated diseases in recent 3 years were analyzed retrospectively. Results. 1the clinical manifestations of 19 patients with acute glomerulonephritis with progressive renal failure and progressive anemia were 15 cases with acute nephritis syndrome, 3 cases with normal renal function and 1 case with pulmonary hemorrhage. 213 / 19 / 19 cases (68.4%) patients were only positive for anti GBM antibody and 3 / 19 cases for anti GBM antibody plus anti neutrophil cytoplasmic antibody (ANCA) positive for 3 / 19 cases.) Anti GBM antibody with some antinuclear antibody (Ana) was positive in 19 / 3% of the patients with anti GBM antibody and anti neutrophil cytoplasmic antibody (ANCA) positive in 3 / 19 cases. Among them, one case was accompanied by anti-SSaA, anti-histone antibody (AHA) and anti-histone antibody (anti-histone antibody) in 1 case, and anti-histone antibody (AHA) in 1 case. The concentration of ANCA antibody was lower in the positive group with ANCA. Compared with the other two groups, the age of onset was older and the smoking rate was higher. One of the 19 patients had the most serious condition (severe anemia, the longest negative period of anti-#en2# antibody was 50 days). The number of plasma exchange was up to 24 times and cyclophosphamide shock 6 times. (3) in 18 patients with plasma exchange, the creatinine was less than 500 umoll / L in 14 of the 18 patients who were treated with hormone and cyclophosphamide for the first time, and the creatinine was > 500 umol/ L in 14 patients at the first visit. 4 Renal biopsy was performed in 13 patients with crescene glomerulonephritis (n = 13), crescentin nephritis (n = 1), acute tubular injury (n = 1) and mild Mesangial proliferative glomerulonephritis (n = 1). Immunofluorescence of 9 patients was typical IgG C 3 deposition along the glomerular capillary wall. 3 cases had immunofluorescence distribution along the Mesangial area and capillary wall granulocyte. Only 1 case had the Mesangial area immune complex deposition. Five hundred and eleven patients had pulmonary infection on admission and were treated with antibiotics, and maintenance dialysis patients were mild anemia after discharge. Conclusion. 1. The clinical manifestations of anti-GBM antibody-related diseases are different. 2. Some of the renal immunopathologic manifestations were linear deposition along the glomerular capillary climbing, some were granular deposition, and some other special pathological manifestations. 3.The effect of plasma exchange, glucocorticoid and cyclophosphamide combined immunotherapy is good. Early diagnosis and timely intensive immunosuppressive therapy are very important to improve the prognosis of the patients. 4. Anemia in patients with end-stage nephropathy treated by dialysis was mild as a result of anti-GBM disease. 5. A few patients with some ANA antibody positive, combined with the patients before entering our hospital have many visits, drug-related.
【学位授予单位】：吉林大学
【学位级别】：硕士
【学位授予年份】：2014
【分类号】：R692.6

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