IgA肾病肾病综合征的临床和病理研究

目的：了解IgA肾病肾病综合征的临床和病理的特点及其与预后的关系。方法：观察72例经肾活检确诊为IgA肾病表现为肾病综合征的患的临床表现、实验室检查、病理改变、免疫组化、组织学定量分析及其与转归的关系。结果：患常见表现有高血压、肉眼和镜下血尿、贫血、氮质血症，病理改变多样；治疗后有22例完全缓解，15例部分缓解，16例呈持续性非肾病性蛋白尿，8例呈持续肾病性蛋白尿，10例发展为肾功能衰竭；其转归或激素敏感性与临床表现、蛋白尿选择性、小球病理形态、区膜区增宽和免疫沉着、小管间质病变及肾间质T淋巴和单核细胞分布显相关。结论：IgA肾病肾病综合征可有不同预后，其预后与临床表现、实验室发现、病理特点和免疫发病机理有关。

Clinico-pathological Study on IgA Nephropathy Presenting with Nephrotic Syndrome

Objective:To study the clinical and pathological features of IgA nephropathy presenting with nephrotic syndrome and their relationships with prognogsis.Methods:Clinical,laboratory,pathological,immuo-historical and morphometric studies were performed in 72 biopsied patients with IgA nephropathy presenting with nephrotic syndrome and their associations with outcome were analyzed.Results:These patients had high incidence of hypertension,gross and microscopic hematuria,azotemia,anemia and various pathological manifestations.Complete remission,partial remission,persistent non-nephrotic range proteinuria,persistent nephrotic range protein and progress of chronic renal feilure were found in 22,15,16,8 and 10 patients respectively.patient outcomes were associated with clinical menifestations,laboratory findings and pathological features.Steroid-responsive patients had a lower incidence of glomerular sclerosis than non-responder.Their glomerular area,mesangial matrix index,interstitial collagen area density,diffusion distance from capillary to tubules,CD + 4 cell density were significantly less than those in the responders,but urinary space index,proximal epithelial cell area,glomerular capillary number and monocyte cell density were significantly higher in the responders than those in the non-responders.Conclusions:Nephrotic synerome of IgA nephropathy had different outcomes that are associated with clinical,pathological features and immune pathogenesis.