肾小球毛细血管壁损伤蛋白尿.PPT

肾小球损伤的机制 抗体介导的免疫损伤：主要通过补体和白细胞介导的途径发挥作用 大多数抗体介导的肾炎由循环免疫复合物沉积引起，免疫荧光呈颗粒状 抗GBM成分的自身抗体可引起抗GBM性肾炎，免疫荧光呈线性分布 抗体可与植入肾小球的抗原发生反应，形成原位免疫复合物，免疫荧光呈颗粒状 Acute Proliferative GN Clinical Manifestation Acute Nephritic Syndrome 血尿：30%肉眼血尿，红细胞管型 水肿：肾小球滤过率降低，水、钠潴留 高血压：轻-中度，水、钠潴留，血容量增加 95% of children completely recover 30-50% of adults suffer progressive renal disease Rapidly Progressive GN Crescentic GN Characterized clinically by rapid progressive loss of renal function with severe oliguria Death from renal failure within weeks to months if untreated Is a syndrome associated with severe glomerular injury and does not denote a specific etiologic form of glomerulonephritis Type I RPGN (Anti-GBM Antibody) Idiopathic Goodpasture syndrome (肺出血-肾炎综合征） Type II RPGN (Immune Complex) Idiopathic Postinfectious Systemic lupus erythematosus Henoch-Sch?nlein purpura (IgA) Others Type III RPGN (Pauci-Immune) ANCA associated Idiopathic Wegener granulomatosis Microscopic polyarteritis Morphology The kidneys are enlarged and pale, often with petechial hemorrhages on the cortical surfaces Morphology Crescent formation by proliferation of parietal cells and infiltration of WBC’s fibrin deposition in Bowman’s space EM reveals focal disruptions in the GBM Clinical Manifestation acute nephritic syndrome occasionally, nephrotic Syndrome ANCA, anti-GBM antinuclear Ab’s are helpful in diagnosis Poor prognosis steroids, cytotoxic drugs longterm dialysis or renal transplantation 肾病综合征相关肾炎 Membranous Nephropathy Membranous Nephropathy Main cause of nephrotic syndrome in adults Characterized by diffuse thickening of the GBM the accumulation of electron-dense immunoglobulin-containing deposits Primary membranous GN (85% of cases) Secondary membranous GN Drugs (e.g., penicillamine, NSAIDs) Malignancies (esp. carcinoma of lung colon, melanoma) SLE Infections (e.g. hepatitis B C, syphilis) Hashimoto thyroiditis Primary membranous GN