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目的 :探讨肺出血肾炎综合征 (GPS)的发病机理、诊断和治疗。方法 :观察 2例GPS患者并文献复习 ,总结GPS的临床表现、病理改变、血清学变化和影像学特点。结果 :GPS的主要临床表现为咯血和急进性肾炎 ;肾 (肺 )活检免疫荧光检查显示肾小球基底膜或 (和 )肺毛细血管基底膜IgG、C3 线样沉积 ;血清抗肾小球基底膜抗体阳性 ;影像学上主要表现为中下肺野中外带腺泡结节及其融合斑片状或大片状阴影。结论 :了解GPS临床表现、病理特点、血清学和影像学改变 ,可提高GPS的确诊率。
Objective: To investigate the pathogenesis, diagnosis and treatment of pulmonary hemorrhagic nephritis syndrome (GPS). Methods: Two patients with GPS were reviewed and reviewed. The clinical manifestations, pathological changes, serological changes and imaging features of GPS were summarized. Results: The main clinical manifestations of GPS were hemoptysis and acute nephritis; renal (lung) biopsy immunofluorescence showed glomerular basement membrane or (and) pulmonary capillary basement membrane IgG, C3 linear deposition; serum anti-glomerular basement Membrane antibody positive; imaging mainly in the middle and lower lung field in the alveolar nodules and fusion patchy or large patchy shadow. Conclusion: Understanding of the clinical manifestations of GPS, pathological features, serological and imaging changes can improve the diagnosis of GPS.