【摘要】 目的通过1例典型病例分析结合文献资料的复习探讨膜性肾病合并抗肾小球基底膜(glomerular basement membrane,GBM)病的临床表现、发病机制、治疗方法和预后。方法对本院收治的1例膜性肾病合并抗GBM病患者的病理特点和临床治疗进行回顾性分析,复习相关文献资料。结果本例患者青年男性,既往体健,有吸烟病史,感染20 d后出现肺出血、肉眼血尿、大量蛋白尿、低蛋白血症、浮肿、血肌酐轻度增高,抗GBM抗体阳性,肾脏病理为膜性肾病合并有抗GBM病伴有亚急性肾小管间质肾病。前期给予甲泼尼龙0.5 g/d,共4 d,环磷酰胺0.4 g/d、0.6 g/d,连续2 d冲击治疗后尿蛋白明显减少,肉眼血尿消失,但肾功能和血色素无改善,同时出现胸闷、气短,活动后加重等症状,胸片显示肺出血加重,经8次双重血浆置换、配合激素、环磷酰胺治疗后,抗GBM抗体转为阴性,肺出血完全控制,尿蛋白定量明显降低,肾功能正常。结论膜性肾病合并抗GBM病较少见,临床容易误诊或漏诊,治疗不及时多预后不佳,尤其合并肺出血者病情变化快。对临床可疑者需尽早行抗GBM抗体或肾穿刺检查明确诊断。对伴有肺出血、肾功能轻度受损者经积极血浆置换和激素、环磷酰胺治疗可改善预后。更多还原

【Abstract】 Objective The clinical manifestations,treatment methods,pathogenesis and prognosis of rare membranous nephropathy combined with anti glomerular basement membrane disease were discussed through 1 typical case analysis and literature review. Methods The pathological characteristics and clinical treatment of one cases of membranous nephropathy combined with anti glomerular basement membrane disease were retrospectively analyzed,and the related literature was reviewed. Results The patients were young men and past physical health,smoking history,Twenty days after infection appeared pulmonary hemorrhage,gross hematuria,large proteinuria,hypoproteinemia,edema,blood creatinine slight increase,anti GBM antibody positive renal pathology as membranous nephropathy with anti GBM disease with sub acute tubular interstitial nephropathy. Pre given methylprednisolone 0. 5 g/d. A total of four days,cyclophosphamide 0. 4 g/d,0. 6 g/d,continuous two days after shock treatment urinary protein was significantly reduced,the naked eye hematuria disappeared,but renal function and hemoglobin no improvement,also appear chest tightness,shortness of breath,activities after aggravating symptoms such as chest radiograph shows pulmonary blood increased,urine protein significantly reduced by eight double plasma exchange,with the hormone,cyclophosphamide,anti GBM antibody turn negative and pulmonary hemorrhage in complete control and renal function normal. Conclusions Membranous nephropathy combined with anti GBM disease is rare,clinical easy to misdiagnosis ormissed diagnosis,treatment is not timely prognosis is not good,especially with the rapid change of the patient’s condition. The diagnosis of clinical suspected as early as possible to anti GBM antibody or renal biopsy. The prognosis of patients with pulmonary hemorrhage,mild impairment of renal function by positive plasma exchange and hormone and cyclophosphamide therapy.更多还原