抗肾小球基底膜病的临床特点分析

The clinical characteristics analysis of anti-glomerular basement membrane disease

目的探讨抗肾小球基底膜(GBM)病的临床和病理特点。方法对该院近3年来确诊的22例抗GBM病患者进行回顾性分析。结果 (1)患者平均年龄(63±21)岁,其中12例为男性;(2)16例患者同时进行了血清抗中性粒细胞胞浆抗体(ANCA)的检测,其中2例为抗GBM抗体和ANCA双阳性;(3)8例患者有完整的临床和病理资料,其中8例患者均有贫血及血肌酐的升高,7/8例患者有血尿和蛋白尿;(4)4例患者行肾活检,3/4例为新月体肾炎。免疫荧光呈典型的IgG、C3沿肾小球毛细血管壁线性沉积或颗粒样分布;(5)经免疫抑制治疗后,1例患者病情得到完全缓解,2例患者肾功能明显改善,脱离透析,2例患者肾功能部分缓解,仍需进行透析治疗,2例因并发终末期肾病仍需进行透析治疗,1例患者因合并肺出血而死亡。结论抗GBM病多发于老年人,免疫抑制疗法对其有较好的治疗效果,早诊断、早治疗可明显改善患者预后。

Objective To explore the clinical and pathological characteristics of anti-glomerular basement membrane（GBM）disease.Methods The clinical data of 22 patients with anti-GBM disease diagnosed in our hospital in recent three years were retrospectively analyzed.Results（1）The average age of patients were（63±21）years and 12 of the 22 patients were male;（2）16 of the 22 patients detected anti-neutrophil cytoplasmic antibody（ANCA）at the same time and 14 of the 16 patients had anti-GBM antibody positive only,the other two patients were both anti-GBM antibody and ANCA positive;（3）Among the 22 patients,8 of them with complete clinical and pathological data and all of them had anemia and elevated level of serum creatinine,7 of 8 patients had hematuria and proteinuria;（4）Renal biopsies were performed in 4 patients and 3 of 4 patients were crescentic glomerulonephritis.And typical IgG and C3 linear deposition along glomerular capillary walls or granular deposition with immunofluorescence examination;（5）After immunosuppressive therapy,1 patient received complete remission,2 patients received obvious improved and don′t need to dialysis any more,2 patients received partial remission and need to remain dialysis,2 patients still need to dialysis treatment as combined with end-stage renal disease and 1 patient died as combined with pulmonary hemorrhage.Conclusion Anti-GBM disease are mainly elderly people and immunosuppressive therapy has better therapeutic effect on anti-GBM disease and early diagnosis and treatment can improve the prognosis significantly.