摘要
目的 :通过对抗肾小球基膜抗体疾病 (抗 GBM疾病 )病例的回顾性分析 ,为我国抗 GBM疾病的流行病学、临床及病理表现特征、治疗现状及预后等方面提供更多资料。 方法 :回顾性分析本所肾脏病临床中心在1980～ 2 0 0 0年间 14例经肾活检证实诊断为抗肾小球基膜抗体疾病患者的临床病理资料。 结果 :该 14例抗 GBM疾病患者占全部自体肾肾活检病例的 0 14 % ,占新月体肾炎的 9 1%。 14例患者中男性 13例 ,女性 1例 ,平均起病年龄为 ( 2 6 7± 8 1)岁 ,大多患者在冬春季节起病 ,10例于发病前一月内有呼吸道感染史。 14例患者均在起病时或短期内出现急进性肾功能衰竭 ,13例需肾脏替代治疗 ;8例病程中出现咯血 ,其中 6例以咯血起病 ;全部病例均伴有不同程度的贫血 ;1例患者伴有中枢神经系统受累。肾脏受累主要表现为肉眼血尿 ( 8/ 14 )、少尿 ( 10 / 14 )或无尿性肾功能衰竭 ( 3 / 14 )。起病时血清学检查抗 GBM抗体 (间接免疫荧光法 )阳性者 13例 (另一例在病程后期出现阳性 ) ,血清C3水平均正常 ,抗核抗体、抗双链DNA抗体及抗中性粒细胞胞浆抗体均阴性。病理上均出现大量新月体形成 ,其中 13 / 14表现为新月体肾炎 (新月体比例 >50 % ) ,8/ 14表现为 10 0 %的新月体肾炎 ,4例出现明显血管袢坏死改变。
Objective:To add more data to the epidemiology documento of Chinese patients with anti glomerular basement membrane disease(A GBM disease). Methodology:A retrospective analysis was made about the epidemiological,clinical and renal pathological data of 14 patients with A GBM disease in one center in China. Results:14 hospitalized patients who received percutaneous renal biopsy were diagnosed with A GBM disease for linear IgG deposit along GBM,positive serum anti GBM antibody and clinical features of rapidly progressive glomerulonephritis with or without pulmonary hemorrhage.This group of patients consisted of 13 male and 1 female with an average age of 26 7±8 1 years.These 14 patients accounted for 0 14% of the total patients receiving renal biopsy and 9 1% of the patients diagnosed as crescentic glomerulonephritis in the same period.Respiratory tract infection was identified as an important predisposing factor of this disorder.Presenting manifestations included rapidly progressive glomerulonephritis,hemoptysis,anemia,and central nervous system involvement.Non of these patients had positive ANCA detection in the course of the disease.13 of the 14 patients needed renal replacement therapy at onset of the disease.Hemoptysis occurred in 8 patients,5 with mild hemoptysis and 3 with massive pulmonary hemorrhage primarily.Renal biopsy revealed linear IgG deposits along GBM in all the patients,and crescents above 50% in 13 of the 14 patients.Two cases received re biopsy after 1 and 2 months of treatment,and the re biopsy data showed that crecent percentages decreased and chronic injuries ensued after plasmaphoresis plus immunosuppressive treatment.Pulmonary hemorrhage resolved shortly after pulse prednisolone treatment withor without plasmaphoresis.Patients plasmaphoresis plus immunosuppressive treatments seemed to have better chance of renal improvement as compared with those who received immunosuppressive treatment only. Conclusion:This analysis of 14 cases is a represent of the epidemiology of A GBM disease in China.The disease is a relatively rare but quite grave disorder especially in young male population in China.Progressive renal failure is prominent and closely associated with the prognosis,while severe pulmonary involvement occuring adequare treatment should be taken.
出处
《肾脏病与透析肾移植杂志》
CAS
CSCD
2001年第3期211-217,共7页
Chinese Journal of Nephrology,Dialysis & Transplantation
关键词
抗-GBM疾病
流行病学
新月体肾炎
肺出血
anti glomerular basement disease crescentic glomerulonephritis plasmaphoresis
