抗肾小球基底膜抗体病的诊断、预后影响因素及治疗的研究进展

Progress in diagnosis,prognostic evaluation,and treatment of anti-glomerular basement membrane antibody disease

抗肾小球基底膜(GBM)抗体病是一种以抗GBM抗体沉积于肾脏或肺泡基底膜为特点的自身免疫性血管炎疾病。典型临床表现为肾功能急剧衰竭伴或不伴弥漫性肺泡出血。其主要诊断依据为血清或组织中检测出抗GBM抗体。该病起病急,进展快,预后差,死亡率高,多数未经治疗的患者在疾病早期即进展为终末期肾脏病(ESRD)。少尿、年龄>60岁、诊断时血肌酐>500μmol/L、新月体百分数>75%、抗中性粒细胞胞质抗体(ANCA)阳性等多种因素均与患者及肾脏预后不良相关。尽早采用血浆置换、糖皮质激素及环磷酰胺联合治疗可显著改善预后。早诊断及明确影响患者和肾脏预后的相关因素有助于制定有效的治疗方案,这对控制抗GBM抗体病发展的意义重大。

Anti-glomerular basement membrane(GBM)antibody disease is an autoimmune vasculitis characterized by the deposition of anti-GBM antibody in the renal or alveolar basement membrane.The typical clinical presentation of this disease is acute renal failure with or without diffuse alveolar hemorrhage.Central to the diagnosis of this disease is the detection of anti-GBM antibody,either in circulation or in tissues.It has a fast onset,rapid progression,poor prognosis,and high mortality,and most untreated patients progress early in the course of the disease to end stage renal disease(ESRD).Oliguria,age>60 years,serum creatinine>500μmol/L at diagnosis,crescent>75%,positive anti-neutrophil cytoplasmic antibody(ANCA),and other factors are associated with a poor prognosis of the patients and kidneys.Combination therapy of plasma exchange,glucocorticoid,and cyclophosphamide can improve the prognosis if used earlier.Therefore,early diagnosing and identifying the relevant factors affecting the prognosis of the kidney and patients to develop an effective therapy is of great significance to control the development of the disease.