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>500 μmol/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.