Objective:To report two cases of anti-glomerular basement membrane (GBM) disease with IgA nephropathy (IgAN), and summarize the clinical characteristics of these patients through systematic literature review, for providing clinical data for the diagnosis and treatment of this disease.Methods:It was a case-series analysis. Clinical data of two cases with anti-GBM disease and IgAN diagnosed in the First Affiliated Hospital of Guangxi Medical University were collected. The course of their diagnosis and treatment was described. Besides, the relevant literature of anti-GBM disease with IgAN cases reported in PubMed and CNKI databases from the establishment of the database to June 30, 2024 were retrived, and the relevant clinical data were extracted for analysis and summary. Patients were divided into end-stage renal disease (ESRD) group and non-ESRD group according to renal outcome, and the differences in age, gender, hemoglobin, creatinine at diagnosis, proportion of hypuria/anuria, qualitative grade of urinary protein, and proportion of renal crescent were compared between the two groups.Results:Two patients were both young women. The pathology of renal tissue suggested crescentic glomerulonephritis. The pathological manifestations were linear deposition of IgG along GBM and deposition of immune complex dominated by IgA in mesangial region. At the initial diagnosis, the renal function damage of the case one was not severe. After plasma exchange, glucocorticoid and cyclophosphamide treatment, the renal function recovered. The kidney function of the case two was obviously impaired at the initial diagnosis, and was improved after the above treatment, but aggravated due to infection, and continued to progress into ESRD. Twenty-six literates involving 40 cases of anti-GBM disease with IgAN were retrieved. A total of 42 cases of anti-GBM disease with IgAN, including 2 cases in this report, were analyzed and summarized. The age was (41.3±17.5) years old, 45.2% were male, 39 cases (92.9%) were from Asia. The hemoglobin was (94.4±15.1) g/L, the serum creatinine was 450.8 (284.4, 755.8) μmol/L, 83.3% (35/42) patients had crescent ratio greater than 50% and 37 patients (88.1%) patients were treated with intravenous methylprednisolone and cytotoxic immunosuppressive drugs. Plasma exchange was performed in 34 patients (81.0%) patients, 23 patients (54.8%) had renal function recovered and 17 patients (40.5%) entered ESRD. Compared with the non-ESRD group, the serum creatinine in the ESRD group was higher at initial diagnosis ( P<0.01), and the proportion of hypuria/anuria was higher ( P=0.03). There were no statistically significant differences in age, sex, hemoglobin, urinary protein and crescent proportion between the two groups (all P>0.05). Conclusions:Compared with anti-GBM disease, anti-GBM with IgAN has a smoother course and a better prognosis. Infection should be actively prevented during treatment.

Objective:To report two cases of anti-glomerular basement membrane (GBM) disease with IgA nephropathy (IgAN), and summarize the clinical characteristics of these patients through systematic literature review, for providing clinical data for the diagnosis and treatment of this disease.Methods:It was a case-series analysis. Clinical data of two cases with anti-GBM disease and IgAN diagnosed in the First Affiliated Hospital of Guangxi Medical University were collected. The course of their diagnosis and treatment was described. Besides, the relevant literature of anti-GBM disease with IgAN cases reported in PubMed and CNKI databases from the establishment of the database to June 30, 2024 were retrived, and the relevant clinical data were extracted for analysis and summary. Patients were divided into end-stage renal disease (ESRD) group and non-ESRD group according to renal outcome, and the differences in age, gender, hemoglobin, creatinine at diagnosis, proportion of hypuria/anuria, qualitative grade of urinary protein, and proportion of renal crescent were compared between the two groups.Results:Two patients were both young women. The pathology of renal tissue suggested crescentic glomerulonephritis. The pathological manifestations were linear deposition of IgG along GBM and deposition of immune complex dominated by IgA in mesangial region. At the initial diagnosis, the renal function damage of the case one was not severe. After plasma exchange, glucocorticoid and cyclophosphamide treatment, the renal function recovered. The kidney function of the case two was obviously impaired at the initial diagnosis, and was improved after the above treatment, but aggravated due to infection, and continued to progress into ESRD. Twenty-six literates involving 40 cases of anti-GBM disease with IgAN were retrieved. A total of 42 cases of anti-GBM disease with IgAN, including 2 cases in this report, were analyzed and summarized. The age was (41.3±17.5) years old, 45.2% were male, 39 cases (92.9%) were from Asia. The hemoglobin was (94.4±15.1) g/L, the serum creatinine was 450.8 (284.4, 755.8) μmol/L, 83.3% (35/42) patients had crescent ratio greater than 50% and 37 patients (88.1%) patients were treated with intravenous methylprednisolone and cytotoxic immunosuppressive drugs. Plasma exchange was performed in 34 patients (81.0%) patients, 23 patients (54.8%) had renal function recovered and 17 patients (40.5%) entered ESRD. Compared with the non-ESRD group, the serum creatinine in the ESRD group was higher at initial diagnosis ( P<0.01), and the proportion of hypuria/anuria was higher ( P=0.03). There were no statistically significant differences in age, sex, hemoglobin, urinary protein and crescent proportion between the two groups (all P>0.05). Conclusions:Compared with anti-GBM disease, anti-GBM with IgAN has a smoother course and a better prognosis. Infection should be actively prevented during treatment.