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Abstract: SA-PO671

Therapy and Outcome of Anti-Glomerular Basement Membrane Disease: A Single-Center Experience

Session Information

Category: Glomerular Diseases

  • 1203 Glomerular Diseases: Clinical, Outcomes, and Trials

Authors

  • Lan, Ping, Kidney Hospital, the First Affiliated Hospital of Xi'an Jiaotong University, Xi'an, China
  • Xie, Liyi, Kidney Hospital, the First Affiliated Hospital of Xi'an Jiaotong University, Xi'an, China
Background

Patients with anti-glomerular basement membrane (GBM) disease are at high risk of morbidity and mortality from renal failure or severe complications. Reviews revealed one-third patients have circulating anti-neutrophil cytoplasmic antibodies (ANCA). Phospholipase A2 receptor (PLA2R) antibody can also be positive. The aim of this study was to analyze the therapy differences of patient and renal outcomes in anti-GBM disease with or without ANCA or PLA2R antibody.

Methods

We screened the patients from December 2014 to April 2019 in the First Affiliated Hospital of Xi’an Jiaotong University. The patients with anti-GBM disease were reviewed. Renal biopsies were scored for the presence of active and chronic lesions. The correlation between survival with clinical characteristics and regimens were analyzed.

Results

A total of 40 patients (18 M/ 22 F) were identified with anti-GBM glomerulonephritis. The average age was 57.4±16.8 years old. The duration of symptoms before diagnosing was 1.3±1.5 months. 35 patients (87.5%) presented with rapidly progressive glomerulonephritis. The SCr level was 10.0±5.2 mg/dL. Serological screening showed anti-GBM antibody titer 180.2±87.4 RU/mL, 11 cases with ANCA positivity (27.5%, 10 cases of pANCA-MPO, 1 case of cANCA-PR3), 5 cases with PLA2R antibody positivity (12.5%). 23 patients accepted renal biopsy. 82.6% were compatible with crescentic glomerulonephritis. 30 patients (75%) received Plasmapheresis (PE). The patients were followed up for 9.98±10.5 months (range 0.5-28.5 months). Except 3 cases lost data and 14 death, 14 patients underwent maintenance hemodialysis, 2 patients underwent peritoneal dialysis, 3 cases had kidney transplantation, renal function recovered in 4 cases and 4 cases to chronic kidney disease. Multivariate Cox regression revealed female (hazard ratio [HR], 3.21; 95% confidence interval [95% CI], 1.41 to 7.30; P=0.005) and regimen with PE (HR, 2.89; 95% CI, 1.32 to6.35; P=0.008) were independent predictors of survival. SCr >6mg/dL was a risk factor for the ESRD and all-cause mortality.

Conclusion

Unlike previous data, our study shows that 55% of the patients with anti-GBM disease were female (considered a district bias). Multivariate analysis reveals female and PE therapy are predictors of renal and patient survival while high SCr (>6mg/dL) is associated with poor renal outcome and high risk of all-cause mortality.

Funding

  • Government Support - Non-U.S.