Predictors of Kidney Outcomes of Anti-Glomerular Basement Membrane Disease in a Large Chinese Cohort

Abstract

<b><i>Introduction:</i></b> Anti-glomerular basement membrane (GBM) disease is a rare but the most aggressive form of glomerulonephritis. To dissect the prognostic factors, we retrospectively analyzed the clinical features of a large cohort and compared the clinical features and prognosis during decades. <b><i>Methods:</i></b> Data on clinical manifestation, treatment, and prognosis were collected. Cox models and receiver operating characteristic (ROC) curve were used to investigate the predictors for outcomes. The Kaplan-Meier curve and log-rank test were used to compare kidney and patient survival. <b><i>Results:</i></b> A total of 448 patients were enrolled. Patient survival and kidney survival at 1 year was 69.4% and 37.7%, respectively. During the past 3 decades, mortality at 3 months and 1 year significantly dropped from 37.5% and 57.1% in 1991–2000 to 2.8% and 6.9% in 2011–2020 (<i>p</i> < 0.001), respectively; kidney prognosis showed a tendency of improvement as well. Serum creatinine (Scr) on diagnosis (HR, 1.16; 95% CI, 1.05–1.29) and crescent percentage (HR, 1.73; 95% CI, 1.34–2.24) were independent predictors for end-stage kidney disease. ROC curve showed that the optimal cutoff point of Scr on diagnosis for prediction of dialysis dependency at 1 year was 536.4 μmol/L (sensitivity 88.3% and specificity 80.8%). Antineutrophil cytoplasmic antibodies (ANCAs) positivity (HR, 4.43; 95% CI, 1.72–11.38) was a predictor for mortality. Plasma exchange was associated with a better patient prognosis (HR, 0.40; 95% CI 0.16–0.95). <b><i>Conclusion:</i></b> Scr on diagnosis and percentage of crescents were predictors for kidney outcomes. Positive ANCA was a predictor for mortality. Overall patient prognosis of anti-GBM disease was improved during the past 3 decades.

Evaluation of the Risk Prediction Models in Predicting Kidney Outcomes in Antiglomerular Basement Membrane Disease

“…Several clinical indicators have been demonstrated to be independently associated with kidney survival, including the levels of anti-GBM antibodies, kidney function, oligoanuria, and dialysis at presentation in previous studies. 6 , 8 Likewise, histological parameters have been studied, highlighting the importance of the proportion of normal glomeruli and crescents. 6 , 7 , 8 In the present cohort, we found that the percentage of normal glomeruli at the time of kidney biopsy and initial need for RRT comprised the 2 strongest independent predictors for kidney outcomes, which was in line with the original publication.…”

“… 2 Approximately 60% to 80% patients entered ESKD with maintenance RRT within the first year of follow-up. 2 , 3 , 4 , 5 , 6 , 7 …”

“…Clinical indicators, such as oligoanuria, serum creatinine on diagnosis, and initial RRT, are important parameters linked to kidney outcome. 6 , 7 , 8 , 9 Kidney biopsy findings, particularly the number of normal glomeruli and the percentage of crescents, have also been identified as independent factors influencing kidney outcome. 5 , 6 , 7 , 8 Given the high risk of mortality and rapid loss of kidney function in untreated anti-GBM disease, immunosuppressive treatment should be initiated in most patients as soon as possible.…”

“… 6 , 7 , 8 , 9 Kidney biopsy findings, particularly the number of normal glomeruli and the percentage of crescents, have also been identified as independent factors influencing kidney outcome. 5 , 6 , 7 , 8 Given the high risk of mortality and rapid loss of kidney function in untreated anti-GBM disease, immunosuppressive treatment should be initiated in most patients as soon as possible. However, the decision to initiate an intensive immunosuppressive regimen should consider the low likelihood of kidney recovery in patients requiring RRT at presentation or those with a high proportion of crescents (85%–100%) on kidney biopsy, as outlined by the Kidney Disease Improving Global Outcomes guidelines.…”

Immunity in the balance: Fatal disseminated adenovirus infection in a patient undergoing plasma exchange and immunosuppressive chemotherapy for anti‐glomerular basement membrane disease

“…10,14,18 Importantly, some studies have suggested that patients with both ANCA and anti-GBM antibodies may be at higher risk of severe disease, and similar to the patient described here, this "double-positivity" may contribute to worse outcomes. [19][20][21] Nonetheless, we believe one consideration that warrants revisiting is the recommended frequency of TPE.…”