Abstract: SA-PO669
Anti-Glomerular Basement Membrane Disease: A Real-World Experience
Session Information
- Glomerular Diseases: ANCA, Anti-GBM, Kidney Biopsy
November 09, 2019 | Location: Exhibit Hall, Walter E. Washington Convention Center
Abstract Time: 10:00 AM - 12:00 PM
Category: Glomerular Diseases
- 1203 Glomerular Diseases: Clinical, Outcomes, and Trials
Authors
- Scott, Jennifer, Trinity Health Kidney Centre, Dublin, Ireland
- Hruskova, Zdenka, Department of Nephrology,General University Hospital and First Faculty of Medicine, Charles University, Prague 2, Czechia
- Van der laan, Sarah, Trinity Health Kidney Centre, Dublin, Ireland
- Chevarria, Julio L., Trinity Health Kidney Centre, Dublin, Ireland
- Rosenberg, Avi Z., Johns Hopkins University, Baltimore, Maryland, United States
- Bartonova, Lenka, Charles University, Prague, Czech Republic, Prague, Czechia
- Frausova, Doubravka, Charles University, Prague, Czech Republic, Prague, Czechia
- Little, Mark Alan, Trinity Health Kidney Centre, Dublin, Ireland
- Geetha, Duvuru, John Hopkins Bayview Medical Center, Baltimore, Maryland, United States
Background
Anti-glomerular basement membrane (Anti-GBM) disease is associated with deleterious renal outcomes, with the majority of patients remaining dialysis dependent. There is a paucity of evidence regarding optimal treatment and factors predicting outcomes in this cohort. We aim to describe our real-world experience and evaluate factors associated with end-stage renal disease (ESRD).
Methods
A multi-centre, retrospective cohort study was performed using existing databases from 3 centers in Ireland, Czech Republic and North America (N= 52). All patients, recruited between 1998-2018, had biopsy proven Anti-GBM disease. We describe the clinical characteristics and evaluate factors associated with ESRD using chi-square and independent sample t-tests.
Results
48 (92%) were Caucasian and 33 (64%) female, with a mean (SD) age of 58 (16) years. Table 1 depicts baseline characteristics for the total cohort stratified for presence of ESRD. 43 (83%) required renal replacement therapy (RRT) at presentation and 22 (42%) displayed ANCA positivity. Patients reaching ESRD had higher need for RRT at entry, were more often ANCA negative and had a lower percentage of normal glomeruli compared to those who did not. Overall, renal recovery occurred in 14 (33%), over a median follow-up of 39 months (IQR 79.5).
Conclusion
The need for RRT at diagnosis, ANCA negativity and a lower percentage of normal glomeruli are associated with an increased trend towards ESRD. Renal recovery occurred in 1/3 of patients, suggesting a possible beneficial role in modulating acute renal inflammation, even with apparent poor prognostic features. Individualization of treatment, stratified by prognostic factors is paramount and requires larger scale collaborative studies to explore this further.
| Variable | ESRD | ESRD | p value |
| Yes (n = 31) | No (n = 21) | ||
| Female (%) | 51 | 48 | 0.12 |
| RRT requirement at diagnosis (%) | 72 | 28 | 0.0001 |
| Alveolar haemorrhage (%) | 47 | 53 | 0.23 |
| eGFR (MDRD) at diagnosis, ml/min/1.73m2 (SD) | 7 (6) | 14 (15) | 0.06 |
| ANCA negative (%) | 70 | 30 | 0.08 |
| Percentage normal glomeruli (SD) | 5 (8) | 15 (22) | 0.05 |
| Crescentic Berden class (%) | 67 | 33 | 0.07 |
| Immunosuppression use (%) | 58 | 42 | 0.24 |
| Plasmapheresis use (%) | 60 | 40 | 0.89 |