Abstract: SA-PO670
Intravenous Cyclophosphamide for Treatment of Anti-GBM Disease
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
- Lorde, Nathan R., Epsom and St Helier University Hospitals NHS Trust, Sutton, United Kingdom
- Harris, Fiona E., Epsom and St Helier University Hospitals NHS Trust, Sutton, United Kingdom
- Condon, Marie B., Imperial College Healthcare, Surrey, United Kingdom
- Sood, Bhrigu Raj, Epsom and St Helier University Hospitals NHS Trust, Sutton, United Kingdom
- Makanjuola, David, St. Helier Hospital, Carshalton, Surrey, United Kingdom
Background
Anti-GBM disease is a condition caused by antibodies to type IV collagen. We report a single centre experience of the use of pulsed intravenous cyclophosphamide (IV Cyc), steroids and plasma exchange (PEX) for induction treatment. There is a paucity of data on the use of IV Cyc for induction. Standard therapy involves daily oral cyclophosphamide.
Methods
Retrospective review of patient records from Jan 2006 to Dec 2018. Primary aim was to compare the outcomes of our treatment regimen on mortality and preservation of renal function with published outcomes.
Results
33 patients were identified. Complete data was not available in 4. Of the remaining 29,
24 had renal disease, 2 had pulmonary disease and 3 had both renal and pulmonary involvement. Iv Cyclophosphamide was dosed adjusted for age and renal function. Maintenance was predominantly with steroids and Azathioprine. The median age was 67 years (range 17-86), 41% were female. Plasma exchange was done in 25 patients who received between 4 and 25 sessions.
14 patients were dialysis dependent at presentation. Renal survival was 10% in dialysis dependent patients and in those who did not require dialysis, it was 75%.
Patient survival was 90% at 1-year for treated patients. 8 out of 29 patients were deemed too frail to receive induction therapy.
14 of 27 (52%) with renal involvement required dialysis at the time of discharge after their first presentation. 3 of the 13 dialysis independent patients later went on to require dialysis.
Conclusion
Published data suggests 1 year patient survival of 87 to 100% and 1 year renal survival of 94% in those with creatinine < 500 umol/1-3. Our cohort had higher proportion of patients who were dialysis dependent at presentation.Our data suggest that pulsed intravenous cyclophosphamide is as effective a therapy as oral cyclophosphamide with comparable outcomes at 1 year. This may make it a suitable option when consideration to cumulative dose exposure is made. As it is a rare disease further collaborative prospective study with other centres will likely be needed.
1. Huart A et al. J Autoimmun 2016;73:24-29
2. Alchi B et al Nephrol Dial Transplant. 2015;30(5):814-821.
3. Levy JB et al Ann Intern Med. 2001;134(11):1033