Late Renal Recovery in a Patient with Solitary Kidney and ANCA-Positive Glomerulonephritis (GN) Treated with Rituximab
- Glomerular Diseases: From Inflammation to Fibrosis - III
November 04, 2023 | Location: Exhibit Hall, Pennsylvania Convention Center
Abstract Time: 10:00 AM - 12:00 PM
Category: Glomerular Diseases
- 1401 Glomerular Diseases: From Inflammation to Fibrosis
- Kae, Soo Hyun, Trinity Health of New England, Hartford, Connecticut, United States
- Cheema, Alina, UConn Health, Farmington, Connecticut, United States
- Min, Matthew, UConn Health, Farmington, Connecticut, United States
- Geller, Ari B., Greater Hartford Nephrology, Bloomfield, Connecticut, United States
- Niranjan, Sankar Narayan, Greater Hartford Nephrology, Bloomfield, Connecticut, United States
ANCA vasculitis is the most common form of new-onset GN in adults >50. It is often associated with high rate of morbidity and mortality, especially when it causes significant renal injury requiring RRT. These patients often do not come off RRT, primarily when they have known CKD.
82yoF with CKD stage IIIa baseline SCr 0.9-1.1 with solitary right kidney due to renal cell cancer s/p left nephrectomy was found to have rapidly progressive AKI with SCr rising from 2.2 to 3.7 in a span of 2 weeks with associated dyspnea, leg edema, and fatigue. She was found to have focal necrotizing and crescentic pauci-immune GN with vascular findings consistent with vasculitis on her kidney biopsy. The crescents were limited to 4 of 46 glomeruli but had 13 globally sclerosed glomeruli with moderate interstitial fibrosis and 40-50% tubular atrophy. Along with positive c-ANCA and anti-MPO antibodies, she was confirmed to have ANCA vasculitis. Although she remained non-oliguric, her SCr continued to rise at 4.6 requiring hemodialysis (HD), which was continued even after hospital discharge. She was pulsed with steroids and was started on rituximab infusion after discharge. She was continued on rituximab and was able to come off HD after 8 months due to improvement in her SCr to 2.0-2.2 range. She remains off HD with last SCr of 2.1 from earlier this month.
ANCA vasculitis remains a rare yet lethal disease that is yet to be fully understood as it is still unknown how ANCA antibodies develop. One study has shown that only 43% of patients who required RRT at the time of diagnosis were able to come off at 1 year. The prognosis is even poorer when they have known CKD with much lower rates of meaningful kidney recovery after initiating RRT at around 5%. Xu et al. emphasize how pathologic severity may determine renal recovery for ANCA vasculitis requiring RRT at disease onset with high proportion of fibrous crescent and global glomerulosclerosis being a predictor of dialysis dependence. Our patient’s renal recovery indicates that even with advanced age, baseline CKD, and moderate tubulointerstitial involvement, it is worth treating the active vasculitis in the form of crescentic glomerular lesions with rituximab. We also make a case for biopsy of a solitary kidney when absolutely indicated despite the risks involved.