Abstract: SA-PO693
Renal Medullary Angiitis and Arteritis in ANCA Vasculitis: Clinico-Pathologic Features and Outcomes
Session Information
- Glomerular Diseases: Clinical, Outcomes, Trials - III
November 05, 2022 | Location: Exhibit Hall, Orange County Convention Center‚ West Building
Abstract Time: 10:00 AM - 12:00 PM
Category: Glomerular Diseases
- 1303 Glomerular Diseases: Clinical‚ Outcomes‚ and Trials
Authors
- Kirby, Grant, Johns Hopkins Medicine, Baltimore, Maryland, United States
- Dasgupta, Alana, Johns Hopkins Medicine, Baltimore, Maryland, United States
- Alabdulsalam, Abdulrahman K., King Abdulaziz Medical City, Riyadh, Al Riyadh Province, Saudi Arabia
- Rosenberg, Avi Z., Johns Hopkins Medicine, Baltimore, Maryland, United States
- Geetha, Duvuru, Johns Hopkins Medicine, Baltimore, Maryland, United States
Group or Team Name
- Johns Hopkins Nephrology
Background
The hallmark of ANCA associated glomerulonephritis is a pauci-immune glomerulonephritis, with renal medullary angiitis (RMA) and arteritis (AR) reported infrequently. We sought to compare the clinico-pathologic characteristics, renal risk score (RRS) and outcomes of RMA and AR in ANCA-associated vasculitis (AAV) patients who underwent a diagnostic kidney biopsy.
Methods
AAV patients with a kidney biopsy diagnosis of RMA and AR were included. RMA was defined by the presence of interstitial hemorrhage in the medulla associated, polymorphonuclear leukocyte infiltrate and karyorrhectic debris. AR was defined by fibrinoid necrosis of the vessel wall and/or inflammatory infiltrate involving the media of the artery. Demographic, clinical and treatment details were extracted by record review. Descriptive statistics were analyzed to evaluate variables of interest.
Results
Of 136 AAV biopsies, 13 had RMA and 13 had AR. The mean (SD) age was 65 (19) yrs for RMA and 72 (9) yrs in AR. At entry all had severe disease with-GFR of ≤20. There were no differences in ANCA type, entry e-GFR, proteinuria or extra-renal vasculitis between the groups. All biopsies included cortex and medulla with a mean (SD) glomeruli of 9 (5) in RMA and 27 (30) in AR. There were no significant differences in the % of normal glomeruli, global sclerosis, glomerular necrosis, or crescents between RMA and RA. RRS was moderate/high in 69% of patients. All patients were treated with glucocorticoids and either cyclophosphamide or rituximab or mycophenolate. All patients with RMA achieved remission while 2 with AR had early mortality secondary to active vasculitis. Similar improvements in e-GFR were noted at 12 M in both groups.
Conclusion
RMA and AR was seen in 10% of biopsies in our cohort and clinically presents with severely impaired renal function. Glomerular crescents, necrosis and interstitial inflammation with less chronicity are observed in both RMA and AR. Although, majority of RMA patients respond to immunosuppression with good kidney recovery, refractory vasculitis is seen 15% of patients with AR.
Funding
- Clinical Revenue Support