Abstract: PO1394
ANCA Negative Medullary Angiitis
Session Information
- Glomerular Diseases: Fibrosis and Extracellular Matrix
November 04, 2021 | Location: On-Demand, Virtual Only
Abstract Time: 10:00 AM - 12:00 PM
Category: Glomerular Diseases
- 1201 Glomerular Diseases: Fibrosis and Extracellular Matrix
Authors
- Kapoor, Tarun, Vanderbilt University Medical Center, Nashville, Tennessee, United States
- Birchmore, Daniel, Vanderbilt University Medical Center, Nashville, Tennessee, United States
- Lusco, Mark, Vanderbilt University Medical Center, Nashville, Tennessee, United States
- Arroyo Ornelas, Juan Pablo, Vanderbilt University Medical Center, Nashville, Tennessee, United States
- Bhave, Gautam B., Vanderbilt University Medical Center, Nashville, Tennessee, United States
Introduction
Medullary angiitis is a rare cause of renal failure. Most cases of medullary angiitis are found to be ANCA positive. We present a rare case of ANCA negative medullary angiitis with no identifiable secondary causes.
Case Description
A 55-year-old male with a medical history significant for uncontrolled diabetes complicated by retinopathy, Chronic kidney disease, HTN, and compensated cirrhosis of unclear etiology, presented to the hospital with shortness of breath and lower extremity edema. Initial workup revealed a creatinine of 7.6 (baseline creatinine 1.5 eighteen months earlier), and significant proteinuria estimated at 8.4g/day along with a urinalysis showing 3 RBC/high power field and 6 WBC/high power field. Serological workup including Hepatitis B/C, HIV, SPEP, serum free light chains, C3/C4, ANA, ANCA was negative. He underwent a renal biopsy revealing medullary angiitis with multifocal medullary hemorrhage with perivascular PMNs and eosinophils along with findings of long-standing diabetes and HTN.
In the absence of IgA deposits, infection, or offending drugs, he was started on high steroids and treated similarly to ANCA positive medullary angiitis. His creatinine during the hospitalization peaked at 8.69 before and fell to 7.6 at discharge. He was continued to oral prednisone at discharge. Outpatient follow-up showed a decrease in Cr to 5.8 before slowing climbing back up to 8.5. He is currently maintained on an immunosuppressive regimen to help delay the initiation of renal replacement therapy.
Discussion
Medullary angiitis is a rare renal disorder that has largely been associated with ANCA positivity. ANCA negative medullary angiitis has been documented in the literature with a recent case series showing IgA nephropathy and recent antibiotics use to be the most common etiologies. This is one of the first case reports of ANCA negative medullary angiitis not associated with IgA nephropathy and recent antibiotic use. Currently, we have no standardized treatment options available for these patients. Given the rapid progression of the renal dysfunction, we decided to start our patient on high-dose steroids. Our patient had an initial improvement in his renal function with subsequent decline. However, treatment may have afforded time for AVF creation and patient education before the initiation of hemodialysis, which was the main goal of therapy.