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Abstract: FR-PO0791

A Case of Anti-Glomerular Basement Membrane Disease with Possible Secondary ANCA-Associated Vasculitis in a Patient with HIV

Session Information

Category: Glomerular Diseases

  • 1402 Glomerular Diseases: Clinical, Outcomes, and Therapeutics

Authors

  • Koch, Allegra Perkins, University of California Irvine School of Medicine, Irvine, California, United States
  • Gee, Caroline A., University of California Irvine School of Medicine, Irvine, California, United States
  • Hanna, Ramy Magdy, UCI Health, Orange, California, United States
Introduction

Anti-glomerular basement membrane disease is a rare autoimmune disorder in which antibodies attack collagen in the kidneys and lungs. ANCA-vasculitis is another rare autoimmune disorder in which antibodies damage small and medium vessels, including those in the kidneys. We present a rare case with elements of both these diseases in the setting of existing HIV infection

Case Description

A 42-year-old female with a history of CKD3, HIV (on dolutegravir/rilpivirine), and hypertension presented to the hospital with acute-on-chronic renal injury. Her presenting complaint was a two-day history of vomiting and poor PO tolerance, preceded by two months of left-sided flank pain. The patient’s vital signs were within normal limits other than a slightly elevated blood pressure of 156/96. Creatinine at presentation was 9.0 mg/dL, increased from a baseline of 2.6mg/dL. Initial GFR was 5. Workup was also notable for nephrotic range proteinuria (24h urine 10.5g). Renal biopsy showed features of anti-glomerular basement membrane (GBM) crescenteric glomerulonephritis. Workup showed atypical ANCA with 1:40 ANCA and negative MPO/PR3. The patient was treated with five sessions of PLEX and pulse dose solumedrol followed by a prednisone taper, and a three-day course of cyclophosphamide that was discontinued due to increasing viral load. The patient had initial improvement in renal function but ultimately progressed to ESRD and required hemodialysis. Rheumatology is considering DMARDS for suspected ANCA if the patient’s viral load improves.

Discussion

This case presents a unique overlap between HIV, anti-glomerular basement membrane disease, and a possible component of atypical ANCA-vasculitis. The patient did not display typical symptoms of ANCA-vasculitis, but renal biopsy showed both acute and chronic changes not typical to isolate anti-GMB and indicated a possible atypical ANCA overlap. Steroids were started in conversation with the Infectious Disease team, who believed the risk would be attenuated by the patient’s viral suppression. The patient’s treatment course was complicated by the presence of a left renal staghorn calculus and pyelonephritis after initial discharge. This case highlights the complicated nature of treating an autoimmune condition in the setting of HIV, as it requires a balance between immunosuppression and viral suppression.