Abstract: SA-PO789
Pauci-Immune Crescentic Glomerulonephritis Linked to Candida parapsilosis Fungemia
Session Information
- ANCA-Associated Vasculitis, Anti-GBM Disease, and Other RPGN
October 26, 2024 | Location: Exhibit Hall, Convention Center
Abstract Time: 10:00 AM - 12:00 PM
Category: Glomerular Diseases
- 1402 Glomerular Diseases: Clinical, Outcomes, and Therapeutics
Authors
- Qian, Long, Yale New Haven Hospital, New Haven, Connecticut, United States
- Carey, Hugh, Metabolism Associates, New Haven, Connecticut, United States
Introduction
Infectious endocarditis (IE) typically correlates with infectious glomerulonephritis (GN), presenting as membranoproliferative GN (MPGN) with positive C3 and immunoglobulin staining. However, there are rare instances of pauci-immune GN associated with infectious endocarditis, sometimes with ANCA positivity, but occasionally negative for any ANCA or PR3 or MPO. Here we present a case of Candida parapsilosis endocarditis associated with p-ANCA positive, PR3-negative, MPO-negative pauci-immune GN.
Case Description
A 59-year-old man with a history of intravenous drug use on methadone, compensated cirrhosis due to HCV and alcohol, and HIV, presented with abdominal pain. Creatinine was newly elevated to 4.4 mg/dL (baseline 0.8). Echocardiogram revealed a 1.1 x 0.7cm aortic valve vegetation. Blood cultures grew Candida parapsilosis. Serological testing showed negative PR3 and MPO, positive p-ANCA (1:40), low C3, normal C4, negative cryoglobulin, negative HIV, HBV, and HCV PCR. Kidney biopsy showed crescentic necrotizing GN with negative to trace immunofluorescence for immunoglobulins, C3, and kappa and lambda, and electron microscopy showed no deposits. Immunosuppression was withheld due to fungemia persistent for 4 weeks despite receiving liposomal amphotericin B, flucytosine, and fluconazole. Hemodialysis (HD) was started 3 weeks post-presentation for uremia. After blood cultures cleared, he was started on 60mg/day of prednisone. However, he later developed methicillin-sensitive Staphylococcus aureus bacteremia, thus prednisone was tapered. He received cefazolin with continued antifungals. Aortic valve replacement was performed 2 months post-presentation without complications. He received one session of HD post-surgery but had kidney recovery and HD was stopped. He completed 6 weeks of amphotericin; fluconazole continues for lifelong suppression. He remains off HD.
Discussion
Pauci-immune necrotizing GN is a rare cause of AKI associated with IE. To our knowledge this is the second reported case of MPO, PR3 negative GN associated with Candida parapsilosis IE. The potential mechanism of IE triggering ANCA is unknow and has been hypothesized to be via autoantigen complementarity or molecular mimicry. For treatment, balancing immunosuppression against ongoing infection can be challenging. Favorable prognosis may be achieved with definitive infection control and cautious immunosuppressive therapy.