An Atypical Case of Immune Complex-Mediated Glomerulonephritis

Document Type

Conference Proceeding

Publication Date

11-1-2023

Publication Title

J Am Soc Nephrol

Abstract

Introduction: Rapidly progressive crescentic glomerulonephritis is an aggressive clinical syndrome characterized by massive loss in kidney function in a relatively short period of time, days to weeks. The etiology of this disease is varied, with subtypes including anti-glomerular membrane disease, immune complex-mediated injury, or pauci-immune. We report a rare case of acute exudative and crescentic glomerulonephritis leading to end-stage renal disease due to an unknown infection. Case Description: A 28-year-old African American male with a medical history only remarkable for asthma presents with nausea, vomiting, diarrhea, and severe abdominal pain for 3-4 days. On admission, the patient's lab data showed a serum creatinine level of 12.28 mg/dL, BUN of 71, and WBC of 23,000. Renal ultrasound showed increased echogenicity of bilateral kidneys suggestive of medical renal disease. Non-emergent HD and kidney biopsy were ordered. COVID-19, flu A/B, hepatitis panel, ANA, c-ANCA, p-ANCA, and anti-GBM were all negative. Serum protein electrophoresis showed mildly elevated IgG lambda. C3 was mildly decreased, and C4 was normal. Blood cultures were negative. The patient was initiated on vancomycin/Cefepime/Metronidazole. Kidney biopsy demonstrated 14/16 glomeruli with necrotizing crescents, C3 predominant mesangial and capillary loop staining, and numerous mesangial and segmental endothelial humps. Discussion: Patients with postinfectious glomerulonephritis and <50% glomerular involvement have a higher chance of mild disease and potential recovery, with >80% glomerular involvement documented as severe disease requiring therapy. This case represents ∼87% glomerular exudative crescents due to an unknown cause, likely post-infectious. The patient was treated with pulse steroids x3 days, but no other immunosuppression was started as data is lacking in severe post-infectious GN as well as idiopathic immune complex RPGN. Initiation of oral or IV cyclophosphamide or rituximab was considered, however, was deferred and the patient was treated with steroid monotherapy. The patient remains on hemodialysis with no evidence of renal recovery. This case highlights the need for further studies in immunosuppression guidelines for patients with this debilitating disease.

Volume

34

First Page

1117

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