Abstract: TH-PO519
When the Eyes Become the Window for the Kidneys: A Case of Rapidly Progressive C3 Glomerulonephritis
Session Information
- Glomerular Diseases: Clinical, Outcomes, Trials - I
November 03, 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
- Azuero, Andres Jose, Mount Sinai Medical Center, Miami Beach, Florida, United States
- Caldwell, Jillian, Stanford University, Stanford, California, United States
- Taiwo, Adetokunbo A., Stanford University, Stanford, California, United States
- Velar, Alex A., Mount Sinai Medical Center, Miami Beach, Florida, United States
- Yu, Margaret K., Stanford University, Stanford, California, United States
Introduction
C3 glomerulonephritis (C3GN) is a rare disease caused by dysregulation of the alternative complement pathway. We present a case of rapidly progressive C3GN with visual symptoms.
Case Description
A 21 year old woman presented to the Ophthalmology clinic for worsening blurry vision over 3 months. Blood pressure was 220/144 mmHg. Fundoscopic exam revealed bilateral central venous and arterial vascular occlusion, extensive cotton wool spots, few hemorrhages, and hypopigmented choroidal spots. Laboratory data was notable for hemoglobin 10.8 g/dL and serum creatinine of 3.49 mg/dL. Urinalysis had 3+ protein and 2+ blood. Urine protein/creatinine ratio was 5.3 g/g and 24 hour urine protein was 4.6 g. Serologic evaluation for glomerulonephritis was unremarkable. Kidney biopsy showed 75% global glomerulosclerosis, few fibrocellular crescents, 90% interstitial fibrosis and tubular atrophy. Immunofluorescence showed 3+ diffuse granular staining in the mesangium for C3, consistent with C3GN. Immunosuppression was not given due to the advanced fibrosis on her biopsy. The patient had rapid progression of disease and required initiation of hemodialysis 7 months after her diagnosis as a bridge to renal transplant. Genetic testing was inconclusive; several variants of uncertain significance were identified and thought to be benign. No gammopathy was identified.
Discussion
Most cases of C3GN manifest as nephritic or nephrotic syndrome and it is common for patients to have preserved kidney function at presentation. ESKD tends to develop over 10 years. It is unusual for C3GN to be a rapidly progressive disease, as observed in this case. In a series of 114 patients with C3GN, only 10% progressed to ESKD over a median of 22 months. This patient’s eye exam was suggestive of hypertensive disease and Purtscher-like retinopathy, a rare angiopathy that may result from complement activation; its correlation with severity of renal disease remains uncertain. In addition to nonspecific therapy for proteinuria, MMF plus steroids or eculizumab alone may be considered based on the severity of proteinuria and degree of renal fibrosis. Additional studies are needed to establish if rapidly progressive cases are treatment responsive or have a higher recurrence risk after kidney transplantation.