C3 Glomerulonephritis Presenting With Nephritic And Nephrotic Syndromes: Spontaneous Remission After Six Months On Dialysis
Gonçalves, F., Marques, N., Silva, R., Mendonça, L., & Faria, B. (2023). C3 Glomerulonephritis Presenting With Nephritic and Nephrotic Syndromes: Spontaneous Remission After Six Months on Dialysis. Cureus, 15(12), e50396. https://doi.org/10.7759/cureus.50396
View Original Source →Abstract
C3 glomerulopathy is a rare and complex renal disease driven by complement dysregulation, with variable presentation and pathophysiology. We report the case of a middle-aged male patient presenting with nephritic and nephrotic syndromes and low serum C3, whose biopsy established the diagnosis of C3 glomerulonephritis. He was found to be homozygous for the complement factor H-related protein (CFHR)3-CFHR1 deletion, which has been associated with the development of anti-factor H autoantibodies. However, the lack of consistent and accessible nephritic factor assays prevented full clarification of the mechanisms involved in the disease. Interestingly, despite not receiving treatment due to suspicion of malignancy and perceived poor renal prognosis, there was spontaneous recovery after six months on hemodialysis. This case reflects the enduring challenges in establishing the diagnosis and prognosis of C3 glomerulonephritis.
Case Details
Disease Location
Kidney, peritoneum
Personal Characteristics
48-year-old man. He was a former smoker with arterial hypertension under control, treated dyslipidemia, and alcoholic child-a cirrhosis.
Clinical Characteristics
Admitted for dyspnea and anasarca. Initial investigations revealed severe acute kidney injury and hypoalbuminemia. The presence of nephritic and nephrotic syndromes was established, and a kidney biopsy was performed. Optical microscopy revealed mesangial and endocapillary hypercellularity with glomerular tuft lobulation with thickened capillary walls and double contours. The histological diagnosis was membranoproliferative glomerulonephritis with c3 dominant staining, compatible with c3gn on electron microscopy. Computed tomography raised suspicion of peritoneal carcinomatosis. Hemodialysis was started due to oliguria and worsening renal function.
Remission Characteristics
On follow-up, ascites cytology and peritoneal biopsy were negative for neoplasia. Surprisingly, spontaneous recovery of renal function was documented six months post-diagnosis. At one year of follow-up, the patient had preserved kidney function, no hematuria, no proteinuria, and c3 levels were normal.
Treatment & Mechanisms
Clinical Treatment
Biopsy, dialysis