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C3 Glomerulonephritis Presenting With Nephritic and Nephrotic Syndromes: Spontaneous Remission After Six Months on
Francisco Gonçalves1, Nídia Marques1, Roberto Silva2
1Nephrology, Centro Hospitalar Universitário de São João, Porto, PRT.
Insights
C3 glomerulopathy, a rare kidney disease, was diagnosed in a patient with a CFHR3-CFHR1 deletion. Despite a poor prognosis, the patient experienced spontaneous recovery after hemodialysis, highlighting diagnostic challenges.
Area of Science:
- Nephrology
- Immunology
- Genetics
Background:
- C3 glomerulopathy is a rare renal disease characterized by complement dysregulation.
- It presents with variable clinical and pathological features, making diagnosis challenging.
Observation:
- A middle-aged male presented with nephritic/nephrotic syndromes and low C3 levels.
- Genetic analysis revealed homozygosity for the CFHR3-CFHR1 deletion, a known risk factor for C3 glomerulopathy.
- The patient experienced spontaneous renal recovery after six months of hemodialysis, despite initial concerns about malignancy and prognosis.
Findings:
- The case highlights the diagnostic complexity of C3 glomerulonephritis, particularly in the absence of definitive assays for certain complement-related factors.
- The CFHR3-CFHR1 deletion was identified as a significant genetic factor.
- Spontaneous remission occurred despite a perceived poor prognosis and lack of specific treatment.
Implications:
- This case underscores the need for improved diagnostic tools and understanding of complement-mediated kidney diseases.
- It suggests that some cases of C3 glomerulopathy may have a more variable prognosis than previously assumed.
- Further research into the mechanisms of spontaneous recovery could inform future therapeutic strategies.
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.
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