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C3 glomerulonephritis associated with monoclonal gammopathy: a retrospective case series study from a single
Xin Zhang1,2,3, Xiao-Juan Yu1,2,3, Dan-Yang Li1,2,3
1Renal Division, Department of Medicine, Institute of Nephrology, Peking University First Hospital, Peking University, Beijing, China.
Insights
Chinese patients with C3 glomerulonephritis and monoclonal gammopathy have poor renal outcomes. Immunosuppressants and supportive therapies showed similar efficacy, highlighting the need for further research into clone-targeted treatments for this condition.
Area of Science:
- Nephrology
- Immunology
- Oncology
Background:
- C3 glomerulonephritis (C3GN) is a rare kidney disease.
- Monoclonal gammopathy can be associated with various kidney diseases, including C3GN.
- Understanding the interplay between C3GN and monoclonal gammopathy is crucial for patient management.
Purpose of the Study:
- To investigate the demographic, clinicopathological features, and renal outcomes of Chinese patients with C3GN in the setting of monoclonal gammopathy.
- To analyze the effectiveness of different treatment strategies in this patient cohort.
Main Methods:
- Retrospective analysis of 19 patients with biopsy-proven C3GN and detectable monoclonal immunoglobulin from 2006 to 2018.
- Collection and analysis of clinical data, renal pathology, treatment, and prognosis.
- Evaluation of renal survival based on treatment modalities.
Main Results:
- C3GN with monoclonal gammopathy represented 24% of C3GN cases in the study period, with a mean age of onset of 55 years.
- Nephrotic syndrome, anemia, and hypocomplementemia were common; IgG was the most frequent monoclonal immunoglobulin isotype.
- Median renal survival was similar for conservative and immunosuppressant therapies (12 vs. 15 months), and clone-targeted therapy efficacy requires further investigation.
Conclusions:
- This is the first case series on C3GN with monoclonal immunoglobulin in northern China.
- Patients with C3GN and monoclonal gammopathy exhibit poor renal prognosis.
- Current immunosuppressant and supportive therapies offer no significant advantage in renal outcomes; clone-targeted therapy warrants further study.
Objective:
To investigate the demographic and clinicopathological features and renal outcomes of Chinese patients with C3 glomerulonephritis in the setting of monoclonal gammopathy.
Methods:
Patients with renal biopsy-proven C3 glomerulonephritis and detectable serum and/or urine monoclonal immunoglobulin from 2006 to 2018 in Peking University First Hospital were included, their clinical data, renal pathology type, treatment, and prognosis were collected and analyzed.
Results:
Nineteen patients were enrolled, accounting for 24% of C3GN patients in the study period. The mean age of onset was 55 years old and the gender ratio was 4/15 (female/male). The mean eGFR at biopsy was 49.55 ± 29.81 ml/min/1.73m2. The prominent clinical manifestations included nephrotic syndrome (58%), anemia (68%), microscopic hematuria and leukocyturia (58%), and hypocomplementemia (13, 68%). The IgG was the most common isotype of monoclonal Ig on immunofixation electrophoresis. Kidney biopsies revealed a relatively prominent MPGN pattern. Only two patients had direct evidence of monocle immunoglobulins acting as C3GN pathogenic factors. Two patients had concurrent TMA-like renal injuries. The median renal survival was 12 and 15 months, respectively in patients receiving conservative therapy and immunosuppressant therapy, without statistical significance. The efficacy of clone-targeted therapy needed further investigation. Plasma exchange therapy only improved one patient's renal outcome.
Conclusions:
This is the first case series report of C3GN combined with monoclonal Ig in northern China. The renal prognosis of these patients is poor, and immunosuppressant therapies show no advantage over supportive therapy in renal prognosis, while the benefit of clone-targeted chemotherapy is still requiring investigation.
