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Published on: November 1, 2018
Successful therapy of C3Nef-positive C3 glomerulopathy with plasma therapy and immunosuppression
Karsten Häffner1, Stefan Michelfelder2, Martin Pohl2
1Center for Pediatrics and Adolescent Medicine, University Hospital Freiburg, Mathildenstr. 1, 79106, Freiburg, Germany. karsten.haeffner@uniklinik-freiburg.de.
Insights
A new multimodal therapy, including plasma exchange, corticosteroids, and mycophenolate mofetil, effectively treated C3 glomerulopathies (C3G) in four patients. This approach offers a promising option for severe C3G cases lacking other identified causes.
Area of Science:
- Nephrology
- Immunology
- Complement System Biology
Background:
- C3 glomerulopathies (C3G) result from dysregulated alternative complement pathway activation.
- C3G often leads to end-stage renal disease and has a high recurrence rate post-transplant.
- Antibodies against C3 convertase (C3Nef) are implicated in most C3G cases, yet effective therapies are lacking.
Purpose of the Study:
- To evaluate a multimodal therapeutic regimen for C3 glomerulopathies (C3G) in patients with identified C3Nef.
- To assess the efficacy of plasma therapy, corticosteroids, and mycophenolate mofetil in treating C3G.
- To explore treatment options for severe C3G cases where complement factor mutations are excluded.
Main Methods:
- A consecutive series of four C3G patients with identified C3Nef and excluded complement mutations were treated.
- The therapeutic regimen included plasma therapy, corticosteroids, and mycophenolate mofetil.
- A single patient with C3 glomerulonephritis (C3GN) and high terminal complement complex levels received additional eculizumab.
Main Results:
- The multimodal regimen normalized renal function in all four patients, achieving complete remission in two.
- Two patients experienced a significant reduction in proteinuria.
- The C3GN patient showed partial remission with eculizumab addition; C3Nef levels did not correlate with outcomes.
Conclusions:
- A multimodal therapeutic approach is effective for C3G patients with suspected autoimmune etiology.
- This regimen provides a viable treatment option for severely affected C3G patients.
- Further research into more specific treatments for C3G is warranted.
Background:
C3 glomerulopathies (C3G) are characterized by uncontrolled activation of the alternative pathway of complement. In most patients these diseases progress towards end-stage renal disease, and the risk of recurrence after renal transplantation is high. In the majority of patients, only antibodies against the C3 convertase, termed C3Nef, can be found as a potential pathogenic factor. Although a large variety of therapeutic approaches have been used, no generally accepted therapy exists.
Methods:
In four consecutive patients with C3G in whom all known complement factor mutations were excluded and only C3Nef could be identified as a potential cause of disease, a multimodal therapeutic regimen with plasma therapy, corticosteroids and mycophenolate mofetil was used.
Results:
The multimodal regimen achieved normalization of renal function in all four patients, with complete remission in two patients and a distinct reduction of proteinuria in the other two patients. The single patient with C3 glomerulonephritis (C3GN) and marked terminal complement complex elevation only showed partial remission; further improvement was achieved following the addition of eculizumab to the therapeutic regimen. Repeatedly measured C3Nef levels did not correlate with disease course or therapeutic response in any of the patients.
Conclusions:
As this multimodal therapeutic approach was effective in all four treated patients with suspected autoimmune etiology of C3G, it offers a treatment option for severely affected patients with this rare disease until more specific regimens are available.
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