WCN25-2948 INTELLECTUAL FREEDOM, OPEN INQUIRY, EQUITY, DIVERSITY, AND INCLUSION: BANFF TRADITIONS FROM THE BEGINNING AND ALSO IMPORTANT TO ISN

Discussion: Conditions such as post-infectious glomerulonephritis are difficult to differentiate from C3 glomerulopathy by renal biopsy alone, which can confound early diagnosis and treatment.No disease-specific treatments are available, although immunosuppressive agents and complement pathway blocking agents (e.g.eculizumab) are helpful in certain patients.Unfortunately, no treatment is universally effective or curative except allogenic liver transplantation in a few select cases.[1] Moreover, recent advances in research have clarified the role of complement in other glomerular diseases in which its role was less established, namely in immune-complex membranoproliferative glomerulonephritis (IC-MPGN), ANCA-vasculitis, IgA nephropathy, and idiopathic membranous nephropathy.As a standard of care, we would advise complement pathway analysis (C3, C4, CH-50, CFH antibody) and autoimmune work up (ANA profile) for all patients with C3G as a histological picture.As the patient reached complete renal remission (normalising creatinine and proteinuria less than 300 mg/ day), we decided to continue with dual immunosuppression.The management required meticulous in-person and telephonic follow up.Due to the rarity of the disease, optimal management options established through randomized clinical trials are not sufficient to guide therapeutic decision-making.[2,3] In this particular case, renal remission was the only parameter that guides the management without the need to perform genetic tests or complement factor analysis. Conclusions: Conclusion:The C3 glomerulonephritis is a recently coined disease entity with variable natural history.It is a rare disease with minimum therapeutic options like eculizumab, plasma exchange or in few cases cyclophosphamide and ACE inhibitors.In this case report, we highlight a case scenario with complete and sustained disease remission with short course (6 months) of glucocorticoids and mycophenolate mofetil.We did not do genetic testing or plasma exchange.This indicated that morphological and IF diagnosis of C3GN need not be a genetic entity always but likely to respond to multi-targeted immunosuppression.Close monitoring of these patients is also equally important to identify and treat relapses.

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Journal
Kidney International Reports
Published
2025-01-27
DOI
https://doi.org/10.1016/j.ekir.2024.11.1309
Primary Topic
Research, Science, and Academia
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article
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article

WCN25-2948 INTELLECTUAL FREEDOM, OPEN INQUIRY, EQUITY, DIVERSITY, AND INCLUSION: BANFF TRADITIONS FROM THE BEGINNING AND ALSO IMPORTANT TO ISN

Kim Solez, Habba Mahal
Kidney International Reports
Research, Science, and Academia
article

WCN25-2948 INTELLECTUAL FREEDOM, OPEN INQUIRY, EQUITY, DIVERSITY, AND INCLUSION: BANFF TRADITIONS FROM THE BEGINNING AND ALSO IMPORTANT TO ISN

Kim Solez, Habba Mahal
article en

Abstract

Discussion: Conditions such as post-infectious glomerulonephritis are difficult to differentiate from C3 glomerulopathy by renal biopsy alone, which can confound early diagnosis and treatment.No disease-specific treatments are available, although immunosuppressive agents and complement pathway blocking agents (e.g.eculizumab) are helpful in certain patients.Unfortunately, no treatment is universally effective or curative except allogenic liver transplantation in a few select cases.[1] Moreover, recent advances in research have clarified the role of complement in other glomerular diseases in which its role was less established, namely in immune-complex membranoproliferative glomerulonephritis (IC-MPGN), ANCA-vasculitis, IgA nephropathy, and idiopathic membranous nephropathy.As a standard of care, we would advise complement pathway analysis (C3, C4, CH-50, CFH antibody) and autoimmune work up (ANA profile) for all patients with C3G as a histological picture.As the patient reached complete renal remission (normalising creatinine and proteinuria less than 300 mg/ day), we decided to continue with dual immunosuppression.The management required meticulous in-person and telephonic follow up.Due to the rarity of the disease, optimal management options established through randomized clinical trials are not sufficient to guide therapeutic decision-making.[2,3] In this particular case, renal remission was the only parameter that guides the management without the need to perform genetic tests or complement factor analysis. Conclusions: Conclusion:The C3 glomerulonephritis is a recently coined disease entity with variable natural history.It is a rare disease with minimum therapeutic options like eculizumab, plasma exchange or in few cases cyclophosphamide and ACE inhibitors.In this case report, we highlight a case scenario with complete and sustained disease remission with short course (6 months) of glucocorticoids and mycophenolate mofetil.We did not do genetic testing or plasma exchange.This indicated that morphological and IF diagnosis of C3GN need not be a genetic entity always but likely to respond to multi-targeted immunosuppression.Close monitoring of these patients is also equally important to identify and treat relapses.

Kidney International ReportsVol. 10(2)
University of Alberta (CA)
Reduced inequalities
Openalex Percentile: Top 99%
Research, Science, and Academia
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