WCN25-1260 CLINICAL MANIFESTATIONS, MORPHOLOGICAL PATTERN AND TREATMENT OUTCOMES OF COMPLEMENT 3 GLOMERULOPATHY ON LONG TERM

She was started on peritoneal dialysis and has been doing very well, actively wait-listed for a kidney transplant.Transplant center is aware of her genetic study and will consider eculizumab to prevent recurrence in the allograft.C3 glomerulopathy (C3G) is a rare set of kidney diseases with 2 patterns: C3GN and dense deposit disease.Pathogenesis of both is due to complement dysregulation in the alternative pathway.Acquired or genetic alterations of the regulatory proteins of the complement pathway result in C3G.Other disease entities such as infectionrelated glomerulonephritis and masked monoclonal deposits can present similarly.Both the C3GN and dense deposit disease variants of C3G are progressive and recur in transplanted kidneys.Conclusions: This patient's presentation has several unique features.She likely had an underlying C3GN years ago but was never biopsied.Subsequently, it appears to have been triggered by an upper respiratory infection prior to her hospitalization leading to a severe kidney injury resulting in ESRD.She also had a positive heterozygous mutation for CFH which is rare in Indian patients (Kumar, Outcome of C3 glomerulopathy patients: largest single-center experience from South Asia, 2020).This case illustrates the importance of obtaining definitive diagnosis with a biopsy, particularly in young patients as well as the importance of genetic testing which may help with planning to prevent recurrence in an allograft.While there is no definitive treatment now, these patients may benefit from immunosuppression or enrollment in clinical trials.Finally, there is limited data on ESRD modalities in C3GN; our patient has remained relatively healthy with a good quality of life on peritoneal dialysis despite her underlying kidney disease.I have no potential conflict of interest to disclose.I did not use generative AI and AI-assisted technologies in the writing process.

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Journal
Kidney International Reports
Published
2025-01-27
DOI
https://doi.org/10.1016/j.ekir.2024.11.378
Primary Topic
Renal Diseases and Glomerulopathies
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article
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article

WCN25-1260 CLINICAL MANIFESTATIONS, MORPHOLOGICAL PATTERN AND TREATMENT OUTCOMES OF COMPLEMENT 3 GLOMERULOPATHY ON LONG TERM

Manas Ranjan Behera, Monica Yachha, Pallavi Prasad, Manoj Jain et al.
Kidney International Reports
Renal Diseases and Glomerulopathies
article

WCN25-1260 CLINICAL MANIFESTATIONS, MORPHOLOGICAL PATTERN AND TREATMENT OUTCOMES OF COMPLEMENT 3 GLOMERULOPATHY ON LONG TERM

Manas Ranjan Behera, Monica Yachha, Pallavi Prasad, Manoj Jain, Nidhi Gupta, Dharmendra Bhadauria, Manas Ranjan Patel, Vinita Agrawal, Anupama Kaul, Ravi Shankar Kushwaha, Narayan Prasad
article en

Abstract

She was started on peritoneal dialysis and has been doing very well, actively wait-listed for a kidney transplant.Transplant center is aware of her genetic study and will consider eculizumab to prevent recurrence in the allograft.C3 glomerulopathy (C3G) is a rare set of kidney diseases with 2 patterns: C3GN and dense deposit disease.Pathogenesis of both is due to complement dysregulation in the alternative pathway.Acquired or genetic alterations of the regulatory proteins of the complement pathway result in C3G.Other disease entities such as infectionrelated glomerulonephritis and masked monoclonal deposits can present similarly.Both the C3GN and dense deposit disease variants of C3G are progressive and recur in transplanted kidneys.Conclusions: This patient's presentation has several unique features.She likely had an underlying C3GN years ago but was never biopsied.Subsequently, it appears to have been triggered by an upper respiratory infection prior to her hospitalization leading to a severe kidney injury resulting in ESRD.She also had a positive heterozygous mutation for CFH which is rare in Indian patients (Kumar, Outcome of C3 glomerulopathy patients: largest single-center experience from South Asia, 2020).This case illustrates the importance of obtaining definitive diagnosis with a biopsy, particularly in young patients as well as the importance of genetic testing which may help with planning to prevent recurrence in an allograft.While there is no definitive treatment now, these patients may benefit from immunosuppression or enrollment in clinical trials.Finally, there is limited data on ESRD modalities in C3GN; our patient has remained relatively healthy with a good quality of life on peritoneal dialysis despite her underlying kidney disease.I have no potential conflict of interest to disclose.I did not use generative AI and AI-assisted technologies in the writing process.

Kidney International ReportsVol. 10(2)
Sanjay Gandhi Post Graduate Institute of Medical Sciences (IN)
Openalex Percentile: Top 98%
Renal Diseases and Glomerulopathies
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