Complement 3 glomerulopathy (C3G) is an ultra-rare, progressive kidney disease resulting from dysregulation of the alternative complement pathway. Clinical presentation of C3G is heterogeneous and definitive diagnosis relies on kidney biopsy and immunofluorescence staining. The term C3G encompasses two subgroups, dense deposit disease and C3 glomerulonephritis, distinguished via electron microscopy. In this podcast article, the authors discuss the challenges associated with C3G diagnosis and the central role of kidney biopsy. Using an illustrative case study, key histological observations are described, and best practices are discussed from the perspectives of a nephrologist and a nephropathologist. Podcast Audio (MP4 141866 KB).

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http://www.ncbi.nlm.nih.gov/pmc/articles/PMC10611840PMC
http://dx.doi.org/10.1007/s12325-023-02654-3DOI Listing

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The Difficulties of Treating Complement-3-Mediated Glomerulopathy.

Am J Ther

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Northwell, New Hyde Park, NY, Department of Medicine, Manhasset, NY.

Background: C3 glomerulopathy (C3G) is a rare disease affecting the complement alternative pathway, categorized into dense deposit disease and C3 glomerulonephritis. Dense deposit disease predominantly affects younger individuals, while C3 glomerulonephritis tends to manifest in older populations. The diseases are characterized by dysregulation of the complement alternative pathway, leading to the deposition of complement components in the glomeruli and subsequent renal dysfunction.

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Complement 3 glomerulopathy (C3G) is an ultra-rare glomerulonephritis caused by dysregulation of the alternative complement pathway. C3G has an estimated incidence of 1-3 cases per million people in the United States. Diagnosing C3G based solely on clinical and laboratory features is challenging because it mimics several other glomerular diseases; therefore, diagnosis requires a kidney biopsy.

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Article Synopsis
  • * The dysregulation of the complement pathway can be triggered by genetic mutations or autoantibodies, leading to further complications like C3 glomerulopathy and thrombotic microangiopathies.
  • * The case study discussed involves a patient with Systemic Lupus Erythematosus (SLE) who developed C3GN in a kidney transplant, highlighting the need for more research on treatment options for this rare condition.
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Background: Complement 3 (C3) glomerulopathy (C3G) is a heterogenous disease characterized by dysregulation of the complement alternative pathway. Within 10 years of a diagnosis, roughly 50% of patients with C3G will progress to end-stage kidney disease. Historically, treatment options have been limited to nonspecific immune suppression with suboptimal response rates to recommended therapies.

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Background: In children, C3 glomerulopathy (C3G) is a heterogeneous disease characterized by diverse clinicopathological profiles and kidney outcomes. However, diagnostic work-up in resource-limited settings is challenging because of the unavailability of complement assays and limited access to electron microscopy or genetic testing.

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