Marzo Aprile 2021

C3 Glomerulopathy, a pathology with scarce evidence. A case report

Abstract

C3 Glomerulonephritis (C3GN) is a rare disease with an estimated incidence of 1-2 cases per million, caused by an alteration in the alternative complement pathway, although its complete physiopathology remains uncertain. Treatment evidence is poor. Immunosuppressive therapy can be initiated in more severe cases. Progression rates to end stage kidney disease are of up to 50% within a decade, and the posttransplant recurrence rates of 45-60%. We describe the case of a young man without any past medical history, with lower extremities edema, dyspnea, and kidney function deterioration. The patient was ultimately diagnosed with C3GN.

Key words: C3 glomerulopathy, glomerulonephritis, alternative complement pathway

Introduction

C3 Glomerulopathy (C3G) is a renal disease caused by a primary alteration in the complement, presenting a dysregulation in the alternative complement pathway, which induces hyperactivity. The term was first described in 2007 in a series of 19 patients characterized by the histopathological finding of C3 deposits in renal tissue without any other significant immunocomplex [1]. The renal symptoms were hypertension, kidney disease, proteinuria, hematuria, and nephrotic syndrome.

 

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