IgA Nephropathy: A case that illustrates its controversies

Abstract

IgA nephropaty (IgAN) is a mesangial proliferative glomerulonephritis characterized by diffuse mesangial deposition of IgA. IgAN is unique among glomerular diseases in being defined by the presence of an inmune reactant rather than by another morphologic feature found on renal biopsy. Light microscopic changes are so variable that there is no universal score and it is not possible to correlate pathology with clinical manifestations.
Although primary IgAN was considered a benign condition for many years, it is now clear that a large number of cases eventually progress to renal failure (up to 15%). Indeed, this nephropathy is the main cause of end stage renal disease in patients with primary glomerular disease who require renal replacement therapy (up to 25%).
Clinical presentation is extremely variable (from benign recurrent hematuria up to a rapidly progressive glomerulonephritis), so treatment also varies widely. The intention of this paper is to illustrate that this nephritis can be, in many occasions, a puzzle for nephrologists.