Rapidly progressive glomerulonephritis in autosomal dominant polycystic kidney disease

Patients with autosomal dominant polycystic kidney disease (ADPKD) can suffer from the same causes of acute kidney injury as the general population. Affected individuals may present with hematuria and proteinuria (usually less than 1g/day). However, nephrotic syndrome and proliferative glomerulonephritis are uncommon in patients with polycystic kidney disease. Development of nephrotic syndrome and / or rapid deterioration in kidney function suggest the presence of another, more aggressive disorder, requiring prompt diagnosis and appropriate interventions to mitigate further injury and progression to end stage kidney disease. In this chapter, we will discuss rapidly progressive glomerulonephritis in association with ADPKD.