The Evidence-Based Approach to Adult-Onset Idiopathic Nephrotic Syndrome

Abstract

Adult-onset nephrotic syndrome (NS) differs from its pediatric counterpart in several important ways. Most importantly, NS in adults is more etiologically heterogeneous compared to children, and thus treatment approaches rely heavily on the histological diagnosis provided by renal biopsy. The evidence-based approach to treatment of adult NS has been critically examined by the Kidney Disease Improving Global Outcomes (KDIGO) guidelines in glomerulonephritis, published in 2012. Here, we examine the strengths and limits of those guidelines and review recent work that expands the evidence-based approach.

Keywords: KDIGO guidelines; clinical practice guidelines; focal segmental glomerulosclerosis; minimal-change disease; nephrotic syndrome.

Figure 1

A suggested algorithm for the clinical approach to adult FSGS. *Note that KDIGO guidelines explicitly recommend against genetic testing. **Highlighted are some of the monogenic causes of FSGS more likely to be found in adults with genetic disease; see text and highlighted references for further discussion. NPHS2, Podocin; CD2AP, CD2-associated protein; ACTN4, α-actinin-4; INF2, inverted formin-2; TRPC6, transient receptor potential channel 6; WT1, Wilms tumor protein.