What is the natural history of nephrotic syndrome?
The natural history of the primary nephrotic syndrome depends on the underlying cause.
Patients have an excellent long-term prognosis. Most patients are responsive to therapy, and while the majority will follow a relapsing course, eventually most patients outgrow the disease without permanent kidney injury.
However, there are some children with MCNS who continue to have relapsing disease well into adulthood.
Nearly half of patients with primary FSGS progress to end-stage kidney disease (ESKD) over 5 to 10 years.
Additionally, there is recurrent disease in 25% to 30% following a kidney transplant.
Nearly 50% of patients with MPGN will progress to ESKD over 10 to 15 years and 20% to 25% of these patients develop recurrent disease in a transplanted kidney.
The long-term course of MN is more variable.
Approximately one-third will go into remission, another third have persistent proteinuria with stable kidney function, and the remaining patients will experience a steady decline in kidney function.
Recent findings suggest that monitoring the level of antibody to M-type phospholipase A(2) receptor (PLA(2)R) can be useful to predict prognosis and response to therapy.