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What is the natural history of nephrotic syndrome?
The natural history of the primary nephrotic syndrome depends on the underlying cause.
MCNS:
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.
FSGS:
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.
MPGN:
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.
Membranous nephropathy:
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.
