Natural history of nephrotic syndrome

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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.

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