A review synthesises long-term outcomes of idiopathic nephrotic syndrome, the commonest chronic glomerular disease of childhood, where the response to corticosteroids, steroid-sensitive versus steroid-resistant, remains the strongest prognostic signal.
About half of steroid-sensitive patients have frequent relapses or steroid dependence, and roughly a quarter relapse at least once in adulthood, so the old assumption that disease remits by puberty is too optimistic. Up to a third of steroid-resistant cases are monogenic, and monogenic or multidrug-resistant disease carries a three- to fourfold higher risk of kidney failure, alongside the growth, metabolic, bone and nephrotoxicity harms of prolonged immunosuppression.
For practice, risk-stratify by steroid response and, in resistant disease, by genetic testing, and plan deliberate transition to adult nephrology rather than discharging at puberty, because relapses and complications continue. Kidney failure and death remain uncommon in steroid-sensitive disease, which is the reassuring counterpoint.
- Steroid response (sensitive vs resistant) is the strongest prognostic factor in childhood nephrotic syndrome.
- About a quarter of steroid-sensitive patients still relapse in adulthood.
- Up to a third of steroid-resistant cases are monogenic.
- Monogenic or multidrug-resistant disease carries a three- to fourfold higher kidney-failure risk.
- Plan structured transition to adult care rather than discharge at puberty.
Why it matters
It overturns the reassurance that childhood nephrotic syndrome reliably remits by puberty, with direct implications for follow-up.
Don't overread it
This is a narrative review summarising outcomes, not new trial data; genetic and outcome risks vary widely by phenotype.
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