Childhood nephrotic syndrome: a quarter relapse in adulthood, and steroid resistance carries lasting risk

Design

Narrative review

Population

Children with idiopathic nephrotic syndrome followed into adulthood

Primary outcome

Long-term relapse, complications and kidney outcomes

Effect

~25% of steroid-sensitive patients relapse in adulthood; kidney failure 3–4× higher in monogenic or multidrug-resistant SRNS

This Kidney International review brings together long-term outcomes of childhood idiopathic nephrotic syndrome. Initial steroid response remains the strongest prognostic factor. About half of children with steroid-sensitive disease have frequent relapses or steroid dependence, and around a quarter have at least one relapse as adults. Childhood frequent relapses and the need for second-line immunosuppression predict persistent disease. Fewer than 10% develop secondary steroid resistance; kidney failure and death are uncommon.

Steroid-resistant disease is different. Up to a third of children with initial resistance have a monogenic cause, and complications are frequent — growth impairment, obesity, hypertension, cardiovascular risk, osteoporosis, cataracts, calcineurin inhibitor nephrotoxicity and progressive CKD. Kidney failure is three to four times more likely with monogenic or multidrug-resistant forms.

For adult nephrologists receiving these patients, relapse in adulthood should be expected rather than treated as a surprise, and the treatment burden carried from childhood needs active screening.

• Expect relapses in adults with childhood frequently relapsing or steroid-dependent nephrotic syndrome.
• Screen transitioned patients for bone density loss, cataracts, hypertension and obesity from cumulative steroids.
• Offer genetic testing in steroid-resistant disease; up to a third have a monogenic cause.
• Monitor kidney function closely in those on long-term calcineurin inhibitors.
• Build a structured transition clinic rather than a single handover letter.

Why it matters

It overturns the reassurance that steroid-sensitive nephrotic syndrome reliably burns out by puberty.

Don't overread it

This is a narrative review; the relapse and complication figures come from cohorts of varying quality.

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