What follow‑up is recommended in adulthood for a patient with a history of childhood minimal‑change disease?

Follow-Up for Adults with Childhood-Onset Minimal Change Disease

Adults who had minimal change disease (MCD) as children and achieved sustained remission require periodic monitoring every 3–6 months to detect early relapse, with particular attention to urine protein screening and renal function assessment. 1

Understanding Long-Term Prognosis

The excellent news is that long-term kidney survival is outstanding in patients with MCD who responded to glucocorticoids during childhood. 2 Patient survival reaches 83–98% at 15 years, and fewer than 5% progress to end-stage renal disease. 3 However, the disease course differs significantly based on age of onset—childhood-onset MCD demonstrates higher relapse rates compared to adult-onset disease (hazard ratio 1.69), though children also show higher remission probabilities. 4

Relapse Risk and Monitoring Strategy

High-Risk Period for Relapse

• Most relapses occur within the first 6–12 months after achieving remission, particularly in younger adults under age 45. 3
• Overall relapse rates range from 34–85% in adults who initially responded to steroids, with more than half of all steroid-responsive patients experiencing at least one relapse. 1, 3
• Childhood-onset patients who underwent biopsy (typically those less responsive to initial therapy) show even higher relapse rates than adolescent- or adult-onset cases. 4

Recommended Monitoring Schedule

For patients in sustained remission:

• Visit frequency: Every 3–6 months to balance early relapse detection against unnecessary healthcare utilization. 1
• Extending intervals beyond 6 months risks missing early relapses when timely intervention is most effective. 1

At each visit, assess:

• Urine protein screening (dipstick or spot urine protein-to-creatinine ratio) to detect nephrotic-range proteinuria recurrence 1
• Serum creatinine and estimated GFR to monitor kidney function 2
• Blood pressure measurement, as hypertension can develop even in previously normotensive MCD patients 2
• Signs of edema or volume overload suggesting relapse 1

Special Considerations for Former Pediatric Patients

If Previously Treated with Long-Term Immunosuppression

For patients who required calcineurin inhibitors (CNIs) during childhood:

• Monitor serum creatinine closely, as CNIs carry risk of chronic nephrotoxicity even after discontinuation. 2
• If creatinine rises >30% above baseline and doesn't plateau after dose reduction (if still on therapy), the CNI should be discontinued. 2
• Consider repeat renal biopsy at 12–24 months if creatinine remains elevated or if maintenance CNI dose exceeded 3.5 mg/kg/day during treatment, to assess for CNI-related scarring. 1

If Previously Treated with Cyclophosphamide

• Be aware of cumulative gonadal toxicity and long-term oncogenicity risk, though routine cancer screening beyond standard guidelines is not specifically indicated. 1
• Fertility counseling may be appropriate for patients of reproductive age who received cyclophosphamide. 1

Patient Education and Self-Monitoring

Instruct patients to contact their nephrologist immediately if they notice:

• Foamy urine (suggesting proteinuria recurrence) 1
• New-onset edema, particularly periorbital or lower extremity swelling 1
• Unexplained weight gain from fluid retention 1

Patients should NOT wait for their next scheduled appointment if these symptoms develop, as early intervention with glucocorticoids can achieve remission more rapidly. 1

Management of Relapse if It Occurs

If relapse is detected:

• Restart the same corticosteroid regimen that induced initial remission: prednisone 1 mg/kg/day (maximum 80 mg) or 2 mg/kg alternate-day (maximum 120 mg). 1
• Maintain high-dose therapy for a minimum of 4 weeks if remission is achieved. 1
• Begin tapering 2 weeks after complete remission is documented. 2

For frequent relapsers or steroid-dependent patients:

• Steroid-sparing agents (cyclophosphamide, CNIs, mycophenolate mofetil, or rituximab) should be employed to avoid cumulative glucocorticoid toxicity. 2, 1
• Rituximab shows particular efficacy in childhood-onset disease and glucocorticoid-sensitive patients (adjusted HR 2.62 for achieving complete remission). 4

Common Pitfalls to Avoid

Do not assume childhood MCD is "cured" simply because the patient achieved remission and has been asymptomatic for years—relapse rates remain substantial even decades later. 3

Do not delay biopsy if relapse occurs in adulthood—unlike children, adults require biopsy confirmation to distinguish MCD from focal segmental glomerulosclerosis (FSGS), as clinical features overlap and FSGS may develop over time. 2

Do not use statins solely to treat hyperlipidemia or ACE inhibitors/ARBs solely to lower proteinuria in normotensive patients during remission, as evidence does not support these interventions for MCD specifically. 2