IgA Nephropathy in Children: What to Do When Glucocorticoid Pulse Therapy Has Not Achieved Proteinuria Targets
This protocol addresses children under 18 years of age with confirmed IgA nephropathy whose proteinuria has not been adequately controlled following a course of glucocorticoid pulse therapy.
Clinical scenario
Patient is aged under 18 years with a diagnosis of IgA nephropathy. Children may differ from adults in disease course and response to treatment, and this includes postpubertal patients.
Escalation trigger — previous treatment did not reach its target
The prior treatment step involved intravenous methylprednisolone pulse therapy. This protocol is indicated when that step has not achieved the target of proteinuria ≤200 mg/d or a urine protein-to-creatinine ratio ≤200 mg/g.
Next-step approach — partial overview
When glucocorticoid pulse therapy has not controlled proteinuria in a child with IgA nephropathy, the approach involves adding an immunosuppressant alongside glucocorticoids. The choice of agent and the specific regimen — including any considerations for rapidly progressive disease — are defined in the full protocol.
References
In this guideline, we define children as those aged <18 years, but it is acknowledged that postpubertal children may in some respects have a similar course and response to treatment as adults with IgAN.
Another potential approach in these children is the use of immunosuppressants (e.g., calcineurin inhibitors, cyclophosphamide, mizoribine where available, mycophenolate mofetil, or rituximab) in addition to glucocorticoids.
As in adults, children with rapidly progressive IgAN have a poor outcome, and despite limited evidence, this subgroup should be offered treatment with systemic glucocorticoids (usually as methylprednisolone pulses) and cyclophosphamide.
View source ↗