Children under 18 years of age with a confirmed diagnosis of IgA nephropathy who have not reached adequate proteinuria control after a course of systemic oral corticosteroids.
Age under 18 years with IgA nephropathy. Children, including postpubertal adolescents, may have a distinct disease course and treatment response compared with adults, and management is tailored to this age group accordingly.
The previous step used systemic oral glucocorticoids (prednisone or prednisolone, or equivalent). That regimen did not achieve the target of proteinuria ≤200 mg/d or PCR ≤200 mg/g, which triggers escalation to this protocol.
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.
Regimens including intravenous methylprednisolone are also used on an individual basis in patients with higher clinical and histologic risk of progression, such as in (i) children with acute onset of IgAN and worsening of kidney function (eGFR <90 ml/min per 1.73 m²) and/or PCR >1000 mg/g (100 mg/mmol) with active severe MEST-C scores (≥2 of the following scores: M1, E1, S1 with podocyte lesions, and/or C1) or (ii) children with crescentic forms of IgAN (C2).
Dosing regimens may be as follows: 3 methylprednisolone intravenous pulses given at the dose of 15 mg/kg/d each (maximum dose 500 mg) on 3 consecutive or alternate days followed by oral prednisone/prednisolone as indicated above.
Alternatively, the intravenous pulses can be repeated 3 times at 2-month intervals, with oral prednisone/prednisolone given at 0.5 mg/kg/d for 2 months between pulse cycles for a total of 6 months.
Aim for proteinuria ≤200 mg/d (≤400 mg/1.73 m² per day) or PCR ≤200 mg/g (≤20 mg/mmol).
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