IgA Nephropathy
ICD-10 N02.8 · ICD-11 MF8Y.1

Treatment of IgA Nephropathy with Rapidly Progressive Glomerulonephritis and ≥50% Decline in eGFR

Clinical Scenario

A patient with IgA nephropathy who experiences a rapid, severe loss of kidney function — defined as a 50% or greater fall in eGFR within three months — with histological features on biopsy consistent with rapidly progressive glomerulonephritis.

Defining Features: Rapidly Progressive Glomerulonephritis

Rapidly progressive IgAN is defined as a ≥50% decline in eGFR over ≤3 months, after excluding other causes of RPGN (such as ANCA-associated vasculitis and anti-GBM disease) and reversible causes. Kidney biopsy is essential in these cases and commonly demonstrates mesangial and endocapillary hypercellularity, a high proportion of glomeruli affected by crescents, and areas of focal necrosis.

Treatment Approach (partial overview)

Management involves immunosuppressive therapy with cyclophosphamide combined with systemic glucocorticoids, following established vasculitis practice guidelines, alongside prophylactic measures accompanying immunosuppression.

The complete structured regimen — including sequencing, monitoring, and all prophylactic components — is available in the full protocol.

Instant Access to Structured Evidence-Based Regimens

References

  1. Rapidly progressive IgAN is defined as a ≥50% decline in eGFR over ≤3 months, where other causes of RPGN (e.g., AAV and anti-GBM disease) and reversible causes (e.g., drug toxicity and common pre- and post-kidney causes) have been excluded.
  2. A kidney biopsy is essential in these cases and will commonly demonstrate mesangial and endocapillary hypercellularity as well as a high proportion of glomeruli affected by crescents with areas of focal necrosis.
  3. Patients with rapidly progressive IgAN should be offered treatment with cyclophosphamide and systemic glucocorticoids in accordance with the KDIGO 2024 Clinical Practice Guideline for the Management of Antineutrophil Cytoplasmic Antibody (ANCA)-Associated Vasculitis.
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