Immunoglobulin A Nephropathy (IgAN) management

Primary GNeasy

A 32-year-old man is found to have IgA nephropathy on kidney biopsy (mild mesangial hypercellularity). His eGFR is 65 mL/min/1.73 m2, urine protein is 0.9 g/day, and he has hypertension. According to the KDIGO 2025 IgAN guideline, which is the most appropriate initial management step?

  1. A.Start a maximally tolerated ACE inhibitor or ARBCorrect
  2. B.Start high-dose systemic glucocorticoid therapy
  3. C.Start cyclophosphamide for aggressive immunosuppression
  4. D.Perform tonsillectomy to reduce mucosal IgA

Explanation

Optimized supportive care is the foundation of IgAN management. KDIGO 2025 recommends a maximally tolerated dose of an ACE inhibitor or ARB in patients with proteinuria and/or hypertension to reduce intraglomerular pressure and proteinuria, alongside blood-pressure and cardiovascular-risk control and (increasingly) an SGLT2 inhibitor. Immunosuppression (glucocorticoids) is considered only for patients who remain at high risk of progression (proteinuria >0.75-1 g/day) despite ~90 days of optimized supportive care; cyclophosphamide is reserved for rapidly progressive/crescentic disease, and tonsillectomy is not a treatment for IgAN outside Japan.

Why each option

A.
Correct. Maximally tolerated RAS inhibition is first-line supportive care for IgAN with proteinuria/hypertension.
B.
Glucocorticoids are reserved for persistent high-risk proteinuria despite optimized supportive care, not initial therapy.
C.
Cyclophosphamide is only for rapidly progressive/crescentic IgAN.
D.
Tonsillectomy is not recommended as IgAN treatment (except possibly in Japan).

Reference: KDIGO 2025 Clinical Practice Guideline for the Management of IgA Nephropathy (IgAN) and IgA Vasculitis (IgAV). Kidney International, 2025.

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