Minimal Change Disease

Primary GNmedium

A 28-year-old woman presents with sudden periorbital and pedal edema, nephrotic-range proteinuria (8 g/day), and hypoalbuminemia (1.9 g/dL). There is no hematuria and kidney function is normal. Biopsy shows normal light microscopy, negative immunofluorescence, and diffuse podocyte foot-process effacement on electron microscopy, confirming minimal change disease. She receives prednisone 1 mg/kg/day. After 16 weeks of full-dose therapy her proteinuria has fallen only to 3.5 g/day. Which of the following best characterizes her disease, and what is the next step?

  1. A.Steroid-resistant disease — repeat the biopsy and switch to a calcineurin inhibitorCorrect
  2. B.Partial remission — simply continue the same full-dose prednisone for another 4 weeks
  3. C.Frequently relapsing disease — add mycophenolate mofetil as a steroid-sparing agent
  4. D.Steroid-dependent disease — taper the prednisone and add rituximab therapy

Explanation

In adults, KDIGO defines steroid resistance in minimal change disease as failure to achieve complete remission after 16 weeks of full-dose corticosteroids. This patient still has nephrotic-range proteinuria (3.5 g/day) at 16 weeks, meeting that definition. The appropriate next steps are to consider a repeat biopsy to exclude under-sampled focal segmental glomerulosclerosis and to switch to a calcineurin inhibitor (cyclosporine or tacrolimus), the preferred second-line agents. Frequent relapse and steroid dependence are patterns defined only after an initial complete remission, which never occurred here, so they do not apply.

Why each option

A.
Correct. No complete remission after 16 weeks of full-dose steroids defines steroid resistance; repeat biopsy to exclude FSGS and switch to a calcineurin inhibitor.
B.
Continuing full-dose steroids beyond 16 weeks in a non-responder adds toxicity without established benefit.
C.
Frequent relapse requires a prior complete remission followed by relapses; that pattern is absent here.
D.
Steroid dependence also requires a prior complete remission (relapse during taper or shortly after stopping); this patient never remitted.

Reference: KDIGO 2021 Clinical Practice Guideline for the Management of Glomerular Diseases. Kidney Int. 2021;100(4S):S1-S276 (Minimal Change Disease).

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