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For further information, see CMDT Part 22-15: Minimal Change Disease

Key Features

  • Nephrotic range proteinuria

  • Most common in children; occurs occasionally in adults

  • Can be idiopathic

  • May also occur

    • Following viral upper respiratory infections (especially in children)

    • In association with neoplasms, such as Hodgkin disease

    • With drugs (lithium)

    • With hypersensitivity reactions (especially to nonsteroidal anti-inflammatory drugs and bee stings)

Clinical Findings

  • Nephrotic syndrome

Diagnosis

  • Biopsy should be considered for children

    • With nephrotic syndrome who exhibit unusual features (such as signs of other systemic illness)

    • Who are corticosteroid-resistant

    • Who relapse upon withdrawal of corticosteroid therapy

  • Kidney biopsy

    • Light microscopy and immunofluorescence: no changes

    • Electron microscopy: characteristic effacement of podocyte foot processes

Treatment

  • Prednisone, 60 mg/m2/day orally

    • Can take up to 16 weeks for patient to respond to treatment

    • Continue for several weeks after complete remission of proteinuria

  • Cyclophosphamide, a calcineurin inhibitor, or rituximab for patients with corticosteroid resistance or relapses

  • Progression to end-stage renal disease is rare

  • Complications most often related to prolonged corticosteroid use

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