Minimal change disease

Question 1

Minimal change disease?

Answer 1

Minimal change disease nearly always presents as nephrotic syndrome, accounting for 75% of cases in children and 25% in adults.

• The majority of cases are idiopathic, but in around 10-20% a cause is found:
• drugs: NSAIDs, rifampicin
• Hodgkin’s lymphoma, thymoma
• infectious mononucleosis

Question 2

Pathophysiology?

Answer 2

• T-cell and cytokine mediated damage to the glomerular basement membrane → polyanion loss
• the resultant reduction of electrostatic charge → increased glomerular permeability to serum albumin

Question 3

Features?

Answer 3

• nephrotic syndrome
• normotension - hypertension is rare
• highly selective proteinuria*
• renal biopsy: electron microscopy shows fusion of podocytes

Question 4

Management?

Answer 4

• majority of cases (80%) are steroid responsive
• cyclophosphamide is the next step for steroid resistant cases

Question 5

Prognosis?

Answer 5

Prognosis is overall good, although relapse is common. Roughly:

• 1/3 have just one episode
• 1/3 have infrequent relapses
• 1/3 have frequent relapses which stop before adulthood