Minimal change disease

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

Pathophysiology

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

Features

nephrotic syndrome
normotension - hypertension is rare
highly selective proteinuria
- only intermediate-sized proteins such as albumin and transferrin leak through the glomerulus
renal biopsy
- normal glomeruli on light microscopy
- electron microscopy shows fusion of podocytes and effacement of foot processes

Management

oral corticosteroids: majority of cases (80%) are steroid-responsive
cyclophosphamide is the next step for steroid-resistant cases

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

+PassMedicine Notes

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

Pathophysiology

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

Features