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:
Pathophysiology
Features
Management
Prognosis is overall good, although relapse is common. Roughly:
*only intermediate-sized proteins such as albumin and transferrin leak through the glomerulus
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*
- renal biopsy: electron microscopy shows fusion of podocytes
Management
- 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
*only intermediate-sized proteins such as albumin and transferrin leak through the glomerulus