Minimal change disease



Introduction
Epidemiology
Aetiology
Pathophysiology
Clinical features
Management
Prognosis


Introduction

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

Epidemiology

  • Incidence: 1.00 cases per 100,000 person-years
  • Peak incidence: 1-5 years
  • Sex ratio: 1:1
<1 1-5 6+ 16+ 30+ 40+ 50+ 60+ 70+ 80+

Aetiology

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

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

Clinical features

Management

  • Majority of cases (80%) are steroid-responsive
  • Cyclophosphamide is the next step for steroid-resistant cases

Prognosis

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