Focal segmental proliferative glomerulonephritis - Glomerular disease

Focal segmental proliferative glomerulonephritis

Definition

Focal segmental proliferative glomerulonephritis is characterised by cellular proliferation affecting only one segment of the glomerulus and occurring in only a proportion of all glomeruli.

Aetiology

This histological pattern is caused by:

·        Primary glomerular diseases such as IgA nephropathy (also called mesangial IgA disease or Berger’s disease) and Goodpasture’s disease (anti-GBM disease).

·        Systemic diseases such as systemic lupus erythematosus (SLE), microscopic polyarteritis, Wegener’s granulomatosis, infective endocarditis and Henoch– Schonlein¨ purpura.

Pathophysiology

There are immune complexes deposited in the glomerular tuft. The reaction to this is localised inflammation and mesangial proliferation, causing reduction of renal blood flow, leading to haematuria and in some acute cases, nephritic syndrome. In severe cases, the necrosis of the glomerular cells stimulates crescent formation (rapidly progressive GN). Whereas IgA nephropathy tends to follow a slower, more benign course, a more florid form occurs in Goodpasture’s disease and the systemic causes in particular.