This is a disease of unknown aetiology which affects all age groups.
It is characterized by segmental glomerular hyalinisation on light microscopy. Initially focal the condition progresses to global sclerosis. Glomeruli at the corticomedullary junction are affected first. As a result renal biopsy often results in a diagnosis of minimal change disease.
Electon microscopy shows fusion of epithelial foot processes, an observation common to almost all causes of proteinuria including minimal change disease.
Immunofluorescence reveals IgM and C3 in a segmental distribution.
The condition is usually steroid resistant. About 50% of patients develop renal failure within 10 years of diagnosis. Sadly the condition often recurs in transplanted kidneys.
Last reviewed 01/2018