Nephrotic focal segmental glomerulosclerosis in 2004: an update

Writing on the treatment of primary focal segmental glomerulosclerosis (FSGS) is a challenge. The jaded nephrologist is tempted to consider that the issue could be summarized as ‘Treatment of a disease which is not a disease but a lesion of obscure pathophysiology, with drugs the mechanism of action of which is poorly understood’ and flinch from the task. However, the knowledge on this topic has so tremendously progressed over recent years that obsolescence looms with each passing year. Let us try to analyse the subject using a Cartesian approach, that is, dissecting the problem step by step. This review will focus on the nephrotic forms of this nephropathy, as mildly proteinuric FSGS is probably a different entity in terms of pathophysiology, prognosis and treatment.