Pathogenesis of focal glomerulosclerosis

Focal segmental glomerulosclerosis (FSGS) is the histologic expression of d iverse processes affecting the renal glomeruli and occurring in primary and secondary forms. A number of pathogenic factors have been identified in pr imary FSGS, and multiple etiologies have been defined as contributing facto rs for the development of secondary FSGS, There is a complex interplay betw een etiologic and pathogenic factors, progression factors and intervention in the phenotypic expression of FSGS. Key components include genetic predis position, environmental influences and the impact on phenotype of pharmacol ogic intervention. The phenotypic spectrum for FSGS ranges from mild protei nuria and slow progression to a devastating clinical syndrome characterized by heavy proteinuria and rapid loss of renal function over a period of mon ths. While the pathogenesis is unknown, much is known about factors which a re involved in the development and progression of both primary and secondar y FSGS, The ultimate goal of understanding pathogenesis is to provide speci fic nontoxic therapy for those patients who have a definable form of FSGS, While this goal is not yet in sight, many types of intervention, not addres sed in the current chapter, can influence the course of various diseases pr esenting as FSGS, Until specific therapy can be fashioned, it is necessary for the clinician caring for these patients to appreciate the complex inter action of pathogenetic factors involved in the development and pregression of FSGS, as a rationale for providing intervention to prevent development o f the disease and to slow its course. Copyright (C) 2001 S. Karger AG, Base l.