New insights into the pathogenesis and the therapy of recurrent focal glomerulosclerosis.
Recurrent focal glomerulosclerosis (FSGS) in renal allografts has remained a frustrating and enigmatic disease. Recent studies on gene mutations encoding podocin and other components of the slit-diaphragm in patients with native kidney nephrotic syndrome have underscored the heterogenecity of the idiopathic form of FSGS. While familial FSGS rarely recurs following transplantation, the sporadic variety of FSGS is associated with a 30% recurrence rate. The patients with the sporadic variety of FSGS who have homozygous or complex heterozygous podocin mutations have a low recurrence rate. In the other patients with sporadic FSGS, a more complex and likely multifactorial etiology accounts for the recurrence of FSGS. The role of CD80 expression on podocytes is intriguing but requires confirmation in kidney biopsies of patients with recurrent FSGS. Recent findings on podocin genomics, the permeability factor and CD80 expression may ultimately lead to a better understanding of recurrent FSGS as well as a more effective approach to its prevention and treatment.
Vincenti
Flavio
F
Kidney Transplant Service, University of California, San Francisco, USA. vincentif@surgery.ucsf.edu
Ghiggeri
Gian Marco
GM
D020022:Genetic Predisposition to Disease; D005923:Glomerulosclerosis, Focal Segmental / Q000209:etiology* / Q000628:therapy*; D006801:Humans; D047908:Intracellular Signaling Peptides and Proteins; D016030:Kidney Transplantation; D008565:Membrane Proteins / Q000235:genetics; D009154:Mutation / Q000235:genetics; D012008:Recurrence
D047908:Intracellular Signaling Peptides and Proteins; D008565:Membrane Proteins; C407223:NPHS2 protein