2016 Volume 51 Issue 2-3 Pages 100-107
Focal segmental glomerulosclerosis (FSGS) is a histological pattern of injury in the kidney that has multiple different etiology, including obesity, viral infection, drug, and genetic disease. Primary idiopathic FSGS, its etiology is still not well defined, and it commonly progresses to end stage renal disease (ESRD). When a transplantation is performed, primary idiopathic FSGS may recur in the renal allograft. The reported FSGS recurrence rate averages approximately 30%, and patients who develop recurrent disease in the first transplant are at very high risk （>80%） for recurrence in subsequent allografts. Five-year graft survival rate for recurrent FSGS is about 50% with persistent nephrotic syndrome. Major risk factors for recurrence of FSGS include childhood onset of initial disease, rapid progression of initial disease, and a history of recurrence in a prior allograft. The histologic subtype of FSGS does not appear to predict the risk of recurrence, and a family history generally predicts a low risk of recurrence. Patients who have recurrent primary FSGS typically present with the rapid onset of proteinuria, which is frequently in the nephrotic range. The diagnosis of FSGS is made by renal biopsy findings in the setting of significant proteinuria （>1 gram/day）. No immunosuppressive therapies have been conclusively shown to prevent FSGS in the transplanted kidney. Though no standard treatment for recurrent FSGS is verified yet, prolonged beneficial results have been reported with plasmapheresis and rituximab. Although definitive data are not available, early initiation of these therapies may be recommended after the onset of recurrent disease before development of irreversible histologic changes.
