From the Guidelines
The long-term prognosis for a patient with Focal Segmental Glomerulosclerosis (FSGS) who has failed two kidney transplants is generally poor, with significant challenges ahead, and management typically involves maintenance dialysis and supportive care to mitigate complications and improve quality of life 1. The patient faces a high risk of end-stage renal disease requiring ongoing dialysis, with limited options for subsequent transplantation due to the high recurrence rate of FSGS in transplanted kidneys (approximately 30-50%) 1. Key considerations in the management of these patients include:
- Maintenance dialysis (either hemodialysis three times weekly or peritoneal dialysis daily)
- Supportive care including blood pressure control with ACE inhibitors or ARBs
- Dietary sodium and protein restrictions
- Management of complications like anemia, mineral bone disease, and cardiovascular issues Some centers may consider another transplant attempt with intensive pre-transplant plasmapheresis, high-dose rituximab, and maintenance immunosuppression with tacrolimus, mycophenolate mofetil, and prednisone 1. The poor prognosis stems from FSGS's aggressive nature, its tendency to recur rapidly in transplanted kidneys, and the development of allosensitization after multiple transplants, which makes finding compatible donors increasingly difficult 1. Five-year survival on dialysis is approximately 40-50% for these complex patients, significantly lower than the general dialysis population. It is essential to prioritize the patient's quality of life, provide psychological support, and ensure coordination of care among teams to address the complex needs of these patients 1.
From the Research
Long-Term Prognosis of FSGS Patients with Failed Kidney Transplants
- The long-term prognosis for patients with Focal Segmental Glomerulosclerosis (FSGS) who have failed two kidney transplants is generally poor, with a high risk of recurrence of the disease in subsequent transplants 2, 3.
- Studies have shown that patients who experience recurrence of FSGS after a first transplant are likely to experience recurrence in subsequent transplants as well 3, 4.
- The risk factors for recurrence of FSGS include onset of the disease during childhood, rapid progression of the disease to end-stage renal disease, and history of recurrent FSGS in previous transplants 2, 5.
- Treatment strategies for recurrent FSGS are limited, and there is no established effective treatment for preventing or treating the disease 2, 6.
- Plasmapheresis and high doses of cyclosporine are commonly used to treat recurrent FSGS, but their effectiveness is limited, and new treatments such as rituximab are being explored 4, 6.
Factors Affecting Prognosis
- Age at diagnosis: Younger patients are more likely to experience recurrence of FSGS 3, 4.
- Rate of progression to end-stage renal disease: Patients with rapid progression are more likely to experience recurrence 2, 5.
- History of recurrent FSGS: Patients who have experienced recurrence in previous transplants are more likely to experience recurrence in subsequent transplants 3, 4.
- Type of FSGS: Certain types of FSGS, such as the collapsing variant, are associated with a higher risk of recurrence 5.
Treatment Options
- Plasmapheresis: A procedure that removes circulating permeability factors from the blood, which may help to reduce proteinuria and slow disease progression 4, 6.
- Cyclosporine: An immunosuppressive medication that may help to reduce proteinuria and slow disease progression 4, 6.
- Rituximab: A monoclonal antibody that targets B cells and may help to reduce proteinuria and slow disease progression 6.
- Renin-angiotensin-aldosterone system inhibition: Medications that block the renin-angiotensin-aldosterone system, which may help to reduce proteinuria and slow disease progression 2, 6.