From the Guidelines
The latest treatment for Focal Segmental Glomerulosclerosis (FSGS) typically involves a combination of medications targeting different aspects of the disease, with first-line therapy consisting of corticosteroids such as prednisone at 1 mg/kg/day (maximum 80 mg) for 4-16 weeks, followed by a slow taper if response occurs, as recommended by the most recent guideline commentary 1.
Treatment Overview
The treatment approach for FSGS is multifaceted, aiming to reduce proteinuria, preserve kidney function, and prevent progression to end-stage kidney disease.
- First-line therapy: Corticosteroids like prednisone are commonly used, with a dosage of 1 mg/kg/day (maximum 80 mg) for 4-16 weeks, followed by a slow taper if response occurs.
- Steroid-resistant cases: Calcineurin inhibitors such as cyclosporine (3-5 mg/kg/day divided into two doses) or tacrolimus (0.05-0.1 mg/kg/day divided into two doses) are often added, with the most recent commentary suggesting CNIs as the preferred second-line agent for primary FSGS 1.
- Supportive care: Essential for reducing proteinuria and controlling blood pressure, typically starting with medications like lisinopril (10-40 mg daily) or losartan (50-100 mg daily).
Considerations and Recent Updates
- The importance of proteinuria reduction is highlighted, with benefits potentially outweighing the negative side effects of CNI, provided close monitoring of kidney function and drug levels is maintained 1.
- Recent updates to guidelines recommend considering CNIs for patients with a relative contraindication to steroids and emphasize the importance of individualizing treatment based on FSGS subtype, disease severity, and patient characteristics.
Investigational Therapies
- Newer investigational therapies include sparsentan (a dual endothelin and angiotensin receptor antagonist) and adrenocorticotropic hormone gel, offering potential future treatment options for FSGS.
Clinical Decision Making
Treatment decisions should be guided by the most recent and highest quality evidence, prioritizing morbidity, mortality, and quality of life outcomes, as emphasized in the latest commentary on the management of glomerular diseases 1.
From the Research
Latest Treatments for Focal Segmental Glomerulosclerosis (FSGS)
- Immunosuppressive therapy is a common treatment approach for FSGS, with various regimens used to manage the disease 2, 3, 4.
- Steroids, such as prednisolone, are often used as the initial treatment for FSGS, with or without additional immunosuppressive agents like azathioprine, cyclosporin, or mycophenolate mofetil 2, 3, 4.
- Combination therapies, such as cyclosporin-combined steroids or leflunomide-combined steroids, have shown efficacy in achieving total remission and reducing proteinuria in FSGS patients 3.
- Extracorporeal therapies, including plasma exchange therapy, immunoadsorption, and low-density lipoprotein apheresis, have emerged as potential treatment options for drug-resistant and recurrent FSGS patients 5.
Treatment Outcomes and Prognosis
- Remission of nephrotic syndrome is a key predictor of kidney survival in FSGS patients, with complete remission associated with a 5-year kidney survival rate of 90% in children 6.
- Treatment with corticosteroids and immunosuppressive agents can achieve remission rates of up to 80% in adult FSGS patients, with remission being an independent predictor of survival off dialysis 4.
- Baseline serum creatinine and remission are independently associated with survival off dialysis in FSGS patients, highlighting the importance of early and effective treatment 4.
Ongoing Research and Future Directions
- Further studies are needed to clarify the optimal treatment regimens for FSGS, including the use of combination therapies and extracorporeal treatments 3, 4, 5.
- Research into the pathogenesis of FSGS and the development of new therapeutic agents may lead to improved treatment outcomes and prognosis for patients with this disease 3, 6, 5.