Focal segmental glomerulosclerosis medical therapy
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]Ali Poyan Mehr, M.D. [2] Associate Editor(s)-in-Chief: Olufunmilola Olubukola M.D.[3]
Overview
Treatment of Idiopathic or primary FSGS is very difficult. Treatment should be tailored towards relieving symptoms and preventing disease progression. The use of loop diuretics to manage the edema and corticosteroids to suppress immune function has been successful in some. However, secondary causes of FSGS are treated by treating the underlying causes. Highly Active Anti-Retroviral Therapy can be used in HIV patients with FSGS. Immunosuppressant like prednisone can be used in immune mediated FSGS. Loop diuretics like furosemide can be used to control the edema and proteinuria. FSGS patients who progress to End Stage Kidney disease would need lifelong dialysis.
Randomized clinical trials have only addressed patients with primary FSGS and have nephrotic-range proteinuria. For those patients, the use of corticosteroids and immunosuppressive therapy followed by conservative therapy with ACE-I or ARBs is recommended, according to the 2012 KDIGO guidelines.[1] For patients with relapse, guidelines for relapsing minimal change disease are recommended for patients with FSGS. Finally, patients with resistance to steroids are recommended to use cyclosporine, or a combination of mycophenolate mofetil and high dose dexamethasone for patients who cannot tolerate cyclosporine.
Medical Therapy
The mainstay of treatment for focal segmental glomerulosclerosis is medical therapy, however dietary therapy is used to control hypertension and proteinuria.
Dietary therapy
Dietary therapy is used to control hypertension, edema, and proteinuria, which include:
- Salt intake should be reduced 2 g of sodium per day
- Protein intake should be 1-1.3 g/kg of body weight
- There should be reduction of fat intake
- Daily exercise is important to reduce weight for obese patients
For symptomatic control
The use of medical therapy in focal segmental glomerulosclerosis (FSGS) is based on the Kidney Disease - Improve Global Outcomes (KDIGO) guidelines of 2012.[1]
Initial Treatment[1]
The use of both corticosteroids and immunosuppressive therapy is recommended for initial treatment in patients with idiopathic FSGS and who have nephrotic syndrome. For patients with secondary FSGS or non-nephrotic range proteinuria, there are currently no reliable clinical trials to support the use of steroids.[2]
Corticosteroids
Either prednisone or prednisolone may be used. Both have equivalent dosage and duration.
- Dose: Single dose of 1 mg/kg/d (maximum 80 mg/d) or alternate-day dose of 2 mg/kg (maximum 120 mg).
- Duration: Minimum 4 weeks - maximum 16 weeks, as tolerated, or until complete remission is achieved.
Steroids should be tapered slowly over a period of 6 months after achieving complete remission, ie. reduction in dose by 10 mg per 2 weeks down to 0.15 mg/kg/d, then every 2-4 weeks by 2.5 mg. Patients with uncontrolled diabetes mellitus, psychiatric conditions, and severe osteoporosis have a relative contraindication to the use of steroids and may benefit more from calcineurin inhibitors (CNI).
In patients with tip lesions, good renal function, and low degrees of proteinuria, the time for initiation of corticosteroids is controversial, and may occur following the use of renin-angiotensin-system (RAS) blockers, such as ACE-inhibitors or ARBs, to assess for possible spontaneous remission.
Calcineurin Inhibitors (CNI)[1]
For patients with relative contraindication to steroids, such as patients with uncontrolled diabetes mellitus, psychiatric conditions, or severe osteoporosis, the use of CNI might be more helpful.
Conservative Treatment[1]
Renin-angiotensin-system (RAS) blockade is also necessary for patients with proteinuria, including those with FSGS. The initiation of RAS blockade may take place following initial corticosteroid therapy in patients with nephrotic syndrome to assess for remission on steroids, which is especially implicated in patients with severe nephrotic syndrome.
Treatment for Relapse[1]
Treatment for relapse is based upon the guidelines for the management of relapse of minimal change disease in adults.
Treatment for Steroid-Resistant FSGS
Cyclosporine[1]
- Dose: 3-5 mg/kg/d in divided doses (initial target: 125-175 ng/ml)
- Duration: At least 4-6 months. Cyclosporine therapy is to be continued for at least 12 months only if there is partial or complete remission with use. For patients who have not achieved any remission by 6 months, discontinuing cyclosporine is recommended.
Slow tapering of cyclosporine should be achieved following the initial duration of use. Tapering should be a reduction in dose by 25% every 2 months. For patients who cannot tolerate cyclosporine, the use of a combination of mycophenolate mofetil and high-dose dexamethasone is recommended.
Tacrolimus and Prednisone
Tacrolimus
- Dose: 0.1-0.2 mg/kg/d in two divided doses (initial target 5-10 ng/ml)
If remission is achieved, follow cyclosporine recommendations.
Prednisone
- Dose: 0.15 mg/kg/d
- Duration: 4-6 months
Prednisone is to be tapered off over 4-8 weeks.
Other
For patients who cannot tolerate cyclosporine, the use of a combination of mycophenolate mofetil (MMF) and high-dose dexamethasone is recommended.[1]
References
- ↑ 1.0 1.1 1.2 1.3 1.4 1.5 1.6 1.7 Beck L, Bomback AS, Choi MJ, Holzman LB, Langford C, Mariani LH; et al. (2013). "KDOQI US commentary on the 2012 KDIGO clinical practice guideline for glomerulonephritis". Am J Kidney Dis. 62 (3): 403–41. doi:10.1053/j.ajkd.2013.06.002. PMID 23871408.
- ↑ Gupta K, Iskandar SS, Daeihagh P, Ratliff HL, Bleyer AJ (2008). "Distribution of pathologic findings in individuals with nephrotic proteinuria according to serum albumin". Nephrol Dial Transplant. 23 (5): 1595–9. doi:10.1093/ndt/gfm833. PMID 18065791.