Focal segmental glomerulosclerosis medical therapy

Jump to navigation Jump to search

Focal segmental glomerulosclerosis Microchapters

Home

Patient Information

Overview

Historical Perspective

Classification

Pathophysiology

Causes

Differentiating Focal segmental glomerulosclerosis from other Diseases

Epidemiology and Demographics

Risk Factors

Screening

Natural History, Complications and Prognosis

Diagnosis

Diagnostic Study of Choice

History and Symptoms

Physical Examination

Laboratory Findings

Electrocardiogram

Chest X-Ray

CT

MRI

Echocardiography or Ultrasound

Other Imaging Findings

Other Diagnostic Studies

Treatment

Medical Therapy

Surgery

Primary Prevention

Secondary Prevention

Cost-Effectiveness of Therapy

Future or Investigational Therapies

Case Studies

Case #1

Focal segmental glomerulosclerosis medical therapy On the Web

Most recent articles

Most cited articles

Review articles

CME Programs

Powerpoint slides

Images

American Roentgen Ray Society Images of Focal segmental glomerulosclerosis medical therapy

All Images
X-rays
Echo & Ultrasound
CT Images
MRI

Ongoing Trials at Clinical Trials.gov

US National Guidelines Clearinghouse

NICE Guidance

FDA on Focal segmental glomerulosclerosis medical therapy

CDC on Focal segmental glomerulosclerosis medical therapy

Focal segmental glomerulosclerosis medical therapy in the news

Blogs on Focal segmental glomerulosclerosis medical therapy

Directions to Hospitals Treating Focal segmental glomerulosclerosis

Risk calculators and risk factors for Focal segmental glomerulosclerosis medical therapy

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 The use of both corticosteroids and immunosuppressive therapy is recommended for initial treatment in patients with idiopathic FSGS and those who have nephrotic syndrome.[2]

Corticosteroids

Preferred regimen (1) prednisone : Single dose of 1 mg/kg/d (maximum 80 mg/d) or alternate-day dose of 2 mg/kg (maximum 120 mg) PO for minimum 4 weeks - maximum 16 weeks, as tolerated, or until complete remission is achieved.[3]

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.

Contraindication to the use of steroids:

  • Uncontrolled diabetes mellitus
  • Psychiatric conditions
  • Severe osteoporosis

Calcineurin Inhibitors (CNI)

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.[1]

Treatment for Relapse

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. 1.0 1.1 1.2 1.3 1.4 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.
  2. 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.
  3. Banfi G, Moriggi M, Sabadini E, Fellin G, D'Amico G, Ponticelli C (August 1991). "The impact of prolonged immunosuppression on the outcome of idiopathic focal-segmental glomerulosclerosis with nephrotic syndrome in adults. A collaborative retrospective study". Clin. Nephrol. 36 (2): 53–9. PMID 1934660.

Template:WH Template:WS