DISEASE SCANNER

Global Incurable Diseases Tracker

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Nephrologic Disease

Focal Segmental Glomerulosclerosis (FSGS)

HIGH SEVERITY

A rare kidney disorder characterized by scarring (sclerosis) in scattered regions (focal) of the kidney's glomeruli (segmental). It is a leading cause of nephrotic syndrome and end-stage renal disease. Can be primary (idiopathic) or secondary to various conditions including genetic mutations, infections, or drug toxicity.

Global Affected

540.0K

Countries

15

Symptoms

Proteinuria (foamy urine)
Edema (swelling in legs/face)
Hypoalbuminemia
Hyperlipidemia
Fatigue
Weight gain from fluid retention
Decreased urine output

Treatment Options

Corticosteroids
Calcineurin inhibitors (tacrolimus, cyclosporine)
ACE inhibitors/ARBs
Diuretics for edema
Statin therapy
Plasma exchange
Rituximab
Kidney transplantation

Risk Factors

1Genetic mutations (APOL1, TRPC6, ACTN4)
2Obesity
3Sickle cell disease
4HIV infection
5Heroin use
6Certain medications (pamidronate, interferon)
7Family history
8African ancestry (APOL1 risk variants)

Diagnostic Methods

  • 1Urinalysis (proteinuria)
  • 2Blood tests (albumin, lipids)
  • 3Kidney biopsy (gold standard)
  • 4Genetic testing
  • 5Renal ultrasound
  • 624-hour urine protein

Prognosis

Variable; primary FSGS has 50-70% progression to ESRD within 10 years without treatment. Response to steroids improves prognosis. Recurrence common after transplant (20-50%).

Prevention

  • Blood pressure control
  • Weight management
  • Avoid nephrotoxic drugs
  • Genetic counseling for familial forms

Research Status

Immunosuppressive therapy with corticosteroids, calcineurin inhibitors. ACE inhibitors/ARBs for proteinuria control. Plasma exchange for recurrent FSGS post-transplant. Novel therapies targeting APOL1 variants in development.

Affected Countries

AustraliaBrazilCanadaChinaGermanySpainFranceUnited KingdomIndiaItalyJapanNigeriaNew ZealandUnited StatesSouth Africa

Sources

  • https://www.kidney.org/atoz/content/focal-segmental-glomerulosclerosis
  • https://www.ncbi.nlm.nih.gov/books/NBK507702
  • https://www.niddk.nih.gov/health-information/kidney-disease/focal-segmental-glomerulosclerosis

Medical Disclaimer

This information is for educational purposes only. Always consult healthcare professionals for medical advice, diagnosis, and treatment.