🔬 Other

Focal Segmental Glomerulosclerosis (FSGS)

Also known as: FSGS, Focal Sclerosis, Segmental Glomerular Sclerosis

Focal Segmental Glomerulosclerosis (FSGS) is a pathological lesion of the kidney characterized by sclerosis (scarring) of some glomeruli and in only segments of each glomerulus. It is a relatively common cause of nephrotic syndrome in children and adults, potentially leading to end-stage renal disease. FSGS can be primary (idiopathic) or secondary to other conditions such as infections, drugs, or genetic mutations.

ICD-10: N04 Orphanet: 79447 OMIM: 603278
👥 Approximately 7 per million people Prevalence
🔬 50 Active Trials

Imagine your kidney has tiny filters that clean your blood. FSGS is when some of these filters get clogged and scarred, letting protein leak into your pee. This can cause swelling and other problems, so doctors try to help the filters work better with medicine and diet.

Signs & Symptoms

  • Proteinuria (protein in the urine)
  • Edema (swelling, especially in the ankles and feet)
  • Weight gain
  • High blood pressure
  • Fatigue
  • Elevated cholesterol levels
  • Decreased kidney function

Treatment Options

MEDICATION FDA Approved

Corticosteroids (e.g., Prednisone)

MODERATELY EFFECTIVE
MEDICATION FDA Approved

Immunosuppressants (e.g., Cyclosporine, Tacrolimus, Mycophenolate Mofetil)

MODERATELY EFFECTIVE
MEDICATION FDA Approved

ACE inhibitors or ARBs (e.g., Lisinopril, Losartan)

HIGHLY EFFECTIVE
MEDICATION FDA Approved

Diuretics (e.g., Furosemide)

SUPPORTIVE
SUPPORTIVE

Dietary Modifications (low-sodium diet)

SUPPORTIVE
SUPPORTIVE FDA Approved

Dialysis

SUPPORTIVE
SURGERY FDA Approved

Kidney Transplantation

HIGHLY EFFECTIVE
MEDICATION FDA Approved

Lipid-lowering medications (Statins)

SUPPORTIVE

Diagnosis

  • Urinalysis (to detect proteinuria)
  • Blood tests (to assess kidney function, cholesterol levels, and albumin levels)
  • Kidney biopsy (to confirm the diagnosis and determine the type of FSGS)

History

FSGS was first described as a distinct entity in the mid-20th century. Over time, understanding of its various causes and subtypes has evolved, leading to more targeted treatment approaches. Research continues to focus on identifying the underlying mechanisms and developing more effective therapies.

Recent Breakthroughs

2022

Identification of Novel Genetic Mutations Associated with FSGS

Recent studies have identified new genetic mutations that contribute to the development of FSGS, providing potential targets for future therapies.

2023

Development of Targeted Therapies for Specific FSGS Subtypes

Researchers are developing targeted therapies based on the underlying cause of FSGS, such as gene therapies for genetic forms of the disease.