Focal Segmental Glomerulosclerosis

 

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Original Editors - Jessie O'Donley from Bellarmine University's Pathophysiology of Complex Patient Problems project.

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Definition/Description

Focal Segmental Glomerulosclerosis (FSGS) is a kidney disease that involves the formation of scar tissue in the glomeruli. “Sclerosis” meaning scarring and “glomerulo-” which are tiny filters that filter circulating blood producing urine as an end-product.1 When these filters are damaged they become scarred and are no longer able to filter blood appropriately.2 “Focal” meaning that only some of the filters are damaged while some are normal and “segmental” is included because often only parts of the filters are scarred.2

Glomerular damage produces two types of syndromes: the nephrotic syndrome and the nephritic syndrome.1 The nephrotic syndrome is not a specific kidney disease rather it occurs as a result of any disease that causes damage to the kidney-filtering units.1 Nephrotic syndrome is commonly associated with proteinuria, protein in the urine, which occurs in diseases such as diabetes, amyloidosis, and membranous glomerulopathy.1 FSGS is a nephrotic syndrome due to massive amounts of protein found in the urine. The nephritic syndrome is commonly associated with hematuria, blood in the urine, which occurs in diseases such as lupus nephritis, immunoglobulin A (IgA) nephropathy, and acute diffuse proliferative glomerulonephritis.1

Prevalence

Typically, idiopathic FSGS is observed in people the age of 18-45 years, although no age group is free from from the disease. In children, FSGS is found in 7-10% of renal biopsies.3 In adults, the lesion is more common in men and is observed in 20-30% of patients.3 FSGS is 3-7 times higher in young African American males when compared to Caucasian males.3

Characteristics/Clinical Presentation

It is possible for there to be no signs or symptoms when in the early stages of FSGS. Typically the first sign that a patient commonly recognizes is edema, especially in the legs, and sudden weight gain.2 

Signs and Symptoms: 2,3,4,5
• Massive proteinuria and foamy urine appearance
• Edema, swelling and associated weight gain
• Hypertension
• Renal dysfunction with increased creatinine levels
• Hypoalbuminemia
• Hyperlipidemia
• Microscopic hematuria is occasionally present
• Fatigue
• Poor appetite
• Headache
• Itchy skin
• Shortness of air
• Nausea

Associated Co-morbidities

See etiology/causes for a full list of co-morbidities associated with primary and secondary FSGS.

Medications

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Diagnostic Tests/Lab Tests/Lab Values

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Etiology/Causes

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Systemic Involvement

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Medical Management (current best evidence)

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Physical Therapy Management (current best evidence)

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Alternative/Holistic Management (current best evidence)

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Differential Diagnosis

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Case Reports/ Case Studies

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Resources

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Recent Related Research (from Pubmed)

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References

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