Focal Segmental Glomerulosclerosis: Difference between revisions
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== Definition/Description == | == 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.<ref name="Goodman">Goodman C, Fuller K. Pathology. Implications for the Physical Therapist. 3rd edition. St. Louis, MO: Saunders Elseveir; 2009. 949-950.</ref> When these filters are damaged they become scarred and are no longer able to filter blood appropriately.<ref name="University">University of North Carolina Kidney Center. Focal Segmental Glomerulosclerosis (FSGS). Available at: http://www.unckidneycenter.org/kidneyhealthlibrary/fsgs.html. Accessed March 16, 2011.</ref> “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.<ref name="University" />[[Image:FSGS.jpg|frame|right|FSGS Scar Tissue]] | 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.<ref name="Goodman">Goodman C, Fuller K. Pathology. Implications for the Physical Therapist. 3rd edition. St. Louis, MO: Saunders Elseveir; 2009. 949-950.</ref> When these filters are damaged they become scarred and are no longer able to filter blood appropriately.<ref name="University">University of North Carolina Kidney Center. Focal Segmental Glomerulosclerosis (FSGS). Available at: http://www.unckidneycenter.org/kidneyhealthlibrary/fsgs.html. Accessed March 16, 2011.</ref> “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.<ref name="University" />[[Image:FSGS.jpg|frame|right|435x300px|FSGS Scar Tissue]] | ||
Glomerular damage produces two types of syndromes: the nephrotic syndrome and the nephritic syndrome.<ref name="Goodman" /> 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.<ref name="Goodman" /> Nephrotic syndrome is commonly associated with proteinuria, protein in the urine, which occurs in diseases such as diabetes, amyloidosis, and membranous glomerulopathy.<ref name="Goodman" /> 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.<ref name="Goodman" /> | Glomerular damage produces two types of syndromes: the nephrotic syndrome and the nephritic syndrome.<ref name="Goodman" /> 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.<ref name="Goodman" /> Nephrotic syndrome is commonly associated with proteinuria, protein in the urine, which occurs in diseases such as diabetes, amyloidosis, and membranous glomerulopathy.<ref name="Goodman" /> 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.<ref name="Goodman" /> | ||
Revision as of 00:05, 5 April 2011
Original Editors - Jessie O'Donley from Bellarmine University's Pathophysiology of Complex Patient Problems project.
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Definition/Description[edit | edit source]
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[edit | edit source]
Typically, idiopathic FSGS is observed in people the age of 18-45 years, although no age group is free 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 with nephrotic syndrome.[3] FSGS is 3-7 times higher in young African American males when compared to Caucasian males.[3] The annual incidence of FSGS in patients aged 18-45 years was 20 cases per one million in African Americans.[3]
Characteristics/Clinical Presentation[edit | edit source]
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[edit | edit source]
See etiology/causes for a full list of co-morbidities associated with primary and secondary FSGS.
Medications[edit | edit source]
The goals of pharmacotherapy are to preserve renal function, reduce morbidity, and to prevent complications.[3] Patients with FSGS should be treated with angiotensin inhibition, an ACE inhibitor or an angiotensin II receptor blocker in order to help decrease protein loss and blood pressure.[4] Patients with nephrotic syndrome should be treated with a statin. The primary disorder should be treated in patients with secondary FSGS. A trial of immunosuppressive therapy, corticosteroids and sometimes cytotoxic drugs, is indicated in idiopathic FSGS if proteinuria reaches the nephrotic range or if renal dysfunction is present.[4]
Diagnostic Tests/Lab Tests/Lab Values[edit | edit source]
A Urinalysis is commonly used to evaluate Blood Urea Nitrogen (BUN), serum creatinine, and 24-hour urinary protein excretion.[4] A Urinalysis usually reveals large amounts of protein, along with hyaline and broad waxy casts, whereas RBC casts are generally absent.[3] A Kidney biopsy however, is the most definitive way to establish the diagnosis of FSGS. The biopsy typically shows focal and segmental hyalinization of the glomeruli, often with immunostaining showing IgM and complement (C3) deposits in a nodular and coarse granular pattern.[4] In HIV-associated FSGS, an ultrasound generally reveals large echogenic kidneys.[3]
Etiology/Causes[edit | edit source]
FSGS can be placed into two categories: primary and secondary. Primary FSGS is idiopathic without an exact known cause. Secondary FSGS can have many different causes/associations. The scarring may be the result of an infection, or drug toxicity, or a disease that affects the entire body, such as diabetes, HIV infection, sickle cell disease or lupus disease.[6]
• Primary (idiopathic) FSGS [2][3][4][6][7]
• FSGS with hyalinosis
• Progression from minimal-change disease
• Progression from immunoglobulin M (IgM) nephropathy
• Progression from mesangial proliferative glomerulonephritis
• Superimposed on other primary glomerulonephritis conditions
• Variants of primary FSGS
• Collapsing form
• Cellular variant (endocapillary and extracapillary hypercellularity)
• FSGS with mesangial hypercellularity
• FSGS with glomerular tip lesions
• Secondary FSGS [2][3][4][6][7]
• Drugs
• Intravenous heroin
• Analgesics
• Viruses
• Hepatitis B
• HIV
• Parvovirus
• Hemodynamic factors - With reduced renal mass
• Solitary kidney
• Renal allograft
• Renal dysplasia
• Renal agenesis
• Oligomeganephronia
• Segmental hypoplasia
• Vesicoureteric reflux
• Hemodynamic causes - Without reduced renal mass
• Massive obesity
• Sickle cell nephropathy
• Congenital cyanotic heart disease
• Malignancies
• Lymphomas
• Other malignancies
• Scarring - Post inflammatory in post infectious glomerulonephritis
• Miscellaneous
• Hypertensive nephrosclerosis
• Alport syndrome
• Sarcoidosis
• Radiation nephritis
Systemic Involvement[edit | edit source]
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Medical Management (current best evidence)[edit | edit source]
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Physical Therapy Management (current best evidence)[edit | edit source]
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Alternative/Holistic Management (current best evidence)[edit | edit source]
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Differential Diagnosis[edit | edit source]
Potential differential diagnoses:[3]
• minimal-change disease
• mesangial proliferative glomerulonephritis
• membranoproliferative glomerulonephritis
• membranous glomerulonephritis
Case Reports/ Case Studies[edit | edit source]
add links to case studies here (case studies should be added on new pages using the case study template)
Resources
[edit | edit source]
The Nephcure Foundation: http://www.nephcure.org/
National Kidney Foundation: http://www.kidney.org/
Recent Related Research (from Pubmed)[edit | edit source]
References[edit | edit source]
see adding references tutorial.
- ↑ 1.0 1.1 1.2 1.3 1.4 Goodman C, Fuller K. Pathology. Implications for the Physical Therapist. 3rd edition. St. Louis, MO: Saunders Elseveir; 2009. 949-950.
- ↑ 2.0 2.1 2.2 2.3 2.4 2.5 University of North Carolina Kidney Center. Focal Segmental Glomerulosclerosis (FSGS). Available at: http://www.unckidneycenter.org/kidneyhealthlibrary/fsgs.html. Accessed March 16, 2011.
- ↑ 3.00 3.01 3.02 3.03 3.04 3.05 3.06 3.07 3.08 3.09 3.10 Rao TS, Soman AS. eMedicine from WebMD. Focal Segmental Glomerulosclerosis. February 17, 2009. Available at: http://emedicine.medscape.com/article/245915-overview. Accessed March 16, 2011.
- ↑ 4.0 4.1 4.2 4.3 4.4 4.5 4.6 McMillan JI. The Merck Manuals Online Medical Library. Nephrotic Syndrome (Focal Segmental Glomerulosclerosis). January 2010. Available at: http://www.merckmanuals.com/professional/sec17/ch235/ch235c.html#sec17-ch235-ch235c-879. Accessed March 16, 2011.
- ↑ Patel P. Medline Plus. Focal Segmental Glomerulosclerosis. May 20, 2009. Available at: http://www.nlm.nih.gov/medlineplus/ency/article/000478.htm. Accessed March 16, 2011.
- ↑ 6.0 6.1 6.2 National Kidney Foundation. Focal Segmental Glomerulosclerosis. September 2008. Available at: http://www.kidney.org/atoz/content/focal.cfm. Accessed March 17, 2011.
- ↑ 7.0 7.1 National Institute of Diabetes and Digestive and Kidney Diseases. Glomerular Disease Primer: Selected Glomerular Diseases (Focal Segmental Glomerulosclerosis (FSGS)). February 25, 2011. Available at: http://www2.niddk.nih.gov/NIDDKLabs/Glomerular_Disease_Primer/SelectedGlomerularDiseases.htm#d. Accessed March 16, 2011.
