Materiały źródłowe

• #1 FSGS: Diagnosis and Diagnostic Work-Up

  https://pmc.ncbi.nlm.nih.gov/articles/PMC4894996/

  Focal segmental glomerulosclerosis is a histologic lesion, rather than a clinical disease. FSGS is common cause of nephrotic syndrome in both adults and children worldwide. […] Currently the incidence is estimated to be 7 per million. […] Upon identification of FSGS in a renal biopsy a thorough examination should be initiated to identify the underlying cause. The distinction between primary and secondary FSGS is important for both prognostic and therapeutic reasons. […] A biopsy is necessary to establish the diagnosis of FSGS and determine the subtype of FSGS. […] The distinction between primary and secondary FSGS has important therapeutic implications as primary FSGS often requires immunomodulatory treatment whereas treatment in secondary FSGS should be focused on the reduction of intraglomerular hypertension using RAAS blockade.

• #2 Focal Segmental Glomerulosclerosis: Symptoms & Treatment

  https://my.clevelandclinic.org/health/diseases/21149-focal-segmental-glomerulosclerosis-fsgs

  FSGS is a rare disease. Healthcare providers diagnose it in about 7 out of 1 million people per year. […] Your healthcare provider will listen to your symptoms and take your medical history. Several types of kidney tests may help your provider diagnose FSGS. […] However, providers can only definitively diagnose FSGS by doing a kidney biopsy. This is when a provider uses a needle to take a small sample of tissue from your kidney to look for signs of FSGS under a microscope. […] Treatment for FSGS depends on the type and cause, your age and whether you have other health conditions. The goal of treatment is to manage your symptoms to help you maintain a good quality of life and slow scarring so that it doesn’t lead to kidney failure. […] FSGS is a chronic disease that can’t be undone or reversed. This means treatment can only slow the progression of kidney disease, not cure you from the disease.

• #3 Focal Segmental Glomerulosclerosis – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK532272/

  Focal segmental glomerulosclerosis (FSGS) is histologically characterized by segmental scarring that affects only a portion of the glomerulus and involves some, but not all, glomeruli in a biopsy sample. […] Accurate identification of the underlying etiology is crucial for guiding treatment decisions, predicting prognosis, and assessing transplant risks. […] Diagnosis requires histopathological confirmation, as proteinuria, low serum albumin, and casts on urinalysis can support clinical suspicion. […] Although clinical signs may suggest FSGS, the diagnosis can only be confirmed through histopathological findings. […] The definitive diagnosis is typically made through a kidney biopsy in these cases. […] Genetic testing is recommended for patients with steroid-resistant FSGS, a family history of FSGS, a history of Wilms tumor (nephroblastoma), or other systemic signs suggestive of a genetic syndrome, such as Alport syndrome, Fabry disease, or nail-patella syndrome.

• #4 FSGS: Diagnosis and Diagnostic Work-Up

  https://pmc.ncbi.nlm.nih.gov/articles/PMC4894996/

  Focal segmental glomerulosclerosis is a histologic lesion, rather than a clinical disease. FSGS is common cause of nephrotic syndrome in both adults and children worldwide. […] Currently the incidence is estimated to be 7 per million. […] Upon identification of FSGS in a renal biopsy a thorough examination should be initiated to identify the underlying cause. The distinction between primary and secondary FSGS is important for both prognostic and therapeutic reasons. […] A biopsy is necessary to establish the diagnosis of FSGS and determine the subtype of FSGS. […] The distinction between primary and secondary FSGS has important therapeutic implications as primary FSGS often requires immunomodulatory treatment whereas treatment in secondary FSGS should be focused on the reduction of intraglomerular hypertension using RAAS blockade.

• #5 Focal Segmental Glomerulosclerosis – Specific Pathologies – Glomerular Diseases – Nephrology – Diseases – McMaster Textbook of Internal Medicine

  https://empendium.com/mcmtextbook/chapter/B31.II.14.3.21.6.

  FSGS is more prevalent in African Americans and in males. Edema and proteinuria are the most common clinical manifestations. Nephrotic syndrome is often found and occurs in up to 80% of patients. […] Diagnosis is based on histologic examination of kidney biopsy specimens. At least 20 glomeruli are required to exclude FSGS, given the focal nature of lesions.

• #6 Focal Segmental Glomerulosclerosis: Practice Essentials, Pathophysiology, Etiology

  https://emedicine.medscape.com/article/245915-overview

  Focal segmental glomerulosclerosis (FSGS) is one of the most common causes of primary glomerular disease in adults. In adults undergoing kidney biopsy for evaluation of proteinuria, FSGS accounts for 35% of all cases and up to 80% of cases in Black patients. However, no age group is exempt. […] A diagnosis of FSGS is established only by histopathology findings. Detailed medical history and pertinent laboratory tests are essential to distinguish between the different types of FSGS. In massively obese patients, FSGS mostly is a diagnosis of exclusion. […] Laboratory testing includes urinalysis, serum creatinine concentration or creatinine clearance, albumin levels, and lipid studies. In patients with suspected secondary FSGS, the search for an underlying etiology may include testing for conditions such as viral infections and systemic lupus erythematosus.

• #7 Focal Segmental Glomerulosclerosis (FSGS) | UNC Kidney Center

  https://unckidneycenter.org/kidneyhealthlibrary/glomerular-disease/focal-segmental-glomerulosclerosis-fsgs/

  FSGS stands for Focal Segmental Glomerulosclerosis. It is a relatively common form of kidney disease, especially in the US. In order to be diagnosed with FSGS, you must undergo a kidney biopsy. […] None of the above symptoms, or even all of them together, is specific for FSGS. If you or your doctor are concerned about FSGS, the only way to know for sure is to have a kidney biopsy. […] FSGS can have several different appearances, or variants, when seen under the microscope. There is still some debate between doctors about the importance of separating FSGS into these different variants. However, these different appearances can provide clues about what caused the disease or how it will behave. […] FSGS is not an easy disease to treat. Anyone with this disease should be seen regularly by a kidney specialist, also called a nephrologist. If it is associated with any of the illnesses mentioned above, treating that particular illness becomes a priority.

• #8 Focal Segmental Glomerulosclerosis: Symptoms & Treatment

  https://my.clevelandclinic.org/health/diseases/21149-focal-segmental-glomerulosclerosis-fsgs

  FSGS is a rare disease. Healthcare providers diagnose it in about 7 out of 1 million people per year. […] Your healthcare provider will listen to your symptoms and take your medical history. Several types of kidney tests may help your provider diagnose FSGS. […] However, providers can only definitively diagnose FSGS by doing a kidney biopsy. This is when a provider uses a needle to take a small sample of tissue from your kidney to look for signs of FSGS under a microscope. […] Treatment for FSGS depends on the type and cause, your age and whether you have other health conditions. The goal of treatment is to manage your symptoms to help you maintain a good quality of life and slow scarring so that it doesn’t lead to kidney failure. […] FSGS is a chronic disease that can’t be undone or reversed. This means treatment can only slow the progression of kidney disease, not cure you from the disease.

• #9 Focal segmental glomerulosclerosis (FSGS) – Diagnosis and treatment – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/fsgs/diagnosis-treatment/drc-20562383

  For possible focal segmental glomerulosclerosis (FSGS), your healthcare professional reviews your medical history and orders lab tests to see how well your kidneys work. Testing may include: […] A biopsy usually involves placing a needle through the skin to take a tiny sample from the kidney. The results of the biopsy can confirm a diagnosis of FSGS. […] For focal segmental glomerulosclerosis (FSGS), some basic questions to ask your healthcare professional include: What tests do I need? […] FSGS is a disease that may return. Because scarring in the glomeruli might be lifelong, you need to follow up with your healthcare team is to see how well your kidneys work.

• #10 Focal Segmental Glomerulosclerosis: Practice Essentials, Pathophysiology, Etiology

  https://emedicine.medscape.com/article/245915-overview

  Focal segmental glomerulosclerosis (FSGS) is one of the most common causes of primary glomerular disease in adults. In adults undergoing kidney biopsy for evaluation of proteinuria, FSGS accounts for 35% of all cases and up to 80% of cases in Black patients. However, no age group is exempt. […] A diagnosis of FSGS is established only by histopathology findings. Detailed medical history and pertinent laboratory tests are essential to distinguish between the different types of FSGS. In massively obese patients, FSGS mostly is a diagnosis of exclusion. […] Laboratory testing includes urinalysis, serum creatinine concentration or creatinine clearance, albumin levels, and lipid studies. In patients with suspected secondary FSGS, the search for an underlying etiology may include testing for conditions such as viral infections and systemic lupus erythematosus.

• #11 Focal Segmental Glomerulosclerosis Workup: Laboratory Studies, Histologic Findings, Ultrasonography

  https://emedicine.medscape.com/article/245915-workup

  In patients with focal segmental glomerulosclerosis (FSGS), urinalysis reveals large amounts of protein, along with hyaline and broad waxy casts, whereas red blood cell (RBC) casts are generally absent. Broad casts may be observed in patients with advanced cases. Serum creatinine (SCr) concentration or creatinine clearance (CrCl) is usually within reference ranges in early stages. […] Kidney biopsy is the most definitive way to establish the diagnosis. The characteristic lesion in FSGS is segmental solidification of the glomerular tuft, usually in the perihilar region and sometimes in the peripheral areas, including the tubular pole. In the affected glomeruli, capillaries are segmentally obliterated by accumulation of acellular matrix and hyaline deposits, along with adhesion to the Bowman capsule.

• #12 Focal segmental glomerulosclerosis – Symptoms, diagnosis and treatment | BMJ Best Practice

  https://bestpractice.bmj.com/topics/en-gb/939

  Key diagnostic factors include presence of risk factors, oedema, foamy urine, hypertension, Muehrcke’s lines, xanthelasma and xanthomata. […] 1st investigations to order include serum urea, creatinine, GFR, urinalysis with microscopy, urine protein-to-creatinine ratio, 24-hour urine collection for protein, serum albumin, and serum lipid profile. […] Investigations to consider include serum HIV enzyme-linked immunosorbent assay, CD4 count and viral load studies, parvovirus DNA polymerase chain reaction (PCR), cytomegalovirus DNA PCR, hepatitis B and C serologies, antinuclear antibody, anti-double-stranded DNA, serum and urine protein electrophoresis, and renal biopsy.

• #13 Focal segmental glomerulosclerosis – Symptoms, diagnosis and treatment | BMJ Best Practice

  https://bestpractice.bmj.com/topics/en-gb/939

  Key diagnostic factors include presence of risk factors, oedema, foamy urine, hypertension, Muehrcke’s lines, xanthelasma and xanthomata. […] 1st investigations to order include serum urea, creatinine, GFR, urinalysis with microscopy, urine protein-to-creatinine ratio, 24-hour urine collection for protein, serum albumin, and serum lipid profile. […] Investigations to consider include serum HIV enzyme-linked immunosorbent assay, CD4 count and viral load studies, parvovirus DNA polymerase chain reaction (PCR), cytomegalovirus DNA PCR, hepatitis B and C serologies, antinuclear antibody, anti-double-stranded DNA, serum and urine protein electrophoresis, and renal biopsy.

• #14 Focal segmental glomerulosclerosis – Symptoms, diagnosis and treatment | BMJ Best Practice

  https://bestpractice.bmj.com/topics/en-gb/939

  Key diagnostic factors include presence of risk factors, oedema, foamy urine, hypertension, Muehrcke’s lines, xanthelasma and xanthomata. […] 1st investigations to order include serum urea, creatinine, GFR, urinalysis with microscopy, urine protein-to-creatinine ratio, 24-hour urine collection for protein, serum albumin, and serum lipid profile. […] Investigations to consider include serum HIV enzyme-linked immunosorbent assay, CD4 count and viral load studies, parvovirus DNA polymerase chain reaction (PCR), cytomegalovirus DNA PCR, hepatitis B and C serologies, antinuclear antibody, anti-double-stranded DNA, serum and urine protein electrophoresis, and renal biopsy.

• #15 Focal Segmental Glomerulosclerosis: Practice Essentials, Pathophysiology, Etiology

  https://emedicine.medscape.com/article/245915-overview

  Focal segmental glomerulosclerosis (FSGS) is one of the most common causes of primary glomerular disease in adults. In adults undergoing kidney biopsy for evaluation of proteinuria, FSGS accounts for 35% of all cases and up to 80% of cases in Black patients. However, no age group is exempt. […] A diagnosis of FSGS is established only by histopathology findings. Detailed medical history and pertinent laboratory tests are essential to distinguish between the different types of FSGS. In massively obese patients, FSGS mostly is a diagnosis of exclusion. […] Laboratory testing includes urinalysis, serum creatinine concentration or creatinine clearance, albumin levels, and lipid studies. In patients with suspected secondary FSGS, the search for an underlying etiology may include testing for conditions such as viral infections and systemic lupus erythematosus.

• #16 Focal segmental glomerulosclerosis – Symptoms, diagnosis and treatment | BMJ Best Practice

  https://bestpractice.bmj.com/topics/en-gb/939

  Key diagnostic factors include presence of risk factors, oedema, foamy urine, hypertension, Muehrcke’s lines, xanthelasma and xanthomata. […] 1st investigations to order include serum urea, creatinine, GFR, urinalysis with microscopy, urine protein-to-creatinine ratio, 24-hour urine collection for protein, serum albumin, and serum lipid profile. […] Investigations to consider include serum HIV enzyme-linked immunosorbent assay, CD4 count and viral load studies, parvovirus DNA polymerase chain reaction (PCR), cytomegalovirus DNA PCR, hepatitis B and C serologies, antinuclear antibody, anti-double-stranded DNA, serum and urine protein electrophoresis, and renal biopsy.

• #17 Focal Segmental Glomerulosclerosis Workup: Laboratory Studies, Histologic Findings, Ultrasonography

  https://emedicine.medscape.com/article/245915-workup

  In the early stages of FSGS, ultrasound examination reveals normal or large kidneys with increased echogenicity, suggesting diffuse intrinsic medical renal disease. In patients with advanced kidney failure, kidneys are small and shrunken, indicating severe glomerular scarring and interstitial fibrosis. In HIV-associated FSGS, ultrasound generally reveals large echogenic kidneys.

• #18 Focal Segmental Glomerulosclerosis: Symptoms, Treatments, and Outlook

  https://resources.healthgrades.com/right-care/kidneys-and-the-urinary-system/focal-segmental-glomerulosclerosis

  Focal segmental glomerulosclerosis (FSGS) occurs when clumps of scar tissue form in the kidneys’ glomeruli, which filter waste products from the bloodstream. […] While symptoms may suggest FSGS as a potential diagnosis, your doctor will likely confirm a diagnosis with a kidney biopsy. […] If you have FSGS, your biopsy will show scarring in some but not all of the glomeruli. […] Aside from a biopsy, your physician will order lab testing of your blood and urine to check protein levels. They may also order a kidney ultrasound to check the size and anatomy of your kidneys.

• #19 Focal Segmental Glomerulosclerosis Workup: Laboratory Studies, Histologic Findings, Ultrasonography

  https://emedicine.medscape.com/article/245915-workup

  In the early stages of FSGS, ultrasound examination reveals normal or large kidneys with increased echogenicity, suggesting diffuse intrinsic medical renal disease. In patients with advanced kidney failure, kidneys are small and shrunken, indicating severe glomerular scarring and interstitial fibrosis. In HIV-associated FSGS, ultrasound generally reveals large echogenic kidneys.

• #20 Focal segmental glomerulosclerosis (FSGS) – Diagnosis and treatment – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/fsgs/diagnosis-treatment/drc-20562383

  For possible focal segmental glomerulosclerosis (FSGS), your healthcare professional reviews your medical history and orders lab tests to see how well your kidneys work. Testing may include: […] A biopsy usually involves placing a needle through the skin to take a tiny sample from the kidney. The results of the biopsy can confirm a diagnosis of FSGS. […] For focal segmental glomerulosclerosis (FSGS), some basic questions to ask your healthcare professional include: What tests do I need? […] FSGS is a disease that may return. Because scarring in the glomeruli might be lifelong, you need to follow up with your healthcare team is to see how well your kidneys work.

• #21 Focal segmental glomerulosclerosis (FSGS) | UM Health-Sparrow

  https://www.uofmhealthsparrow.org/departments-conditions/conditions/focal-segmental-glomerulosclerosis-fsgs

  For possible focal segmental glomerulosclerosis (FSGS), your healthcare professional reviews your medical history and orders lab tests to see how well your kidneys work. Testing may include: […] A biopsy usually involves placing a needle through the skin to take a tiny sample from the kidney. The results of the biopsy can confirm a diagnosis of FSGS.

• #22 Focal Segmental Glomerulosclerosis Workup: Laboratory Studies, Histologic Findings, Ultrasonography

  https://emedicine.medscape.com/article/245915-workup

  In patients with focal segmental glomerulosclerosis (FSGS), urinalysis reveals large amounts of protein, along with hyaline and broad waxy casts, whereas red blood cell (RBC) casts are generally absent. Broad casts may be observed in patients with advanced cases. Serum creatinine (SCr) concentration or creatinine clearance (CrCl) is usually within reference ranges in early stages. […] Kidney biopsy is the most definitive way to establish the diagnosis. The characteristic lesion in FSGS is segmental solidification of the glomerular tuft, usually in the perihilar region and sometimes in the peripheral areas, including the tubular pole. In the affected glomeruli, capillaries are segmentally obliterated by accumulation of acellular matrix and hyaline deposits, along with adhesion to the Bowman capsule.

• #23 Focal Segmental Glomerulosclerosis – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK532272/

  A kidney biopsy is the most specific diagnostic modality for FSGS, as it is for many other glomerulopathies. The characteristic finding in FSGS is segmental solidification of the glomeruli. […] In idiopathic FSGS, investigations for an underlying etiology are usually negative. […] FSGS is a diagnosis of exclusion in patients with morbid obesity.

• #24 Focal Segmental Glomerulosclerosis – Specific Pathologies – Glomerular Diseases – Nephrology – Diseases – McMaster Textbook of Internal Medicine

  https://empendium.com/mcmtextbook/chapter/B31.II.14.3.21.6.

  FSGS is more prevalent in African Americans and in males. Edema and proteinuria are the most common clinical manifestations. Nephrotic syndrome is often found and occurs in up to 80% of patients. […] Diagnosis is based on histologic examination of kidney biopsy specimens. At least 20 glomeruli are required to exclude FSGS, given the focal nature of lesions.

• #25

  https://journals.lww.com/cjasn/fulltext/2017/03000/focal_segmental_glomerulosclerosis.18.aspx

  Genetic testing is beneficial in particular clinical settings. Identifying the etiology of FSGS guides selection of therapy and provides prognostic insight. […] The diagnosis and evaluation of FSGS rely on integration of the clinical history (family history, birth history, peak weight and body mass, and medication use), clinical laboratory findings (serum albumin, urine protein, and viral serologies), and renal histopathology. […] The distinction between MCD and FSGS is critical, because both present with proteinuria, podocyte injury, and minimal immune deposits. […] The involvement of glomeruli in focal glomerular processes follows a binomial distribution, and thus, for focal disease, more glomeruli are necessary to observe an affected glomerulus. […] The Columbia classification of FSGS recognized five morphologic patterns, all of which involve obliteration of the capillary lumens and for the most part, have good reproducibility across independent observers.

• #26

  https://journals.lww.com/cjasn/fulltext/2017/03000/focal_segmental_glomerulosclerosis.18.aspx

  Genetic testing is beneficial in particular clinical settings. Identifying the etiology of FSGS guides selection of therapy and provides prognostic insight. […] The diagnosis and evaluation of FSGS rely on integration of the clinical history (family history, birth history, peak weight and body mass, and medication use), clinical laboratory findings (serum albumin, urine protein, and viral serologies), and renal histopathology. […] The distinction between MCD and FSGS is critical, because both present with proteinuria, podocyte injury, and minimal immune deposits. […] The involvement of glomeruli in focal glomerular processes follows a binomial distribution, and thus, for focal disease, more glomeruli are necessary to observe an affected glomerulus. […] The Columbia classification of FSGS recognized five morphologic patterns, all of which involve obliteration of the capillary lumens and for the most part, have good reproducibility across independent observers.

• #27

  https://www.omim.org/entry/603278

  D’Agati et al. (2011) provided a detailed review of FSGS, emphasizing that the disorder results from defects of the podocyte. […] Focal segmental glomerulosclerosis and nephrotic syndrome are genetically heterogeneous disorders representing a spectrum of hereditary renal diseases. […] The genetic contribution to FSGS etiology is indicated by reports of its occurrence in multiple members of families (Conlon et al., 1995; Faubert and Porush, 1997). Both autosomal dominant and recessive patterns of inheritance have been proposed (Conlon et al., 1995). […] The inheritance pattern in the family reported by Mathis et al. (1998) was autosomal dominant with reduced penetrance and variable expressivity. […] D’Agati et al. (2004) proposed a pathologic classification of FSGS, defining 5 morphologic variants based entirely on assessment of glomerular light microscopic alterations: collapsing variant, tip variant, cellular variant, perihilar variant, and 'not otherwise specified,’ with classification into a given category requiring that all preceding categories, as listed, be excluded.

• #28 Focal Segmental Glomerulosclerosis: Histopathology Discussion – European Medical Journal

  https://www.emjreviews.com/nephrology/article/focal-segmental-glomerulosclerosis-histopathology-discussion-j120121/

  KDIGO guidelines recommend high-dose oral glucocorticoids as first-line immunosuppressive therapy in primary FSGS. Cyclosporin or tacrolimus are recommended in steroid-resistant primary FSGS. Histopathologic classification of FSGS helps in prognostic terms. The collapsing variant has worse prognosis, with approximately 70% of patients with this variant progressing to Stage 5 chronic kidney disease. Such progression in tip lesion, FSGS not otherwise specified (classic), perihilar, and cellular variants is less common, with 520%, 3040%, 3050%, and around 30% of patients progressing to Stage 5 chronic kidney disease, respectively. […] Electron microscopy is an important tool to differentiate primary and secondary FSGS. Podocyte foot process effacement is more severe in primary FSGS, generally involving 80% of the basement membranes surface area. Secondary FSGS effacement is more uneven and patchy.

• #29 Focal Segmental Glomerulosclerosis: Histopathology Discussion – European Medical Journal

  https://www.emjreviews.com/nephrology/article/focal-segmental-glomerulosclerosis-histopathology-discussion-j120121/

  KDIGO guidelines recommend high-dose oral glucocorticoids as first-line immunosuppressive therapy in primary FSGS. Cyclosporin or tacrolimus are recommended in steroid-resistant primary FSGS. Histopathologic classification of FSGS helps in prognostic terms. The collapsing variant has worse prognosis, with approximately 70% of patients with this variant progressing to Stage 5 chronic kidney disease. Such progression in tip lesion, FSGS not otherwise specified (classic), perihilar, and cellular variants is less common, with 520%, 3040%, 3050%, and around 30% of patients progressing to Stage 5 chronic kidney disease, respectively. […] Electron microscopy is an important tool to differentiate primary and secondary FSGS. Podocyte foot process effacement is more severe in primary FSGS, generally involving 80% of the basement membranes surface area. Secondary FSGS effacement is more uneven and patchy.

• #30

  https://journals.lww.com/cjasn/fulltext/2017/03000/focal_segmental_glomerulosclerosis.18.aspx

  The use of all three renal pathology modalities (light microscopy, electron microscopy, and staining for Ig and complement) is critical in making the distinction between MCD and FSGS. […] FSGS comprises a complex set of syndromes, most with multiple causes, and hence, FSGS represents a diagnostic challenge that requires close and thoughtful collaboration between patient, family, nephrologist, pathologist, and geneticist to gather the necessary data.

• #31

  https://journals.lww.com/cjasn/fulltext/2017/03000/focal_segmental_glomerulosclerosis.18.aspx

  The use of all three renal pathology modalities (light microscopy, electron microscopy, and staining for Ig and complement) is critical in making the distinction between MCD and FSGS. […] FSGS comprises a complex set of syndromes, most with multiple causes, and hence, FSGS represents a diagnostic challenge that requires close and thoughtful collaboration between patient, family, nephrologist, pathologist, and geneticist to gather the necessary data.

• #32 FSGS: Diagnosis and Diagnostic Work-Up

  https://pmc.ncbi.nlm.nih.gov/articles/PMC4894996/

  Focal segmental glomerulosclerosis is a histologic lesion, rather than a clinical disease. FSGS is common cause of nephrotic syndrome in both adults and children worldwide. […] Currently the incidence is estimated to be 7 per million. […] Upon identification of FSGS in a renal biopsy a thorough examination should be initiated to identify the underlying cause. The distinction between primary and secondary FSGS is important for both prognostic and therapeutic reasons. […] A biopsy is necessary to establish the diagnosis of FSGS and determine the subtype of FSGS. […] The distinction between primary and secondary FSGS has important therapeutic implications as primary FSGS often requires immunomodulatory treatment whereas treatment in secondary FSGS should be focused on the reduction of intraglomerular hypertension using RAAS blockade.

• #33 Focal Segmental Glomerulosclerosis: Histopathology Discussion – European Medical Journal

  https://www.emjreviews.com/nephrology/article/focal-segmental-glomerulosclerosis-histopathology-discussion-j120121/

  In focal segmental glomerulosclerosis (FSGS), some (not all) glomeruli are affected with sclerosis (focal), and each diseased glomerulus is only partially affected (segmental). If kidney biopsy reveals FSGS lesions, and patient has nephrotic syndrome (24 hours proteinuria 3.5 g and presence of serum albumin 30 g/L, with or without oedema), then there is more probability of primary FSGS. This is especially true if the electron microscopy shows diffuse foot process effacement. If the patient does not satisfy this criterion, then they should be investigated for secondary FSGS. Genetic testing to exclude genetic forms of FSGS (mutations in podocyte and glomerular basement membrane proteins) may be needed when subject does not meet the criteria for primary FSGS. […] KDIGO has recommended that when a patient has nephrotic syndrome, and in kidney biopsy, the light microscopy shows focal and segmental glomerulosclerosis lesions, and the electron microscopy shows diffuse foot process effacement, then it satisfies the recommended definition of primary FSGS. However, please note that no histopathological finding is pathognomonic of primary FSGS. Nephrotic syndrome is defined as 24-hour proteinuria of greater than 3.5 g, plus hypoalbuminaemia of less than 30 g/L, often with or without dyslipidaemia and oedema. There should not be any established pathophysiologic entity that may cause FSGS (no secondary factors that can cause FSGS should have been identified).

• #34 Focal Segmental Glomerulosclerosis: Histopathology Discussion – European Medical Journal

  https://www.emjreviews.com/nephrology/article/focal-segmental-glomerulosclerosis-histopathology-discussion-j120121/

  In focal segmental glomerulosclerosis (FSGS), some (not all) glomeruli are affected with sclerosis (focal), and each diseased glomerulus is only partially affected (segmental). If kidney biopsy reveals FSGS lesions, and patient has nephrotic syndrome (24 hours proteinuria 3.5 g and presence of serum albumin 30 g/L, with or without oedema), then there is more probability of primary FSGS. This is especially true if the electron microscopy shows diffuse foot process effacement. If the patient does not satisfy this criterion, then they should be investigated for secondary FSGS. Genetic testing to exclude genetic forms of FSGS (mutations in podocyte and glomerular basement membrane proteins) may be needed when subject does not meet the criteria for primary FSGS. […] KDIGO has recommended that when a patient has nephrotic syndrome, and in kidney biopsy, the light microscopy shows focal and segmental glomerulosclerosis lesions, and the electron microscopy shows diffuse foot process effacement, then it satisfies the recommended definition of primary FSGS. However, please note that no histopathological finding is pathognomonic of primary FSGS. Nephrotic syndrome is defined as 24-hour proteinuria of greater than 3.5 g, plus hypoalbuminaemia of less than 30 g/L, often with or without dyslipidaemia and oedema. There should not be any established pathophysiologic entity that may cause FSGS (no secondary factors that can cause FSGS should have been identified).

• #35 Focal Segmental Glomerulosclerosis: Histopathology Discussion – European Medical Journal

  https://www.emjreviews.com/nephrology/article/focal-segmental-glomerulosclerosis-histopathology-discussion-j120121/

  When a patient has FSGS lesions in presence of secondary factors that are known to cause FSGS, then it is termed as secondary FSGS. Electron microscopy may or may not show diffuse foot process effacement. Secondary causes of FSGS include, but are not limited to, diabetic nephropathy, hypertensive nephrosclerosis, obesity, viral infections (including severe acute respiratory syndrome coronavirus 2, HIV, and Hepatitis C), drug induced (including due to nonsteroidal anti-inflammatory drugs, steroids, calcineurin inhibitors, mammalian target of rapamycin inhibitors, heroin, interferon-, and pamidronate), and reduced nephron number (as in age-related FSGS, renal dysplasia, surgical ablation, and reflux nephropathy). […] Mutations in podocyte or glomerular basement membrane proteins may result in genetic FSGS. The mutations may occur in proteins including podocin, nephrin, -actinin-4, and -integrin. Patients with genetic forms of FSGS may have a family history of kidney disease, and they are often young.

• #36 Focal segmental glomerulosclerosis (FSGS) – Symptoms, causes, treatment | National Kidney Foundation

  https://www.kidney.org/kidney-topics/focal-segmental-glomerulosclerosis-fsgs

  FSGS is not caused by a single disease. It can have many different causes. The scarring may happen because of an infection, or drug, or a disease that affects the entire body, like diabetes, HIV infection, sickle cell disease or lupus. FSGS can also be caused by another glomerular disease that you had before you got FSGS. FSGS has different types based on the cause. […] A blood test, urine test, and a kidney biopsy will determine if you have FSGS. […] The type of treatment you get depends on the cause. Everyone is different and your doctor will make a treatment plan that is right for your type of FSGS. Usually, treatments for FSGS include: Corticosteroids (often called steroids), Immunosuppressive drugs, Plasmapheresis, ACE inhibitors and ARBs, Diuretics, Diet change. […] You should talk with your doctor about your condition because the progression of the disease depends on many factors. FSGS is a chronic disease, because the scarred glomeruli cannot be repaired. Treatment can slow the process of kidney disease. Everyone is different in how they respond to treatment. Over time, some patients with FSGS gradually get worse until they reach kidney failure. If this occurs, they will need a kidney transplant or dialysis to stay alive. Some people respond well to treatment and may live with the disease for many years while being monitored for any signs of change.

• #37 Focal Segmental Glomerulosclerosis: Practice Essentials, Pathophysiology, Etiology

  https://emedicine.medscape.com/article/245915-overview

  Kidney biopsy is the most definitive way to confirm the diagnosis. Findings include segmental solidification of the glomerular tuft, segmental obliteration of glomerular capillaries, and fusion of diffuse foot process. […] Management of secondary FSGS is also directed toward the etiology or associated disorder.

• #38 Focal Segmental Glomerulosclerosis: Histopathology Discussion – European Medical Journal

  https://www.emjreviews.com/nephrology/article/focal-segmental-glomerulosclerosis-histopathology-discussion-j120121/

  When a patient has FSGS lesions in presence of secondary factors that are known to cause FSGS, then it is termed as secondary FSGS. Electron microscopy may or may not show diffuse foot process effacement. Secondary causes of FSGS include, but are not limited to, diabetic nephropathy, hypertensive nephrosclerosis, obesity, viral infections (including severe acute respiratory syndrome coronavirus 2, HIV, and Hepatitis C), drug induced (including due to nonsteroidal anti-inflammatory drugs, steroids, calcineurin inhibitors, mammalian target of rapamycin inhibitors, heroin, interferon-, and pamidronate), and reduced nephron number (as in age-related FSGS, renal dysplasia, surgical ablation, and reflux nephropathy). […] Mutations in podocyte or glomerular basement membrane proteins may result in genetic FSGS. The mutations may occur in proteins including podocin, nephrin, -actinin-4, and -integrin. Patients with genetic forms of FSGS may have a family history of kidney disease, and they are often young.

• #39 Focal Segmental Glomerulosclerosis: Histopathology Discussion – European Medical Journal

  https://www.emjreviews.com/nephrology/article/focal-segmental-glomerulosclerosis-histopathology-discussion-j120121/

  When a patient has FSGS lesions in presence of secondary factors that are known to cause FSGS, then it is termed as secondary FSGS. Electron microscopy may or may not show diffuse foot process effacement. Secondary causes of FSGS include, but are not limited to, diabetic nephropathy, hypertensive nephrosclerosis, obesity, viral infections (including severe acute respiratory syndrome coronavirus 2, HIV, and Hepatitis C), drug induced (including due to nonsteroidal anti-inflammatory drugs, steroids, calcineurin inhibitors, mammalian target of rapamycin inhibitors, heroin, interferon-, and pamidronate), and reduced nephron number (as in age-related FSGS, renal dysplasia, surgical ablation, and reflux nephropathy). […] Mutations in podocyte or glomerular basement membrane proteins may result in genetic FSGS. The mutations may occur in proteins including podocin, nephrin, -actinin-4, and -integrin. Patients with genetic forms of FSGS may have a family history of kidney disease, and they are often young.

• #40 Genetic Testing Lagging for Patients With Focal Segmental Glomerulosclerosis – Renal and Urology News

  https://www.renalandurologynews.com/reports/genetic-testing-focal-segmental-glomerulosclerosis/

  Identifying a genetic mutation in a patient with focal segmental glomerulosclerosis (FSGS) may help to solidify diagnosis before the often-rapid deterioration in kidney function. […] In a July 2023 online survey of 100 nephrologists in the United States, 71% agreed that genetic testing is an increasingly important aspect of FSGS diagnosis and treatment. […] According to patient chart data, 36% received genetic testing, of whom 34% tested positive for APOL1 gene mutations. […] Patients with high-risk APOL1 variants exhibit FSGS at an earlier age. […] Genetically-linked FSGS patients account for approximately 10% of all primary FSGS cases but are also considered the most challenging to manage according to nephrologists, leaving many patients undertreated with a chronic and progressive disease, the investigators noted.

• #41 Indications for genetic testing in adults with focal segmental glomerulosclerosis | Nefrología

  https://www.revistanefrologia.com/en-indications-for-genetic-testing-in-articulo-S2013251425000173

  Family and personal history and the clinical presentation are not always sufficient to exclude a hereditary cause of the disease. The clinical presentation of FSGS with a genetic cause is extremely variable; with differences in age of presentation, degree of proteinuria, and progression of chronic kidney disease (CKD). […] It is well established that recognizing the diagnosis of genetically caused FSGS is of vital importance for patients. […] A correct classification of patients according to their clinical and histological characteristics is essential for decision making and the planning of a suitable decisions and the planning of an adequate therapeutic scheme. […] In the present document we propose recommendations, based on clinical and anatomopathological criteria, for the performance of a genetic study in adult patients with FSGS.

• #42 Focal Segmental Glomerulosclerosis – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK532272/

  Focal segmental glomerulosclerosis (FSGS) is histologically characterized by segmental scarring that affects only a portion of the glomerulus and involves some, but not all, glomeruli in a biopsy sample. […] Accurate identification of the underlying etiology is crucial for guiding treatment decisions, predicting prognosis, and assessing transplant risks. […] Diagnosis requires histopathological confirmation, as proteinuria, low serum albumin, and casts on urinalysis can support clinical suspicion. […] Although clinical signs may suggest FSGS, the diagnosis can only be confirmed through histopathological findings. […] The definitive diagnosis is typically made through a kidney biopsy in these cases. […] Genetic testing is recommended for patients with steroid-resistant FSGS, a family history of FSGS, a history of Wilms tumor (nephroblastoma), or other systemic signs suggestive of a genetic syndrome, such as Alport syndrome, Fabry disease, or nail-patella syndrome.

• #43 FSGS: Diagnosis and Diagnostic Work-Up

  https://pmc.ncbi.nlm.nih.gov/articles/PMC4894996/

  In infants with steroid-resistant nephrotic syndrome, a genetic defect can be identified in up to two-thirds of patients. […] Genetic screening of patients with steroid-resistant nephrotic syndrome should be performed when FSGS occurs early in life as the likelihood of identifying a genetic basis for FSGS is high. […] The interpretation of negative results especially remains problematic as these patients may have mutation in noncoding regions of candidate genes or mutations in novel genes not yet reported. […] Histologically FSGS is classified in 5 different subtypes: perihilar, tip, collapsing, cellular, and not otherwise specified. […] The histologic subtype was associated with clinical features: patients with NOS FSGS were more likely to present with subnephrotic proteinuria whereas patients with tip or collapsing variants tended to be older and have higher degrees of proteinuria and hypoalbuminemia at presentation.

• #44 Athena Diagnostics

  https://www.athenadiagnostics.com/view-full-catalog/focal-and-segmental-glomerulosclerosis-fsgs-evaluation1

  Test code: 717 Type of disorder: Hereditary Renal Glomerular Disorders Disease(s) tested for: Nephrotic Syndrome, Focal Segmental Glomerulosclerosis (FSGS) Genes Included: INF2, ACTN4, TRPC6, NPHS2, Tests included: ACTN4 DNA Sequencing TestINF2 DNA Sequencing TestNPHS2 DNA Sequencing TestTRPC6 DNA Sequencing Test Informed Consent Required: This test requires physician attestation that patient consent has been received […] Clinical Significance: Detects sequence variants in the INF2, ACTN4, TRPC6, and NPHS2 genes in patients with nephrotic syndrome. Patients exhibit proteinuria, nephrotic syndrome, and frequently the progressive loss of renal function.

• #45

  https://www.omim.org/entry/603278

  D’Agati et al. (2011) reviewed the pathogenesis of FSGS, with emphasis on loss of the glomerular filtration barrier due to defects in the podocyte. […] In 3 families with clear evidence of autosomal dominant inheritance of FSGS, including the family reported by Mathis et al. (1992, 1998), Kaplan et al. (2000) identified heterozygous mutations in the ACTN4 gene (604638.0001-604638.0003). […] In a 13-year-old German girl with FSGS1, Bartram et al. (2016) identified a de novo heterozygous missense mutation in the ACTN4 gene (G195D; 604638.0004). The mutation was found by gene panel analysis and confirmed by Sanger sequencing. Introduction of the mutation into podocytes showed that the mutant protein had altered localization compared to wildtype and formed multiple F-actin-positive aggregates. […] It is important to recognize that FSGS is a histologic pattern of renal injury: some patients with FSGS on biopsy have nephrotic syndrome, whereas others have only mild proteinuria. NPHS and FSGS represent a spectrum of hereditary renal diseases of the podocyte.

• #46 RFSGS – Overview: Focal Segmental Glomerulosclerosis (FSGS) and Nephrotic Syndrome Gene Panel, Varies

  https://www.mayocliniclabs.com/test-catalog/overview/618114

  Providing a genetic evaluation for patients with a personal or family history of steroid resistant nephrotic syndrome (SRNS) […] Establishing a diagnosis of hereditary SRNS […] Guiding treatment decisions in individuals with nephrotic syndrome […] This test utilizes next-generation sequencing to detect single nucleotide, deletion-insertion, and copy number variants in 56 genes associated with focal segmental glomerulosclerosis and nephrotic syndrome […] Identification of a disease-causing variant may assist with diagnosis, prognosis, clinical management, familial screening, and genetic counseling for focal segmental glomerulosclerosis or nephrotic syndrome […] Nephrotic syndrome (NS) is a kidney disorder characterized by proteinuria, hypoalbuminemia, and edema […] Approximately 85% of nephrotic syndrome is steroid sensitive, while the remaining 15% is steroid resistant (SRNS)

• #47 Nephrotic Syndrome (NS)/Focal Segmental Glomerulosclerosis (FSGS) Panel Test – PreventionGenetics

  https://www.preventiongenetics.com/testInfo?val=Nephrotic-Syndrome-%28NS%29%2FFocal-Segmental-Glomerulosclerosis-%28FSGS%29-Panel

  Candidates for this test are patients with nephrotic syndrome (NS) or focal segmental glomerulosclerosis (FSGS). This test especially aids in a differential diagnosis of similar phenotypes by analyzing multiple genes simultaneously. […] A conclusive molecular diagnosis is necessary for better personalized treatment and accurate genetic counselling. […] In the largest international cohort study to date, sequencing for 27 genes known to cause steroid-resistant nephrotic syndrome (SRNS) detected a single-gene cause across 21 genes in 29.5% (526 of 1783) of families with SRNS that manifested before 25 years of age. […] This test is performed using Next-Gen sequencing with additional Sanger sequencing as necessary. […] This panel typically provides 99.8% coverage of all coding exons of the genes plus 10 bases of flanking noncoding DNA in all available transcripts along with other non-coding regions in which pathogenic variants have been identified at PreventionGenetics or reported elsewhere.

• #48 RFSGS – Overview: Focal Segmental Glomerulosclerosis (FSGS) and Nephrotic Syndrome Gene Panel, Varies

  https://www.mayocliniclabs.com/test-catalog/overview/618114

  Genetic SRNS may result from disease-causing variants in genes encoding renal-specific proteins (renal-limited) or syndromic conditions with extrarenal features […] Therefore, identification of a genetic form of SRNS may impact evaluation for extra-renal manifestations, treatment decisions including transplantation, and genetic counselling […] All detected variants are evaluated according to American College of Medical Genetics and Genomics recommendations […] Variants are classified based on known, predicted, or possible pathogenicity and reported with interpretive comments detailing their potential or known significance […] Test results should be interpreted in the context of clinical findings, family history, and other laboratory data […] If testing was performed because of a clinically significant family history, it is often useful to first test an affected family member […] Next-generation sequencing may not detect all types of genomic variants […] This test is not designed to detect low levels of mosaicism or to differentiate between somatic and germline variants […] Genes may be added or removed based on updated clinical relevance

• #49 Indications for genetic testing in adults with focal segmental glomerulosclerosis | Nefrología

  https://www.revistanefrologia.com/en-indications-for-genetic-testing-in-articulo-S2013251425000173

  Focal segmental glomerulosclerosis (FSGS) is a histological pattern of injury that derives from various pathological processes that affect podocytes, resulting in loss of selectivity of the glomerular filtration membrane, proteinuria and the development of renal failure that progresses to end-stage kidney disease in a significant number of patients. […] Genetic testing is crucial to identify FSGS of genetic cause. The prevalence of genetic FSGS is significant in children and considerable in adults, highlighting the importance of early diagnosis to avoid unnecessary treatments and facilitate genetic counselling. […] This document proposes clinical recommendations for carrying out genetic studies in adults with FSGS, highlighting the need for a correct classification for adequate therapeutic planning and improvement of results in clinical trials.

• #50 FSGS: Diagnosis and Diagnostic Work-Up

  https://pmc.ncbi.nlm.nih.gov/articles/PMC4894996/

  In infants with steroid-resistant nephrotic syndrome, a genetic defect can be identified in up to two-thirds of patients. […] Genetic screening of patients with steroid-resistant nephrotic syndrome should be performed when FSGS occurs early in life as the likelihood of identifying a genetic basis for FSGS is high. […] The interpretation of negative results especially remains problematic as these patients may have mutation in noncoding regions of candidate genes or mutations in novel genes not yet reported. […] Histologically FSGS is classified in 5 different subtypes: perihilar, tip, collapsing, cellular, and not otherwise specified. […] The histologic subtype was associated with clinical features: patients with NOS FSGS were more likely to present with subnephrotic proteinuria whereas patients with tip or collapsing variants tended to be older and have higher degrees of proteinuria and hypoalbuminemia at presentation.

• #51 FSGS: Diagnosis and Diagnostic Work-Up

  https://pmc.ncbi.nlm.nih.gov/articles/PMC4894996/

  Both clinical and histologic features have been reported to be predictive towards outcome. […] When confronted with a patient with FSGS a careful medical history and clinical examination should be performed. […] The detection of activated parietal epithelial cells immunohistochemically has been shown to be able to make a distinction between early FSGS and minimal change disease. […] Several genes, instrumental in podocyte homeostasis, have been reported to be associated with genetic forms of FSGS and these findings have propelled the field of podocyte biology in the last decade. […] The usefulness of genetic testing in the setting is disputed and is dependent on the presence of a familial history and onset in early life. […] It is well established that FSGS is more prevalent and the course of disease is more severe as well in African American individuals.

• #52 Novel diagnostic and therapeutic techniques reveal changed metabolic profiles in recurrent focal segmental glomerulosclerosis | Scientific Reports

  https://www.nature.com/articles/s41598-021-83883-w

  We report an innovative zebrafish model to detect the presence of circulating permeability factors causing FSGS that might be used as non-invasive diagnostic test in the future. […] We hypothesize that these metabolomic signatures indirectly reflect the effects of the unknown circulating factors in the kidney or that these metabolome changes might even represent the pathogenic components themselves. […] We measured proposed circulating permeability factors in the patients serum before and after first, second and third CytoSorb apheresis. […] Even though published circulating factors were in normal range or not detectable in our patient, the early disease recurrence after kidney transplantation and the immediate response to CytoSorb treatment were still suggestive for a disease-causing factor in the patients circulation.

• #53 Pathology Outlines – Focal segmental glomerulosclerosis-general

  https://www.pathologyoutlines.com/topic/kidneyfsgs.html

  There is consensus on performing genetic tests in steroid resistant FSGS in patients with positive family history or extrarenal physical signs suggestive of a genetic disorder (Fabry disease, nail patella syndrome or Alport syndrome). New markers such as suPAR have been proposed to diagnose and predict FSGS recurrence after transplantation and monitor treatment response in patients with primary FSGS.

• #54 Novel diagnostic and therapeutic techniques reveal changed metabolic profiles in recurrent focal segmental glomerulosclerosis | Scientific Reports

  https://www.nature.com/articles/s41598-021-83883-w

  Idiopathic forms of Focal Segmental Glomerulosclerosis (FSGS) are caused by circulating permeability factors, which can lead to early recurrence of FSGS and kidney failure after kidney transplantation. […] Therefore, there is an increased demand in FSGS research for the use of novel technologies that allow us to study FSGS from a yet unexplored angle. […] Here, we report CytoSorb apheresis as a novel therapeutic approach to bring recurrent FSGS in remission in a patient that was nonresponsive to immunosuppressive therapy and plasmapheresis. […] There is an unmet clinical need for early and specific biomarkers to detect FSGS and to predict its prognosis and response to therapy. […] However, even by histology the actual disease of the patients might be misinterpreted, as FSGS is a focal disease vulnerable to sampling errors in the biopsy.

• #55 Novel diagnostic and therapeutic techniques reveal changed metabolic profiles in recurrent focal segmental glomerulosclerosis | Scientific Reports

  https://www.nature.com/articles/s41598-021-83883-w

  We report an innovative zebrafish model to detect the presence of circulating permeability factors causing FSGS that might be used as non-invasive diagnostic test in the future. […] We hypothesize that these metabolomic signatures indirectly reflect the effects of the unknown circulating factors in the kidney or that these metabolome changes might even represent the pathogenic components themselves. […] We measured proposed circulating permeability factors in the patients serum before and after first, second and third CytoSorb apheresis. […] Even though published circulating factors were in normal range or not detectable in our patient, the early disease recurrence after kidney transplantation and the immediate response to CytoSorb treatment were still suggestive for a disease-causing factor in the patients circulation.

• #56

  https://journals.lww.com/cjasn/fulltext/2017/03000/focal_segmental_glomerulosclerosis.18.aspx

  Genetic testing is beneficial in particular clinical settings. Identifying the etiology of FSGS guides selection of therapy and provides prognostic insight. […] The diagnosis and evaluation of FSGS rely on integration of the clinical history (family history, birth history, peak weight and body mass, and medication use), clinical laboratory findings (serum albumin, urine protein, and viral serologies), and renal histopathology. […] The distinction between MCD and FSGS is critical, because both present with proteinuria, podocyte injury, and minimal immune deposits. […] The involvement of glomeruli in focal glomerular processes follows a binomial distribution, and thus, for focal disease, more glomeruli are necessary to observe an affected glomerulus. […] The Columbia classification of FSGS recognized five morphologic patterns, all of which involve obliteration of the capillary lumens and for the most part, have good reproducibility across independent observers.

• #57 Kidney Biopsy of the Month: Focal Segmental Glomerulosclerosis – Renal Fellow Network

  https://www.renalfellow.org/2021/01/26/kidney-biopsy-of-the-month-focal-segmental-glomerulosclerosis/

  A group of podocytopathies (of varying etiologies) share a common morphologic feature of focal segmental glomerulosclerosis (FSGS), typically with moderate to heavy proteinuria. […] FSGS was recognized as distinct entity by International Study of Kidney Diseases in Children in 1970s. […] Most common cause of nephrotic syndrome in adults. […] Adequate sample of glomeruli is crucial to make diagnosis of FSGS since focal lesions may be missed with small samples. […] Numerous globally sclerotic glomeruli and vascular changes in patient with nephrotic syndrome are suggestive of FSGS even if no segmental lesions are seen. […] Difference in outcome between MCD and FSGS equivalent to difference between benign and malignant.

• #58 Kidney Biopsy of the Month: Focal Segmental Glomerulosclerosis – Renal Fellow Network

  https://www.renalfellow.org/2021/01/26/kidney-biopsy-of-the-month-focal-segmental-glomerulosclerosis/

  A group of podocytopathies (of varying etiologies) share a common morphologic feature of focal segmental glomerulosclerosis (FSGS), typically with moderate to heavy proteinuria. […] FSGS was recognized as distinct entity by International Study of Kidney Diseases in Children in 1970s. […] Most common cause of nephrotic syndrome in adults. […] Adequate sample of glomeruli is crucial to make diagnosis of FSGS since focal lesions may be missed with small samples. […] Numerous globally sclerotic glomeruli and vascular changes in patient with nephrotic syndrome are suggestive of FSGS even if no segmental lesions are seen. […] Difference in outcome between MCD and FSGS equivalent to difference between benign and malignant.

• #59 Focal Segmental Glomerulosclerosis: Histopathology Discussion – European Medical Journal

  https://www.emjreviews.com/nephrology/article/focal-segmental-glomerulosclerosis-histopathology-discussion-j120121/

  In focal segmental glomerulosclerosis (FSGS), some (not all) glomeruli are affected with sclerosis (focal), and each diseased glomerulus is only partially affected (segmental). If kidney biopsy reveals FSGS lesions, and patient has nephrotic syndrome (24 hours proteinuria 3.5 g and presence of serum albumin 30 g/L, with or without oedema), then there is more probability of primary FSGS. This is especially true if the electron microscopy shows diffuse foot process effacement. If the patient does not satisfy this criterion, then they should be investigated for secondary FSGS. Genetic testing to exclude genetic forms of FSGS (mutations in podocyte and glomerular basement membrane proteins) may be needed when subject does not meet the criteria for primary FSGS. […] KDIGO has recommended that when a patient has nephrotic syndrome, and in kidney biopsy, the light microscopy shows focal and segmental glomerulosclerosis lesions, and the electron microscopy shows diffuse foot process effacement, then it satisfies the recommended definition of primary FSGS. However, please note that no histopathological finding is pathognomonic of primary FSGS. Nephrotic syndrome is defined as 24-hour proteinuria of greater than 3.5 g, plus hypoalbuminaemia of less than 30 g/L, often with or without dyslipidaemia and oedema. There should not be any established pathophysiologic entity that may cause FSGS (no secondary factors that can cause FSGS should have been identified).

• #60 Focal segmental glomerulosclerosis

  https://www.kidneypathology.com/English_version/Focal_segmental_GS.html

  Focal and segmental glomerulosclerosis (FSGS) is a disease characterized morphologically by segments of sclerosis in some glomeruli. […] The histologic features of FSGS do not allow differentiating the primary forms from the secondary ones. In order to make this differentiation we must help us with clinical findings and laboratory data, and it is very important evaluate alterations in other histologic compartments: vessels, interstitium, and tubules. […] The prognosis of FSGS is different to minimal change disease (MCD): there is greater incidence of chronic renal failure, very little response to steroids, and greater incidence of hematuria and hypertension. […] The etiology of FSGS is still far from being explained, to a great extent because this disease seems to be a pathological expression of different types of injury.

• #61

  https://journals.lww.com/cjasn/fulltext/2017/03000/focal_segmental_glomerulosclerosis.18.aspx

  The use of all three renal pathology modalities (light microscopy, electron microscopy, and staining for Ig and complement) is critical in making the distinction between MCD and FSGS. […] FSGS comprises a complex set of syndromes, most with multiple causes, and hence, FSGS represents a diagnostic challenge that requires close and thoughtful collaboration between patient, family, nephrologist, pathologist, and geneticist to gather the necessary data.

• #62 FSGS: Diagnosis and Diagnostic Work-Up

  https://pmc.ncbi.nlm.nih.gov/articles/PMC4894996/

  Focal segmental glomerulosclerosis is a histologic lesion, rather than a clinical disease. FSGS is common cause of nephrotic syndrome in both adults and children worldwide. […] Currently the incidence is estimated to be 7 per million. […] Upon identification of FSGS in a renal biopsy a thorough examination should be initiated to identify the underlying cause. The distinction between primary and secondary FSGS is important for both prognostic and therapeutic reasons. […] A biopsy is necessary to establish the diagnosis of FSGS and determine the subtype of FSGS. […] The distinction between primary and secondary FSGS has important therapeutic implications as primary FSGS often requires immunomodulatory treatment whereas treatment in secondary FSGS should be focused on the reduction of intraglomerular hypertension using RAAS blockade.

• #63

  https://journals.lww.com/cjasn/fulltext/2017/03000/focal_segmental_glomerulosclerosis.18.aspx

  The use of all three renal pathology modalities (light microscopy, electron microscopy, and staining for Ig and complement) is critical in making the distinction between MCD and FSGS. […] FSGS comprises a complex set of syndromes, most with multiple causes, and hence, FSGS represents a diagnostic challenge that requires close and thoughtful collaboration between patient, family, nephrologist, pathologist, and geneticist to gather the necessary data.

• #64 Focal segmental glomerulosclerosis (FSGS) – Diagnosis and treatment – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/fsgs/diagnosis-treatment/drc-20562383

  For possible focal segmental glomerulosclerosis (FSGS), your healthcare professional reviews your medical history and orders lab tests to see how well your kidneys work. Testing may include: […] A biopsy usually involves placing a needle through the skin to take a tiny sample from the kidney. The results of the biopsy can confirm a diagnosis of FSGS. […] For focal segmental glomerulosclerosis (FSGS), some basic questions to ask your healthcare professional include: What tests do I need? […] FSGS is a disease that may return. Because scarring in the glomeruli might be lifelong, you need to follow up with your healthcare team is to see how well your kidneys work.

• #65 Focal segmental glomerulosclerosis (FSGS) – Symptoms, causes, treatment | National Kidney Foundation

  https://www.kidney.org/kidney-topics/focal-segmental-glomerulosclerosis-fsgs

  FSGS is not caused by a single disease. It can have many different causes. The scarring may happen because of an infection, or drug, or a disease that affects the entire body, like diabetes, HIV infection, sickle cell disease or lupus. FSGS can also be caused by another glomerular disease that you had before you got FSGS. FSGS has different types based on the cause. […] A blood test, urine test, and a kidney biopsy will determine if you have FSGS. […] The type of treatment you get depends on the cause. Everyone is different and your doctor will make a treatment plan that is right for your type of FSGS. Usually, treatments for FSGS include: Corticosteroids (often called steroids), Immunosuppressive drugs, Plasmapheresis, ACE inhibitors and ARBs, Diuretics, Diet change. […] You should talk with your doctor about your condition because the progression of the disease depends on many factors. FSGS is a chronic disease, because the scarred glomeruli cannot be repaired. Treatment can slow the process of kidney disease. Everyone is different in how they respond to treatment. Over time, some patients with FSGS gradually get worse until they reach kidney failure. If this occurs, they will need a kidney transplant or dialysis to stay alive. Some people respond well to treatment and may live with the disease for many years while being monitored for any signs of change.

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