Materiały źródłowe

• #1 Focal Segmental Glomerulosclerosis Treatment & Management: Approach Considerations, Nonspecific Treatment, Specific Treatment

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

  Treatment of focal segmental glomerulosclerosis (FSGS) can be divided into nonspecific and specific therapy. […] In patients with primary or secondary FSGS (non-nephrotic or nephrotic) and proteinuria, the initial approach consists of optimal blood pressure (BP) control and the use of angiotensin-converting enzyme inhibitors (ACEIs) or angiotensin receptor blockers (ARBs). For patients who remain non-nephrotic or become non-nephrotic after 6 months of therapy, this remains the primary therapeutic approach. Patients who are persistently nephrotic after a course of conservative therapy or who present with complications from nephrotic syndrome require more aggressive treatment with prednisone or immunosuppressive agents. […] In patients with secondary FSGS and nephrotic-range proteinuria, the mainstay of therapy remains BP control with ACEIs and ARBs, along with disease-specific treatment if available (eg, antiretroviral therapy in HIV-associated nephropathy).

• #2 How I Treat Focal Segmental Glomerulosclerosis

  https://pmc.ncbi.nlm.nih.gov/articles/PMC9718025/

  Corticosteroids remain the mainstay of treatment in people with primary FSGS, a condition currently thought to be caused by a yet identified circulating permeability factor that is toxic to the podocytes. […] In the absence of a convincing diagnosis of primary FSGS, we ensure that efforts are made to look for an underlying cause of the FSGS lesion and to treat such a condition, if so detected, rather than starting corticosteroids at the outset. […] We suggest that the management for this group of patients should follow general supportive care for proteinuric kidney diseases, including maximizing renin-angiotensin system (RAS) blockade and control of hypertension for at least 6 months. […] Although corticosteroid therapy is accepted universally as the first-line therapy for primary FSGS, calcineurin inhibitors (CNIs) should be considered as the initial treatment in patients with medical conditions where corticosteroid use may be detrimental.

• #3 Focal Segmental Glomerulosclerosis: Symptoms & Treatment

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

  Focal segmental glomerulosclerosis (FSGS) is a rare disease that affects the filters in your kidneys. When these filters are scarred, they cant remove waste from your blood, which can lead to kidney damage and failure. Treatment for FSGS manages symptoms and prevents the disease from worsening. […] 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 doesnt lead to kidney failure. […] In some people with secondary FSGS, treating the underlying condition may stop kidney damage from progressing. Your healthcare provider cant repair damaged glomeruli, but over time, kidney function may improve. […] Some treatments for FSGS include: Angiotensin-converting enzyme inhibitors (ACE-Is). Angiotensin receptor blockers (ARBs). Mineralocorticoid receptor blockers (MRBs). Antibiotics. Diuretics. Immunosuppressive drugs. Plasmapheresis. Corticosteroids.

• #4 Focal Segmental Glomerulosclerosis: Symptoms & Treatment

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

  FSGS is a chronic disease that cant be undone or reversed. This means treatment can only slow the progression of kidney disease, not cure you from the disease. Everyone responds differently to treatment. Some people go on to need a kidney transplant or kidney dialysis to live. […] FSGS is a rare kidney disease that can cause kidney failure in some people. Luckily, there are treatments that can help stop this disease from progressing.

• #5 Focal segmental glomerulosclerosis (FSGS)

  https://www.kidneyfund.org/all-about-kidneys/other-kidney-diseases/focal-segmental-glomerulosclerosis-fsgs

  FSGS is a long-term (chronic) condition that cannot be reversed. Scarring can make it hard for your kidneys to filter out waste from your body. While treatments can help slow its progress, some people with FSGS eventually develop kidney failure. If this happens, you will need a kidney transplant or dialysis to live. […] The type of treatment plan your doctor will choose depends on what caused your FSGS. The goal of treatment is to stop proteinuria, the leaking of protein into your urine. For primary FSGS, treatment usually includes: […] Medicines called immunosuppressants that will stop your body from attacking your kidneys. This can include glucocorticoids (often called „steroids”) or another type of medicine called calcineurin inhibitors. […] For secondary FSGS, treatment involves managing the condition that caused FSGS. This can include stopping a certain medicine or treating the viral infection.

• #6 Focal Segmental Glomerulosclerosis Treatment & Management: Approach Considerations, Nonspecific Treatment, Specific Treatment

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

  Nonspecific treatment goals in patients with nephrotic syndrome include maintenance of adequate nutrition, minimization or elimination of proteinuria, and prevention of complications resulting from edema. Control of hypertension is one of the most important aspects of overall management. Lowering of lipid levels is necessary to reduce cardiovascular risk and to possibly delay the progression of kidney disease. […] The mainstay of treatment is reduction in daily salt intake to 2 g of sodium (6 g of salt) and the use of diuretics in varying doses and combinations. Because a high level of protein intake may further aggravate proteinuria, adversely affecting kidney function, current recommendations call for an intake of 1-1.3 g of high biologic value protein per kilogram of body weight. A reduction of dietary fat intake may help with dyslipidemia.

• #7 Focal Segmental Glomerulosclerosis Treatment & Management: Approach Considerations, Nonspecific Treatment, Specific Treatment

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

  Treatment of focal segmental glomerulosclerosis (FSGS) can be divided into nonspecific and specific therapy. […] In patients with primary or secondary FSGS (non-nephrotic or nephrotic) and proteinuria, the initial approach consists of optimal blood pressure (BP) control and the use of angiotensin-converting enzyme inhibitors (ACEIs) or angiotensin receptor blockers (ARBs). For patients who remain non-nephrotic or become non-nephrotic after 6 months of therapy, this remains the primary therapeutic approach. Patients who are persistently nephrotic after a course of conservative therapy or who present with complications from nephrotic syndrome require more aggressive treatment with prednisone or immunosuppressive agents. […] In patients with secondary FSGS and nephrotic-range proteinuria, the mainstay of therapy remains BP control with ACEIs and ARBs, along with disease-specific treatment if available (eg, antiretroviral therapy in HIV-associated nephropathy).

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

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

  Treatment for focal segmental glomerulosclerosis (FSGS) depends on the type and the cause. […] Depending on symptoms, medicines to treat FSGS might include: […] An angiotensin-converting enzyme (ACE) inhibitor or an angiotensin II receptor blocker (ARB). These can lower blood pressure and reduce protein in the urine. […] Medicines to lower cholesterol levels. People with FSGS often have high cholesterol. […] Medicines to help the body get rid of salt and water, called diuretics. These can improve blood pressure and swelling. […] Medicines to lower the body’s immune response. For primary FSGS, these medicines may stop the immune system from damaging the kidneys. These medicines include corticosteroids. They can have serious side effects, so they’re used with caution. […] For people who have kidney failure, treatments include dialysis and kidney transplant.

• #9 Emerging drugs for treatment of focal segmental glomerulosclerosis

  https://pmc.ncbi.nlm.nih.gov/articles/PMC7508791/

  Existing treatment of glomerulosclerosis and FSGS focus on non-specific treatment of blood pressure and agents that reduce proteinuria. Normalization of blood pressure is associated with delayed disease progression. Blood pressure control involves dietary control of sodium, potassium, and calcium intake and prescription of safe and tolerated anti-hypertensive drugs. Implementation of drugs that inhibit the activity of the renin-angiotensin-aldosterone system (RAAS) are especially useful in this regard because they not only lower blood pressure but they reduce proteinuria and are renoprotective. […] The application of these promising findings to other forms of glomerular disease and causes of CKD such as FSGS is under current investigation. However, they are generic interventions that are applicable to virtually all types of kidney disease and do not address the underlying mechanism of glomerular injury. Specifically, they do not target the podocyte which is the major cell involved in primary glomerular disease.

• #10 Focal Segmental Glomerulosclerosis Treatment & Management: Approach Considerations, Nonspecific Treatment, Specific Treatment

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

  In most patients, loop diuretics (eg, furosemide) are needed to promote diuresis. Patients with massive edema with impaired oral absorption may require intravenous administration. In patients with refractory conditions, addition of other diuretics (eg, metolazone) and potassium-sparing agents (eg, spironolactone, triamterene) facilitates diuresis and prevents hypokalemia. […] ACEIs and ARBs are nonspecific agents that reduce proteinuria because of their antihypertensive and intrarenal hemodynamic effects of reducing glomerular capillary pressure and resistance. ACEIs and ARBs are effective in reducing protein loss even in normotensive patients. These agents do not eliminate proteinuria completely or reverse the primary glomerular disease process. […] Current evidence favors prolonged corticosteroid therapy (approximately 6 months) to induce remission in patients with idiopathic FSGS. […] In adults, the current approach calls for initiating therapy with prednisone in a dose of 1 mg/kg daily (maximum 80 mg) or 2 mg/kg on alternate days. This dosage is continued for at least 4 weeks and until complete remission is reached, or to a maximum of 16 weeks. Studies indicate that 30-60% of patients may undergo complete or partial remission with such a regimen, and relapses are frequent when steroids are discontinued.

• #11 Focal segmental glomerulosclerosis (FSGS) – Care at Mayo Clinic – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/fsgs/care-at-mayo-clinic/mac-20354696

  Successful treatment of FSGS can be challenging. Some people with FSGS also develop nephrotic syndrome, which may affect treatment choice. Nephrotic syndrome is a kidney disorder that causes your body to pass too much protein in the urine. […] Depending on the type of FSGS you have, your doctor may recommend: […] An angiotensin-converting enzyme (ACE) inhibitor or an angiotensin II receptor blocker (ARB) medication to lower blood pressure and reduce protein in the urine. […] Medication to lower cholesterol levels. People with FSGS and nephrotic syndrome often have high cholesterol. […] Diuretic medications to help your body get rid of salt and water, which can improve blood pressure and swelling. […] Medication to suppress the immune system if you have primary FSGS. These drugs, including corticosteroids, may stop the immune system from attacking the glomeruli and further damaging kidney function. But these medications can have serious side effects, so they’re used with caution.

• #12 Focal Segmental Glomerulosclerosis Treatment & Management: Approach Considerations, Nonspecific Treatment, Specific Treatment

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

  Nonspecific treatment goals in patients with nephrotic syndrome include maintenance of adequate nutrition, minimization or elimination of proteinuria, and prevention of complications resulting from edema. Control of hypertension is one of the most important aspects of overall management. Lowering of lipid levels is necessary to reduce cardiovascular risk and to possibly delay the progression of kidney disease. […] The mainstay of treatment is reduction in daily salt intake to 2 g of sodium (6 g of salt) and the use of diuretics in varying doses and combinations. Because a high level of protein intake may further aggravate proteinuria, adversely affecting kidney function, current recommendations call for an intake of 1-1.3 g of high biologic value protein per kilogram of body weight. A reduction of dietary fat intake may help with dyslipidemia.

• #13 Focal Segmental Glomerulosclerosis Treatment & Management: Approach Considerations, Nonspecific Treatment, Specific Treatment

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

  In most patients, loop diuretics (eg, furosemide) are needed to promote diuresis. Patients with massive edema with impaired oral absorption may require intravenous administration. In patients with refractory conditions, addition of other diuretics (eg, metolazone) and potassium-sparing agents (eg, spironolactone, triamterene) facilitates diuresis and prevents hypokalemia. […] ACEIs and ARBs are nonspecific agents that reduce proteinuria because of their antihypertensive and intrarenal hemodynamic effects of reducing glomerular capillary pressure and resistance. ACEIs and ARBs are effective in reducing protein loss even in normotensive patients. These agents do not eliminate proteinuria completely or reverse the primary glomerular disease process. […] Current evidence favors prolonged corticosteroid therapy (approximately 6 months) to induce remission in patients with idiopathic FSGS. […] In adults, the current approach calls for initiating therapy with prednisone in a dose of 1 mg/kg daily (maximum 80 mg) or 2 mg/kg on alternate days. This dosage is continued for at least 4 weeks and until complete remission is reached, or to a maximum of 16 weeks. Studies indicate that 30-60% of patients may undergo complete or partial remission with such a regimen, and relapses are frequent when steroids are discontinued.

• #14 Focal segmental glomerulosclerosis (FSGS) – Care at Mayo Clinic – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/fsgs/care-at-mayo-clinic/mac-20354696

  Cyclosporine may be used when corticosteroids such as prednisone are not working in people with nephrotic syndrome. […] Your doctor may also recommend certain lifestyle strategies for overall kidney health: […] Avoid medications that have the potential to damage your kidneys, for example, some pain relievers such as nonsteroidal anti-inflammatory drugs (NSAIDs) […] Follow a healthy, low-sodium, moderate-protein diet to help protect your kidneys and lower your blood pressure […] Stop smoking […] Lose weight if you’re overweight […] Be active on most days. […] FSGS is a disease that may relapse. Because scarring in the glomeruli may be permanent, follow-up monitoring is important to assess kidney function. […] For people who develop kidney failure, treatment options include: […] Dialysis. Mayo Clinic offers state-of-the-art dialysis treatment, including the option for in-home dialysis. […] Kidney transplant. All three Mayo Clinic campuses offer kidney transplants through the Kidney Transplant Program. Mayo Clinic has performed more than 17,500 kidney transplants with excellent results.

• #15 How to Treat Corticosteroid-Resistant Idiopathic Focal Segmental Glomerulosclerosis? | Nefrología

  https://www.revistanefrologia.com/en-how-treat-corticosteroid-resistant-idiopathic-focal-articulo-X2013251411052060

  Focal segmental glomerulosclerosis (FSGS) is defined as an increase in the mesangial matrix in some glomeruli with obliteration of capillary lumens, sclerosis, hyalinosis, foam cells, and adhesions to the Bowmans capsule. […] Fortunately, FSGS is an uncommon disease that is found only in 9% of all kidney biopsies in our country, with stable rates in recent years. […] As with other glomerular diseases, its treatment must be based on the best evidence available through controlled clinical trials. However, few studies have been performed to resolve the current problems with treatment, and many of them are of poor quality, as reported by Quereda and Ballarn in an excellent systematic review published in Nefrologa in 2007. […] Patients with idiopathic FSGS that do not develop a nephrotic syndrome, as well as those with secondary FSGS, do not receive immunosuppressive treatment (recommendation 1C, GRADE system). In these cases, the standard treatment consists of: 1) maintain blood pressure below 130/80mm Hg; 2) administer angiotensin-converting enzyme (ACE) inhibitors, angiotensin II receptor antagonists, or both; 3) administer statins; 4) administer antiplatelet or anticoagulation therapy; and 5) diet for renal protection.

• #16 Focal Segmental Glomerulosclerosis Treatment & Management: Approach Considerations, Nonspecific Treatment, Specific Treatment

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

  In most patients, loop diuretics (eg, furosemide) are needed to promote diuresis. Patients with massive edema with impaired oral absorption may require intravenous administration. In patients with refractory conditions, addition of other diuretics (eg, metolazone) and potassium-sparing agents (eg, spironolactone, triamterene) facilitates diuresis and prevents hypokalemia. […] ACEIs and ARBs are nonspecific agents that reduce proteinuria because of their antihypertensive and intrarenal hemodynamic effects of reducing glomerular capillary pressure and resistance. ACEIs and ARBs are effective in reducing protein loss even in normotensive patients. These agents do not eliminate proteinuria completely or reverse the primary glomerular disease process. […] Current evidence favors prolonged corticosteroid therapy (approximately 6 months) to induce remission in patients with idiopathic FSGS. […] In adults, the current approach calls for initiating therapy with prednisone in a dose of 1 mg/kg daily (maximum 80 mg) or 2 mg/kg on alternate days. This dosage is continued for at least 4 weeks and until complete remission is reached, or to a maximum of 16 weeks. Studies indicate that 30-60% of patients may undergo complete or partial remission with such a regimen, and relapses are frequent when steroids are discontinued.

• #17 How to Treat Corticosteroid-Resistant Idiopathic Focal Segmental Glomerulosclerosis? | Nefrología

  https://www.revistanefrologia.com/en-how-treat-corticosteroid-resistant-idiopathic-focal-articulo-X2013251411052060

  The initial treatment is based on a prednisone cycle (1mg/kg/day, maximum of 80mg/day or 2mg/kg/day on alternate days, maximum of 120 mg), which should be maintained for 16 weeks before the condition is declared corticosteroid-resistant. […] Approximately 30%-40% of patients are corticosteroid-resistant, presenting problems for developing a treatment plan, since resistance to corticosteroids is the strongest predictor for the development of chronic kidney disease: 35% at 5 years and 70% at 10 years. […] Cyclosporin A (CsA) is the best documented method of treatment in controlled trials and observational studies. […] The recommended dose is 2-5mg/kg/day administered in two doses (with levels of 125-175ng/ml) during a minimum of 6 months. […] In the case of a partial or complete response, which appears in 60%-70% of cases, treatment should be maintained for at least 12 months, and can then be progressively reduced by 25% every 2 months.

• #18 Focal Segmental Glomerulosclerosis Treatment & Management: Approach Considerations, Nonspecific Treatment, Specific Treatment

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

  A variety of other immunosuppressive agents have been used in patients with contraindications, unacceptable toxicity, or inadequate response to corticosteroids. These have included cyclophosphamide, calcineurin inhibitors, mycophenolate mofetil, and rituximab, among others. […] Guidelines from Kidney Disease/Improving Global Outcomes (KDIGO) suggest considering calcineurin inhibitors (cyclosporine or tacrolimus) as first-line therapy for patients who fail to respond to corticosteroids or experience significant adverse effects, as well as for patients with relative contraindications to high-dose corticosteroids. […] Rituximab has proved effective for maintenance of remission in frequently relapsing FSGS and for treatment of remission, including in kidney transplant recipients. […] Small studies have found benefit with use of galactose, abatacept, and adalimumab. Subcutaneously injected adrenocorticotropic hormone (ACTH) gel may have some efficacy for treatment of FSGS, especially in treatment-resistant or relapsing cases.

• #19 How I Treat Focal Segmental Glomerulosclerosis

  https://pmc.ncbi.nlm.nih.gov/articles/PMC9718025/

  CNI remains the only alternative therapeutic option with acceptable efficacy from clinical studies on primary FSGS and is indicated in those with intolerance, dependence, or resistance to corticosteroids. […] Treatment choices after exhausting the option of corticosteroids and CNIs remain poorly evaluated, with conflicting outcomes from clinical studies. […] Genetic testing in FSGS should also be considered in patients being evaluated for a kidney transplant as it is valuable in informing the unlikely risk of recurrence of nephrotic syndrome due to a genetic cause, where in contrast, the risk of recurrent disease after transplantation is high in primary FSGS.

• #20 How to Treat Corticosteroid-Resistant Idiopathic Focal Segmental Glomerulosclerosis? | Nefrología

  https://www.revistanefrologia.com/en-how-treat-corticosteroid-resistant-idiopathic-focal-articulo-X2013251411052060

  Fewer studies exist regarding the use of tacrolimus in the treatment of corticosteroid-resistant FSGS, but Segarra et al obtained positive results from using tacrolimus (0.15mg/kg/day; levels of 5-10ng/l) and low-dose steroids (0.15mg/kg/day) in 25 patients with previous resistance to steroids and CsA. […] Experience with this drug is scarce, with an approximately 44% of cases showing a partial response, but half of these patients suffer a recurrence when medication is suspended. […] In a recent study that was also sponsored by GLOSEN, involving 8 patients with FSGS resistant to other drug treatments, rituximab had a positive effect in only 3 cases, making it an unattractive alternative, at least as a monotherapy. […] Some experience has been gained in the administration of cyclophosphamide and chlorambucil, as they were used before the advent of calcineurin inhibitors.

• #21 Focal segmental glomerulosclerosis: Treatment and prognosis – UpToDate

  https://www.uptodate.com/contents/treatment-of-primary-focal-segmental-glomerulosclerosis

  Focal segmental glomerulosclerosis: Treatment and prognosis […] This topic will review the treatment and prognosis of the FSGS lesion in adults in all of its forms but will focus on patients with presumed primary FSGS. […] TREATMENT MEASURES FOR ALL FORMS OF FSGS […] TREATMENT OF PRIMARY FSGS […] Initial therapy […] General approach […] Dosing and duration of initial therapy […] Glucocorticoids […] Calcineurin inhibitors as alternative initial therapy […] Monitoring the response to therapy […] Relapsing disease […] Glucocorticoid-dependent or glucocorticoid-resistant disease […] Calcineurin inhibitors as preferred second-line therapy […] Other therapies for patients resistant or intolerant to a calcineurin inhibitor […] Mycophenolate mofetil/sodium […] Rituximab […] Cytotoxic therapy […] Adrenocorticotropic hormone (ACTH) […] Plasmapheresis […] LDL apheresis […] TREATMENT OF SECONDARY FSGS OR FSGS OF UNDETERMINED CAUSE […] TREATMENT OF GENETIC FSGS […] INVESTIGATIONAL THERAPIES

• #22 Update on the treatment of focal segmental glomerulosclerosis in renal transplantation

  https://www.wjgnet.com/2220-3230/full/v6/i1/54.htm

  In FSGS recurrence, Kershaw et al treated 3 pediatric patients with CYC (1-2 mg/kg per day, adjusted for white blood cell count) for 8-12 wk and obtained two complete remissions and one partial; the patient with the longest follow-up (125 mo) experienced two additional relapses, each of which were treated successfully with pulse intravenous steroids. A more recent report described a series of 6 patients with FSGS recurrence all of whom were treated with a combination of CYC and PE (10 sessions over 2 wk, followed by 1 session per week for 2 mo), with complete remission being achieved in 3 of the patients and partial remission in the other 3. […] MMF inhibits the inosine monophosphate dehydrogenase-mediated reduction of T and B lymphocyte proliferation. Gbadegesin et al suggested MMF for treatment of steroid-dependent/resistant FSGS on native kidneys. Subsequently, a randomized controlled trial including 138 patients (both children and adults) with primary FSGS compared CyA and MMF plus dexamethasone, but no difference was observed in complete or partial remission rates after 52 wk of follow-up and both groups showed poor outcome (remission in 46% vs 33%, respectively). At the present time, as reported by Lau et al, no randomized controlled trial has yet to demonstrate the efficacy of MMF in association with other therapies or as a single agent in FSGS treatment on native or transplanted kidneys.

• #23 Rituximab treatment of adults with primary focal segmental glomerulosclerosis | Scientific Reports

  https://www.nature.com/articles/s41598-023-33678-y

  Rituximab (RTX) is a human-mouse chimeric monoclonal antibody, which can specifically bind to CD20 molecules on the surface of B cells, eliminate B cells through antibody or complement-mediated cytotoxicity, apoptosis induction and other ways, and is safe and effective for the treatment of a variety of immune diseases. […] We retrospectively analyzed the clinical data of patients with primary FSGS treated with RTX to evaluate the efficacy and safety of RTX in the treatment of FSGS. […] The primary outcome measures of follow-up were the remission and recurrence of renal disease, and the secondary outcome measures included adverse events (AEs) and renal outcomes. […] The recurrence rate of 9 SDNS/FRNS patients was magnificently reduced after the use of RTX, and the dose of glucocorticoid/immunosuppressant was withdrawn or reduced.

• #24 Rituximab treatment of adults with primary focal segmental glomerulosclerosis | Scientific Reports

  https://www.nature.com/articles/s41598-023-33678-y

  In this study, 8 adult patients with SDNS/FRNS were relieved after using RTX, the recurrence rate was significantly reduced, and the dosage of glucocorticoid and immunosuppressant was significantly reduced, which was consistent with the above results, and further confirmed the efficacy of RTX in the treatment of adult glucocorticoid-dependent FSGS. […] RTX can effectively induce remission of glucocorticoid-dependent/recurrent primary FSGS nephrotic syndrome in adults, reduce the risk of recurrence, and reduce the dosage of glucocorticoid and immunosuppressant or withdraw them. […] More prospective randomized controlled trials of RTX are needed to truly assess efficacy and durability of this drug compared with traditional specific immunosuppressive therapy.

• #25 Rituximab therapy for focal segmental glomerulosclerosis and minimal change disease in adults: a systematic review and meta-analysis | BMC Nephrology | Full Text

  https://bmcnephrol.biomedcentral.com/articles/10.1186/s12882-020-01797-7

  Use of rituximab (RTX) for focal segmental glomerulosclerosis (FSGS) and minimal change disease (MCD) is widely described in children. Clinical evidence in adults is limited. The objective of this study was to determine the treatment outcomes of RTX in adults with FSGS and MCD. […] A total of 16 studies were included in our review and analysis. All studies were observational studies and included a total of 221 patients (23.1% FSGS, 76.9% MCD). Mean follow-up duration was 26.312.8 months. From the analysis of five studies with FSGS patients (n=51), the overall remission rate and relapse rate of RTX therapy was 53.6% (95% CI, 15.887.6%) and 47.3% (95% CI, 25.470.2%), respectively. Complete remission occurred in 42.9%. In contrast, from the analysis of 11 studies with MCD patients (n=170), the overall remission rate and relapse rate of RTX therapy was 80.3% (95% CI, 68.588.5%) and 35.9% (95% CI, 25.148.4), respectively. Complete remission occurred in 74.7%.

• #26 Rituximab therapy for focal segmental glomerulosclerosis and minimal change disease in adults: a systematic review and meta-analysis | BMC Nephrology | Full Text

  https://bmcnephrol.biomedcentral.com/articles/10.1186/s12882-020-01797-7

  Rituximab may be considered as an additional treatment to the standard therapy for adult patients with FSGS and MCD. Remissions and relapses are similar between FSGS and MCD. Serious adverse effects of rituximab were uncommon. We encourage further randomized controlled trials to confirm the efficacy of rituximab therapy in these patients.

• #27 How to Treat Corticosteroid-Resistant Idiopathic Focal Segmental Glomerulosclerosis? | Nefrología

  https://www.revistanefrologia.com/en-how-treat-corticosteroid-resistant-idiopathic-focal-articulo-X2013251411052060

  Given the limitations presented by the previous drug treatments, some authors have incorporated the concept of synergistic effects of immunosuppressive drugs into the treatment of glomerular diseases. […] Although the treatment of corticosteroid-resistant idiopathic FSGS is still an unresolved issue, we must not be pessimistic. […] In conclusion, the treatment of idiopathic FSGS patients with nephrotic syndrome is still unresolved, and clinicians are faced with a challenge in producing some type of response in the patient and slowing or halting the evolution towards kidney failure. […] 1. FSGS is a non-specific glomerular lesion that is classified as idiopathic (primary) or secondary. […] 2. Patients with idiopathic FSGS that do not develop nephrotic syndrome and cases of secondary FSGS should not be treated using steroids or immunosuppressive drugs.

• #28 How to Treat Corticosteroid-Resistant Idiopathic Focal Segmental Glomerulosclerosis? | Nefrología

  https://www.revistanefrologia.com/en-how-treat-corticosteroid-resistant-idiopathic-focal-articulo-X2013251411052060

  3. Idiopathic FSGS patients with nephrotic syndrome should be initially treated with a cycle of steroids (or steroids and CsA in the case of intolerance to high levels of steroids) for a minimum of 4 weeks and a maximum of 16 weeks. […] 4. Patients with corticosteroid-resistant FSGS should be treated using CsA and low doses of steroids for at least 6 months, monitoring blood levels and nephrotoxicity. […] 5. Mycophenolate mofetil and rituximab can be effective alternatives in the case of resistance to CsA, although little evidence exists. […] 6. Alkylating agents (cyclophosphamide and chlorambucil) are only barely indicated and their use is not recommended, except for in very severe cases of dependence on corticosteroids. […] 7. Until now, combined treatments have not produced any positive results. […] 8. New treatments are needed based on controlled clinical trials that can induce a response in proteinuria and avoid the evolution of the disease towards kidney failure.

• #29 Focal segmental glomerulosclerosis (FSGS)

  https://www.kidneyfund.org/all-about-kidneys/other-kidney-diseases/focal-segmental-glomerulosclerosis-fsgs

  For all forms of FSGS, doctors recommend: Blood pressure medicines to lower the amount of protein lost and to control your blood pressure. […] Medicines called diuretics (water pills) that help your kidneys get rid of salt and water and make you urinate more to help reduce swelling in the body. […] Healthy life changes, such as following a kidney-friendly eating plan, being active for 30 minutes most days of the week and quitting smoking or other kinds of tobacco. […] In some cases of FSGS especially when FSGS is recurrent, and after a kidney transplant, plasmapheresis (also known as plasma exchange or „PLEX”) is a treatment option. […] This procedure has shown to help decrease protein loss in the urine (proteinuria) and may also help to improve response to other FSGS treatments. Some people may have several treatments over a few weeks depending on the response to the treatments. […] Your treatment plan will be unique to you and your condition. Talk with your doctor about other medicines and your options to help treat FSGS.

• #30 Focal Segmental Glomerulosclerosis Treatment & Management: Approach Considerations, Nonspecific Treatment, Specific Treatment

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

  Management of secondary FSGS is directed toward the etiology or associated disorder. For example, in HIV-associated FSGS, highly active antiretroviral therapy (HAART) is associated with remission of proteinuria and preservation of kidney function. Current guidelines call for initiation of HAART therapy in patients with HIV who have nephropathy irrespective of CD4 count and viral load.

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

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

  Management of secondary FSGS is also directed toward the etiology or associated disorder, such as the following: HIV-associated FSGS: Highly active antiretroviral therapy (HAART); corticosteroids, Heroin- or other drug-associated FSGS: Discontinuation of the offending agent. […] Some patients with FSGS continue to deteriorate and progress to ESKD. Treatment options include the following: Maintenance hemodialysis, Continuous ambulatory peritoneal dialysis, Cadaver or living donor kidney transplantation.

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

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

  How is FSGS treated? 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. […] Corticosteroids and immunosuppressive drugs: These medications are used to calm your immune system (your body’s defense system) and stop it from attacking your glomeruli. […] ACE inhibitors and ARBs: These are blood pressure medications used to reduce protein loss and control blood pressure. […] Diuretics: These medications help your body get rid of excess fluid and swelling. These can be used to lower your blood pressure too. […] Diet changes: Some diet changes may be needed, such as reducing salt (sodium) and protein in your food choices to lighten the load of wastes on the kidneys.

• #33 Focal Segmental Glomerulosclerosis FSGS Causes & Treatment

  https://nephcure.org/intro-to-rkd/types-of-rkd/focal-segmental-glomerulosclerosis-fsgs/

  FSGS is a term that describes a pattern of scarring in the kidneys. This scarring can be seen on a biopsy in patients with increased levels of protein in their urine. […] Your doctor recommends treatment based on your: Kidney function, Protein in the urine, Type of FSGS. […] If you have primary FSGS, your doctor is likely to prescribe medicines that modify your immune system. These include: Steroids, Medicines that target T and B cell function. […] Clinical trials are another treatment option. Your doctor helps you find the right study for you. These trials test more targeted treatments with potentially lower risks of side effects. Some clinical trials enroll patients, based on their genetic testing results, to test the effectiveness of precision-based therapies targeted to specific genetic changes. Knowing your genetic test results is important because patients with genetic FSGS generally do not show a meaningful clinical response to immunosuppressive medicines that suppress the immune response to reduce inflammation.

• #34 FSGS | Children’s Hospital Colorado

  https://www.childrenscolorado.org/conditions-and-advice/conditions-and-symptoms/conditions/focal-segmental-glomerulosclerosis/

  FSGS caused by genetic abnormalities is often irreversible, and doctors can only offer supportive treatment to contain the amount of urinary protein loss and body swelling. […] The treatment of FSGS in children is highly specialized and often complex. Details depend on individual findings such as the severity of nephrotic syndrome, degree of kidney function impairment and other aspects. The worst-case scenario is destruction of the kidneys by FSGS, at which point dialysis or kidney transplantation would be required. The Kidney Center at Childrens Colorado has the only pediatric dialysis and kidney transplant programs in the region. From testing and diagnosis to kidney transplantation, all these factors help make us the optimal choice for comprehensive pediatric FSGS care.

• #35

  https://link.springer.com/article/10.1007/s00467-024-06430-5

  The management of focal segmental glomerulosclerosis (FSGS) is challenging, and a definitive long-term cure remains elusive. It is associated with poor outcomes due to underlying irreversible glomerular fibrosis, scarring, different degrees of interstitial injury, and a variable rate of chronic kidney disease (CKD) progression. […] In non-genetic primary FSGS, complete or partial remission (CR or PR) of proteinuria is associated with a better prognosis, with 90% kidney survival at 5 years follow-up. Conversely, approximately 50% of non-responders have kidney failure (KF) by 5 years. […] Thus, the challenge is to provide optimal therapy to all affected children with the aim of achieving proteinuria remission and thereby improving short-term morbidities and long-term outcomes. […] Roughly, 1/3 of patients with primary FSGS may have proteinuria remission with steroid courses and renin-angiotensin system inhibitors (RASi), without the addition of other immunosuppressives (IS).

• #36

  https://link.springer.com/article/10.1007/s00467-024-06430-5

  The recent IPNA evidence-based guidelines for steroid-resistant (SR) NS recommend that, if available, genetic testing should be performed in all children diagnosed with primary SRNS, even before a kidney biopsy. […] After diagnostic evaluation, it is recommended to initiate RASi therapy and treatment with calcineurin inhibitor (CNI) for at least 6 months. Cyclophosphamide (CP) is suggested as an alternative first-line IS in low-resource settings if CNI is unavailable. […] Other IS agents used in non-responders include mycophenolate mofetil (MMF) and rituximab (RTX). […] Despite multiple trials and guidelines, several challenges in managing primary FSGS remain. At onset, it is difficult to predict response to IS, and the best options for long-term therapy and benefits for kidney survival remain unknown for the individual patient.

• #37 Pathophysiology and treatment of focal segmental glomerulosclerosis: the role of animal models | BMC Nephrology | Full Text

  https://bmcnephrol.biomedcentral.com/articles/10.1186/1471-2369-14-74

  Focal segmental glomerulosclerosis (FSGS) is a kidney disease with progressive glomerular scarring and a clinical presentation of nephrotic syndrome. […] Although kidney transplantation is a potentially curative treatment, 40% of patients have recurrence of FSGS after transplantation. […] To date, there is no exact understanding of the pathogenesis of idiopathic FSGS, and there is no definite curative treatment. […] We conclude that there is a need for a better animal model to investigate the pathogenesis and potential treatment options of FSGS. […] Ever since FSGS was first described by Arnold Rich in 1959, many studies have been conducted to understand its pathogenesis and to identify risk factors and/or possible treatments for this disease. […] Studies using the remnant kidney model are conducted for the development of preventive treatment strategies as well as for gaining more insight in underlying pathologies.

• #38 How to treat recurrent focal segmental glomerulosclerosis after kidney transplantation in children

  https://www.chikd.org/journal/view.php?number=819&viewtype=pubreader

  Focal segmental glomerulosclerosis (FSGS), a significant cause of kidney failure in children, is a common pathological diagnosis in cases of idiopathic nephrotic syndrome, especially steroid-resistant ones. […] However, advancements in management, including intensive plasmapheresis and immunosuppressive therapies like rituximab, have increased remission rates in cases of recurrence. Identifying patients at high risk of recurrence, such as those with an initial treatment response or previous failed transplantation, is crucial. These children require close monitoring of proteinuria and prompt, intensive treatment upon recurrence to improve outcomes. […] The primary treatment involves plasmapheresis or immunoadsorption to eliminate circulating permeability factors. Initiating early therapy is crucial to averting irreversible glomerular damage.

• #39 How to treat recurrent focal segmental glomerulosclerosis after kidney transplantation in children

  https://www.chikd.org/journal/view.php?number=819&viewtype=pubreader

  The CERTAIN group suggested that the initial approaches should include plasmapheresis or immunoadsorption, rituximab, and angiotensin-converting enzyme inhibitor/angiotensin II receptor blocker (ACEI/ARB) therapy. If these are ineffective, apheresis should be considered if the patient becomes dependent on plasmapheresis, high-dose cyclosporin, rituximab, and low-density lipoprotein apheresis. […] Plasmapheresis typically involves removing 1.5 plasma volumes and replacing them with 5% albumin, while immunoadsorption employs columns to selectively eliminate immunoglobulins from the plasma. […] Rituximab, a monoclonal antibody targeting CD20-positive B cells, has shown promise, with remission rates of up to 79% in recurrent FSGS. […] In addition to the aforementioned treatments, adjunctive immunosuppressive therapies are frequently used.

• #40 How to treat recurrent focal segmental glomerulosclerosis after kidney transplantation in children

  https://www.chikd.org/journal/view.php?number=819&viewtype=pubreader

  An important consideration is that treatment failure in the native kidneys does not necessarily predict posttransplantation treatment failure. Early intervention after transplantation can prevent sclerosis formation, emphasizing the importance of prompt treatment upon recurrence. […] For high-risk patients, such as children with a history of recurrence in a previous allograft, preemptive strategies are considered. These include pretransplant plasmapheresis or immunoadsorption sessions, sometimes combined with immunosuppression. […] Until such biomarkers are available, close monitoring and prompt, intensive treatment of recurrence is essential. Importantly, a diagnosis of FSGS should not be considered a contraindication to kidney transplantation, as many patients can achieve remission and enjoy long-term graft survival.

• #41 Emerging drugs for treatment of focal segmental glomerulosclerosis

  https://pmc.ncbi.nlm.nih.gov/articles/PMC7508791/

  The potent vasoconstrictor peptide, Endothelin, is produced by endothelial cells within the glomerulus. It interacts locally with podocytes to disrupt mitochondrial function and cellular bioenergetics. […] SGLT2 inhibitors are a novel class of drugs that have been shown to significantly reduce the risk of cardiovascular events and renal complications in patients with type 2 diabetes. […] Taken together, it is likely that over the next 5-10 years, we will witness significant advances in the scope of effective therapeutics that will be available to treat patients with glomerulosclerosis and FSGS in particular.

• #42 Advances in FSGS Treatment from the Perspective of the Newest Mechanis | DDDT

  https://www.dovepress.com/advances-in-focal-segmental-glomerulosclerosis-treatment-from-the-pers-peer-reviewed-fulltext-article-DDDT

  The DUPLEX study provides an updated evaluation of the efficacy of sparsentan, a dual endothelin-angiotensin receptor antagonist, in the treatment of FSGS patients. […] Moreover, recent findings suggest that sodium-glucose cotransporter 2 (SGLT2) inhibitors may benefit non-diabetic proteinuric chronic kidney disease (CKD) patients by mitigating hemodynamic injury. […] Studies on high-risk FSGS renal transplant recipients have shown that patients treated with rituximab have a significantly reduced risk of proteinuria recurrence after transplantation, which may be related to the direct podocyte protective effect of rituximab. […] Recent evidence suggests that there may be a specific subset of FSGS cases activated by an intrinsic TNF–podocyte pathway. […] Urinary MCP-1 and TIMP-1 may serve as non-invasive biomarkers for identifying the subset of FSGS patients with TNF- activation as a key driver of kidney injury. However, their potential for predicting the therapeutic response to TNF- blockers needs to be confirmed in a larger patient cohort.

• #43 Azthena logo with the word Azthena

  https://www.news-medical.net/news/20231103/Sparsentan-may-be-a-new-treatment-option-for-focal-segmental-glomerulosclerosis.aspx

  Focal segmental glomerulosclerosis (FSGS) is a rare kidney disorder that affects children and adults, and can lead to kidney failure. New findings from a team led by the University of Minnesota Medical School show patients with FSGS who were treated with the medication sparsentan experienced improved kidney function – making it a potential new treatment option for the disorder. […] The research, published today in the New England Journal of Medicine, suggests sparsentan may provide kidney protection by significantly reducing excess protein in urine – known as proteinuria, a proven indicator of kidney damage. […] FSGS patients in this study who were treated with sparsentan through two years of treatment had lower protein in their urine and were more likely to achieve complete remission compared to patients treated with irbesartan, the current standard treatment. […] These findings suggest sparsentan could be a potential new treatment option for FSGS. […] Further analysis is ongoing to determine which patients with FSGS may benefit the most from sparsentan.

• #44 Update on the treatment of focal segmental glomerulosclerosis in renal transplantation

  https://www.wjgnet.com/2220-3230/full/v6/i1/54.htm

  Abatacept is a biologic agent, specifically the CTLA4-Ig recombinant fusion protein derived from the extracellular portion of CTLA4-Ig and genetically fixated to the high and constant portion of the IgG1 immunoglobulin. Its effect is exerted by interfering with lymphocyte co-stimulation upon binding to the APC protein ligands B71 (CD80) or B72 (CD86) and displacing their T cell counterpart or CD28. In some experimental models of organ transplantation, the systemic administration of CTLA4-Ig effectively dampened the immune response, preventing experimental acute and chronic rejection and resulting in prolonged graft survival and tolerance. On the basis of these findings, different biological T cell co-stimulation blockers became the subject of clinical trials. A high affinity variant of CTLA4-Ig, named LEA29Y (belatacept, Nulojix; Bristol-Myers Squibb Pharma, Uxbridge, United Kingdom), has been developed and was awarded approval by the Federal Drug Administration (FDA) in 2011 for prophylactic use for organ rejection in adult kidney recipients.

• #45 Advances in FSGS Treatment from the Perspective of the Newest Mechanis | DDDT

  https://www.dovepress.com/advances-in-focal-segmental-glomerulosclerosis-treatment-from-the-pers-peer-reviewed-fulltext-article-DDDT

  Podocyte injury was widely recognized as a fundamental mechanism driving the progression of focal segmental glomerulosclerosis (FSGS). Recent research has therefore focused on the development of targeted therapies aimed at disrupting specific pathogenic signaling cascades within podocytes, resulting in noteworthy advancements. […] Corresponding targeted medications such as Abatacept, chemokine receptor (CCR) inhibitors, CDDO-Im (2-Cyano-3,12-dioxooleana-1,9-dien-28-imidazolide), adenosine monophosphate-activated protein kinase (AMPK) activators, and Adalimumab are currently under investigation. Notably, some medications such as Rituximab and Sparsentan, may simultaneously target multiple downstream mechanisms. […] Hence, a critical aspect of managing FSGS patients is to identify those who may benefit from specific treatments and to offer more targeted therapeutic options. In recent years, the focus of research has shifted towards therapies targeting podocyte-specific pathogenic signaling cascades.

• #46 Young Man Becomes First in World to Be Cured of FSGS With New Treatment – Stanford Medicine Children’s Health Blog

  https://healthier.stanfordchildrens.org/en/young-man-becomes-first-in-world-to-be-cured-of-fsgs-with-new-treatment/

  Combined stem cell transplant and kidney transplant prove a winning combination against autoimmune FSGS. […] Unfortunately, traditional kidney transplants fail in children with autoimmune FSGS over 90% of the time. […] In 2016, Traejens nephrologist (kidney doctor) put the family in touch with Dr. Grimm to talk over solutions. […] A groundbreaking procedure called dual immune/solid organ transplant (DISOT) is a two-transplant approach, pioneered at Stanford Medicine Childrens Health for certain kidney diseases, including autoimmune FSGS. […] The idea has been around for decades, yet it has been difficult for researchers to implement. Until now. […] The plan was for Traejen to receive his kidney transplant in the summer of 2021. […] Thankfully, the kidney transplant went well. […] A huge benefit of the DISOT approach is that the recipient doesnt have to take anti-rejection medicines for life, only for a short period of time right after transplant.

• #47 Autoimmune Focal Segmental Glomerulosclerosis (FSGS) – Stanford Medicine Children’s Health

  https://www.stanfordchildrens.org/en/services/transplant/disot/fsgs.html

  Read more about our new revolutionary approach to treating and potentially curing FSGS, called DISOT. […] Autoimmune FSGS can be treated with diet changes, medicines, and high doses of immunosuppressive drugs and corticosteroids given intravenously to suppress the immune system. […] We have researched, developed, and had initial success with a two-transplant approach to treating autoimmune FSGS called dual immune/solid organ transplant (DISOT). […] DISOT is a stem cell transplant, which provides your child with a new immune system, followed by a kidney transplant from the same donor, usually a parent. […] When a new immune system is adopted from a stem cell donorone that recognizes the transplanted kidney as its ownit essentially reboots your childs immune system. […] The donor kidney could last a long time, maybe even a lifetime.

• #48 Young Man Becomes First in World to Be Cured of FSGS With New Treatment – Stanford Medicine Children’s Health Blog

  https://healthier.stanfordchildrens.org/en/young-man-becomes-first-in-world-to-be-cured-of-fsgs-with-new-treatment/

  Best of all, Traejens FSGS is gone. […] If FSGS was going to come back, it should have come back long ago. […] Because we changed his immune system and gave him the immune system of his father, we consider him cured, Dr. Bertaina adds. […] We are grateful to Traejen and his family, Dr. Bertaina says. They helped us broaden the use of our dual-transplant approach, empowering us to potentially improve the lives of thousands of children with FSGS and other diseases.

• #49 Advances in FSGS Treatment from the Perspective of the Newest Mechanis | DDDT

  https://www.dovepress.com/advances-in-focal-segmental-glomerulosclerosis-treatment-from-the-pers-peer-reviewed-fulltext-article-DDDT

  Despite gaining new understanding of the pathogenesis of FSGS, there remains a paucity of effective targeted therapies. […] It is crucial that immunosuppressive regimens be tailored to focus on patients who are likely to benefit, while avoiding their use in those who are unlikely to respond favorably. […] Consequently, there is an imperative to explore innovative therapeutic approaches that specifically target the pathophysiological mechanisms underlying FSGS, in order to pave the way for new treatment modalities that overcome the limitations and adverse effects associated with conventional treatments. […] Innovative therapeutic agents, like the slit guidance ligand 2 (SLIT2) antagonists, CDDO-Im (2-Cyano-3,12-dioxooleana-1,9-dien-28-imidazolide), CC chemokine receptor 2 (CCR2) inhibitors and adenosine monophosphate-activated protein kinase (AMPK) activators, are under development, aiming to expand the treatment repertoire for patients suffering from focal segmental glomerulosclerosis (FSGS).

• #50 Focal Segmental Glomerulosclerosis (FSGS) | GlomCon

  https://www.glomcon.org/kidney-diseases/focal-segmental-glomerulosclerosis-(fsgs)

  Treatment for FSGS is dependent on the cause of the disease, either primary or secondary such as due to genetic cause. […] Typically primary FSGS treatment involves immunosuppressive medications. Sometimes, several immunosuppressive medications may be required. […] For APOL1-mediated kidney disease, several clinical trials are currently under way to find a treatment. […] For secondary forms of the disease, treatment is usually directed at the underlying cause. For example, if your doctor suspects an infection causes the disease, treatment is targeted at the infection. […] For any form of FSGS, your doctor will likely give you medications to control your blood pressure, reduce the amount of protein leaking into the urine, reduce swelling (using diuretics), maintain your cholesterol levels, and prevent blood clots (using anti-coagulant medications). Your doctor will also advise you to maintain a healthy lifestyle, follow a low-salt diet, quit smoking, and exercise regularly.

• #51 Focal Segmental Glomerulosclerosis – MD Searchlight

  https://mdsearchlight.com/kidney-health/focal-segmental-glomerulosclerosis/

  In some cases, other drugs or approaches might be used if patients are intolerant or resistant to the glucocorticoid or the calcineurin inhibitors. These can include Mycophenolate mofetil, high-dose dexamethasone, rituximab, adrenocorticotropic hormone, mTOR inhibitors, and for select patients, plasmapheresis. […] For less severe cases, lifestyle changes like reduced salt intake and certain medications can be tried. It’s also important to manage other conditions like high blood pressure, manage symptoms like swelling with water pills (diuretics), use cholesterol-lowering drugs for high cholesterol, and in some cases, use blood thinners to prevent blood clots. […] The effectiveness of treatment can vary based on age. Children often respond to treatment within a few weeks, while adults may take months to see improvement. About 30% of patients show improvements through glucocorticoids, while around half show improvement with calcineurin inhibitors.

• #52 Azthena logo with the word Azthena

  https://www.news-medical.net/health/Focal-Segmental-Glomerular-Sclerosis-Diagnosis-and-Treatment.aspx

  Therapy for FSGS is aimed towards inducing total remission of urinary excretion of proteins, which leads to greater chances of preserving the long-term function of the kidney. It is crucial to make a distinction between primary and secondary FSGS, because different therapeutic approaches appear to be more effective in one as opposed to the other and vice versa. […] Primary FSGS is presumed to be due to dysregulation of an autoimmune response and as such, immunosuppressive agents are advocated for treating this group of patients. These agents include calcineurin inhibitors, rituximab, and high-dose prednisone. […] Attempts are made to eliminate the causative factor in secondary FSGS. In addition to treating the underlying condition, intraglomerular and systemic pressures are closely managed with agents, such as inhibitors of the renin-angiotensin system.

• #53 Azthena logo with the word Azthena

  https://www.news-medical.net/health/Focal-Segmental-Glomerular-Sclerosis-Diagnosis-and-Treatment.aspx

  However, regardless of the etiology involved, all patients with nephrotic syndrome signs and symptoms and FSGS should be managed with diuretics, low-salt diets, and inhibitors of the renin-angiotensin system. These assist in reducing the risk of cardiovascular disease and long-term damage to the kidney.

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

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

  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. It is also important for someone with either primary OR secondary FSGS to be on medication that reduces the amount of protein in the urine. These medications are called ACE-inhibitors (angiotensin converting enzyme inhibitors) and ARBs (angiotensin II receptor blockers). If urine protein levels are high, the complications of the Nephrotic Syndrome should also be considered. Patients should receive routine cholesterol screening/treatment. Their physicians should always remember their tendency to form clots. […] In addition to the above, many different types of drugs that suppress the immune system are also being used to treat FSGS. The most common of these are steroids. Unfortunately, all of these drugs have significant side effects. Their use must be considered on a patient-by-patient basis.

• #55

  https://link.springer.com/article/10.1007/s00467-024-06430-5

  In the last three decades, the increasing availability of a large number of IS and biological agents has improved the prognosis of patients with primary non-genetic FSGS. Further research into more precise phenotyping to guide therapy, as well as less expensive and less toxic therapeutic agents will hopefully continue to improve outcomes in this difficult condition.

• #56 Focal Segmental Glomerulosclerosis (FSGS) | GlomCon

  https://www.glomcon.org/kidney-diseases/focal-segmental-glomerulosclerosis-(fsgs)

  If the disease progresses or presents in an advanced stage, your doctor will discuss kidney replacement therapy options with you, including dialysis and kidney transplantation. Your doctor may also offer you the option of enrolling in a clinical trial. […] It is important for anyone experiencing symptoms suspicious of FSGS to see a doctor for proper diagnosis and treatment. Early intervention may help prevent complications and improve the chances of a successful outcome. […] Developing kidney failure is, unfortunately, a possibility for those suffering from FSGS. Depending on the underlying cause, about 40-50% of patients may not have an adequate response to treatment and may progress to kidney failure, in which case your doctor will discuss the options of dialysis and/or kidney transplantation. […] New treatments and therapies are being developed for kidney diseases. Ask your doctor if you are interested in participating in a clinical trial for Focal Segmental Glomerulosclerosis.

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