Materiały źródłowe

• #1 Lupus Nephritis – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK499817/

  Lupus nephritis is a severe manifestation of systemic lupus erythematosus (SLE) a chronic autoimmune disease that causes inflammation and damage to multiple organ systems, with the kidneys as a primary target. […] The primary treatment goal is to preserve kidney function, slow disease progression, and improve outcomes, with therapies tailored to the specific pathological lesion. […] Treatment may include hydroxychloroquine for all patients and immunosuppressive or steroid therapy for more severe forms. […] This activity emphasizes the importance of interprofessional collaboration among healthcare providers in managing lupus nephritis and delivering integrated care. […] The treatment of lupus nephritis is primarily guided by histopathological class. All patients should initiate therapy with hydroxychloroquine at baseline unless contraindicated, with regular ophthalmological exams to assess for retinal toxicity. […] Generally, classes I and II may require monitoring and may not need treatment, particularly if proteinuria is under 500 mg/d. Immunosuppressive therapy and steroids are necessary for classes III and IV, while renal replacement therapy is considered for class VI, where most glomeruli are sclerotic.

• #2 Lupus Nephritis: What Is It, Causes, Symptoms and Treatment

  https://my.clevelandclinic.org/health/diseases/21809-lupus-nephritis

  Lupus nephritis is kidney inflammation due to lupus, an autoimmune disease. […] About half of adults and 80% of children with lupus will develop lupus nephritis. […] Medication and diet changes are the most common treatments for lupus nephritis. Your healthcare provider may recommend: […] Corticosteroids and immunosuppressive drugs: These medications prevent your immune system from attacking the blood vessels in your kidneys. […] Diet changes: You may need to reduce your sodium (salt) intake. Eating less protein, such as meat and dairy, can also make it easier for your kidneys to work. […] Diuretics: These medications help treat edema (excess fluid and swelling). Diuretics can also lower your blood pressure. […] Kidney failure develops in 10% to 30% of people with lupus nephritis. If this happens, you may need:

• #3 Lupus and kidney disease (lupus nephritis) – Symptoms, treatment, & stages | National Kidney Foundation

  https://www.kidney.org/kidney-topics/lupus-nephritis

  Lupus nephritis is a disease that affects the kidneys, making it difficult for them to clean blood well. […] Most medications for LN work to prevent the overactive immune system from attacking the kidneys. Other medicines are used to keep your kidneys healthy. Treatments differ depending on the class of the disease. […] The goals of treatment for lupus nephritis are to: Reduce inflammation in your kidneys, Decrease immune system activity, Block your body’s immune cells from attacking the kidneys directly or making antibodies that attack the kidneys. […] No matter the class, LN is usually treated with Plaquenil (hydroxychloroquine), which helps to keep your immune system activity well-balanced. In addition, blood pressure medications, such as angiotensin-converting enzyme (ACE) inhibitors or angiotensin receptor blockers (ARBs), are often recommended, even if you don’t have high blood pressure.

• #4 Lupus Nephritis: What Is It, Causes, Symptoms and Treatment

  https://my.clevelandclinic.org/health/diseases/21809-lupus-nephritis

  Lupus nephritis is kidney inflammation due to lupus, an autoimmune disease. […] About half of adults and 80% of children with lupus will develop lupus nephritis. […] Medication and diet changes are the most common treatments for lupus nephritis. Your healthcare provider may recommend: […] Corticosteroids and immunosuppressive drugs: These medications prevent your immune system from attacking the blood vessels in your kidneys. […] Diet changes: You may need to reduce your sodium (salt) intake. Eating less protein, such as meat and dairy, can also make it easier for your kidneys to work. […] Diuretics: These medications help treat edema (excess fluid and swelling). Diuretics can also lower your blood pressure. […] Kidney failure develops in 10% to 30% of people with lupus nephritis. If this happens, you may need:

• #5 Treatment of lupus nephritis: consensus, evidence and perspectives | Nature Reviews Rheumatology

  https://www.nature.com/articles/s41584-023-00925-5

  Despite the continuing development of immunomodulatory agents and supportive care, the prognosis associated with lupus nephritis (LN) has not improved substantially in the past decade, with end-stage kidney disease still developing in 530% of patients within 10 years of LN diagnosis. […] Modalities that better preserve kidney function and reduce the toxicities of concomitant glucocorticoids are unmet needs in the development of therapeutics for LN. […] In addition to the conventional recommended therapies for LN, there are newly approved treatments as well as investigational drugs in the pipeline, including the newer generation calcineurin inhibitors and biologic agents. […] Belimumab and voclosporin are the two most recently approved drugs for the treatment of LN in most parts of the world.

• #6 Lupus and kidney disease (lupus nephritis) – Symptoms, treatment, & stages | National Kidney Foundation

  https://www.kidney.org/kidney-topics/lupus-nephritis

  Lupus nephritis is a disease that affects the kidneys, making it difficult for them to clean blood well. […] Most medications for LN work to prevent the overactive immune system from attacking the kidneys. Other medicines are used to keep your kidneys healthy. Treatments differ depending on the class of the disease. […] The goals of treatment for lupus nephritis are to: Reduce inflammation in your kidneys, Decrease immune system activity, Block your body’s immune cells from attacking the kidneys directly or making antibodies that attack the kidneys. […] No matter the class, LN is usually treated with Plaquenil (hydroxychloroquine), which helps to keep your immune system activity well-balanced. In addition, blood pressure medications, such as angiotensin-converting enzyme (ACE) inhibitors or angiotensin receptor blockers (ARBs), are often recommended, even if you don’t have high blood pressure.

• #7 Lupus Nephritis Treatment & Management: Approach Considerations, Pharmacotherapy for Lupus Nephritis Based on Stage, New Therapies

  https://emedicine.medscape.com/article/330369-treatment

  The principal goal of therapy in lupus nephritis is to normalize kidney function or, at least, to prevent the progressive loss of kidney function. Therapy differs depending on the pathologic lesion. It is important to treat extrarenal manifestations and other variables that may affect the kidneys. Patients should be on hydroxychloroquine if possible, as data suggest that this improves outcomes in patients who have lupus nephritis, in addition to reducing lupus-related flares and disease damage accrual. […] Corticosteroid therapy should be instituted if the patient has clinically significant renal disease. Use immunosuppressive agents, particularly cyclophosphamide, azathioprine, or mycophenolate mofetil, if the patient has aggressive proliferative renal lesions, as they improve the renal outcome. Immunosuppressives can also be used if the patient has an inadequate response or excessive sensitivity to corticosteroids.

• #8 Lupus Nephritis Treatment & Management: Approach Considerations, Pharmacotherapy for Lupus Nephritis Based on Stage, New Therapies

  https://emedicine.medscape.com/article/330369-treatment

  The principal goal of therapy in lupus nephritis is to normalize kidney function or, at least, to prevent the progressive loss of kidney function. Therapy differs depending on the pathologic lesion. It is important to treat extrarenal manifestations and other variables that may affect the kidneys. Patients should be on hydroxychloroquine if possible, as data suggest that this improves outcomes in patients who have lupus nephritis, in addition to reducing lupus-related flares and disease damage accrual. […] Corticosteroid therapy should be instituted if the patient has clinically significant renal disease. Use immunosuppressive agents, particularly cyclophosphamide, azathioprine, or mycophenolate mofetil, if the patient has aggressive proliferative renal lesions, as they improve the renal outcome. Immunosuppressives can also be used if the patient has an inadequate response or excessive sensitivity to corticosteroids.

• #9 What is Lupus Nephritis? Treatment and Overview | HSS

  https://www.hss.edu/conditions_lupus-nephritis-what-you-need-to-know-about-lupus-kidney-disease.asp

  People with the more severe forms of lupus nephritis, classes III, IV and V (with nephrotic proteinuria), need to be started on an aggressive course of treatment that is also referred to as induction therapy, since the goal is to induce a remission of the inflammation and related symptoms. […] During induction therapy, immunosuppressive drugs are administered at high doses. The duration of induction therapy can range from three months to one year. A key point here is to start induction therapy as soon as possible without losing time, in order to minimize loss of kidney tissue from the inflammation. […] As the condition improves, the patient switches to maintenance therapy, in which a lower dose of the drug or drugs is administered. Maintenance therapy continues for at least two years, though the optimal duration is not known yet.

• #10 Lupus Nephritis – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK499817/

  Lupus nephritis is a severe manifestation of systemic lupus erythematosus (SLE) a chronic autoimmune disease that causes inflammation and damage to multiple organ systems, with the kidneys as a primary target. […] The primary treatment goal is to preserve kidney function, slow disease progression, and improve outcomes, with therapies tailored to the specific pathological lesion. […] Treatment may include hydroxychloroquine for all patients and immunosuppressive or steroid therapy for more severe forms. […] This activity emphasizes the importance of interprofessional collaboration among healthcare providers in managing lupus nephritis and delivering integrated care. […] The treatment of lupus nephritis is primarily guided by histopathological class. All patients should initiate therapy with hydroxychloroquine at baseline unless contraindicated, with regular ophthalmological exams to assess for retinal toxicity. […] Generally, classes I and II may require monitoring and may not need treatment, particularly if proteinuria is under 500 mg/d. Immunosuppressive therapy and steroids are necessary for classes III and IV, while renal replacement therapy is considered for class VI, where most glomeruli are sclerotic.

• #11 Lupus Nephritis Guidelines: Guidelines Summary

  https://emedicine.medscape.com/article/330369-guidelines

  Patients with clinical evidence of active, previously untreated lupus nephritis should have a kidney biopsy to classify the disease according to International Society of Nephrology/Renal Pathology Society criteria. […] All patients with lupus nephritis should receive background therapy with hydroxychloroquine, unless contraindicated; this recommendation was based on a prospective controlled trial showing lower flare rates in those who continued hydroxychloroquine, compared with those who switched to placebo. […] Glucocorticoids plus either cyclophosphamide intravenously (IV) or mycophenolate mofetil orally for induction in patients with class III and IV disease; patients with class I and II nephritis do not require immunosuppressive therapy. […] Administer ACEIs or ARBs if proteinuria is 0.5 g/24 h or more.

• #12 Lupus Nephritis Treatment & Management: Approach Considerations, Pharmacotherapy for Lupus Nephritis Based on Stage, New Therapies

  https://emedicine.medscape.com/article/330369-treatment

  The principal goal of therapy in lupus nephritis is to normalize kidney function or, at least, to prevent the progressive loss of kidney function. Therapy differs depending on the pathologic lesion. It is important to treat extrarenal manifestations and other variables that may affect the kidneys. Patients should be on hydroxychloroquine if possible, as data suggest that this improves outcomes in patients who have lupus nephritis, in addition to reducing lupus-related flares and disease damage accrual. […] Corticosteroid therapy should be instituted if the patient has clinically significant renal disease. Use immunosuppressive agents, particularly cyclophosphamide, azathioprine, or mycophenolate mofetil, if the patient has aggressive proliferative renal lesions, as they improve the renal outcome. Immunosuppressives can also be used if the patient has an inadequate response or excessive sensitivity to corticosteroids.

• #13 What is lupus nephritis? | Lupus Foundation of America

  https://www.lupus.org/resources/what-is-lupus-nephritis

  Lupus nephritis is one of the most serious complications of systemic lupus erythematosus (SLE). […] If lupus nephritis is not treated symptoms can get worse and lead to kidney failure. […] Getting tested and treated as soon as possible is extremely important. […] Your doctor or a special doctor called a nephrologist can help you find the right treatment if you have lupus nephritis. […] There are medications used to help control the inflammation and reduce kidney damage like immunosuppressive drugs, corticosteroids, and even blood thinning supplements. Two drugs, Benlysta and Lupkynis, have been approved specifically to treat lupus nephritis. […] Every person with lupus is different, make sure to talk to your doctor about the treatment plan that is right for you. […] All patients with lupus nephritis usually take a medicine called hydroxychloroquine. Hydroxychloroquine has anti-inflammatory properties, decreasing inflammation and subsequent damage, which can help prevent flare-ups of lupus disease.

• #14 Lupus Nephritis – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK499817/

  Lupus nephritis is a severe manifestation of systemic lupus erythematosus (SLE) a chronic autoimmune disease that causes inflammation and damage to multiple organ systems, with the kidneys as a primary target. […] The primary treatment goal is to preserve kidney function, slow disease progression, and improve outcomes, with therapies tailored to the specific pathological lesion. […] Treatment may include hydroxychloroquine for all patients and immunosuppressive or steroid therapy for more severe forms. […] This activity emphasizes the importance of interprofessional collaboration among healthcare providers in managing lupus nephritis and delivering integrated care. […] The treatment of lupus nephritis is primarily guided by histopathological class. All patients should initiate therapy with hydroxychloroquine at baseline unless contraindicated, with regular ophthalmological exams to assess for retinal toxicity. […] Generally, classes I and II may require monitoring and may not need treatment, particularly if proteinuria is under 500 mg/d. Immunosuppressive therapy and steroids are necessary for classes III and IV, while renal replacement therapy is considered for class VI, where most glomeruli are sclerotic.

• #15 Lupus and kidney disease (lupus nephritis) – Symptoms, treatment, & stages | National Kidney Foundation

  https://www.kidney.org/kidney-topics/lupus-nephritis

  Lupus nephritis is a disease that affects the kidneys, making it difficult for them to clean blood well. […] Most medications for LN work to prevent the overactive immune system from attacking the kidneys. Other medicines are used to keep your kidneys healthy. Treatments differ depending on the class of the disease. […] The goals of treatment for lupus nephritis are to: Reduce inflammation in your kidneys, Decrease immune system activity, Block your body’s immune cells from attacking the kidneys directly or making antibodies that attack the kidneys. […] No matter the class, LN is usually treated with Plaquenil (hydroxychloroquine), which helps to keep your immune system activity well-balanced. In addition, blood pressure medications, such as angiotensin-converting enzyme (ACE) inhibitors or angiotensin receptor blockers (ARBs), are often recommended, even if you don’t have high blood pressure.

• #16 Lupus Nephritis Guidelines: Guidelines Summary

  https://emedicine.medscape.com/article/330369-guidelines

  Patients with clinical evidence of active, previously untreated lupus nephritis should have a kidney biopsy to classify the disease according to International Society of Nephrology/Renal Pathology Society criteria. […] All patients with lupus nephritis should receive background therapy with hydroxychloroquine, unless contraindicated; this recommendation was based on a prospective controlled trial showing lower flare rates in those who continued hydroxychloroquine, compared with those who switched to placebo. […] Glucocorticoids plus either cyclophosphamide intravenously (IV) or mycophenolate mofetil orally for induction in patients with class III and IV disease; patients with class I and II nephritis do not require immunosuppressive therapy. […] Administer ACEIs or ARBs if proteinuria is 0.5 g/24 h or more.

• #17 Lupus nephritis – Symptoms, treatment and complications – American Kidney Fund (AKF)

  https://www.kidneyfund.org/all-about-kidneys/other-kidney-diseases/lupus-nephritis-symptoms-treatment-and-complications

  Lupus nephritis is an autoimmune disease that occurs when the immune system attacks the kidneys. The treatment for lupus nephritis focuses on preventing more damage to your kidneys. Finding and treating lupus nephritis early can help prevent serious damage. To prevent this from happening, your health care provider might want you to take a medicine called an immunosuppressant. These types of medicines weaken your immune system so that it cannot harm your kidneys as much. […] Your health care provider might also want you to take medicine to lower your blood pressure. High blood pressure is the second most common cause of kidney failure (end-stage renal disease, or ESRD). Two common types of blood pressure medicine are ACE (angiotensin converting enzyme) inhibitors and ARBs (angiotensin receptor blockers). These medicines lower the amount of protein in the urine and protect the kidneys from further damage.

• #18 Lupus Nephritis Guidelines: Guidelines Summary

  https://emedicine.medscape.com/article/330369-guidelines

  Maintain blood pressure at or below 130/80 mm Hg. […] Initial treatment for class III-IV LN: Mycophenolate mofetil (MMF) or low-dose IV cyclophosphamide plus glucocorticoids. […] Alternate option for class III-IV LN: Combination therapy of MMF with a calcineurin inhibitor (CNI), especially tacrolimus, particularly in patients with nephrotic-range proteinuria. […] To reduce cumulative glucocorticoid dose: Intravenous pulses of methylprednisolone (total dose 500-2500mg, depending on disease severity), followed by oral prednisone (0.3-0.5mg/kg/day) for up to 4 weeks, tapered to 7.5mg/day by 3 to 6 months. […] Initial treatment for pure class V disease with nephrotic-range proteinuria: MMF in combination with methylprednisolone followed by oral prednisone. […] In all LN patients: Hydroxychloroquine (HCQ) should be coadministered at a dose not to exceed 5mg/kg/day and adjusted for the GFR, in the absence of contraindications.

• #19 Lupus Nephritis Guidelines: Guidelines Summary

  https://emedicine.medscape.com/article/330369-guidelines

  Patients with clinical evidence of active, previously untreated lupus nephritis should have a kidney biopsy to classify the disease according to International Society of Nephrology/Renal Pathology Society criteria. […] All patients with lupus nephritis should receive background therapy with hydroxychloroquine, unless contraindicated; this recommendation was based on a prospective controlled trial showing lower flare rates in those who continued hydroxychloroquine, compared with those who switched to placebo. […] Glucocorticoids plus either cyclophosphamide intravenously (IV) or mycophenolate mofetil orally for induction in patients with class III and IV disease; patients with class I and II nephritis do not require immunosuppressive therapy. […] Administer ACEIs or ARBs if proteinuria is 0.5 g/24 h or more.

• #20 Lupus Nephritis – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK499817/

  Lupus nephritis is a severe manifestation of systemic lupus erythematosus (SLE) a chronic autoimmune disease that causes inflammation and damage to multiple organ systems, with the kidneys as a primary target. […] The primary treatment goal is to preserve kidney function, slow disease progression, and improve outcomes, with therapies tailored to the specific pathological lesion. […] Treatment may include hydroxychloroquine for all patients and immunosuppressive or steroid therapy for more severe forms. […] This activity emphasizes the importance of interprofessional collaboration among healthcare providers in managing lupus nephritis and delivering integrated care. […] The treatment of lupus nephritis is primarily guided by histopathological class. All patients should initiate therapy with hydroxychloroquine at baseline unless contraindicated, with regular ophthalmological exams to assess for retinal toxicity. […] Generally, classes I and II may require monitoring and may not need treatment, particularly if proteinuria is under 500 mg/d. Immunosuppressive therapy and steroids are necessary for classes III and IV, while renal replacement therapy is considered for class VI, where most glomeruli are sclerotic.

• #21 Lupus and kidney disease (lupus nephritis) – Symptoms, treatment, & stages | National Kidney Foundation

  https://www.kidney.org/kidney-topics/lupus-nephritis

  Depending on your class/type of LN, your nephrologist will likely recommend a treatment plan that is right for you and will help keep your kidneys working well. These treatments can range from simple monitoring (watching and testing) to stronger medicines that help manage any symptoms and prevent more harm to your kidneys. […] Classes I II: Usually, no treatment is needed unless there is a large amount of protein in the urine (pee). If protein is in your pee, medicines like prednisone (a type of steroid) may be used. […] Active Classes III, IV V: Usually, the first phase (3 to 12 months) of treatment includes steroids and one or more medications like these: Mycophenolate mofetil (MMF), Mycophenolic acid (MPA), Cyclophosphamide, Belimumab (Benlysta), Calcineurin inhibitors, such as voclosporin (Lupkynis), tacrolimus, or cyclosporine, Rituximab (Class V only). […] Stable Classes III, IV, V: Usually, the first phase of treatment includes hydroxychloroquine and/or blood pressure medications. MMF or MPA may be recommended. […] Relapse (Symptoms return or get worse): Treat with the same medications that worked before.

• #22 Lupus and kidney disease (lupus nephritis) – Symptoms, treatment, & stages | National Kidney Foundation

  https://www.kidney.org/kidney-topics/lupus-nephritis

  Depending on your class/type of LN, your nephrologist will likely recommend a treatment plan that is right for you and will help keep your kidneys working well. These treatments can range from simple monitoring (watching and testing) to stronger medicines that help manage any symptoms and prevent more harm to your kidneys. […] Classes I II: Usually, no treatment is needed unless there is a large amount of protein in the urine (pee). If protein is in your pee, medicines like prednisone (a type of steroid) may be used. […] Active Classes III, IV V: Usually, the first phase (3 to 12 months) of treatment includes steroids and one or more medications like these: Mycophenolate mofetil (MMF), Mycophenolic acid (MPA), Cyclophosphamide, Belimumab (Benlysta), Calcineurin inhibitors, such as voclosporin (Lupkynis), tacrolimus, or cyclosporine, Rituximab (Class V only). […] Stable Classes III, IV, V: Usually, the first phase of treatment includes hydroxychloroquine and/or blood pressure medications. MMF or MPA may be recommended. […] Relapse (Symptoms return or get worse): Treat with the same medications that worked before.

• #23 Lupus Nephritis – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK499817/

  Lupus nephritis is a severe manifestation of systemic lupus erythematosus (SLE) a chronic autoimmune disease that causes inflammation and damage to multiple organ systems, with the kidneys as a primary target. […] The primary treatment goal is to preserve kidney function, slow disease progression, and improve outcomes, with therapies tailored to the specific pathological lesion. […] Treatment may include hydroxychloroquine for all patients and immunosuppressive or steroid therapy for more severe forms. […] This activity emphasizes the importance of interprofessional collaboration among healthcare providers in managing lupus nephritis and delivering integrated care. […] The treatment of lupus nephritis is primarily guided by histopathological class. All patients should initiate therapy with hydroxychloroquine at baseline unless contraindicated, with regular ophthalmological exams to assess for retinal toxicity. […] Generally, classes I and II may require monitoring and may not need treatment, particularly if proteinuria is under 500 mg/d. Immunosuppressive therapy and steroids are necessary for classes III and IV, while renal replacement therapy is considered for class VI, where most glomeruli are sclerotic.

• #24 Lupus Nephritis Treatment & Management: Approach Considerations, Pharmacotherapy for Lupus Nephritis Based on Stage, New Therapies

  https://emedicine.medscape.com/article/330369-treatment

  Patients with either focal (class III) or diffuse (class IV) lupus nephritis are at high risk of progressing to ESRD and thus require aggressive therapy. Administer prednisone 1 mg/kg/day for at least 4 weeks, depending on clinical response. Then, taper it gradually to a daily maintenance dose of 5-10 mg/day for approximately 2 years. In acutely ill patients, intravenous (IV) methylprednisolone at a dosage of up to 1000 mg/day for 3 days may be used to initiate corticosteroid therapy. […] In patients who do not respond to corticosteroids alone, who have unacceptable toxicity to corticosteroids, who have worsening renal function, who have severe proliferative lesions, or who have evidence of sclerosis on renal biopsy specimens, use immunosuppressive drugs in addition to corticosteroids. […] Both cyclophosphamide and azathioprine are effective in proliferative lupus nephritis, although cyclophosphamide is apparently more effective in preventing progression to ESRD. Mycophenolate mofetil has been shown to be at least as effective as intravenous (IV) cyclophosphamide, with less toxicity, in patients with focal or diffuse lupus nephritis who have stable renal function.

• #25 Lupus and kidney disease (lupus nephritis) – Symptoms, treatment, & stages | National Kidney Foundation

  https://www.kidney.org/kidney-topics/lupus-nephritis

  Depending on your class/type of LN, your nephrologist will likely recommend a treatment plan that is right for you and will help keep your kidneys working well. These treatments can range from simple monitoring (watching and testing) to stronger medicines that help manage any symptoms and prevent more harm to your kidneys. […] Classes I II: Usually, no treatment is needed unless there is a large amount of protein in the urine (pee). If protein is in your pee, medicines like prednisone (a type of steroid) may be used. […] Active Classes III, IV V: Usually, the first phase (3 to 12 months) of treatment includes steroids and one or more medications like these: Mycophenolate mofetil (MMF), Mycophenolic acid (MPA), Cyclophosphamide, Belimumab (Benlysta), Calcineurin inhibitors, such as voclosporin (Lupkynis), tacrolimus, or cyclosporine, Rituximab (Class V only). […] Stable Classes III, IV, V: Usually, the first phase of treatment includes hydroxychloroquine and/or blood pressure medications. MMF or MPA may be recommended. […] Relapse (Symptoms return or get worse): Treat with the same medications that worked before.

• #26

  https://journals.lww.com/kidney360/fulltext/2023/10000/management_of_lupus_nephritis__new_treatments_and.23.aspx

  The 2011 Kidney Disease: Improving Global Outcomes (KDIGO) guidelines recommended first-line induction therapy for LN classes III and IV with either CYC or mycophenolate and maintenance therapy with AZA or MMF and low-dose oral corticosteroids. […] The role of calcineurin inhibitors (CNIs) in lupus has been largely in combination with other SOC therapy. […] Rituximab, a chimeric anti-CD20 monoclonal antibody, has been investigated in combination with MMF for induction treatment of proliferative LN. […] A 2018 Cochrane review concluded that MMF provides equivalent disease remission compared with IV CYC and may avoid drug-related toxicity, supporting its use as first-line induction therapy. […] On the basis of these studies, the 2019 EULAR/ERA-EDTA guidelines recommend treatment of active class III or IV LN with induction therapy consisting of either MMF/MPA or low-dose IV CYC as first-line agents alongside GCs.

• #27 Lupus and kidney disease (lupus nephritis) – Symptoms, treatment, & stages | National Kidney Foundation

  https://www.kidney.org/kidney-topics/lupus-nephritis

  Depending on your class/type of LN, your nephrologist will likely recommend a treatment plan that is right for you and will help keep your kidneys working well. These treatments can range from simple monitoring (watching and testing) to stronger medicines that help manage any symptoms and prevent more harm to your kidneys. […] Classes I II: Usually, no treatment is needed unless there is a large amount of protein in the urine (pee). If protein is in your pee, medicines like prednisone (a type of steroid) may be used. […] Active Classes III, IV V: Usually, the first phase (3 to 12 months) of treatment includes steroids and one or more medications like these: Mycophenolate mofetil (MMF), Mycophenolic acid (MPA), Cyclophosphamide, Belimumab (Benlysta), Calcineurin inhibitors, such as voclosporin (Lupkynis), tacrolimus, or cyclosporine, Rituximab (Class V only). […] Stable Classes III, IV, V: Usually, the first phase of treatment includes hydroxychloroquine and/or blood pressure medications. MMF or MPA may be recommended. […] Relapse (Symptoms return or get worse): Treat with the same medications that worked before.

• #28 New Treatment Regimens, New Drugs, and New Treatment Goals for Lupus Nephritis

  https://www.mdpi.com/2077-0383/14/2/584

  According to the EULAR 2023 guidelines, patients with active proliferative LN should be treated with low-dose intravenous cyclophosphamide or mycophenolate combined with glucocorticoids, including initial pulses of intravenous methylprednisolone followed by oral tapering. Combination therapy with belimumab or calcineurin inhibitors, such as voclosporin or tacrolimus, may be considered alongside mycophenolate or cyclophosphamide. Maintenance therapy should continue for at least three years, with mycophenolate or azathioprine replacing cyclophosphamide for those initially treated with it. For high-risk patients, characterized by reduced glomerular filtration rate (eGFR < 45 mL/min/1.73 m²), cellular crescents, fibrinoid necrosis, or severe interstitial inflammation, high-dose intravenous cyclophosphamide (NIH regimen) with pulse methylprednisolone may be appropriate.

• #29

  https://journals.lww.com/kidney360/fulltext/2023/10000/management_of_lupus_nephritis__new_treatments_and.23.aspx

  The 2011 Kidney Disease: Improving Global Outcomes (KDIGO) guidelines recommended first-line induction therapy for LN classes III and IV with either CYC or mycophenolate and maintenance therapy with AZA or MMF and low-dose oral corticosteroids. […] The role of calcineurin inhibitors (CNIs) in lupus has been largely in combination with other SOC therapy. […] Rituximab, a chimeric anti-CD20 monoclonal antibody, has been investigated in combination with MMF for induction treatment of proliferative LN. […] A 2018 Cochrane review concluded that MMF provides equivalent disease remission compared with IV CYC and may avoid drug-related toxicity, supporting its use as first-line induction therapy. […] On the basis of these studies, the 2019 EULAR/ERA-EDTA guidelines recommend treatment of active class III or IV LN with induction therapy consisting of either MMF/MPA or low-dose IV CYC as first-line agents alongside GCs.

• #30 New Treatment Regimens, New Drugs, and New Treatment Goals for Lupus Nephritis

  https://www.mdpi.com/2077-0383/14/2/584

  According to the EULAR 2023 guidelines, patients with active proliferative LN should be treated with low-dose intravenous cyclophosphamide or mycophenolate combined with glucocorticoids, including initial pulses of intravenous methylprednisolone followed by oral tapering. Combination therapy with belimumab or calcineurin inhibitors, such as voclosporin or tacrolimus, may be considered alongside mycophenolate or cyclophosphamide. Maintenance therapy should continue for at least three years, with mycophenolate or azathioprine replacing cyclophosphamide for those initially treated with it. For high-risk patients, characterized by reduced glomerular filtration rate (eGFR < 45 mL/min/1.73 m²), cellular crescents, fibrinoid necrosis, or severe interstitial inflammation, high-dose intravenous cyclophosphamide (NIH regimen) with pulse methylprednisolone may be appropriate.

• #31 Lupus Nephritis Guideline Encourages Triple Therapy – The Rheumatologist

  https://www.the-rheumatologist.org/article/lupus-nephritis-guideline-encourages-triple-therapy/

  Lupus nephritis requires extended treatment to decrease the risk of flares. Thus, it is conditionally recommended that patients who have achieved a complete renal response extend their treatment period to at least three to five years, whether they were receiving triple or dual therapy. […] A key element of the guideline is its emphasis on triple therapy for new onset or flaring lupus nephritis. In this context, triple therapy consists of glucocorticoids (i.e., high-dose, pulse intravenous [IV] glucocorticoids followed by an oral taper) given with two additional immunosuppressive therapies. […] Typically, these additional immunosuppressives should be: MMF or an analogue, such as mycophenolic acid plus belimumab; MMF with a calcineurin inhibitor (e.g., voclosporin, tacrolimus and cyclosporine); or low-dose cyclophosphamide as established in the EuroLupus Nephritis Trial plus belimumab, with MMF switched in after the initial course of cyclophosphamide is complete.

• #32 How Are the New Lupus Nephritis Guidelines Impacting Treatment? – Lupus Research Alliance

  https://www.lupusresearch.org/how-are-the-new-lupus-nephritis-guidelines-impacting-treatment/

  Recognized as National Kidney Month, March is the ideal time to learn more about the 2024 American College of Rheumatology (ACR) Guideline for the Screening, Treatment, and Management of Lupus Nephritis. Recently issued, the guidelines are designed to provide healthcare providers with up-to-date guidance on the care and treatment of adults and children with lupus nephritis the most common and severe manifestation of systemic lupus erythematosus (SLE). […] The new guidance represents the recent shift in lupus nephritis treatment that involves moving away from separate initial and maintenance therapy phases to a continuous combination or triple therapy approach. This new strategy targets multiple parts of the immune system at the same time, providing a more comprehensive and sustained treatment approach.

• #33 How Are the New Lupus Nephritis Guidelines Impacting Treatment? – Lupus Research Alliance

  https://www.lupusresearch.org/how-are-the-new-lupus-nephritis-guidelines-impacting-treatment/

  The primary goal is to safeguard kidney function and reduce the long-term health impacts and mortality rates associated with chronic kidney disease, while minimizing the side effects of the medications used. […] The most significant recommendation, and the recommendation that differentiates this guideline from all other current lupus nephritis guidelines, is that patients be considered for triple therapy right from the time of diagnosis. Triple therapy incorporates a background therapy with steroids and the immunosuppressant mycophenolate (which most patients with LN are currently taking), plus the addition of one of the more recently approved drugs for lupus nephritis either voclosporin or belimumab. […] This recommendation is based on clinical trials that showed more patients were able to achieve a complete kidney response with triple therapy than with steroids and mycophenolate alone.

• #34 Lupus Nephritis Guideline Encourages Triple Therapy – The Rheumatologist

  https://www.the-rheumatologist.org/article/lupus-nephritis-guideline-encourages-triple-therapy/

  Lupus nephritis requires extended treatment to decrease the risk of flares. Thus, it is conditionally recommended that patients who have achieved a complete renal response extend their treatment period to at least three to five years, whether they were receiving triple or dual therapy. […] A key element of the guideline is its emphasis on triple therapy for new onset or flaring lupus nephritis. In this context, triple therapy consists of glucocorticoids (i.e., high-dose, pulse intravenous [IV] glucocorticoids followed by an oral taper) given with two additional immunosuppressive therapies. […] Typically, these additional immunosuppressives should be: MMF or an analogue, such as mycophenolic acid plus belimumab; MMF with a calcineurin inhibitor (e.g., voclosporin, tacrolimus and cyclosporine); or low-dose cyclophosphamide as established in the EuroLupus Nephritis Trial plus belimumab, with MMF switched in after the initial course of cyclophosphamide is complete.

• #35 Lupus Nephritis Guideline Encourages Triple Therapy – The Rheumatologist

  https://www.the-rheumatologist.org/article/lupus-nephritis-guideline-encourages-triple-therapy/

  Another specific, conditional recommendation is for MMF plus a calcineurin inhibitor plus corticosteroids to be used if proteinuria is greater than 3g/g creatinine in class III, class IV nephritis or if over 1g/g creatinine in class V (membranous) nephritis, which is less common. […] In line with other recent ACR guidelines, this guideline attempts to limit doses of glucocorticoids and their consequent toxicities. Therefore, pulse IV glucocorticoids followed by low to moderate doses of oral glucocorticoids (i.e., 0.5 mg/kg/day with a max dose of 40 mg/day, tapering to a target dose of less than 5 mg/day by six months) are conditionally recommended. […] Dr. Sammaritano explained the preference for triple over dual therapy. She noted that the best current randomized controlled trials on the topic, such as the BLISS-LN study with belimumab and the AURORA trial with voclosporin, show improved outcomes with the addition of another immunosuppressant without greater adverse events.

• #36 Lupus Nephritis Guideline Encourages Triple Therapy – The Rheumatologist

  https://www.the-rheumatologist.org/article/lupus-nephritis-guideline-encourages-triple-therapy/

  Lupus nephritis requires extended treatment to decrease the risk of flares. Thus, it is conditionally recommended that patients who have achieved a complete renal response extend their treatment period to at least three to five years, whether they were receiving triple or dual therapy. […] A key element of the guideline is its emphasis on triple therapy for new onset or flaring lupus nephritis. In this context, triple therapy consists of glucocorticoids (i.e., high-dose, pulse intravenous [IV] glucocorticoids followed by an oral taper) given with two additional immunosuppressive therapies. […] Typically, these additional immunosuppressives should be: MMF or an analogue, such as mycophenolic acid plus belimumab; MMF with a calcineurin inhibitor (e.g., voclosporin, tacrolimus and cyclosporine); or low-dose cyclophosphamide as established in the EuroLupus Nephritis Trial plus belimumab, with MMF switched in after the initial course of cyclophosphamide is complete.

• #37 Lupus Nephritis Guidelines: Guidelines Summary

  https://emedicine.medscape.com/article/330369-guidelines

  Patients who improve after initial treatment should receive MMF/MPA (especially if it was the initial treatment) or azathioprine (preferred in women who may become pregnant) in combination with low-dose prednisone when needed to control disease activity. […] Gradual withdrawal of treatment (glucocorticoids first, then immunosuppressive drugs) can be attempted after at least 3 to 5 years therapy in patients with complete clinical response. […] HCQ should be continued long-term. […] Patients in whom initial therapy fails should be switched to one of the alternative initial therapies or to rituximab. […] In patients with active class III or IV LN, with or without a membranous component, treat initially with glucocorticoids plus either low-dose intravenous cyclophosphamide or a mycophenolic acid analogue (MPAA; ie, MMF or MPA).

• #38 Lupus Nephritis Treatment Strategies | The Journal of Rheumatology

  https://www.jrheum.org/content/51/6/549.full

  Lupus nephritis (LN) is present in approximately 25% to 50% of patients at the time of systemic lupus erythematosus diagnosis and eventually develops in up to 60% of adults and 80% of children. Over the past 5 decades, major advances in immunosuppressive medications for patients with severe LN (classes III, IV, and V alone or in combination) have resulted in significant improvements in patient survival. The most common approach for the treatment of severe LN is the use of sequential therapies, with an initial induction phase of an intensive immunosuppressive regimen aiming to achieve complete renal remission (CRR). This is followed by the maintenance phase, which uses an immunosuppressive regimen aiming to consolidate remission and reduce the risk of relapse, without increasing the risk of serious adverse events (SAEs). Currently, for the initial induction phase, the Kidney Disease: Improving Global Outcomes (KDIGO) recommends glucocorticoids (GCs) plus one of the following regimens: (1) mycophenolic acid (MPA) analog; (2) low-dose intravenous (IV) cyclophosphamide (CYC); (3) belimumab and either MPA analog or low-dose IV CYC; or (4) MPA analog and a calcineurin inhibitor (CNI). In clinical trials, triple-therapy regimens of either (1) GCs plus belimumab and MPA analog, or (2) GCs plus MPA analog and a CNI achieved the highest CRR rates (34-46%) without excessive risk of SAEs (7.2-26%) and mortality (0-3%). KDIGO also recommends for the maintenance phase that patients should be placed on an MPA analog as the first choice, because mycophenolate mofetil (MMF) was associated with the lowest risk of relapse (13-19%) without excessive risk of SAEs (15-24%) and mortality (0-4%) compared to azathioprine (AZA) in recently completed clinical trials in patients with severe LN treated and followed for 3 to 4 years. KDIGO recommends maintenance with AZA as an alternative to MPA analogs in patients considering pregnancy or in those with intolerance or lack of access to MPA analogs.

• #39

  https://journals.lww.com/kidney360/fulltext/2023/10000/management_of_lupus_nephritis__new_treatments_and.23.aspx

  For maintenance therapy, the task force recommends MMF in those who received MMF for induction and either MMF or AZA in those who received IV CYC for induction. […] Similarly, the 2021 KDIGO glomerular disease guidelines recommend that in patients with active class III or IV LN, either low-dose IV CYC or MMF in conjunction with GCs is used for induction therapy. […] Belimumab is a monoclonal antibody that inhibits soluble human B-lymphocyte stimulator and was approved in 2011 for use in active SLE without severe renal or central nervous system involvement. […] This was followed by approval in December 2020 for the treatment of adults with active LN receiving standard therapy, representing the first FDA-approved therapy for active LN. […] Voclosporin received FDA approval in January 2021 on the basis of the phase 3 AURORA-1 trial, representing the first oral therapy approved for LN.

• #40 Lupus Nephritis Guidelines: Guidelines Summary

  https://emedicine.medscape.com/article/330369-guidelines

  Patients who improve after initial treatment should receive MMF/MPA (especially if it was the initial treatment) or azathioprine (preferred in women who may become pregnant) in combination with low-dose prednisone when needed to control disease activity. […] Gradual withdrawal of treatment (glucocorticoids first, then immunosuppressive drugs) can be attempted after at least 3 to 5 years therapy in patients with complete clinical response. […] HCQ should be continued long-term. […] Patients in whom initial therapy fails should be switched to one of the alternative initial therapies or to rituximab. […] In patients with active class III or IV LN, with or without a membranous component, treat initially with glucocorticoids plus either low-dose intravenous cyclophosphamide or a mycophenolic acid analogue (MPAA; ie, MMF or MPA).

• #41 Lupus Nephritis Guidelines: Guidelines Summary

  https://emedicine.medscape.com/article/330369-guidelines

  Patients who improve after initial treatment should receive MMF/MPA (especially if it was the initial treatment) or azathioprine (preferred in women who may become pregnant) in combination with low-dose prednisone when needed to control disease activity. […] Gradual withdrawal of treatment (glucocorticoids first, then immunosuppressive drugs) can be attempted after at least 3 to 5 years therapy in patients with complete clinical response. […] HCQ should be continued long-term. […] Patients in whom initial therapy fails should be switched to one of the alternative initial therapies or to rituximab. […] In patients with active class III or IV LN, with or without a membranous component, treat initially with glucocorticoids plus either low-dose intravenous cyclophosphamide or a mycophenolic acid analogue (MPAA; ie, MMF or MPA).

• #42 Management of Lupus Nephritis: New Treatments and Updated Guidelines

  https://pmc.ncbi.nlm.nih.gov/articles/PMC10617804/

  Belimumab is a monoclonal antibody that inhibits soluble human B-lymphocyte stimulator and was approved in 2011 for use in active SLE without severe renal or central nervous system involvement. […] In the 2-year phase 3 Belimumab International Study in Lupus Nephritis (BLISS-LN), patients with active class III or IV LN were randomized to receive IV belimumab 10 mg/kg or placebo on a background of SOC therapy. […] Voclosporin received FDA approval in January 2021 on the basis of the phase 3 AURORA-1 trial, representing the first oral therapy approved for LN. […] The results of these studies also highlight the increasing use of reduced doses of GCs in the management of LN. […] Both the 2019 EULAR/ERA-EDTA and 2021 KDIGO guidelines recommend that a regimen of reduced dose GCs after a short course of IV methylprednisolone pulses be considered during the initial treatment of active LN.

• #43

  https://journals.lww.com/kidney360/fulltext/2023/10000/management_of_lupus_nephritis__new_treatments_and.23.aspx

  For maintenance therapy, the task force recommends MMF in those who received MMF for induction and either MMF or AZA in those who received IV CYC for induction. […] Similarly, the 2021 KDIGO glomerular disease guidelines recommend that in patients with active class III or IV LN, either low-dose IV CYC or MMF in conjunction with GCs is used for induction therapy. […] Belimumab is a monoclonal antibody that inhibits soluble human B-lymphocyte stimulator and was approved in 2011 for use in active SLE without severe renal or central nervous system involvement. […] This was followed by approval in December 2020 for the treatment of adults with active LN receiving standard therapy, representing the first FDA-approved therapy for active LN. […] Voclosporin received FDA approval in January 2021 on the basis of the phase 3 AURORA-1 trial, representing the first oral therapy approved for LN.

• #44 Management of Lupus Nephritis: New Treatments and Updated Guidelines

  https://pmc.ncbi.nlm.nih.gov/articles/PMC10617804/

  Belimumab is a monoclonal antibody that inhibits soluble human B-lymphocyte stimulator and was approved in 2011 for use in active SLE without severe renal or central nervous system involvement. […] In the 2-year phase 3 Belimumab International Study in Lupus Nephritis (BLISS-LN), patients with active class III or IV LN were randomized to receive IV belimumab 10 mg/kg or placebo on a background of SOC therapy. […] Voclosporin received FDA approval in January 2021 on the basis of the phase 3 AURORA-1 trial, representing the first oral therapy approved for LN. […] The results of these studies also highlight the increasing use of reduced doses of GCs in the management of LN. […] Both the 2019 EULAR/ERA-EDTA and 2021 KDIGO guidelines recommend that a regimen of reduced dose GCs after a short course of IV methylprednisolone pulses be considered during the initial treatment of active LN.

• #45

  https://link.springer.com/article/10.1007/s40744-025-00752-y

  Based on the available literature, cyclophosphamide is the only treatment for LN that currently satisfies the confirmed disease modification criteria. […] Belimumab met more criteria across the three time points than any other biologic treatment, but lacked sufficient data beyond year 5 to confirm disease modification. […] All LN treatments had data indicating disease modification potential, and these will need formal evaluation in longer-term studies to confirm their disease modification status and to aid informed treatment decisions by physicians and patients. […] The concept of disease modification in SLE and LN is based on the ability of a therapy to impact disease activity and outcomes, indicating a modification of the natural course of the disease. […] The following working definition of kidney disease modification in LN was proposed: minimizing disease activity with the fewest treatment-associated toxicities and slowing or preventing the progression to ESKD.

• #46

  https://link.springer.com/article/10.1007/s40744-025-00752-y

  To demonstrate kidney disease modification potential during years 25, at least one of the following criteria was required to be fulfilled: (1) sustained improvement in uPCR; (2) reduced decline in eGFR (i.e., decline no greater than 30% from baseline); (3) reduction of kidney flare rates; and 4) continued reduction in glucocorticoids and/or immunosuppressants. […] Confirmed disease modification in LN at 5 years was defined as slowing or preventing organ damage assessed by total SDI, ESKD, and/or doubling of serum creatinine to indicate nephroprotection, per the proposed criteria. […] Although there are no 5-year SDI, ESKD or serum creatinine data available in LN for belimumab, the authors note that the positive 5-year SDI data for belimumab in SLE, as well as belimumab having met seven of the eight LN disease modification criteria at years 1-5, indicate that belimumab is likely to also have disease modification potential beyond year 5 in LN.

• #47 Management of Lupus Nephritis: New Treatments and Updated Guidelines

  https://pmc.ncbi.nlm.nih.gov/articles/PMC10617804/

  Belimumab is a monoclonal antibody that inhibits soluble human B-lymphocyte stimulator and was approved in 2011 for use in active SLE without severe renal or central nervous system involvement. […] In the 2-year phase 3 Belimumab International Study in Lupus Nephritis (BLISS-LN), patients with active class III or IV LN were randomized to receive IV belimumab 10 mg/kg or placebo on a background of SOC therapy. […] Voclosporin received FDA approval in January 2021 on the basis of the phase 3 AURORA-1 trial, representing the first oral therapy approved for LN. […] The results of these studies also highlight the increasing use of reduced doses of GCs in the management of LN. […] Both the 2019 EULAR/ERA-EDTA and 2021 KDIGO guidelines recommend that a regimen of reduced dose GCs after a short course of IV methylprednisolone pulses be considered during the initial treatment of active LN.

• #48 First drugs ever approved for lupus nephritis | Ohio State Medical Center

  https://wexnermedical.osu.edu/departments/innovations/nephrology/lupus-nephritis-drugs

  RovinPhysicians have new treatment options for patients with lupus nephritis. Investigators at The Ohio State University Wexner Medical Center helped lead clinical trials that resulted in approval of two new drugsthe first drugs ever approved for lupus nephritis by the Food and Drug Administration. […] Other than steroids, there had been no drugs approved specifically for lupus nephritisever, says Brad Rovin, MD, director of the Division of Nephrology and medical director of the Center for Clinical Research Management at the Ohio State Wexner Medical Center. […] In December 2020, belimumab (Benlysta), which was previously approved for the treatment of systemic lupus erythematosus, was approved for lupus nephritis. In January 2021, voclosporin (Lupkynis), a calcineurin inhibitor, was approved as the first oral drug for lupus nephritis.

• #49 Lupus Nephritis Treatment & Management: Approach Considerations, Pharmacotherapy for Lupus Nephritis Based on Stage, New Therapies

  https://emedicine.medscape.com/article/330369-treatment

  Belimumab (Benlysta) is an antiB-lymphocyte stimulator monoclonal antibody. It is approved by the US Food and Drug Administration (FDA) to treat adults with active lupus nephritis who are receiving standard therapy. […] Voclosporin (Lupkynis), a calcineurin inhibitor, is the first FDA-approved oral therapy used in combination with immunosuppressive therapy for lupus nephritis. Approval was based on data from 2 clinical trials, the phase III AURORA trial and the phase II AURA-LV trial. […] Patients with ESRD require dialysis and are good candidates for kidney transplantation. Patients with ESRD secondary to SLE represent 1.5% of all patients on dialysis in the United States. The survival rate among patients on dialysis is fair (5-year survival rate, 60-70%) and is comparable with that among patients on dialysis who do not have SLE.

• #50 Management of Lupus Nephritis: New Treatments and Updated Guidelines

  https://pmc.ncbi.nlm.nih.gov/articles/PMC10617804/

  Belimumab is a monoclonal antibody that inhibits soluble human B-lymphocyte stimulator and was approved in 2011 for use in active SLE without severe renal or central nervous system involvement. […] In the 2-year phase 3 Belimumab International Study in Lupus Nephritis (BLISS-LN), patients with active class III or IV LN were randomized to receive IV belimumab 10 mg/kg or placebo on a background of SOC therapy. […] Voclosporin received FDA approval in January 2021 on the basis of the phase 3 AURORA-1 trial, representing the first oral therapy approved for LN. […] The results of these studies also highlight the increasing use of reduced doses of GCs in the management of LN. […] Both the 2019 EULAR/ERA-EDTA and 2021 KDIGO guidelines recommend that a regimen of reduced dose GCs after a short course of IV methylprednisolone pulses be considered during the initial treatment of active LN.

• #51 New Treatment Regimens, New Drugs, and New Treatment Goals for Lupus Nephritis

  https://www.mdpi.com/2077-0383/14/2/584

  According to the 2024 KDIGO guidelines, for the initial treatment of active Class III or IV LN, with or without a membranous component, glucocorticoids are recommended alongside one of the following: mycophenolic acid analogs (MPAA), low-dose intravenous cyclophosphamide, a combination of belimumab with MPAA or cyclophosphamide, or MPAA with a calcineurin inhibitor (CNI) for patients with preserved kidney function (eGFR > 45 mL/min/1.73 m²). […] The so-called multitarget treatment involves a combination of tacrolimus, MMF, and prednisone. […] Belimumab is a fully human recombinant monoclonal antibody of the IgG1 lambda (IgG1λ) isotype that targets and inhibits the soluble B-lymphocyte stimulator (BLyS), also known as B-cell activating factor (BAFF). […] Voclosporin, an oral calcineurin inhibitor (CNI) derived from cyclosporine, features a cyclic undecapeptide structure with a single carbon extension at amino acid-1, enhancing its efficacy, metabolic stability, and safety while eliminating the need for therapeutic drug monitoring.

• #52 Management of Lupus Nephritis: New Treatments and Updated Guidelines

  https://pmc.ncbi.nlm.nih.gov/articles/PMC10617804/

  Multiple immune pathways are implicated in LN, allowing for different treatment targets for emerging therapies. […] The AURORA-2 continuation study was designed to assess long-term safety and tolerability of the combination of voclosporin, MMF, and low-dose GCs over an additional 2 years of treatment after completion of AURORA-1. […] The identification of downstream urinary or serum biomarkers of specific pathways may permit a precision medicine approach targeting therapeutic agents to the individual. […] Concerns remain regarding the cost-effectiveness of lupus therapy. […] In summary, LN outcomes have improved over recent decades, but there is a great need for safer and more effective therapies.

• #53 Lupus Nephritis Treatment & Management: Approach Considerations, Pharmacotherapy for Lupus Nephritis Based on Stage, New Therapies

  https://emedicine.medscape.com/article/330369-treatment

  Calcineurin inhibitors, especially tacrolimus, have demonstrated benefit in lupus nephritis. However, most studies have been limited to Asian patients, and further research is required on long-term benefits and disadvantages. The calcineurin inhibitor voclosporin is the first oral therapy approved by the US Food and Drug Administration (FDA) for lupus nephritis in conjunction with immunosuppressive treatment. […] Leflunomide, a pyrimidine synthesis inhibitor that is approved by the FDA for use in rheumatoid arthritis, has shown efficacy in proliferative lupus nephritis in Chinese patients. More recent evidence indicates that leflunomide may also have some efficacy in lupus nephritis in patients of other ethnic groups. […] Treat hypertension aggressively. On the basis of beneficial effects in other nephropathies, angiotensin-converting enzyme inhibitors (ACEIs) and angiotensin II receptor blockers (ARBs) have been routinely used to treat proteinuria in lupus nephritis.

• #54 Treatment of Lupus Nephritis: Practical Insights – GlomCon (pubs)

  https://pubs.glomcon.org/treatment-of-lupus-nephritis-practical-insights/

  In a recent study, biopsy-guided maintenance immunosuppression was shown to be informative in identifying patients whose immunosuppressive therapy can be safely withdrawn. […] For rare cases of refractory LN, the following treatment options may be considered: switch between MMF and CYC, rituximab as an add-on or alternative therapy, a combined calcineurin inhibitor (CNI) multitarget therapy, prolonged courses of IV CYC, or enrollment in clinical trials. […] Calcineurin inhibitors are currently being used as an alternative therapy for lupus nephritis, especially in those with a membranous component (i.e., class V ± III/IV). […] B cell-depleting agents are also being used in the management of LN. Although rituximab failed to show an increase in renal response rates in the LUNAR study, belimumab added to standard therapy was shown in BLISS-LN to have a better primary efficacy renal response in patients with active LN than standard therapy alone.

• #55 Lupus Nephritis | Diagnosis & Disease Information

  https://www.rheumatologyadvisor.com/ddi/lupus-nephritis/

  The Table summarizes the 2024 KDIGO lupus nephritis treatment recommendations. […] According to the ACR guidelines, mycophenolate mofetil 2 to 3 grams orally per day for induction therapy of Class III/IV/V lupus nephritis is recommended. […] For maintenance dosing, mycophenolate mofetil 750 mg to 1000 mg orally twice daily or mycophenolic acid 540 mg to 720 mg twice daily is recommended, according to the KDIGO guidelines. […] Cyclophosphamide is an immunosuppressant that may be used as initial therapy for Class III/IV lupus nephritis in patients who may have difficulty adhering to an oral therapy. […] Belimumab is a human monoclonal antibody that acts as a B-lymphocyte stimulator (BLyS)-specific inhibitor. It is approved by the US Food and Drug Administration for the treatment of SLE and active lupus nephritis in people age 5 years and older. […] According to the KDIGO guidelines, a CNI-containing regimen may be preferred for some patients, including those who have preserved kidney function; proteinuria caused by podocyte injury; contraindications to cyclophosphamide-based regimens; or who cant tolerate standard-dose MPAA.

• #56 Update on available therapies for refractory lupus nephritis | Revista Colombiana de Reumatología (English Edition)

  https://www.elsevier.es/en-revista-revista-colombiana-reumatologia-english-edition–474-articulo-update-on-available-therapies-for-S2444440523000912

  Update on available therapies for refractory lupus nephritis […] Currently, standard immunosuppression treatment (cyclophosphamide or mycophenolate) has managed to significantly preserve kidney function and improve survival. […] Unfortunately, a subset of patients with LN do not respond to initial immunosuppression treatment and is defined as refractory, constituting a great challenge given the scarce evidence of alternative therapies based on controlled clinical trials and that sometimes obtain unfavorable results. […] We also provide an update on the available therapies for refractory LN. […] The therapeutic approach for patients who are considered resistant to initial therapy, that is, those with clinical or histological evidence of active nephritis, varies with the drug used for induction and the severity of the disease.

• #57 Lupus Nephritis Guidelines: Guidelines Summary

  https://emedicine.medscape.com/article/330369-guidelines

  Patients who improve after initial treatment should receive MMF/MPA (especially if it was the initial treatment) or azathioprine (preferred in women who may become pregnant) in combination with low-dose prednisone when needed to control disease activity. […] Gradual withdrawal of treatment (glucocorticoids first, then immunosuppressive drugs) can be attempted after at least 3 to 5 years therapy in patients with complete clinical response. […] HCQ should be continued long-term. […] Patients in whom initial therapy fails should be switched to one of the alternative initial therapies or to rituximab. […] In patients with active class III or IV LN, with or without a membranous component, treat initially with glucocorticoids plus either low-dose intravenous cyclophosphamide or a mycophenolic acid analogue (MPAA; ie, MMF or MPA).

• #58

  https://journals.lww.com/kidney360/fulltext/2023/10000/management_of_lupus_nephritis__new_treatments_and.23.aspx

  The results of these studies also highlight the increasing use of reduced doses of GCs in the management of LN. […] Both the 2019 EULAR/ERA-EDTA and 2021 KDIGO guidelines recommend that a regimen of reduced dose GCs after a short course of IV methylprednisolone pulses be considered during the initial treatment of active LN. […] The traditional therapeutic approach to a suboptimal response to induction therapy with MMF and GCs has been to switch to a CYC-based induction regimen. […] With the success of CNIs or belimumab in combination with MMF as induction agents, these are now commonly used as additive therapies when the initial response is suboptimal. […] The addition of rituximab to MMF has been widely used for refractory LN and is supported by multiple observational and uncontrolled studies. […] In summary, LN outcomes have improved over recent decades, but there is a great need for safer and more effective therapies.

• #59 Update on available therapies for refractory lupus nephritis | Revista Colombiana de Reumatología (English Edition)

  https://www.elsevier.es/en-revista-revista-colombiana-reumatologia-english-edition–474-articulo-update-on-available-therapies-for-S2444440523000912

  Those who do not achieve remission after 6 months with cyclophosphamide are switched to mycophenolate, or vice versa. […] Other therapeutic options such as calcineurin inhibitors, plasmapheresis or mesenchymal stem cell transplantation have been proposed, and a case report informs a favorable outcome of CAR-T cells targeting CD19. […] Rituximab has been considered one of the first treatment alternatives when there is therapeutic failure with mycophenolate and cyclophosphamide. […] According to a meta-analysis, the mean follow-up of the patients has been 60 weeks, and rituximab has achieved a surprising overall response rate of 74% in refractory cases. […] Belimumab has been demonstrated that an increase in BAFF is associated with an early relapse of the disease. […] The evidence for calcineurin inhibitors has also changed significantly in terms of their efficacy and safety in refractory LN.

• #60 Treatment of Lupus Nephritis: Practical Insights – GlomCon (pubs)

  https://pubs.glomcon.org/treatment-of-lupus-nephritis-practical-insights/

  In a recent study, biopsy-guided maintenance immunosuppression was shown to be informative in identifying patients whose immunosuppressive therapy can be safely withdrawn. […] For rare cases of refractory LN, the following treatment options may be considered: switch between MMF and CYC, rituximab as an add-on or alternative therapy, a combined calcineurin inhibitor (CNI) multitarget therapy, prolonged courses of IV CYC, or enrollment in clinical trials. […] Calcineurin inhibitors are currently being used as an alternative therapy for lupus nephritis, especially in those with a membranous component (i.e., class V ± III/IV). […] B cell-depleting agents are also being used in the management of LN. Although rituximab failed to show an increase in renal response rates in the LUNAR study, belimumab added to standard therapy was shown in BLISS-LN to have a better primary efficacy renal response in patients with active LN than standard therapy alone.

• #61 Update on available therapies for refractory lupus nephritis | Revista Colombiana de Reumatología (English Edition)

  https://www.elsevier.es/en-revista-revista-colombiana-reumatologia-english-edition–474-articulo-update-on-available-therapies-for-S2444440523000912

  Those who do not achieve remission after 6 months with cyclophosphamide are switched to mycophenolate, or vice versa. […] Other therapeutic options such as calcineurin inhibitors, plasmapheresis or mesenchymal stem cell transplantation have been proposed, and a case report informs a favorable outcome of CAR-T cells targeting CD19. […] Rituximab has been considered one of the first treatment alternatives when there is therapeutic failure with mycophenolate and cyclophosphamide. […] According to a meta-analysis, the mean follow-up of the patients has been 60 weeks, and rituximab has achieved a surprising overall response rate of 74% in refractory cases. […] Belimumab has been demonstrated that an increase in BAFF is associated with an early relapse of the disease. […] The evidence for calcineurin inhibitors has also changed significantly in terms of their efficacy and safety in refractory LN.

• #62 Rituximab therapy for lupus nephritis: A meta‑analysis

  https://www.spandidos-publications.com/10.3892/wasj.2021.119

  The aim of the present study was to assess the clinical efficacy and safety of rituximab (RTX) therapy in the treatment of lupus nephritis by performing a metaanalysis. […] The findings of the present study demonstrate that RTX exhibits favorable clinical efficacy in the treatment of lupus nephritis, which can significantly reduce the level of proteinuria and SLEDAI, and increase the level of serum albumin. Compared with traditional immunosuppressive therapy (corticosteroids + cyclophosphamide and/or mycophenolate), RTX was more effective in the treatment of lupus nephritis. Additionally, rituximab exhibited good safety. […] The present study aimed to assess the clinical efficacy and safety of RTX as a novel immunosuppressive treatment for LN by performing a meta-analysis of the available literature.

• #63

  https://journals.lww.com/kidney360/fulltext/2023/10000/management_of_lupus_nephritis__new_treatments_and.23.aspx

  The results of these studies also highlight the increasing use of reduced doses of GCs in the management of LN. […] Both the 2019 EULAR/ERA-EDTA and 2021 KDIGO guidelines recommend that a regimen of reduced dose GCs after a short course of IV methylprednisolone pulses be considered during the initial treatment of active LN. […] The traditional therapeutic approach to a suboptimal response to induction therapy with MMF and GCs has been to switch to a CYC-based induction regimen. […] With the success of CNIs or belimumab in combination with MMF as induction agents, these are now commonly used as additive therapies when the initial response is suboptimal. […] The addition of rituximab to MMF has been widely used for refractory LN and is supported by multiple observational and uncontrolled studies. […] In summary, LN outcomes have improved over recent decades, but there is a great need for safer and more effective therapies.

• #64 From sequential to combination and personalised therapy in lupus nephritis: moving towards a paradigm shift? | Annals of the Rheumatic Diseases

  https://ard.bmj.com/content/81/1/15

  The current treatment paradigm in lupus nephritis consists of an initial phase aimed at inducing remission and a subsequent remission maintenance phase. […] With this so-called sequential treatment approach, complete renal response is achieved in a disappointing proportion of 20-30% of the patients within 6-12 months, and 5-20% develop end-stage kidney disease within 10 years. […] In this viewpoint, we discuss the pros and cons of voclosporin and belimumab as add-on agents to standard therapy, the first drugs to be licenced for lupus nephritis after recent successful randomised phase III clinical trials. […] Undoubtably, the treatment landscape in lupus nephritis is changing, with combination treatment regimens challenging the sequential concept. […] After many years of trial failures, we may now anticipate a heartening future for patients with lupus nephritis.

• #65 Lupus Nephritis Treatment & Management: Approach Considerations, Pharmacotherapy for Lupus Nephritis Based on Stage, New Therapies

  https://emedicine.medscape.com/article/330369-treatment

  Belimumab (Benlysta) is an antiB-lymphocyte stimulator monoclonal antibody. It is approved by the US Food and Drug Administration (FDA) to treat adults with active lupus nephritis who are receiving standard therapy. […] Voclosporin (Lupkynis), a calcineurin inhibitor, is the first FDA-approved oral therapy used in combination with immunosuppressive therapy for lupus nephritis. Approval was based on data from 2 clinical trials, the phase III AURORA trial and the phase II AURA-LV trial. […] Patients with ESRD require dialysis and are good candidates for kidney transplantation. Patients with ESRD secondary to SLE represent 1.5% of all patients on dialysis in the United States. The survival rate among patients on dialysis is fair (5-year survival rate, 60-70%) and is comparable with that among patients on dialysis who do not have SLE.

• #66 Lupus Nephritis Treatment & Management: Approach Considerations, Pharmacotherapy for Lupus Nephritis Based on Stage, New Therapies

  https://emedicine.medscape.com/article/330369-treatment

  Patients with SLE account for 3% of all kidney transplantations in the United States. It is important to ensure that the patient does not have active SLE disease at the time of transplantation. A 3-month period of dialysis is usually prudent in the event of spontaneous renal recovery. […] Substantial evidence shows that patients with SLE fare worse than patients without SLE in terms of graft survival. Living-related allografts show better outcomes than cadaveric allografts. In patients with SLE, reasons for a more severe outcome after transplantation include recurrent lupus nephritis and concomitant antiphospholipid antibody syndrome resulting in allograft loss.

• #67 Lupus Nephritis Treatment & Management: Approach Considerations, Pharmacotherapy for Lupus Nephritis Based on Stage, New Therapies

  https://emedicine.medscape.com/article/330369-treatment

  Belimumab (Benlysta) is an antiB-lymphocyte stimulator monoclonal antibody. It is approved by the US Food and Drug Administration (FDA) to treat adults with active lupus nephritis who are receiving standard therapy. […] Voclosporin (Lupkynis), a calcineurin inhibitor, is the first FDA-approved oral therapy used in combination with immunosuppressive therapy for lupus nephritis. Approval was based on data from 2 clinical trials, the phase III AURORA trial and the phase II AURA-LV trial. […] Patients with ESRD require dialysis and are good candidates for kidney transplantation. Patients with ESRD secondary to SLE represent 1.5% of all patients on dialysis in the United States. The survival rate among patients on dialysis is fair (5-year survival rate, 60-70%) and is comparable with that among patients on dialysis who do not have SLE.

• #68 Lupus Nephritis Treatment & Management: Approach Considerations, Pharmacotherapy for Lupus Nephritis Based on Stage, New Therapies

  https://emedicine.medscape.com/article/330369-treatment

  Patients with SLE account for 3% of all kidney transplantations in the United States. It is important to ensure that the patient does not have active SLE disease at the time of transplantation. A 3-month period of dialysis is usually prudent in the event of spontaneous renal recovery. […] Substantial evidence shows that patients with SLE fare worse than patients without SLE in terms of graft survival. Living-related allografts show better outcomes than cadaveric allografts. In patients with SLE, reasons for a more severe outcome after transplantation include recurrent lupus nephritis and concomitant antiphospholipid antibody syndrome resulting in allograft loss.

• #69 New ACR Guideline Summary: Lupus Nephritis

  https://rheumatology.org/press-releases/new-acr-guideline-summary-provides-guidance-to-screen-treat-and-manage-lupus-nephritis

  Today, the American College of Rheumatology (ACR) released a summary of the 2024 ACR Guideline for the Screening, Treatment, and Management of Lupus Nephritis during ACR Convergence 2024. This is the Colleges first lupus nephritis guideline since 2012 and provides evidence-based, expert guidance for the condition in adults and children. […] The goal of lupus nephritis treatments is to preserve kidney function and reduce morbidity and mortality of chronic kidney disease while minimizing medication-related toxicities. […] Key recommendations include: […] Conditional recommendation for a triple immunosuppressive regimen in patients with active Class III IV lupus nephritis: Triple therapy for Class III/IV lupus nephritis includes glucocorticoid, and one of three immunosuppressive combination regimens: mycophenolate plus belimumab, mycophenolate plus calcineurin inhibitor therapy, or low dose cyclophosphamide plus belimumab.

• #70 Lupus Nephritis – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK499817/

  The 2019 EULAR guidelines recommend initiating treatment with MMF (2-3 g/d or equivalent mycophenolic acid) or cyclophosphamide (500 mg for 6 biweekly doses), combined with 3 days of intravenous glucocorticoids, followed by a prednisone taper to the lowest dose. […] Cyclophosphamide is the preferred treatment for life-threatening complications of SLE, such as pulmonary involvement or rapidly progressive lupus nephritis. […] Immunosuppressive therapy should be considered alongside hydroxychloroquine for patients with proteinuria exceeding 1 g/d. […] If clinical improvement is achieved, maintenance therapy is initiated with either a reduced dose of MMF or azathioprine. […] Patients with ESRD due to lupus nephritis who initiate dialysis experience outcomes similar to those of dialysis patients without lupus nephritis. […] The risk of recurrent lupus pathology in the renal allograft is estimated at 2% to 11%, with a median follow-up of 4 years.

• #71 Lupus Nephritis Guidelines: Guidelines Summary

  https://emedicine.medscape.com/article/330369-guidelines

  An MPAA-based regimen is the preferred initial therapy of proliferative LN for patients at high risk of infertility; patients who have a moderate to high prior cyclophosphamide exposure; and patients of Asian, Hispanic, or African ancestry. […] After completion of initial therapy for class III-IV LN, patients should be placed on MPAA for maintenance. […] The total duration of initial immunosuppression plus combination maintenance immunosuppression for proliferative LN should not be 36 months. […] For class V LN, the guidelines provide algorithms for management, based on proteinuria level, and on treatment of patients whose response to therapy is deemed unsatisfactory. […] Patients with LN who develop kidney failure may be treated with hemodialysis, peritoneal dialysis, or kidney transplantation; however, kidney transplantation is preferred over long-term dialysis.

• #72 Management of Lupus Nephritis: New Treatments and Updated Guidelines

  https://pmc.ncbi.nlm.nih.gov/articles/PMC10617804/

  Belimumab is a monoclonal antibody that inhibits soluble human B-lymphocyte stimulator and was approved in 2011 for use in active SLE without severe renal or central nervous system involvement. […] In the 2-year phase 3 Belimumab International Study in Lupus Nephritis (BLISS-LN), patients with active class III or IV LN were randomized to receive IV belimumab 10 mg/kg or placebo on a background of SOC therapy. […] Voclosporin received FDA approval in January 2021 on the basis of the phase 3 AURORA-1 trial, representing the first oral therapy approved for LN. […] The results of these studies also highlight the increasing use of reduced doses of GCs in the management of LN. […] Both the 2019 EULAR/ERA-EDTA and 2021 KDIGO guidelines recommend that a regimen of reduced dose GCs after a short course of IV methylprednisolone pulses be considered during the initial treatment of active LN.

• #73

  https://journals.lww.com/kidney360/fulltext/2023/10000/management_of_lupus_nephritis__new_treatments_and.23.aspx

  The results of these studies also highlight the increasing use of reduced doses of GCs in the management of LN. […] Both the 2019 EULAR/ERA-EDTA and 2021 KDIGO guidelines recommend that a regimen of reduced dose GCs after a short course of IV methylprednisolone pulses be considered during the initial treatment of active LN. […] The traditional therapeutic approach to a suboptimal response to induction therapy with MMF and GCs has been to switch to a CYC-based induction regimen. […] With the success of CNIs or belimumab in combination with MMF as induction agents, these are now commonly used as additive therapies when the initial response is suboptimal. […] The addition of rituximab to MMF has been widely used for refractory LN and is supported by multiple observational and uncontrolled studies. […] In summary, LN outcomes have improved over recent decades, but there is a great need for safer and more effective therapies.

• #74 Lupus Nephritis Guideline Encourages Triple Therapy – The Rheumatologist

  https://www.the-rheumatologist.org/article/lupus-nephritis-guideline-encourages-triple-therapy/

  Another specific, conditional recommendation is for MMF plus a calcineurin inhibitor plus corticosteroids to be used if proteinuria is greater than 3g/g creatinine in class III, class IV nephritis or if over 1g/g creatinine in class V (membranous) nephritis, which is less common. […] In line with other recent ACR guidelines, this guideline attempts to limit doses of glucocorticoids and their consequent toxicities. Therefore, pulse IV glucocorticoids followed by low to moderate doses of oral glucocorticoids (i.e., 0.5 mg/kg/day with a max dose of 40 mg/day, tapering to a target dose of less than 5 mg/day by six months) are conditionally recommended. […] Dr. Sammaritano explained the preference for triple over dual therapy. She noted that the best current randomized controlled trials on the topic, such as the BLISS-LN study with belimumab and the AURORA trial with voclosporin, show improved outcomes with the addition of another immunosuppressant without greater adverse events.

• #75 New ACR Guideline Summary: Lupus Nephritis

  https://rheumatology.org/press-releases/new-acr-guideline-summary-provides-guidance-to-screen-treat-and-manage-lupus-nephritis

  Conditional recommendation for treatment with a specific triple therapy as the most desirable therapy for pure Class V lupus nephritis with proteinuria greater than 1 g/g: Triple therapy for Class V lupus nephritis includes glucocorticoid, mycophenolate, and calcineurin inhibitor therapy. […] Conditional recommendation for a lower dose glucocorticoid regimen (after initial intravenous pulse therapy) to minimize toxicity, starting at 0.5 mg/kg/day prednisone (maximum of 40 mg daily) with taper to a prednisone goal of less than or equal to 5 mg/day by six months of treatment. […] Guideline recommendations are based on systematic evidence reviews, values and preferences from a lupus nephritis patient panel, and input from adult and pediatric rheumatologists and nephrologists and a rheumatology physician assistant. The recommendations are intended to promote optimal outcomes for the most commonly encountered lupus nephritis scenarios. The guideline includes therapies available in the United States as of fall 2024 and applies to lupus nephritis in adults, children, and adolescents. This is the first time the ACR has included children and adolescents in their lupus treatment guidelines.

• #76 Lupus Nephritis Guidelines: Guidelines Summary

  https://emedicine.medscape.com/article/330369-guidelines

  Maintain blood pressure at or below 130/80 mm Hg. […] Initial treatment for class III-IV LN: Mycophenolate mofetil (MMF) or low-dose IV cyclophosphamide plus glucocorticoids. […] Alternate option for class III-IV LN: Combination therapy of MMF with a calcineurin inhibitor (CNI), especially tacrolimus, particularly in patients with nephrotic-range proteinuria. […] To reduce cumulative glucocorticoid dose: Intravenous pulses of methylprednisolone (total dose 500-2500mg, depending on disease severity), followed by oral prednisone (0.3-0.5mg/kg/day) for up to 4 weeks, tapered to 7.5mg/day by 3 to 6 months. […] Initial treatment for pure class V disease with nephrotic-range proteinuria: MMF in combination with methylprednisolone followed by oral prednisone. […] In all LN patients: Hydroxychloroquine (HCQ) should be coadministered at a dose not to exceed 5mg/kg/day and adjusted for the GFR, in the absence of contraindications.

• #77 New ACR Guideline Summary: Lupus Nephritis

  https://rheumatology.org/press-releases/new-acr-guideline-summary-provides-guidance-to-screen-treat-and-manage-lupus-nephritis

  Conditional recommendation for treatment with a specific triple therapy as the most desirable therapy for pure Class V lupus nephritis with proteinuria greater than 1 g/g: Triple therapy for Class V lupus nephritis includes glucocorticoid, mycophenolate, and calcineurin inhibitor therapy. […] Conditional recommendation for a lower dose glucocorticoid regimen (after initial intravenous pulse therapy) to minimize toxicity, starting at 0.5 mg/kg/day prednisone (maximum of 40 mg daily) with taper to a prednisone goal of less than or equal to 5 mg/day by six months of treatment. […] Guideline recommendations are based on systematic evidence reviews, values and preferences from a lupus nephritis patient panel, and input from adult and pediatric rheumatologists and nephrologists and a rheumatology physician assistant. The recommendations are intended to promote optimal outcomes for the most commonly encountered lupus nephritis scenarios. The guideline includes therapies available in the United States as of fall 2024 and applies to lupus nephritis in adults, children, and adolescents. This is the first time the ACR has included children and adolescents in their lupus treatment guidelines.

• #78 Management of Lupus Nephritis: New Treatments and Updated Guidelines

  https://pmc.ncbi.nlm.nih.gov/articles/PMC10617804/

  Belimumab is a monoclonal antibody that inhibits soluble human B-lymphocyte stimulator and was approved in 2011 for use in active SLE without severe renal or central nervous system involvement. […] In the 2-year phase 3 Belimumab International Study in Lupus Nephritis (BLISS-LN), patients with active class III or IV LN were randomized to receive IV belimumab 10 mg/kg or placebo on a background of SOC therapy. […] Voclosporin received FDA approval in January 2021 on the basis of the phase 3 AURORA-1 trial, representing the first oral therapy approved for LN. […] The results of these studies also highlight the increasing use of reduced doses of GCs in the management of LN. […] Both the 2019 EULAR/ERA-EDTA and 2021 KDIGO guidelines recommend that a regimen of reduced dose GCs after a short course of IV methylprednisolone pulses be considered during the initial treatment of active LN.

• #79 Diagnosis and treatment of lupus nephritis. Consensus document from the systemic auto-immune disease group (GEAS) of the Spanish Society of Internal Medicine (SEMI) and the Spanish Society of Nephrology (S.E.N.) | N

  https://www.revistanefrologia.com/en-diagnosis-treatment-lupus-nephritis-consensus-articulo-X2013251412000644

  We recommend evaluating cardiovascular risk and implementing both pharmacological and non-pharmacological measures to decrease the probability of developing accelerated arteriosclerosis (1B). […] We recommend the use of drugs for gastric protection in patients with a history of gastrointestinal haemorrhage or ulcerative disease and those under concomitant treatment with corticosteroids and non-steroidal anti-inflammatories (1B). […] We recommend that patients treated with corticosteroids receive oral supplements of calcium and vitamin D, as long as no contraindications exist (1A). […] We recommend that patients with class III or IV lupus nephritis receive maintenance treatment with low doses of steroids and mycophenolate mofetil or enteric-coated mycophenolate sodium (2D) as the first option over azathioprine (2A).

• #80 Lupus Nephritis Guidelines: Guidelines Summary

  https://emedicine.medscape.com/article/330369-guidelines

  Maintain blood pressure at or below 130/80 mm Hg. […] Initial treatment for class III-IV LN: Mycophenolate mofetil (MMF) or low-dose IV cyclophosphamide plus glucocorticoids. […] Alternate option for class III-IV LN: Combination therapy of MMF with a calcineurin inhibitor (CNI), especially tacrolimus, particularly in patients with nephrotic-range proteinuria. […] To reduce cumulative glucocorticoid dose: Intravenous pulses of methylprednisolone (total dose 500-2500mg, depending on disease severity), followed by oral prednisone (0.3-0.5mg/kg/day) for up to 4 weeks, tapered to 7.5mg/day by 3 to 6 months. […] Initial treatment for pure class V disease with nephrotic-range proteinuria: MMF in combination with methylprednisolone followed by oral prednisone. […] In all LN patients: Hydroxychloroquine (HCQ) should be coadministered at a dose not to exceed 5mg/kg/day and adjusted for the GFR, in the absence of contraindications.

• #81 Lupus Nephritis: What Is It, Causes, Symptoms and Treatment

  https://my.clevelandclinic.org/health/diseases/21809-lupus-nephritis

  Lupus nephritis is kidney inflammation due to lupus, an autoimmune disease. […] About half of adults and 80% of children with lupus will develop lupus nephritis. […] Medication and diet changes are the most common treatments for lupus nephritis. Your healthcare provider may recommend: […] Corticosteroids and immunosuppressive drugs: These medications prevent your immune system from attacking the blood vessels in your kidneys. […] Diet changes: You may need to reduce your sodium (salt) intake. Eating less protein, such as meat and dairy, can also make it easier for your kidneys to work. […] Diuretics: These medications help treat edema (excess fluid and swelling). Diuretics can also lower your blood pressure. […] Kidney failure develops in 10% to 30% of people with lupus nephritis. If this happens, you may need:

• #82 Diagnosis and treatment of lupus nephritis. Consensus document from the systemic auto-immune disease group (GEAS) of the Spanish Society of Internal Medicine (SEMI) and the Spanish Society of Nephrology (S.E.N.) | N

  https://www.revistanefrologia.com/en-diagnosis-treatment-lupus-nephritis-consensus-articulo-X2013251412000644

  We recommend evaluating cardiovascular risk and implementing both pharmacological and non-pharmacological measures to decrease the probability of developing accelerated arteriosclerosis (1B). […] We recommend the use of drugs for gastric protection in patients with a history of gastrointestinal haemorrhage or ulcerative disease and those under concomitant treatment with corticosteroids and non-steroidal anti-inflammatories (1B). […] We recommend that patients treated with corticosteroids receive oral supplements of calcium and vitamin D, as long as no contraindications exist (1A). […] We recommend that patients with class III or IV lupus nephritis receive maintenance treatment with low doses of steroids and mycophenolate mofetil or enteric-coated mycophenolate sodium (2D) as the first option over azathioprine (2A).

• #83 Updated EULAR/ERA-EDTA Recommendations for the Management of Lupus Nephritis – Rheumatology Advisor

  https://www.rheumatologyadvisor.com/features/updated-recommendations-for-the-management-of-lupus-nephritis/

  Among patients with lupus nephritis with impaired renal function or arterial hypertension (except in pregnancy), renin-angiotensin-aldosterone system blockers were recommended by the task force. […] The task force recommended the prevention of osteoporosis and bone protection measures based on fracture risk, and suggested the inclusion of both nonpharmacologic (exercise, maintaining normal weight) and pharmacologic therapies (supplementation, antiresorptive agents). […] The task force recommended that all methods of kidney replacement modalities be considered in patients SLE. […] Among patients with antiphospholipid syndrome-associated nephropathy, the task force recommended treatment with antiplatelet/anticoagulant agents, in addition to hydroxychloroquine. […] The task force recommended the 2017 EULAR guidelines for pregnancy planning while managing SLE.

• #84 What is Lupus Nephritis? Treatment and Overview | HSS

  https://www.hss.edu/conditions_lupus-nephritis-what-you-need-to-know-about-lupus-kidney-disease.asp

  Immunosuppressive agents include: High dose glucocorticoids (or steroids) such as prednisone (by mouth) or methylprednisolone administered by mouth, often after IV infusions of methylprednisolone for one to three days. […] Intravenous administration of cyclophosphamide every month for a period of six months. […] Oral mycophenolate. […] Oral azathioprine. This medication is not the first choice for induction or maintenance therapy. However, it is used in certain situations such as pregnancy or intolerance to the other drugs. […] Other agents that may occasionally be used include: cyclosporine, tacrolimus, rituximab, etc. […] Adjunctive therapy that addresses other disease-related concerns may include one or more of the following, depending on the individual case and if there are no contraindications: Diuretics, to reduce swelling and control blood pressure.

• #85 Updated EULAR/ERA-EDTA Recommendations for the Management of Lupus Nephritis – Rheumatology Advisor

  https://www.rheumatologyadvisor.com/features/updated-recommendations-for-the-management-of-lupus-nephritis/

  Among patients with lupus nephritis with impaired renal function or arterial hypertension (except in pregnancy), renin-angiotensin-aldosterone system blockers were recommended by the task force. […] The task force recommended the prevention of osteoporosis and bone protection measures based on fracture risk, and suggested the inclusion of both nonpharmacologic (exercise, maintaining normal weight) and pharmacologic therapies (supplementation, antiresorptive agents). […] The task force recommended that all methods of kidney replacement modalities be considered in patients SLE. […] Among patients with antiphospholipid syndrome-associated nephropathy, the task force recommended treatment with antiplatelet/anticoagulant agents, in addition to hydroxychloroquine. […] The task force recommended the 2017 EULAR guidelines for pregnancy planning while managing SLE.

• #86 What is Lupus Nephritis? Treatment and Overview | HSS

  https://www.hss.edu/conditions_lupus-nephritis-what-you-need-to-know-about-lupus-kidney-disease.asp

  Angiotensin Converting Enzyme (ACE) inhibitors and Angiotensin Receptor Blockers (ARBs), additional drugs that help control high blood pressure and reduce proteinuria. […] Bisphosphonates, such as alendronate, to protect the bones. […] Statins and other drugs that control cholesterol. […] Anticoagulants for people at risk of blood clots. […] Antibiotics such as Trimethoprim/Sulfamethoxazole protect against PJP pneumonia (during periods of heavy immunosuppression) and vaccines protect against other infections such as pneumococcal pneumonia or the flu. […] Treatment for lupus nephritis can be complicated. […] The goal is to control the disease as quickly as possible, and then reduce the dose of medications given, to limit side effects. […] Unfortunately, people who are successfully treated for lupus nephritis remain at risk of recurrence for the condition. Therefore, regular monitoring of kidney function is essential, especially since the early signs of recurrence may only be detectable with laboratory tests.

• #87 Diagnosis and treatment of lupus nephritis. Consensus document from the systemic auto-immune disease group (GEAS) of the Spanish Society of Internal Medicine (SEMI) and the Spanish Society of Nephrology (S.E.N.) | N

  https://www.revistanefrologia.com/en-diagnosis-treatment-lupus-nephritis-consensus-articulo-X2013251412000644

  Given the level of associated morbidity, the use of oral corticosteroids has been recommended only at the lowest doses and shortest time spans possible (1B). In severe cases, intravenous pulses of methylprednisolone at 250mg-1000mg is recommended at the start of treatment and as adjuvant therapy during the induction phase (1B). […] We recommend that patients with SLE receive hydroxychloroquine on a long-term basis if no contraindications exist. Lupus nephritis, sustained remission, and pregnancy should not indicate halting hydroxychloroquine treatment (1B). […] We recommend that patients with lupus nephritis, proteinuria, and/or arterial hypertension receive renin-angiotensin-aldosterone system (RAAS) blockers (1B). We recommend weight loss if the patient is obese due to the beneficial effects on proteinuria and the progression of the renal disease (1C).

• #88 Updated EULAR/ERA-EDTA Recommendations for the Management of Lupus Nephritis – Rheumatology Advisor

  https://www.rheumatologyadvisor.com/features/updated-recommendations-for-the-management-of-lupus-nephritis/

  Among patients with lupus nephritis with impaired renal function or arterial hypertension (except in pregnancy), renin-angiotensin-aldosterone system blockers were recommended by the task force. […] The task force recommended the prevention of osteoporosis and bone protection measures based on fracture risk, and suggested the inclusion of both nonpharmacologic (exercise, maintaining normal weight) and pharmacologic therapies (supplementation, antiresorptive agents). […] The task force recommended that all methods of kidney replacement modalities be considered in patients SLE. […] Among patients with antiphospholipid syndrome-associated nephropathy, the task force recommended treatment with antiplatelet/anticoagulant agents, in addition to hydroxychloroquine. […] The task force recommended the 2017 EULAR guidelines for pregnancy planning while managing SLE.

• #89 Lupus Nephritis: What Is It, Causes, Symptoms and Treatment

  https://my.clevelandclinic.org/health/diseases/21809-lupus-nephritis

  Lupus nephritis is kidney inflammation due to lupus, an autoimmune disease. […] About half of adults and 80% of children with lupus will develop lupus nephritis. […] Medication and diet changes are the most common treatments for lupus nephritis. Your healthcare provider may recommend: […] Corticosteroids and immunosuppressive drugs: These medications prevent your immune system from attacking the blood vessels in your kidneys. […] Diet changes: You may need to reduce your sodium (salt) intake. Eating less protein, such as meat and dairy, can also make it easier for your kidneys to work. […] Diuretics: These medications help treat edema (excess fluid and swelling). Diuretics can also lower your blood pressure. […] Kidney failure develops in 10% to 30% of people with lupus nephritis. If this happens, you may need:

• #90 New Treatment Regimens, New Drugs, and New Treatment Goals for Lupus Nephritis

  https://www.mdpi.com/2077-0383/14/2/584

  In the phase 2 NOBILITY trial, 125 patients with proliferative LN were randomized to receive either obinutuzumab (1 g at week 0, 2, 24 and 26) or placebo in combination with SOC (MMF 2–2.5 g daily or equivalent doses of MPAA, 1–3 g methylprednisolone pulses followed by oral prednisone starting at 0.5 mg/kg daily tapered to 7.5 mg daily by week 12). […] Anifrolumab, a monoclonal antibody targeting the type I interferon (IFN) receptor subunit 1, is approved and recommended for SLE without LN, following the results of a phase 3 trial. However, there is a rationale for its use in LN, stemming from the role of IFN pathways in LN pathogenesis, with over 80% of LN patients displaying elevated IFN gene signatures linked to active kidney disease and treatment resistance.

• #91 Management of Lupus Nephritis: New Treatments and Updated Guidelines

  https://pmc.ncbi.nlm.nih.gov/articles/PMC10617804/

  Multiple immune pathways are implicated in LN, allowing for different treatment targets for emerging therapies. […] The AURORA-2 continuation study was designed to assess long-term safety and tolerability of the combination of voclosporin, MMF, and low-dose GCs over an additional 2 years of treatment after completion of AURORA-1. […] The identification of downstream urinary or serum biomarkers of specific pathways may permit a precision medicine approach targeting therapeutic agents to the individual. […] Concerns remain regarding the cost-effectiveness of lupus therapy. […] In summary, LN outcomes have improved over recent decades, but there is a great need for safer and more effective therapies.

• #92 New Treatment Regimens, New Drugs, and New Treatment Goals for Lupus Nephritis

  https://www.mdpi.com/2077-0383/14/2/584

  In the phase 2 NOBILITY trial, 125 patients with proliferative LN were randomized to receive either obinutuzumab (1 g at week 0, 2, 24 and 26) or placebo in combination with SOC (MMF 2–2.5 g daily or equivalent doses of MPAA, 1–3 g methylprednisolone pulses followed by oral prednisone starting at 0.5 mg/kg daily tapered to 7.5 mg daily by week 12). […] Anifrolumab, a monoclonal antibody targeting the type I interferon (IFN) receptor subunit 1, is approved and recommended for SLE without LN, following the results of a phase 3 trial. However, there is a rationale for its use in LN, stemming from the role of IFN pathways in LN pathogenesis, with over 80% of LN patients displaying elevated IFN gene signatures linked to active kidney disease and treatment resistance.

• #93 New Treatment Regimens, New Drugs, and New Treatment Goals for Lupus Nephritis

  https://www.mdpi.com/2077-0383/14/2/584

  In the phase 2 NOBILITY trial, 125 patients with proliferative LN were randomized to receive either obinutuzumab (1 g at week 0, 2, 24 and 26) or placebo in combination with SOC (MMF 2–2.5 g daily or equivalent doses of MPAA, 1–3 g methylprednisolone pulses followed by oral prednisone starting at 0.5 mg/kg daily tapered to 7.5 mg daily by week 12). […] Anifrolumab, a monoclonal antibody targeting the type I interferon (IFN) receptor subunit 1, is approved and recommended for SLE without LN, following the results of a phase 3 trial. However, there is a rationale for its use in LN, stemming from the role of IFN pathways in LN pathogenesis, with over 80% of LN patients displaying elevated IFN gene signatures linked to active kidney disease and treatment resistance.

• #94 Lupus Nephritis Treatment With CAR T-Cell Therapy | Kyverna

  https://kyvernatx.com/patients/lupus-nephritis/

  Lupus nephritis (LN) is a severe kidney manifestation of Systemic Lupus Erythematosus (SLE) that affects up to 50% of these patients and is a major cause of morbidity and mortality. […] Treatment goals of LN include long-term preservation of kidney function accompanied by improvements in proteinuria, survival, and quality of life, as well as prevention of disease flares and management of comorbidities. Despite recent advancements in treatment for LN, complete response rate (CRR) is achieved by less than 50% of patients and many patients will require dialysis and kidney transplant as their disease progresses. […] We believe our approach may present a significant advantage over current standard-of-care therapies by aiming to directly deplete B cells and potentially resetting disease-contributing B cells.

• #95 Update on available therapies for refractory lupus nephritis | Revista Colombiana de Reumatología (English Edition)

  https://www.elsevier.es/en-revista-revista-colombiana-reumatologia-english-edition–474-articulo-update-on-available-therapies-for-S2444440523000912

  Extracorporeal therapy could be a reasonable option in patients with a refractory disease course or in those who have an absolute contraindication to the intensification of conventional treatment, particularly those who present with a severe infection at the same time as the renal flare. […] MSC transplantation appears to be a promising and safe treatment, with very few side effects. […] According to the available evidence, these medications are considered the first line of treatment in refractory LN. […] It is expected that anifrolumab may have good results in LN to consider it later in studies of refractory LN.

• #96 Treatment of lupus nephritis: consensus, evidence and perspectives | Nature Reviews Rheumatology

  https://www.nature.com/articles/s41584-023-00925-5

  New-generation anti-CD20 biologic agents, type I interferon antagonists and rituximab-belimumab combination regimens show promise for the treatment of LN. […] Molecular profiling, gene-signature fingerprints and urine proteomic panels could enhance the accuracy of patient stratification for treatment personalization. […] The development of novel therapeutics to improve the efficacy-to-toxicity balance for lupus nephritis (LN) has unmet needs. […] Inter-ethnic variation in tolerance to, efficacy of and evidence level for various conventional treatment regimens for LN has led to differences in prioritization in national and international guidelines.

• #97 Lupus Nephritis Treatment Consensus and Future Directions – Renal and Urology News

  https://www.renalandurologynews.com/news/lupus-nephritis-treatment-consensus-and-future-directions/

  Clinical trials are investigating novel biological agents or targeted small molecules in combination with standard of care in LN. […] Voclosporin, a novel calcineurin inhibitor, induces renal response when added to standard of care MMF and glucocorticoids, with no increased risk of infection. […] Belimumab, a monoclonal antibody against B cell activating factor (BAFF), was tested as an add-on therapy to standard regimens of glucocorticoids plus MMF or cyclophosphamide plus azathioprine. […] Patients with lupus nephritis benefit from blood pressure control, reduction of albuminuria, and a healthy lifestyle involving healthy eating, exercise, and smoking cessation, the authors recapitulated. […] In addition to LN histological class, activity and chronicity, selection of LN therapy varies by age, ethnicity, LN history and course, kidney function, and comorbidities, the authors pointed out. […] Molecular profiling, gene-signature fingerprints and urine proteomic panels could enhance the accuracy of patient stratification for treatment personalization in the future, Dr Mok and colleagues wrote.

• #98 Lupus Nephritis Treatment & Management: Approach Considerations, Pharmacotherapy for Lupus Nephritis Based on Stage, New Therapies

  https://emedicine.medscape.com/article/330369-treatment

  Patients with active lupus nephritis should avoid pregnancy, because it may worsen their renal disease and because certain medications used in the treatment may be teratogenic. In women who desire pregnancy, the following approach is advised: a preconception evaluation to establish and inform the patient about pregnancy risks, plan for pregnancy during inactive lupus nephritis, keep the lupus nephritis inactive with the lowest possible dosage of allowed drugs, treat known risk factors (hypertension, antiphospholipid and antibodies), and monitor closely during and after pregnancy to rapidly identify and treat SLE flares and obstetric complications. […] Patients with end-stage renal disease (ESRD), sclerosis, and a high chronicity index based on renal biopsy findings are unlikely to respond to aggressive therapy. In these cases, focus therapy on extrarenal manifestations of systemic lupus erythematosus (SLE) and on possible kidney transplantation.

• #99 Lupus Nephritis Treatment & Management: Approach Considerations, Pharmacotherapy for Lupus Nephritis Based on Stage, New Therapies

  https://emedicine.medscape.com/article/330369-treatment

  Patients with active lupus nephritis should avoid pregnancy, because it may worsen their renal disease and because certain medications used in the treatment may be teratogenic. In women who desire pregnancy, the following approach is advised: a preconception evaluation to establish and inform the patient about pregnancy risks, plan for pregnancy during inactive lupus nephritis, keep the lupus nephritis inactive with the lowest possible dosage of allowed drugs, treat known risk factors (hypertension, antiphospholipid and antibodies), and monitor closely during and after pregnancy to rapidly identify and treat SLE flares and obstetric complications. […] Patients with end-stage renal disease (ESRD), sclerosis, and a high chronicity index based on renal biopsy findings are unlikely to respond to aggressive therapy. In these cases, focus therapy on extrarenal manifestations of systemic lupus erythematosus (SLE) and on possible kidney transplantation.

• #100 Updated EULAR/ERA-EDTA Recommendations for the Management of Lupus Nephritis – Rheumatology Advisor

  https://www.rheumatologyadvisor.com/features/updated-recommendations-for-the-management-of-lupus-nephritis/

  Drugs such as prednisone, azathioprine, calcineurin inhibitors, and hydroxychloroquine are compatible at safe dosages and may be continued during pregnancy and lactation. […] According to the 2012 EULAR/ERA-EDTA recommendations for managing pediatric lupus nephritis, the task force suggested that the diagnosis, treatment (using pediatric doses), and monitoring of children was similar to that of adults.

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