This document summarizes lupus nephritis and its treatment. It discusses how lupus nephritis is defined by the presence of abnormal elements in the urine of patients with systemic lupus erythematosus. It outlines the classification system for lupus nephritis and describes biopsy findings. It also discusses the goals of treatment which are to normalize kidney function, reduce proteinuria, and prevent progressive loss of kidney function. Treatment involves immunosuppressive medications like corticosteroids combined with cyclophosphamide or mycophenolate mofetil to induce remission, followed by maintenance treatment to prevent relapse.
Lupus nephritis is a serious manifestation of systemic lupus erythematosus where the kidneys are damaged. It is characterized by autoantibodies that form immune complexes and deposit in the glomeruli, causing inflammation. A renal biopsy is required for diagnosis and classification. Treatment depends on the class, with classes III and IV requiring aggressive immunosuppression including corticosteroids and other agents to reduce inflammation and prevent loss of renal function. Factors like genetics, estrogen, and ultraviolet light exposure are believed to contribute to lupus development.
Diabetic nephropathy is characterized by persistent albuminuria, declining kidney function, hypertension, and high risk of cardiovascular disease. It is primarily caused by excess accumulation of extracellular matrix in the kidneys over many years due to effects of hyperglycemia. Screening for diabetic nephropathy involves testing for microalbuminuria annually in diabetic patients. Treatment focuses on tight glycemic control, blood pressure control typically using RAAS inhibitors, and management of cardiovascular risk factors. Uncontrolled diabetes and hypertension can lead to a progressive decline in kidney function that ultimately requires renal replacement therapy if left untreated.
- English version of this lecture is available at:
https://youtu.be/t7N2GSXhYwA
- Arabic version of this lecture is available at:
https://youtu.be/WzFZym9hDtQ
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Renal transplantation in patients with lupus nephritis - prof. Ayman Refaie MNDU net
This document discusses renal transplantation in patients with lupus nephritis. It begins with background on lupus nephritis as a cause of end-stage renal disease. It then covers the pre-transplant workup, including screening for cardiovascular disease, infections, and thrombophilia. The timing of transplantation is discussed, noting most centers recommend 3-6 months of dialysis. Recurrence of lupus nephritis after transplantation is evaluated, finding the rate is low at 2-9%. Outcomes are then reviewed, with graft and patient survival found to be similar to other causes of ESRD. In conclusion, kidney transplantation is a good option for lupus nephritis patients and offers better
Management of Lupus Nephritis involves investigating through urine analysis, renal function tests, serological blood tests, and renal biopsy. Treatment depends on histologic findings and includes immunosuppressants like corticosteroids and cyclophosphamide for induction, then azathioprine or mycophenolate mofetil for maintenance. Response is monitored through urine protein levels and renal function. New guidelines recommend early renal biopsy and mycophenolate as initial therapy.
lupus nephritis is a autoimmune disease, commonly seen in adult and child and the medical or nursing care is also very important for this type of disease condition.
This document discusses guidelines for treating lupus nephritis (LN). It defines the 6 classes of LN based on renal biopsy findings and associated clinical features. Class III and IV LN typically cause nephritic syndrome and kidney impairment and are treated initially with corticosteroids combined with cyclophosphamide or mycophenolate mofetil. Relapses should be treated similarly. Class V (membranous) LN can cause nephrotic syndrome and is generally treated with corticosteroids and immunosuppressants. Managing comorbidities like antiphospholipid antibody syndrome and thrombotic thrombocytopenic purpura is also discussed.
17 february lupus nephritis prof ashraf foudaFarragBahbah
This document discusses key points about lupus nephritis. It notes that 35% of adults with SLE have clinical evidence of nephritis at diagnosis, and 50-60% develop nephritis within 10 years. Lupus nephritis reduces survival at 10 years to 88% compared to 92% for SLE alone. Renal biopsy is recommended for confirmed proteinuria over 0.5 g/24h or active urine sediment. Repeat biopsy may be considered for worsening renal function or unexplained changes. Treatment typically involves steroids like prednisone combined with immunosuppressants like MMF or cyclophosphamide. While initial response rates are similar, steroids alone is associated with more relapses. Long term
Lupus nephritis is a serious manifestation of systemic lupus erythematosus where the kidneys are damaged. It is characterized by autoantibodies that form immune complexes and deposit in the glomeruli, causing inflammation. A renal biopsy is required for diagnosis and classification. Treatment depends on the class, with classes III and IV requiring aggressive immunosuppression including corticosteroids and other agents to reduce inflammation and prevent loss of renal function. Factors like genetics, estrogen, and ultraviolet light exposure are believed to contribute to lupus development.
Diabetic nephropathy is characterized by persistent albuminuria, declining kidney function, hypertension, and high risk of cardiovascular disease. It is primarily caused by excess accumulation of extracellular matrix in the kidneys over many years due to effects of hyperglycemia. Screening for diabetic nephropathy involves testing for microalbuminuria annually in diabetic patients. Treatment focuses on tight glycemic control, blood pressure control typically using RAAS inhibitors, and management of cardiovascular risk factors. Uncontrolled diabetes and hypertension can lead to a progressive decline in kidney function that ultimately requires renal replacement therapy if left untreated.
- English version of this lecture is available at:
https://youtu.be/t7N2GSXhYwA
- Arabic version of this lecture is available at:
https://youtu.be/WzFZym9hDtQ
- Visit our website for more lectures: www.NephroTube.com
- Subscribe to our YouTube channel: www.youtube.com/NephroTube
- Join our facebook group: www.facebook.com/groups/NephroTube
- Like our facebook page: www.facebook.com/NephroTube
- Follow us on twitter: www.twitter.com/NephroTube
Renal transplantation in patients with lupus nephritis - prof. Ayman Refaie MNDU net
This document discusses renal transplantation in patients with lupus nephritis. It begins with background on lupus nephritis as a cause of end-stage renal disease. It then covers the pre-transplant workup, including screening for cardiovascular disease, infections, and thrombophilia. The timing of transplantation is discussed, noting most centers recommend 3-6 months of dialysis. Recurrence of lupus nephritis after transplantation is evaluated, finding the rate is low at 2-9%. Outcomes are then reviewed, with graft and patient survival found to be similar to other causes of ESRD. In conclusion, kidney transplantation is a good option for lupus nephritis patients and offers better
Management of Lupus Nephritis involves investigating through urine analysis, renal function tests, serological blood tests, and renal biopsy. Treatment depends on histologic findings and includes immunosuppressants like corticosteroids and cyclophosphamide for induction, then azathioprine or mycophenolate mofetil for maintenance. Response is monitored through urine protein levels and renal function. New guidelines recommend early renal biopsy and mycophenolate as initial therapy.
lupus nephritis is a autoimmune disease, commonly seen in adult and child and the medical or nursing care is also very important for this type of disease condition.
This document discusses guidelines for treating lupus nephritis (LN). It defines the 6 classes of LN based on renal biopsy findings and associated clinical features. Class III and IV LN typically cause nephritic syndrome and kidney impairment and are treated initially with corticosteroids combined with cyclophosphamide or mycophenolate mofetil. Relapses should be treated similarly. Class V (membranous) LN can cause nephrotic syndrome and is generally treated with corticosteroids and immunosuppressants. Managing comorbidities like antiphospholipid antibody syndrome and thrombotic thrombocytopenic purpura is also discussed.
17 february lupus nephritis prof ashraf foudaFarragBahbah
This document discusses key points about lupus nephritis. It notes that 35% of adults with SLE have clinical evidence of nephritis at diagnosis, and 50-60% develop nephritis within 10 years. Lupus nephritis reduces survival at 10 years to 88% compared to 92% for SLE alone. Renal biopsy is recommended for confirmed proteinuria over 0.5 g/24h or active urine sediment. Repeat biopsy may be considered for worsening renal function or unexplained changes. Treatment typically involves steroids like prednisone combined with immunosuppressants like MMF or cyclophosphamide. While initial response rates are similar, steroids alone is associated with more relapses. Long term
1. Systemic lupus erythematosus (SLE) is an autoimmune disease that can affect multiple organ systems.
2. SLE is characterized by the production of autoantibodies directed against cell nuclei.
3. Treatment of lupus nephritis involves induction therapy with corticosteroids and cytotoxic agents to control disease activity, followed by long-term maintenance treatment to prevent flares and preserve renal function.
Focal segmental glomerulosclerosis (FSGS) is a kidney disease characterized by scarring in the glomeruli. It can be primary/idiopathic or secondary to other causes like viral infections, drugs, ischemia, etc. Patients often present with nephrotic syndrome. Treatment involves controlling blood pressure and proteinuria with ACE inhibitors/ARBs initially. For idiopathic FSGS with nephrotic syndrome, the first line treatment is corticosteroids. Mycophenolate mofetil or calcineurin inhibitors can be used as steroid-sparing alternatives. For steroid-resistant FSGS, calcineurin inhibitors like cyclosporine A are recommended.
Hemolytic Uremic Syndrome Induced AKI (From Pathogenesis to Bedside) - Dr. GawadNephroTube - Dr.Gawad
Thrombotic microangiopathy (TMA) refers to intraluminal platelet thrombosis in small blood vessels. TMA can be caused by conditions such as HUS, TTP, HIV, and malignant hypertension. In hemolytic uremic syndrome (HUS), TMA is caused by Shiga toxin from E. coli or other bacteria, which activates the alternative complement pathway and causes endothelial damage. The diagnostic approach to HUS and TMA involves excluding drugs, autoimmune hemolytic anemia, and other systemic diseases as causes before determining if the presentation matches Shiga toxin-HUS, atypical HUS, or TTP. Treatment of Shiga toxin-associated HUS is generally supportive with intravenous
Acute kidney injury (AKI) is a sudden episode of kidney failure or kidney damage that happens within a few hours or a few days.It's most common in those who are critically ill and already hospitalized.
clinical approach to Rapidly Progressive GlomerulonephritisDr santosh km
This document discusses rapidly progressive glomerulonephritis (RPGN). It begins by describing the original use of the term RPGN and the discovery of anti-glomerular basement membrane (GBM) antibody's role in Goodpasture syndrome. It then discusses three main types of RPGN - anti-GBM antibody disease, immune complex disease, and pauci-immune disease. The document provides details on the clinical presentation, pathogenesis, treatment and prognosis of each type. It highlights that plasmapheresis and immunosuppression can benefit 40-45% of patients with anti-GBM antibody RPGN by preventing progression to end-stage renal disease or death.
This document summarizes IgA nephropathy (IgAN), the most common primary glomerulonephritis globally. Key points include: IgAN is characterized by deposition of IgA in the mesangium. Clinical presentations range from asymptomatic hematuria to rapidly progressive glomerulonephritis. Prognosis depends on factors like proteinuria level and hypertension. Treatment involves renin-angiotensin system blockade, glucocorticoids, and immunosuppression. The Oxford classification uses pathological features to predict prognosis.
Tubulointerstitial nephritis involves inflammatory reactions in the renal tubules and interstitium. It can be primary, secondary to other renal diseases, idiopathic, reactive to infections, or infectious itself. Acute tubular necrosis is characterized by tubular epithelial cell death due to ischemia or toxins and can cause acute kidney injury. Chronic tubulointerstitial nephritis develops over months or years and is associated with progressive loss of kidney function. It has many etiologies including drugs, infections, obstructive nephropathy, and autoimmune diseases.
This document provides information on membranous nephropathy (MN), including its epidemiology, pathophysiology, pathology, clinical presentation, secondary causes, clinical course and outcomes, and treatment options. It notes that MN is a common cause of nephrotic syndrome in adults. The pathology involves immune complex deposition on the outer aspect of the glomerular basement membrane. Conservative management focuses on controlling edema, hypertension, and proteinuria. Cyclophosphamide combined with corticosteroids can be effective for idiopathic MN with nephrotic-range proteinuria, while the role of mycophenolate mofetil requires further study.
Lupus nephritis is an inflammation of the kidneys caused by systemic lupus erythematosus (SLE), an autoimmune disease. Approximately 10-30% of SLE patients develop lupus nephritis, which can progress to end-stage renal disease requiring dialysis or transplantation if not properly treated. Lupus nephritis is classified into six classes based on pathological findings. Treatment involves medications to suppress the immune system such as corticosteroids, immunosuppressants, and biologic drugs depending on the class of lupus nephritis. Prognosis is generally good if treatment can control proteinuria, hypertension, and renal dysfunction.
This document discusses the approach and management of thrombocytopenia and immune thrombocytopenic purpura (ITP). It defines thrombocytopenia and its causes, provides diagnostic criteria for ITP, and outlines treatment approaches including corticosteroids, IVIG, anti-D, thrombopoietin receptor agonists, splenectomy, rituximab, and experimental therapies. It also addresses management of severe bleeding, pregnancy-associated thrombocytopenia, and thrombocytopenia in the settings of HIV and hepatitis C infection.
Hepatitis C virus infection is associated with many renal diseases.
Renal disease caused by
• Virus itself
• Drugs used for treatment of hepatitis c
• Associated condition with hepatitisadvanced liver cell failure.
A. The renal disease associated with hepatitis c due to advanced liver cell failure:
• Prerenal (Hypovolemia , shock and hepatorenal syndrome )
• ATN ( sepsis or shock)
B. Drugs used for treatment of hepatitis c:
• Interstitial nephritis secondary to Interferon
C. Hepatitis c itself
o Hepatitis c is RNA flavivirus( single strand)
o Has extrahepatic manifestation like arthritis, DM, cryglobulinemia and glomerulonephritis
o Renal diseases associated with hepatitis C
1. The most common types is MPGN with cryoglobulinemia
2. Others are
MPGN without cryoglobulinemia
Membranous nephropathy (MN)
Focal segmental glomerulosclerosis
IgA nephropathy
Fibrillary glomerulopathy
Immunotactoid glomerulopathy
Thrombotic microangiopathy
Amyloid
Vasculitis
Interstitial nephritis secondary to virus
HCV-associated PAN
Thrombotic Microangiopathies are diverse group of disorders wherein thrombocytopenia, hemolytic anemia and organ dysfunction such as Kidney and brain occur . Major recent advances in this field have occurred which opens up oppurtunities to effectively manage its clinical challenges .
How to retard the progression of ckd dr Tareq tantawyFarragBahbah
The document discusses ways to retard the progression of chronic kidney disease (CKD). It outlines several risk factors that can accelerate CKD progression, including proteinuria, obesity, metabolic syndrome, hypertension, cardiovascular abnormalities, and high uric acid levels. The document recommends general measures to address these risks, such as lifestyle changes to improve cardiovascular health, strict blood pressure control, and interrupting the renin-angiotensin-aldosterone system through medications.
This document describes a case of acute liver failure in a 26-year-old male patient. It provides details of the patient's history, presenting symptoms of jaundice, abdominal pain and confusion. Physical exam findings include jaundice, enlarged liver and abnormal lab values including elevated liver enzymes and coagulopathy. The patient is diagnosed with acute liver failure secondary to anti-tuberculosis medications and hepatic encephalopathy.
Hereditary hemolytic anemias can be caused by defects in the red blood cell's hemoglobin, membrane, metabolic machinery or enzymes. Glucose-6-phosphate dehydrogenase (G6PD) deficiency is the most common enzyme defect causing hemolytic anemia. It results from a lack of the enzyme G6PD, which protects red blood cells from oxidative damage. Patients with G6PD deficiency typically present with neonatal jaundice or acute hemolytic anemia triggered by factors like fava beans, infections or drugs. An acute hemolytic episode is characterized by symptoms like jaundice and dark urine over hours to days, along with signs of intravascular and extravascular hemolysis on lab tests.
Membranoproliferative glomerulonephritis (MPGN) is a type of glomerulonephritis characterized by thickening of the glomerular basement membrane, proliferation of mesangial cells and capillary loops, and activation of the complement system. There are two main types - type I is caused by immune complex deposition and type II (dense deposit disease) results from abnormal alternative complement pathway regulation leading to dense material deposition in the glomerular basement membrane. MPGN follows a progressive clinical course and can lead to end stage renal disease within 10 years if left untreated. Treatment aims to slow disease progression and control symptoms.
pathophysiology and therapy of diabetic nephropathyMuhamed Al Rohani
This document discusses diabetic nephropathy, which is the leading cause of end-stage renal disease. It begins with an overview of the epidemiology and risk factors for diabetic nephropathy. It then covers the pathophysiology and pathological stages, explaining the progression from increased glomerular filtration rate to decreased GFR and kidney damage. Treatment focuses on tight blood glucose and blood pressure control using ACE inhibitors, ARBs, or a combination to slow the progression of kidney disease. Clinical trials demonstrate that these drugs can reduce proteinuria and prevent worsening of renal function in patients with diabetes.
This document lists and briefly discusses six diseases of the kidney: acute pyelonephritis, SLE, diabetic kidney disease, renal cell carcinoma, renal infarct, and vacuolar degeneration. For each disease, there is a brief section with no other details provided.
- A new version of this lecture is available at: https://www.slideshare.net/MohammedGawad/thrombotic-microangiopathy-tma-in-adults-and-acute-kidney-injury-dr-gawad
- Visit our website for more lectures: www.NephroTube.com
- Subscribe to our YouTube channel: www.youtube.com/NephroTube
- Join our facebook group: www.facebook.com/groups/NephroTube
- Like our facebook page: www.facebook.com/NephroTube
- Follow us on twitter: www.twitter.com/NephroTube
1. Systemic lupus erythematosus (SLE) is an autoimmune disease that can affect multiple organ systems.
2. SLE is characterized by the production of autoantibodies directed against cell nuclei.
3. Treatment of lupus nephritis involves induction therapy with corticosteroids and cytotoxic agents to control disease activity, followed by long-term maintenance treatment to prevent flares and preserve renal function.
Focal segmental glomerulosclerosis (FSGS) is a kidney disease characterized by scarring in the glomeruli. It can be primary/idiopathic or secondary to other causes like viral infections, drugs, ischemia, etc. Patients often present with nephrotic syndrome. Treatment involves controlling blood pressure and proteinuria with ACE inhibitors/ARBs initially. For idiopathic FSGS with nephrotic syndrome, the first line treatment is corticosteroids. Mycophenolate mofetil or calcineurin inhibitors can be used as steroid-sparing alternatives. For steroid-resistant FSGS, calcineurin inhibitors like cyclosporine A are recommended.
Hemolytic Uremic Syndrome Induced AKI (From Pathogenesis to Bedside) - Dr. GawadNephroTube - Dr.Gawad
Thrombotic microangiopathy (TMA) refers to intraluminal platelet thrombosis in small blood vessels. TMA can be caused by conditions such as HUS, TTP, HIV, and malignant hypertension. In hemolytic uremic syndrome (HUS), TMA is caused by Shiga toxin from E. coli or other bacteria, which activates the alternative complement pathway and causes endothelial damage. The diagnostic approach to HUS and TMA involves excluding drugs, autoimmune hemolytic anemia, and other systemic diseases as causes before determining if the presentation matches Shiga toxin-HUS, atypical HUS, or TTP. Treatment of Shiga toxin-associated HUS is generally supportive with intravenous
Acute kidney injury (AKI) is a sudden episode of kidney failure or kidney damage that happens within a few hours or a few days.It's most common in those who are critically ill and already hospitalized.
clinical approach to Rapidly Progressive GlomerulonephritisDr santosh km
This document discusses rapidly progressive glomerulonephritis (RPGN). It begins by describing the original use of the term RPGN and the discovery of anti-glomerular basement membrane (GBM) antibody's role in Goodpasture syndrome. It then discusses three main types of RPGN - anti-GBM antibody disease, immune complex disease, and pauci-immune disease. The document provides details on the clinical presentation, pathogenesis, treatment and prognosis of each type. It highlights that plasmapheresis and immunosuppression can benefit 40-45% of patients with anti-GBM antibody RPGN by preventing progression to end-stage renal disease or death.
This document summarizes IgA nephropathy (IgAN), the most common primary glomerulonephritis globally. Key points include: IgAN is characterized by deposition of IgA in the mesangium. Clinical presentations range from asymptomatic hematuria to rapidly progressive glomerulonephritis. Prognosis depends on factors like proteinuria level and hypertension. Treatment involves renin-angiotensin system blockade, glucocorticoids, and immunosuppression. The Oxford classification uses pathological features to predict prognosis.
Tubulointerstitial nephritis involves inflammatory reactions in the renal tubules and interstitium. It can be primary, secondary to other renal diseases, idiopathic, reactive to infections, or infectious itself. Acute tubular necrosis is characterized by tubular epithelial cell death due to ischemia or toxins and can cause acute kidney injury. Chronic tubulointerstitial nephritis develops over months or years and is associated with progressive loss of kidney function. It has many etiologies including drugs, infections, obstructive nephropathy, and autoimmune diseases.
This document provides information on membranous nephropathy (MN), including its epidemiology, pathophysiology, pathology, clinical presentation, secondary causes, clinical course and outcomes, and treatment options. It notes that MN is a common cause of nephrotic syndrome in adults. The pathology involves immune complex deposition on the outer aspect of the glomerular basement membrane. Conservative management focuses on controlling edema, hypertension, and proteinuria. Cyclophosphamide combined with corticosteroids can be effective for idiopathic MN with nephrotic-range proteinuria, while the role of mycophenolate mofetil requires further study.
Lupus nephritis is an inflammation of the kidneys caused by systemic lupus erythematosus (SLE), an autoimmune disease. Approximately 10-30% of SLE patients develop lupus nephritis, which can progress to end-stage renal disease requiring dialysis or transplantation if not properly treated. Lupus nephritis is classified into six classes based on pathological findings. Treatment involves medications to suppress the immune system such as corticosteroids, immunosuppressants, and biologic drugs depending on the class of lupus nephritis. Prognosis is generally good if treatment can control proteinuria, hypertension, and renal dysfunction.
This document discusses the approach and management of thrombocytopenia and immune thrombocytopenic purpura (ITP). It defines thrombocytopenia and its causes, provides diagnostic criteria for ITP, and outlines treatment approaches including corticosteroids, IVIG, anti-D, thrombopoietin receptor agonists, splenectomy, rituximab, and experimental therapies. It also addresses management of severe bleeding, pregnancy-associated thrombocytopenia, and thrombocytopenia in the settings of HIV and hepatitis C infection.
Hepatitis C virus infection is associated with many renal diseases.
Renal disease caused by
• Virus itself
• Drugs used for treatment of hepatitis c
• Associated condition with hepatitisadvanced liver cell failure.
A. The renal disease associated with hepatitis c due to advanced liver cell failure:
• Prerenal (Hypovolemia , shock and hepatorenal syndrome )
• ATN ( sepsis or shock)
B. Drugs used for treatment of hepatitis c:
• Interstitial nephritis secondary to Interferon
C. Hepatitis c itself
o Hepatitis c is RNA flavivirus( single strand)
o Has extrahepatic manifestation like arthritis, DM, cryglobulinemia and glomerulonephritis
o Renal diseases associated with hepatitis C
1. The most common types is MPGN with cryoglobulinemia
2. Others are
MPGN without cryoglobulinemia
Membranous nephropathy (MN)
Focal segmental glomerulosclerosis
IgA nephropathy
Fibrillary glomerulopathy
Immunotactoid glomerulopathy
Thrombotic microangiopathy
Amyloid
Vasculitis
Interstitial nephritis secondary to virus
HCV-associated PAN
Thrombotic Microangiopathies are diverse group of disorders wherein thrombocytopenia, hemolytic anemia and organ dysfunction such as Kidney and brain occur . Major recent advances in this field have occurred which opens up oppurtunities to effectively manage its clinical challenges .
How to retard the progression of ckd dr Tareq tantawyFarragBahbah
The document discusses ways to retard the progression of chronic kidney disease (CKD). It outlines several risk factors that can accelerate CKD progression, including proteinuria, obesity, metabolic syndrome, hypertension, cardiovascular abnormalities, and high uric acid levels. The document recommends general measures to address these risks, such as lifestyle changes to improve cardiovascular health, strict blood pressure control, and interrupting the renin-angiotensin-aldosterone system through medications.
This document describes a case of acute liver failure in a 26-year-old male patient. It provides details of the patient's history, presenting symptoms of jaundice, abdominal pain and confusion. Physical exam findings include jaundice, enlarged liver and abnormal lab values including elevated liver enzymes and coagulopathy. The patient is diagnosed with acute liver failure secondary to anti-tuberculosis medications and hepatic encephalopathy.
Hereditary hemolytic anemias can be caused by defects in the red blood cell's hemoglobin, membrane, metabolic machinery or enzymes. Glucose-6-phosphate dehydrogenase (G6PD) deficiency is the most common enzyme defect causing hemolytic anemia. It results from a lack of the enzyme G6PD, which protects red blood cells from oxidative damage. Patients with G6PD deficiency typically present with neonatal jaundice or acute hemolytic anemia triggered by factors like fava beans, infections or drugs. An acute hemolytic episode is characterized by symptoms like jaundice and dark urine over hours to days, along with signs of intravascular and extravascular hemolysis on lab tests.
Membranoproliferative glomerulonephritis (MPGN) is a type of glomerulonephritis characterized by thickening of the glomerular basement membrane, proliferation of mesangial cells and capillary loops, and activation of the complement system. There are two main types - type I is caused by immune complex deposition and type II (dense deposit disease) results from abnormal alternative complement pathway regulation leading to dense material deposition in the glomerular basement membrane. MPGN follows a progressive clinical course and can lead to end stage renal disease within 10 years if left untreated. Treatment aims to slow disease progression and control symptoms.
pathophysiology and therapy of diabetic nephropathyMuhamed Al Rohani
This document discusses diabetic nephropathy, which is the leading cause of end-stage renal disease. It begins with an overview of the epidemiology and risk factors for diabetic nephropathy. It then covers the pathophysiology and pathological stages, explaining the progression from increased glomerular filtration rate to decreased GFR and kidney damage. Treatment focuses on tight blood glucose and blood pressure control using ACE inhibitors, ARBs, or a combination to slow the progression of kidney disease. Clinical trials demonstrate that these drugs can reduce proteinuria and prevent worsening of renal function in patients with diabetes.
This document lists and briefly discusses six diseases of the kidney: acute pyelonephritis, SLE, diabetic kidney disease, renal cell carcinoma, renal infarct, and vacuolar degeneration. For each disease, there is a brief section with no other details provided.
- A new version of this lecture is available at: https://www.slideshare.net/MohammedGawad/thrombotic-microangiopathy-tma-in-adults-and-acute-kidney-injury-dr-gawad
- Visit our website for more lectures: www.NephroTube.com
- Subscribe to our YouTube channel: www.youtube.com/NephroTube
- Join our facebook group: www.facebook.com/groups/NephroTube
- Like our facebook page: www.facebook.com/NephroTube
- Follow us on twitter: www.twitter.com/NephroTube
Systemic Lupus Erythematosus (SLE) is an autoimmune disease characterized by autoantibodies that can cause injury to multiple organs. The exact cause is unknown but involves genetic, immunological, and environmental factors. Key aspects include the presence of various nuclear and cytoplasmic autoantibodies, defective B and T cell tolerance, and exposure to environmental triggers. The autoantibodies can cause direct tissue damage or form immune complexes that activate complement pathways. This leads to manifestations in various organs like the kidneys, blood vessels, skin, and joints. Renal involvement is a major cause of morbidity and mortality in SLE patients.
This document discusses thrombotic thrombocytopenic purpura (TTP). It begins by presenting a case of a 32-year-old woman presenting with headaches, difficulty speaking and moving her tongue, and numbness. Her exam and labs show thrombocytopenia and schistocytes. The document then discusses the differential diagnosis, epidemiology, terminology, definitions, types, presentations, investigations, and treatment of TTP, with a focus on plasma exchange therapy to remove antibodies and replace deficient ADAMTS13 protease.
Idiopathic thrombocytopenic purpura (ITP) is an autoimmune disorder characterized by low platelet counts and bruising. It most commonly affects children ages 1-4 years old within 4 weeks of a viral infection. An autoantibody develops against platelets, causing their destruction by the spleen. Most cases of childhood ITP are mild and resolve spontaneously within 6 months. Treatment involves observation for mild cases or corticosteroids, IVIG, or anti-D therapy for more severe cases. Splenectomy is reserved for chronic or uncontrolled cases.
Hemolytic-uremic syndrome (HUS) is a disease characterized by hemolytic anemia, low platelet count, and kidney failure. It is predominantly seen in children and can be caused by infections from E. coli or other bacteria. There are typical and atypical forms of HUS. The typical form is usually caused by Shiga toxin-producing bacteria and their toxins damaging the endothelial cells in the kidneys and other organs. Atypical HUS is linked to genetic complement factor abnormalities. Treatment involves supportive care, antibiotics if infection is present, and plasma therapy to replace deficient complement factors for atypical cases. Most patients survive the acute phase but some may have long-term kidney or other organ damage.
This document discusses thrombocytopenic purpura (TP), a condition characterized by low platelet count and bruising. It describes the main types of TP, including immune (idiopathic) TP, thrombotic thrombocytopenic purpura, and drug-induced TP. Epidemiology and pathogenesis are covered. A case study demonstrates the clinical presentation and diagnosis of a 12-year-old male with immune thrombocytopenic purpura. Treatment options are outlined, including steroids, immunoglobulins, thrombopoietin receptor agonists, and splenectomy. Differential diagnoses and references are also provided.
This document discusses lupus nephritis, including its pathophysiology, risk factors, clinical presentation, investigations, classification, treatment, and prognosis. Key points include:
- Lupus nephritis is a serious manifestation of systemic lupus erythematosus caused by autoantibodies forming immune complexes that deposit in the glomeruli.
- It is classified based on renal biopsy findings, with classes III and IV having the highest risk of progressing to end-stage renal disease.
- Treatment involves immunosuppressive induction therapy with corticosteroids and cyclophosphamide or mycophenolate mofetil to rapidly decrease inflammation, followed by long-term maintenance
This document summarizes landmark trials in the treatment of lupus nephritis over 50 years. Early trials in the 1960s established the benefit of high-dose steroids over low-dose. The 1986 NIH trial showed intravenous cyclophosphamide reduced end-stage renal failure compared to oral steroids alone. Subsequent trials tested maintenance therapies like mycophenolate mofetil versus azathioprine, and induction therapies like belimumab and voclosporin. Recent trials explored rituximab and found benefits without oral steroids. While treatment has improved over decades of research, heterogeneity remains a challenge in lupus nephritis clinical trials.
This document discusses recent treatments for lupus nephritis and summarizes a case study. It reviews definitions of glomerular pathology, the pathogenesis of lupus nephritis including the role of immune complexes, and the WHO classification system. It summarizes recent clinical trials comparing cyclophosphamide and mycophenolate mofetil as induction therapies, and azathioprine versus mycophenolate mofetil for maintenance. Rituximab was not found to be superior to placebo as an add-on therapy in one trial. The document recommends treatment and reviews considerations for a specific unemployed African American male patient with new onset nepus nephritis.
This document discusses recent treatment trials for lupus nephritis and provides an example of a patient case. It defines classifications of glomerular pathology and reviews a kidney biopsy specimen. It covers the pathogenesis of lupus nephritis including the role of immune complexes and antibodies. Treatment considerations are outlined for induction therapy with cyclophosphamide or mycophenolate mofetil and maintenance therapy with azathioprine. Clinical trials comparing various regimens are summarized.
This document discusses tubulointerstitial nephritis (TIN), a pattern of renal injury characterized by inflammation and edema of the renal tubules and interstitium. TIN is most commonly caused by drugs (71% of cases) and infections (15% of cases). On biopsy, TIN shows lymphocytic infiltration of the tubules and interstitium with tubular atrophy and normal glomeruli and vessels. Treatment involves withdrawing the offending agent and supportive care. Corticosteroids may aid recovery but their effectiveness is debated. Prognosis depends on factors like duration of the insult and degree of fibrosis - complete recovery is more likely if treatment begins early.
Based on the information provided, the most likely diagnosis is:
Behçet's disease.
Key characteristics that support this diagnosis include:
- Recurring rash on the face, arms and hands. Rash is a common manifestation of Behçet's disease.
- Painless mouth ulcers. Oral ulcers are a hallmark feature seen in over 90% of patients with Behçet's disease.
- No other medical conditions reported. Behçet's disease typically presents in otherwise healthy young adults.
- Sexual activity mentioned. Behçet's disease has been associated with sexual transmission in some cases.
Other conditions that can cause mouth ul
- The document discusses lupus nephritis, its incidence, presentation, pathogenesis, classification, and management.
- Renal biopsy is important for assessing disease activity and chronicity to guide treatment, which typically involves immunosuppressants like mycophenolate or cyclophosphamide combined with corticosteroids.
- Prognosis depends on the WHO classification - classes I-II have excellent prognosis while classes IV-VI have poorer renal outcomes if not treated aggressively.
Immune Thrombocytopenia (ITP) is an immune-mediated acquired disease characterized by low platelet count and increased risk of bleeding. The pathophysiology involves increased platelet destruction mediated by autoantibodies against platelet surface glycoproteins, and possibly decreased platelet production due to cross-reactivity of antibodies with megakaryocytes. Clinical manifestations range from asymptomatic purpura to severe bleeding. First-line therapies include corticosteroids, intravenous immunoglobulin, and anti-D immunoglobulin. Second-line options include splenectomy, rituximab, azathioprine, thrombopoietin receptor agonists, and others. Treatment goals are to maintain a safe platelet count while
Presentation on Sarcoidosis by S.K Jindal | Jindal Chest Clinic, ChandigarhJindal Chest Clinic
When the immune system overreacts, granulomas are formed, leading to a condition known as sarcoidosis. This disorder can cause mild to severe symptoms, or no symptoms at all. This Presentation describes sarcoidosis and gives an overview on Sarcoidosis including causes, symptoms, diagnosis, complications, supplements for sacrcoidosis, and treatment strategies. For more information, please contact us: 9779030507.
This document discusses pediatric kidney transplantation, including its history, indications, recipient and donor evaluation processes, surgical procedure, immunosuppression regimens, complications, and long-term management. Key points include: the first successful human kidney transplant was in 1950; recipients must not have active infection or malignancy; living donors are preferred when possible; tissue typing and crossmatching are important; immunosuppression involves induction agents, steroids, calcineurin inhibitors, and other drugs; and complications can include rejection, infection, graft failure, and malignancy. Long-term follow up is needed to monitor graft function and patient health.
Acute lymphoblastic leukemia (ALL) is a cancer of the lymphoid line of blood cells characterized by increased numbers of immature lymphocytes in the bone marrow. It is the most common cancer in children. Treatment involves chemotherapy given systemically and intrathecally in phases including induction, consolidation and maintenance to achieve and maintain remission. Prognosis depends on risk factors like age, white blood cell count, genetics. Late effects of intensive chemotherapy include secondary cancers, organ dysfunction. Relapse indicates poor prognosis requiring aggressive salvage therapies like stem cell transplant.
Biological therapy in rheumatic diseasesSamar Tharwat
This document discusses biological therapies for rheumatic diseases. It provides a historical overview of biological therapies and reviews various cytokine inhibitors including tumor necrosis factor (TNF) inhibitors, interleukin inhibitors, T-cell and B-cell directed therapies. It summarizes several TNF inhibitors including infliximab, adalimumab, etanercept, golimumab, and certolizumab pegol. The document also reviews strategies for using biologics in rheumatoid arthritis and considerations for special patient populations.
Nuclear medicine in systemic lymphomasGanesh Kumar
Nuclear medicine plays an important role in managing systemic lymphomas through techniques like FDG PET/CT, radioimmunotherapy (RIT), and assessing atypical presentations. PET/CT is useful for staging, prognostication via interim PET, and response evaluation using Deauville criteria. RIT with radiolabeled antibodies like Zevalin and Bexxar is an effective treatment for relapsed lymphomas. Unusual presentations can include neurolymphomatosis where PET/CT helps with diagnosis, and AIDS-related lymphomas where PET/CT aids in differentiation and treatment guidance.
This document provides an overview of thyroid cancer, including:
1. It describes the anatomy of the thyroid gland and risk factors for thyroid cancer such as radiation exposure and family history.
2. Papillary thyroid cancer is the most common type, and the document outlines the classification and presentation of thyroid cancers.
3. Diagnosis involves laboratory tests, ultrasound, and biopsy, while staging uses the TNM system.
4. Treatment typically involves surgery followed by radioactive iodine therapy or radiotherapy, with chemotherapy for advanced cases.
5. Prognosis depends on factors like histology, size, and spread, and follow up involves monitoring blood work and imaging tests.
Recent Advances in Pharmacotherapy of Inflammatory Bowel DiseaseShreya Gupta
This document discusses recent advances in pharmacotherapy for inflammatory bowel disease (IBD). It begins by introducing IBD as consisting of Crohn's disease and ulcerative colitis, which result from a dysregulated immune response in the gut. Recent treatment advances discussed include Janus kinase inhibitors like tofacitinib, sphingosine-1-phosphate receptor modulators like ozanimod, and phosphodiesterase 4 inhibitors. Upcoming therapies discussed are conventional small molecules and more expensive biologic drugs targeting pathways like JAK and integrins. Safety concerns are highlighted for immunomodulators commonly used to treat IBD.
Dr Alison Young, Consultant Medical Oncology, Leeds Teaching Hospitals Trust
Dr Andrew Stewart, Haematologist and Lead for Acute Oncology, University Hospitals of the North Midlands
Ceri Stubbs, Clinical Lead, Velindre NHS Trust
- Guillain-Barré syndrome (GBS) is an acute immune-mediated disorder of the peripheral nervous system.
- It is characterized by progressive weakness and loss of reflexes in the limbs and can potentially lead to respiratory failure.
- Treatment with plasma exchange or intravenous immunoglobulin has reduced mortality from GBS to 3-7% and hastens recovery.
This document lists 18 fashion designers including Danielle Nicole, Bessie, Betsey Johnson, Suarez, Michael Kors, Barry Kieselstein-Cord, Marc Jacobs, Fendi, Gucci, Emilio Pucci, Yves Saint Laurent, Valentino, Prada, Marchesa, Jimmy Choo, Tory Burch, Hélène, Kara Ross, Jardin, and Yves Saint Laurent.
This document lists 18 fashion designers including Danielle Nicole, Bessie, Betsey Johnson, Suarez, Michael Kors, Barry Kieselstein-Cord, Marc Jacobs, Fendi, Gucci, Emilio Pucci, Yves Saint Laurent, Valentino, Prada, Marchesa, Jimmy Choo, Tory Burch, Hélène, Kara Ross, Jardin, and Yves Saint Laurent.
Depression and memory problems are common in patients with lupus. Reasons for depression include symptoms of lupus like pain and fatigue, medications, and possibly the disease affecting the brain itself. While the cause of memory problems is unclear, lifestyle modifications and treatment of depression can help manage mood and cognitive issues related to lupus. Ongoing research aims to better understand and treat neuropsychiatric manifestations of the disease.
Skin Care, Weight Control, and Feeling Good with LupusLupusNY
This document summarizes a talk on managing lupus through skin care, weight control, and feeling good. It discusses how lupus can affect the skin, common skin manifestations, and tips for skin care including managing acne and sun exposure. It covers how lupus and steroids can impact weight and provides dietary and exercise recommendations. Finally, it addresses elements of feeling good such as managing fatigue and sleep.
Bone is living tissue that is constantly broken down and rebuilt. During teenage years, bone growth outpaces breakdown due to hormones, leading to peak bone mass by age 20-30. After age 30, breakdown and rebuilding are balanced until menopause in women causes estrogen loss and more breakdown. People with lupus are at higher risk of osteoporosis due to steroids, inflammation from the disease, and other factors. Regular screening, calcium, vitamin D, exercise, and medications can help prevent or treat osteoporosis in people with lupus.
This document provides information about lupus and its treatments. It begins by noting that lupus can affect the body in many ways and medications are used to protect the body from lupus. It then discusses how lupus is an autoimmune disease where the immune system attacks the body. It outlines the common symptoms and organ systems affected by lupus. The document concludes by describing several common medications used to treat lupus, including their purposes, side effects, and monitoring requirements.
This document appears to be a list of designers and brands from a New York ladies luncheon on April 11, 2013. It includes over 40 fashion labels such as Calvin Klein, Celine, Chloe, Dooney and Burke, Ferragamo, Isabella Fiore, Kenneth Cole, Max Mara, Stella McCartney, Longchamp, and Stuart Weitzman. High end designers like Chanel, Christian Dior, Heremes, Lanvin, Louis Vuitton, Gucci, Prada and Michael Kors are represented.
This document discusses new therapeutics for the treatment of lupus. It begins by describing two example cases of lupus patients with varying symptoms and disease severity. It then outlines the goals of lupus therapy which are to control disease activity, prevent damage, and prevent flares while also doing no harm through treatment. The document discusses principles of treatment design and different drug options for lupus, including their mechanisms of action and toxicities. It also provides an example of how to prioritize treating different disease manifestations in a case. Finally, it discusses challenges with lupus clinical trials and the recent success of belimumab.
Systemic lupus erythematosus (SLE) is a complex autoimmune disease that can affect multiple organ systems in variable ways between patients. Common initial symptoms include rashes and joint pain. Around 60% of SLE patients experience kidney involvement, which is a leading cause of morbidity and mortality. While SLE has no cure, early diagnosis and monitoring disease activity is important to managing symptoms and improving long-term outcomes.
This document discusses systemic lupus erythematosus (SLE) and its effects on the heart. SLE is a chronic inflammatory disease that can affect multiple organs, including the heart. It can cause valvular heart disease, pericarditis, myocarditis, and conduction defects. SLE is also associated with an increased risk of accelerated atherosclerosis and coronary heart disease. Reasons for this include traditional risk factors, steroid use, inflammation from SLE, and certain autoantibodies that affect blood vessels and cholesterol levels. Screening and monitoring of cardiac involvement is important for lupus patients.
The document discusses lupus, an autoimmune disease that commonly affects the kidneys and can lead to end-stage renal disease requiring dialysis or transplantation. Kidney involvement from lupus nephritis is a major cause of illness and death, and the document outlines risk factors for progression to end-stage renal disease as well as treatment options and outcomes for lupus patients with kidney failure.
Systemic lupus erythematosus (SLE) is a chronic inflammatory disease that can affect multiple organ systems. While there is no cure for lupus, treatment can help reduce symptoms. Exercise is beneficial for people with lupus by improving range of motion, strengthening muscles, maintaining bone health, and reducing fatigue and joint stiffness. A balanced exercise program includes range of motion, strengthening, and aerobic exercises. It is important to start slowly, monitor symptoms, and adjust the program as needed to avoid overexertion during flares. Pacing activities and practicing energy conservation techniques can also help manage fatigue.
This document discusses common musculoskeletal complaints seen in systemic lupus erythematosus (SLE) such as arthritis, arthralgias, myalgias, tendonitis, and fibromyalgia. Joint involvement is one of the most common initial manifestations of SLE and can include non-erosive or erosive arthritis. Other joint conditions like Jacoud's arthropathy may present with hypermobile joints and reducible deformities.
This document discusses oral health issues related to lupus and Sjogren's syndrome. It outlines that oral ulcers, dry mouth, and infections are common oral manifestations. Medications used to treat lupus and Sjogren's can also impact oral health. Proper dental care, saliva substitutes, and medications like pilocarpine or cevimelene can help manage dry mouth. Fungal infections like candida and viral infections like herpes simplex need to be treated with anti-fungal or anti-viral medications. Bisphosphonates used for osteoporosis can potentially cause osteonecrosis of the jaw, so dental exams are recommended before starting these therapies.
Systemic lupus erythematosus (SLE) can involve all layers of the heart, including the pericardium, myocardium, valves, and coronary arteries. Patients with SLE have a 4-8 fold increased risk of developing cardiovascular disease compared to the general population. Studies have found significantly higher rates of atherosclerosis in SLE patients compared to age-matched controls, even in younger patients and those without traditional risk factors. Aggressive treatment of modifiable risk factors such as dyslipidemia, hypertension, and smoking is important for reducing cardiovascular risk in SLE patients.
Lupus can affect many organs, including the kidneys, in up to 50% of patients. Lupus nephritis is an inflammation of the kidneys caused by lupus that can lead to scarring and fibrosis if not treated. A kidney biopsy is always indicated to determine the stage of kidney involvement, assess prognosis, and guide treatment decisions. Treatment involves controlling blood pressure and protein levels in the urine with medications, as well as targeted therapies like immunosuppressants depending on biopsy results. Prognosis depends on biopsy findings, with dialysis needed if renal function deteriorates significantly.
Managing Lupus and Maintaining a Healthy LifestyleLupusNY
This document outlines lifestyle recommendations for managing lupus, including avoiding triggers like infections, sun exposure, and certain drugs. It recommends getting adequate sleep, avoiding smoking and excessive alcohol, and exercising regularly. Nutritious eating and maintaining a healthy weight while on steroids is also discussed. The importance of stress management, preventative healthcare, appearance, social support systems, and clinical trial participation are also covered. The overall message is that patients should focus on controlling modifiable factors while not blaming themselves for things outside their control.
by Cono Grasso, MD
Jamaica Hospital Medical Center
Presented at the S.L.E. Lupus Foundation's "Get into the Loop!" New York City Lupus Education Series on October 6, 2010.
Biomarkers for Disease Flare by Emily Baechler Gillespie LupusNY
This document discusses potential biomarkers for disease activity in systemic lupus erythematosus (SLE). It describes how identifying proteins in the blood that correlate with lupus disease activity could lead to a new clinical test to better monitor patients and predict flares. Studies have found that interferon-regulated chemokines are higher during active lupus compared to inactive lupus, correlate with disease activity levels, and may predict future flares. Ongoing work is exploring if measuring these chemokines could form the basis of a new clinical test and help identify those at risk for developing lupus.
This document discusses systemic lupus erythematosus (SLE), also known as lupus, which is a chronic autoimmune disease that can affect multiple systems of the body. It presents in episodes or flares and remissions. Common symptoms in teens include fever, rash, mouth sores, and hair loss. Organs that can be involved include the blood, joints, skin, kidneys, heart, and lungs. Treatment focuses on minimizing symptoms, preventing organ damage, and reducing toxicity through medications like hydroxychloroquine and prednisone. With treatment, long-term outcomes have improved significantly, though organ damage can still occur in severe cases. An optimal treatment approach requires a multidisciplinary team and
2. Systemic Lupus Erythematosis
• The term “Lupus Erythematosis” was introduced in
19th century to describe skin lesions
• 100 years later, it was realized that it is a systemic
disease and it is causes by some aberrant
autoimmunity
• Prevalence- 1 case per 2000 population
• Currently, US 322,000 have SLE
• Incidence higher in African Americans,Hispanics,
and Asian ancestry
• 4 year survival rate- 50% in 1950s
• 15 year survival rate- 80% in 2012
3. Lupus Nephritis
• Multisystem involvement
• Lupus patients are more at risk for development of
kidney disease than people who do not have lupus
• Kidney involvement can occur in upto 50% of patients
with SLE. It usually occurs within first 5 years of
diagnosis of SLE.
• Renal involvement can occur before ACR criterion for
SLE is made
• Patients with SLE can also develop kidney disease due
to other medical problems like Diabetes Mellitus
4. Lupus Nephritis
• Defined as presence of abnormal elements in
the urine of patients with SLE
• red blood cells, white blood cells
• Red blood cell casts in urine
• Presence of protein >0.5gm/Day
• Elevated serum creatinine reflecting kidney
damage
• occurs both in children and
• adults
4
6. Lupus Nephritis- Clinical
Features
• History:
• Patients usually experience other symptoms of active
SLE like rash, fatique, arthritis,serositis or clinical CNS
disease
• Some patients are asymptomatic usually with
mesangial/membranous lupus nephritis.
• Some patients experience swelling of the body due to
proteinuria. Some are hypertensive along with
proteinuria.
• Some have symptoms associated with hypertension like
dizziness,headaches and heart failure
7. Lupus Nephritis- Clinical
Features
• Physical Examination:
• Evidence of rash, oral or nasal ulcers, joint swelling,
brown or foamy urine and changes in amount of
urine
• Patients with active lupus nephritis have
hypertension, peripheral edema, and occasionally
heart failure
• With membranous nephropathy, signs of nephrotic
syndrome( peripheral edema, ascites, pericardial
effusion and pleural effusions can be seen.
8. Diagnosis of Lupus Nephritis
• Blood tests
» BUN
» Serum Creatinine
» Laboratory tests for lupus disease activity like complement
level, antibodies to DNA, ESR,CRP etc
• Urine tests
» Urinalysis- presence of blood or protein in the urine as well
as presence of red blood cell casts
» Spot urine for protein/creatinine ratio
» 24 hour urine for creatinine clearance and protein
• Kidney biopsy
9. International Society of Nephrology/Renal
Pathology Society (ISN/RPS) 2003 classification of
lupus nephritis
• Class I Minimal mesangial lupus nephritis
• Normal glomeruli by light microscopy, but mesangial immune deposits by immunofluorescence
• Class II Mesangial proliferative lupus nephritis
• Purely mesangial hypercellularity of any degree or mesangial matrix expansion by light microscopy, with mesangial
immune deposits
• Class III Focal lupus nephritis
• Active or inactive focal, segmental or global endo- or extracapillary glomerulonephritis involving <50% of all
glomeruli, typically with focal subendothelial immune deposits, with or without mesangial alterations
– A=active lesions, C=chronic lesions
• Class IV Diffuse lupus nephritis
• Active or inactive diffuse, segmental or global endo- or extracapillary glomerulonephritis involving >50% of all
glomeruli, typically with diffuse subendothelial immune deposits, with or without mesangial alterations.
– This class is divided into diffuse segmental(IV-S) lupus nephritis when 50% of the involved glomeruli have segmental lesions, and diffuse
global (IV-G) lupus nephritis when 50% of the involved glomeruli have global lesions.
– A=active lesions, C=chronic lesions
• Class V Membranous lupus nephritis
• Global or segmental subepithelial immune deposits or their morphologic sequelae by light microscopy and by
immunofluorescence or electron microscopy, with or without mesangial alterations
– Class V lupus nephritis may occur in combination with class III or IV in which case both will be diagnosed
14. IF staining for IgG
IgG-TBM deposits IgG vessel wall deposits
15. US NIH renal Pathology System for Lupus
Nephritis
16. Treatment of Lupus Nephritis
• Depends upon class of LN diagnosed on kidney biopsy along with
presence of extra-renal manifestations of SLE
• Goal of treatment is to normalize kidney function, reduce
proteinuria, and prevent progressive loss of kidney function.
• Conservative (Non-immunomodulatory) treatment is appropriate for
Class I and II LN
• RAAS Blockade with ACE/ARB delays progression of Lupus Nephritis
• (Durán-Barragán S et al. Rheumatology 2008;47:1093-1096)
• Spironolactone significantly reduces proteinuria and lowers levels of
anti ds DNA and anti ss DNA
• (Role of aldosterone blockade in murine lupus nephritis Arthritis Res Ther 2008;10:R5)
17. Kaplan–Meier survival curve for the development of renal involvement as a function of the use of
ACE inhibitors.
Durán-Barragán S et al. Rheumatology 2008;47:1093-1096
18. Treatment of Lupus Nephritis
• Patients with proliferative classes of lupus
nephritis need immunomodulatory treatment
to turn off the immune system.
• Induction: usually Corticosteroids with either
Cyclophosphamide or Mycophenalate are used for first
six months
• Maintenance: usually with lowest and best
tolerated immunosuppressive medication. Azathioprine
or Mycophenalate are used based on recent clinical
trials
19. Induction Treatment
• It is initial intense treatment given to induce
remission of active disease
• Corticosteroids
» PO or IV
» 3 day Pulse( 7mg/kg/day) followed by 1mg/kg/day ( not
exceeding 60mg/day) for 8 weeks and then taper
» Glucocorticoids alone is significantly less effective
• Immunosuppressive agents
» Cyclophosphamide (Cytoxan) given IV
» Mycophenalate Mofetil (cellcept) administered orally
» Azathioprine( Imuran)
20. Cyclophosphamide
• Efficacious when used in conjunction with
glucocorticoids
• can be used intravenously or orally
• Used in dose of 0.5- 1.0gm/m2 monthly for six
months and then quarterly for at least two
years
• Can be given in biweekly doses of 500mg/m2
• Toxicity remains an issue
21. Standard “NIH protocol” dosing
Methylprednisolone and cyclophosphamide Alone or in Combination in
Patients w LN
• Randomized Controlled Trial in 85 Pts
• Treatment Remission
• IV Cyt x 6 mo + q 3rd mo 13/21 62%
• Methylpred 1 g/m2 x 1yr 7/24 29%
• Combined Therapy 17/20 85%
• Gourly MF, et al. Ann Int Med. 125:549, 1996
22. Kaplan-Meier Analysis of Failure of Therapy
Illei GG, et al. Ann Intern Med. 2 001
1.0 Cy + MP
Cy alone
0.9 MP alone
0.8
Probability That Therapy
0.7
Would Not Fail
0.6
0.5
0.4
Patients at Risk
0.3 27 25 25 24 23 22 18 18 17 11 7 Cy
0.2
28 27 27 26 23 23 20 20 17 15 12 Cy + MP
27 23 23 19 17 13 11 10 10 8 6 MP
0 24 48 72 96 120
Months From Study Entry
23. Long-term Follow-up of Protocol Completers in
WHO Class IV LN
50 Dialysis
Doubling SCr
40 50% Rise SCr
Patients (%)
30
20
10
0
MP alone CY alone MP + CY
(n = 24*) (n = 21) (n = 20)
*14 of 24 patients received CY after study completion
Illei GG, et al. Ann Intern Med. 2001;135:248-257.
24. • Multicenter Prospective Clinical trial
• Enrolled 90 patients from September 1996 – 2000 in 19
European centers
• Objective: To evaluate efficacy and safety of low dose
IV CYC for remission induction followed by AZA
25. Euro Lupus Trial- Study Design
90 Patients with LN Class
III-V
Low Dose IV CYC
High Dose IV CYC 44
46 (6 pulses of 500mg
(6 monthly pulses and 2 q2wks)
quaterly pulses)
40 Remained in the study 38 Remained in the study
26. Euro Lupus Trial Treatment failure
100
90 LD
Free of Failure (%)
80
HD
70
60
50 HD
LD
0
0 12 24 36 48 60
Follow-up (months)
Houssiau et al., Arthritis Rheum, 2002
28. Efficacy of MMF vs sequential PO CYT-AZA in 42
patients with DPLN
Group 1: MMF
(2 g x 6 mo,
then 1 g x 6 mo)
+ prednisone
(0.8 mg/kg)
Group 2: POCY
(2.5 mg/kg/d
x 6 mo), then
AZA (1.5-2.0
mg/kg/d) +
prednisone
Pts (%)
Chan TM et al. New Engl J Med 2000; 343:1156-62.
29. Multicenter Trial of MMF vs IV CTX for
Induction Therapy of Severe LN
• Multicenter, randomized, non-blinded trial of
induction RX for severe active LN
• Designed as equivalence trial
• Calculated sample size: 64/ Rx arm
• Hypothesis: MMF has equivalent efficacy with
superior toxicity/tolerability profile vs. IVC
•
• Ginzler et al. N Eng J Med 2005
31. Remission Rates: MMF vs. IVC
Intent to treat analysis
P=0.009
Percent Responding
P=0.005 P=NS
32. • Multinational, Prospective, Randomized, Open label trial
comparing MMF to IV Cyclophosphamide
• Background: Smaller studies have shown that MMF may offer
advantages over IV Cyclophosphamide for treatment of Lupus
Nephritis but there is no large International Randomized control
trial
• Objective: To assess the efficacy and safety of MMF as Induction
therapy in Lupus Nephritis
• Hypothesis: More patents with Lupus Nephritis would respond to
MMF than IV Cyclophosphamide during 24 weeks
33. ALMS TRIAL – RCT MMF vs IVC in Severe LN
Appel , Contreras, Dooley et al JASN 2009
Randomized (n = 370)
MMF Open-label treatment IVC
Allocated to MMF Allocated to IVC
(n = 185) (n = 185)
Received MMF (n = 184) Received IVC (n = 180)
Withdrawals (n = 35) Withdrawals (n = 29)
Due to adverse event (n = 24)
Consent withdrawn (n = 6) Due to adverse event (n = 13)
Other reason (n = 5) Consent withdrawn (n = 5)
Other reason (n = 11)
Primary endpoint: responders in randomized population (n = 370)
Responders
Maintenance phase
Double-blind re-randomization to corticosteroids plus MMF or azathioprine for up to 3 years
34. Figure 2. Response rates of study population and by racial group
Appel, G. B. et al. J Am Soc Nephrol 2009;20:1103-1112
35. Summary – Induction Phase
Therapy for Class III or IV LN
• Multiple options
– Standard, NIH protocol IV CTX
• 0.75-1.0 g/m2 IV q4 weeks for 6 doses
– Eurolupus IV CTX
• 500 mg IV q2 weeks for 6 doses
– MMF
• 2000-3000 mg/day for 6 months
• All should be given alongside standard steroid
regimen
– 3 days pulse (7 mg/kg) followed by PO steroids at 1
mg/kg/day (not exceeding 60 mg) for 8 weeks, then taper
36. Treatment of Resistant LN
• Some patients are challenging for induction
• Plasmaphresis may be beneficial in some cases although randomized
controlled trials showed no benefit
• Tacrolimus can by used
• Rituximab - FDA approved for the treatment of relapsed or refractory,
CD20-positive B-cell NH Lymphomas and Rheumatoid Arthritis
• Chimeric murine/human monoclonal antibody
• Used in many glomerular diseases in uncontrolled trials
• LUNAR study showed that induction of complete or partial remission
is higher with rituximab as compared to placebo
Rovin et al LUNAR study Arth Rheu 2012
Lewis et al NEJM 1992
Euler et al Arth Rheum 1994
37. Maintenance Therapy for
Proliferative LN
• Up to 50% of patients with LN relapse during reduction in or cessation of
immunosuppressive therapy
• Relapse rate is 5-15 per 100 patient years with an average of 8 per 100 patient years
for first 5 years of follow up.
• Treatment is usually continued for 24 months to prevent progressive kidney disease
• Usually treatment with cellcept or azathioprine is continued to keep patient in
remission and prevent relapses
• Cyclosporine is not used due to high risk of relapse upon withdrawl of drug
• Low dose oral prednisone is continued in most patients receiving maintenance
therapy with goal to prevent extrarenal symptoms. Usually a dose of 0.05 to
0.2mg/kg/day is used
Houssiau et al Maintain Trial 2010
Chan et al JASN 2005
Contreras et al NEJM 2004
38. Treatment of LN- Membranous
Nephropathy
• Treatment with immunosuppressive drugs is indicated in patients
» Severe symptomatic nephrotic syndrome
» Rising serum creatinine
» Mixed membranous and proliferative lesions on renal biospy
» Non immunosuppressive treatment is continued in these patients along with
immunosuppressive drugs
• Various drugs can be used in patients with class V lupus nephritis,
usually Mycophenalate is used for remission of nephrotic syndrome
because of its safety and better tolerance.
•
• IV cyclophosphamide and cyclosporine can be used as alternative
when MMF cannot be used.
• Non immunosuppressive agents include use of ACEI, statins, aspirin,
non dihydropyridine calcium channel blockers
39. Partial and Complete Remission Rates
Membranous LN
Radhakrishnan J, Moutzouris D, Ginzler E, and Appel GB
Kidney Int 77:152-160, 2009.
40. Prognostic Features- Lupus
Nephritis
• End Stage Renal Disease can occur in some patients with lupus
• Histological Predictors
• Class IV (diffuse proliferative LN)
• High activity and chronicity on Biopsy
• Crescents
• Interstitial fibrosis
• Segmental necrotizing lesions
• Clinical Predictors
• Hypertension
• Anemia
• High baseline serum creatinine
• Higher baseline proteinuria
• Delay in therapy
• Epidemiologic Predictors
• African American Race
• Low socioeconomic status
41. Treatment of LN- ESRD
• Survival rate for individuals with LN after 10 years of diagnosis
of SLE is reduced to 88%.
• This survival rate is further reduced in African Americans
• 15-20% of patients with lupus nephritis start renal replacement
therapy if End Stage Renal Disease occurs
• USRDS data shows that 1.4% of ESRD due to LN
» Hemodialysis
» Peritoneal Dialysis
» Kidney Transplantation
Appel et al: AJKD 1987: 83-877
Ortega LM et al: Lupus 2010: 19-557
42. Hemodialysis
• Patient survival with either hemodialysis or CAPD appears to be similar in
patients with ESRD ( 5 year survival rate is 90%)
• Most lupus nephritis patients with end-stage renal disease opt for
hemodialysis therapy(82%)
• Increased risk of death during the first three months of dialysis due to sepsis
and other complications of high dose of steroids.
Cheigh et al AJKD 1993: 21:2
Devlin et al Arth Care & Resear 2011
43. Peritoneal Dialysis
• Peritoneal dialysis and kidney transplantations lower among African-
Americans, uninsured, and unemployed
• Only 12.2% of LN patients started PD between 1995-2006
• Patients on PD do better than HD after kidney transplantation
• There may be high risk of peritonitis and non catheter related Infections in
PD.
Huang et al PDI 2001 21:143
Siu YP et al NDT 2005 20:2797
Kasiske et al J Transp 2001
44. Kidney Transplantation
• Under utilized in patients with LN
• Only 3% of LN patients undergo renal transplantation
• Graft survival rates at 5 and 10 years in patients with lupus are similar to those in
patients with other diseases.
• Rate of recurrent disease is low ( 2-9% in LN patients)
• Patients with rapidly progressive renal disease
• Should undergo dialysis before kidney transplant
• Measurement of serologic marker such as titres of
• DNA and complement levels do not help predict
• disease recurrence
• Kidney transplant recipients with lupus and
• Antiphospholipid antibodies are at increased
• Risk for thrombotic events.
Stone et al Semin Arth Rheu 1997
Choy et al Am J Transplant 2006
83% of patients enrolled in the study completed the full 24-week course of treatment. Inclusion criteria Age 12–75 years Diagnosis of SLE per American College of Rheumatology (ACR) 1997 criteria Kidney biopsy within the 6 months prior to first randomization Lupus nephritis (International Society of Nephrology/Renal Pathology Society [ISN/RPS] 2003 classification) class III, IV-S, or IV-G, (A) or (A/C), or V, alone or in combination with class III or IV Exclusion criteria Continuous dialysis for more than 2 weeks before randomization and/or expected 8 weeks Pancreatitis, GI hemorrhage within 6 months, active peptic ulcer within 3 months Severe viral infection Severe cardiovascular disease Bone marrow insufficiency, with cytopenias not attributable to SLE Current infection requiring intravenous antibiotics Maintenance phase: Double-blind, double-dummy, randomized (re-randomized), MMF orally 2 g/day or oral azathioprine 2 mg/kg/day with corticosteroids to a maximum of 10 mg/day, to treatment failure (or up to 3 years for last patient)