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A Detailed Study on Lupus
Nephritis
By Shaswat Nayak
Introduction
 Lupus nephritis is a disease which is related to inflammation of Kidneys and is caused due to a condition
called Systemic Lupus Erythematous (SLE). SLE affects many organs in the body which include skin, brain,
lungs and kidneys.
 In Lupus Nephritis, inflammation of kidneys takes place due to binding of immune cells. The extrarenal
etiology of systemic lupus is based on multiple combinations of genetic variants that compromise those
mechanisms normally assuring immune tolerance to nuclear autoantigens. This loss of tolerance becomes
clinically detectable by the presence of antinuclear antibodies. In addition, nucleic acids released from
netting or apoptotic neutrophils activate innate and adaptive immunity via viral nucleic acid-specific Toll-
like receptors.
 Severe form of Lupus Nephritis can cause kidney failure as there is leak of proteins from the inflamed
kidneys particularly inflamed glomeruli.
Pathogenesis
A) Genetic variants of homeostatic cell death (i.e., Fas variants) and the rapid clearance of dead
cell corpses result either in secondary necrosis or incomplete chromatin
digestion, which both promote the exposure of nuclear particles to the immune system.
Pathogenesis
(B) Nuclear particles resemble viral particles and
activate the same viral nucleic acid recognition
receptors on antigen-presenting cells. Genetic
variants of those signaling elements are recognized
to be risk factors for SLE. The activation of antigen-
presenting cells changes (by costimulation) the
immune interpretation of concomitantly presented
antigens of the same particle.
(C) Polyclonal lymphocyte expansion hasmultiple
effects on the disease process and genetic variants
further affect the differentiation of T helper cells. The
complex regulation of lymphocyte activation and
expansion is affected by multiple genetic variants.
Pathogenesis
Symptoms
General symptoms of lupus nephritis include:
 Fever
 Edema
 High blood pressure
 Joint pain
 Muscle pain
 Malar rash
 Foamy urine
Diagnosis
The diagnosis of lupus nephritis begins with a medical history, physical exam, and evaluation of
symptoms. Doctors will likely order tests to make or confirm a diagnosis. Tests used in diagnosing
kidney problems include urine tests, blood tests, imaging tests such as ultrasound, and kidney
biopsy.
There are five different types of lupus nephritis. Treatment is based on the type of lupus nephritis,
which is determined by the biopsy. Since symptoms and severity vary from person to person,
treatments are individually tailored to meet a person's particular circumstances.
Treatment
Medications used in treatment can include:
 Corticosteroids. These strong anti-inflammatory drugs can decrease inflammation. Doctors may prescribe these
until the lupus nephritis improves. Because these drugs can cause a variety of potentially serious side effects, they
must be monitored carefully. Doctors generally taper down the dosage once the symptoms start to improve.
 Immunosuppressive drugs. These drugs, which are related to the ones used to treat cancer or prevent the rejection
of transplanted organs, work by suppressing immune system activity that damages the kidneys. They include
cyclophosphamide (Cytoxan), azathioprine (Imuran) and mycophenolate (Cellcept).
 Medications to prevent blood clots or lower blood pressure if needed.
Even with treatment, loss of kidney function sometimes progresses. If both kidneys fail, people with lupus nephritis
may need dialysis. Dialysis involves filtering the blood through a machine to remove waste products from the body.
Ultimately, it may be necessary to have a kidney transplant. In those cases, people will need additional drugs to keep
their immune system from rejecting the transplanted kidney.
Conclusion
LN is considered to be the major complication or outcome in SLE. Its incidence varies widely between
populations. Over the years, a better understanding of immunopathogenesis and natural history has
developed, which ultimately results in effective therapeutic decisions for the benefit of the patient and
prevent end-stage renal disease.
So far, therapy for LN has shown to be partially effective in terms of renal remission. Directed target
therapy against B and T cells could bring new insights for real effective treatment in LN and thus
achieving a better outcome in patients.
References:
 Maciej Lech and Hans-Joachim Anders-The Pathogenesis of Lupus Nephritis-Journal of the American
Society of Nephrology Vol 24: 1357–1366, 2013
 Alberto de Zubiria Salgado and Catalina Herrera-Diaz-Lupus Nephritis: An Overview of Recent Findings-
Hindawi Publishing Corporation Autoimmune Diseases Volume 2012.

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A Detailed study on Lupus Nephritis

  • 1. A Detailed Study on Lupus Nephritis By Shaswat Nayak
  • 2. Introduction  Lupus nephritis is a disease which is related to inflammation of Kidneys and is caused due to a condition called Systemic Lupus Erythematous (SLE). SLE affects many organs in the body which include skin, brain, lungs and kidneys.  In Lupus Nephritis, inflammation of kidneys takes place due to binding of immune cells. The extrarenal etiology of systemic lupus is based on multiple combinations of genetic variants that compromise those mechanisms normally assuring immune tolerance to nuclear autoantigens. This loss of tolerance becomes clinically detectable by the presence of antinuclear antibodies. In addition, nucleic acids released from netting or apoptotic neutrophils activate innate and adaptive immunity via viral nucleic acid-specific Toll- like receptors.  Severe form of Lupus Nephritis can cause kidney failure as there is leak of proteins from the inflamed kidneys particularly inflamed glomeruli.
  • 3. Pathogenesis A) Genetic variants of homeostatic cell death (i.e., Fas variants) and the rapid clearance of dead cell corpses result either in secondary necrosis or incomplete chromatin digestion, which both promote the exposure of nuclear particles to the immune system.
  • 4. Pathogenesis (B) Nuclear particles resemble viral particles and activate the same viral nucleic acid recognition receptors on antigen-presenting cells. Genetic variants of those signaling elements are recognized to be risk factors for SLE. The activation of antigen- presenting cells changes (by costimulation) the immune interpretation of concomitantly presented antigens of the same particle. (C) Polyclonal lymphocyte expansion hasmultiple effects on the disease process and genetic variants further affect the differentiation of T helper cells. The complex regulation of lymphocyte activation and expansion is affected by multiple genetic variants.
  • 6. Symptoms General symptoms of lupus nephritis include:  Fever  Edema  High blood pressure  Joint pain  Muscle pain  Malar rash  Foamy urine
  • 7. Diagnosis The diagnosis of lupus nephritis begins with a medical history, physical exam, and evaluation of symptoms. Doctors will likely order tests to make or confirm a diagnosis. Tests used in diagnosing kidney problems include urine tests, blood tests, imaging tests such as ultrasound, and kidney biopsy. There are five different types of lupus nephritis. Treatment is based on the type of lupus nephritis, which is determined by the biopsy. Since symptoms and severity vary from person to person, treatments are individually tailored to meet a person's particular circumstances.
  • 8. Treatment Medications used in treatment can include:  Corticosteroids. These strong anti-inflammatory drugs can decrease inflammation. Doctors may prescribe these until the lupus nephritis improves. Because these drugs can cause a variety of potentially serious side effects, they must be monitored carefully. Doctors generally taper down the dosage once the symptoms start to improve.  Immunosuppressive drugs. These drugs, which are related to the ones used to treat cancer or prevent the rejection of transplanted organs, work by suppressing immune system activity that damages the kidneys. They include cyclophosphamide (Cytoxan), azathioprine (Imuran) and mycophenolate (Cellcept).  Medications to prevent blood clots or lower blood pressure if needed. Even with treatment, loss of kidney function sometimes progresses. If both kidneys fail, people with lupus nephritis may need dialysis. Dialysis involves filtering the blood through a machine to remove waste products from the body. Ultimately, it may be necessary to have a kidney transplant. In those cases, people will need additional drugs to keep their immune system from rejecting the transplanted kidney.
  • 9. Conclusion LN is considered to be the major complication or outcome in SLE. Its incidence varies widely between populations. Over the years, a better understanding of immunopathogenesis and natural history has developed, which ultimately results in effective therapeutic decisions for the benefit of the patient and prevent end-stage renal disease. So far, therapy for LN has shown to be partially effective in terms of renal remission. Directed target therapy against B and T cells could bring new insights for real effective treatment in LN and thus achieving a better outcome in patients.
  • 10. References:  Maciej Lech and Hans-Joachim Anders-The Pathogenesis of Lupus Nephritis-Journal of the American Society of Nephrology Vol 24: 1357–1366, 2013  Alberto de Zubiria Salgado and Catalina Herrera-Diaz-Lupus Nephritis: An Overview of Recent Findings- Hindawi Publishing Corporation Autoimmune Diseases Volume 2012.