1921-the kidney in lupus lfgw june08 handout
TRANSCRIPT
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Kidney Disease in SystemicLupus Erythematosus (SLE)
Gabor Illei, MD, MHS
National Institute [email protected]
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Clinical manifestations of SLE
Variable: from mild to life
threatening
Onset: gradual (more
common) or acute
Remitting-relapsing
course
Kidney is the most
common major organ
involved (lupus nephritis)
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Systemic lupus erythematosus
Autoimmune Disease
The immune system is turning against itself and is
attacking the bodys own cells
This leads to inflammation pain, swelling, redness, heat and loss of function
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Lupus is a disease characterized
by inflammation
Arthritis Serositis Dermatitis
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Why should we worry about
kidney problems
50% of all lupus patients will have kidney
involvement during their life of these, 50 % will have serious kidney disease
Patients may not be aware that kidney
problems exist
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What is lupus kidney disease?
Also called lupus nephritis or
glomerulonephritis Inflammation in the kidney
The inflammation may be:
BAD or NOT SO BAD
Could result in scarring
BAD or NOT SO BAD
Worse case = Renal Failure
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Why is inflammation undesirable?
If untreated:
Scarring, dysfunction, and loss of function results.
We dont want to have scarring in the kidney
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The Normal Kidney
kidney
ureter
bladder
urethra
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Function of the Normal Kidney
Major waste management plant of the
body Filters and excretes metabolic waste products
Detoxifies and eliminates toxins
Regulates fluid and electrolyte balance
Important role in blood pressure regulation
Regulates red blood cell production
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Function of the Normal Kidney
Functional unit:nephron
About 1.2 million
nephrons per kidneyEach nephron has 2
parts
Glomerulus: filtering
unit Tubules: reabsorbs and
secretes electrolytes,glucose and aminoacids
glomerulus tubules
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Glomerulus
Blood components arefiltered through amembrane (called a
basement membrane)which acts like a sieve
Small molecules easilypass through this filter
Larger molecules, likecells and proteins abovea certain size, do not passthrough this membranein healthy people
Function of the Normal Kidney
urineBasement
membrane
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How does lupus damage the
kidneys?
Autoantibodies are formed against antigens
in the glomerulus basement membrane
Circulating immune complexes bind to the
basement membrane of the glomeruli (the
sieve)
These result in inflammation of the
glomeruli (glomerulonephritis)
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How does lupus damage the
kidneys?The basement membrane is damaged by the
inflammation and its function as a sieve is lost
Appearance of protein, white and red blood cellsand casts in the urine
Low albumin levels in the blood resulting inleakage of fluid from the vessels into the tissues
(edema)Accumulation of waste proteins (uremia)
Hypertension
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How can we tell the severity
of the inflammation
Patients well-being
Physical examination
Results of blood and urine tests
Kidney biopsy
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Signs and Symptoms
Variable
Fatigue
More lupus symptoms Fluid retention (swelling in the feet, legs, hands)
Foamy urine
need tourinate more often or at night
OR nothing appears different
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Physical Findings
High blood pressure
Inflammation (joints, hair loss, oral ulcers,etc.)
signs that lupus is active
Edema
Feet, legs, hands
OR everything appears normal
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All lupus patients should:
See their health care provider routinely
Have both blood and urine examined
regularly Monitor blood pressure
Report any symptoms of lupus to their
health care provider
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Laboratory
Tests todetermine if lupus is active
Rise in the titers of anti-double stranded DNA
antibodies
Decrease in complement levels
CBC (complete blood count)
Kidney function tests
Increased blood urea nitrogen (BUN) Increased serum creatinine
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Laboratory
Urine Presence of blood in the urine
Protein: best if measured in a 24 hour urine
collection
Abnormal urine sediment (urine is centrifuged
and the analyzed under a microscope)
Red blood cells (esp. if they have an
abnormal shape)
White blood cells
Casts (dead cells retaining the tubules shape)
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Red blood cells and cast in the urine
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What happens if lupus kidney
disease is suspected? Many things will occur:
Blood and urine evaluation
Consultations with Rheumatologist,Nephrologist
Ultrasound of the kidneys
Kidney biopsy
shows a picture of how muchinflammation is present and
where it is occurring
http://www.kidneybiopsy.com/silverman.htm
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Do I really need a biopsy?
Most likely - YES depends upon the treating physician
Kidney biopsies are important to dictate how to treat
predict how long to treat
predict the chance for kidney function recovery
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Kidney Biopsy
Who does it? A kidney specialist (nephrologist)
How is it done?
A needle is inserted into the skin in the lower backguided by ultrasound or CT
A small piece of kidney is removed and sent awayfor analysis
Patients may be admitted for overnight observation
Complications Bleeding around the kidney
Infections
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The biopsy helps determine treatment
Inflammation can occur:
Diffusely
Focally
It can cause Thickening
More cells than normal
Scarring
Loss of function
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Electronmicroscopy
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Types of Lupus Nephritis
6 classes based on a WHO classification
Class I (normal) mild
Class II (mesangial) mild
Class III (focal proliferative glomerulonephritis)
moderately severe
Class IV (diffuse proliferative glomerulonephritis)
severeClass V (membranous glomerulonephritis) variable
Class VI (glomerulosclerosis) irreversible changes
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Cellcept (Mycophenolate mofetil) in
lupus nephritis
Recent study showed that Cellcept is not
better than cytoxan in inducing remission Long-term results with Cellcept are unkown
Higher relapse rate
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Cellcept
Side effects
Infections
Low white blood cell count Diarrhea
Increased risk of lymphoma ?
No effect on fertility
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Time
C3 level
DNA titer
ProteinuriaPrednisone dose
Cytoxan therapy
Monitoring Lupus Activity
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Will I get kidney disease a
second time? Perhaps
To avoid a relapse:
Take care of yourself
eat right, sleep right, avoid stress
Take your medications
Regular follow-up
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Kidney failure in lupus nephritis
Total shutdown of kidneyfunction
Fatal if not treated Treatment of kidney
failure
Dialysis
Some patients recover withtreatment and can come offdialysis
Kidney transplant