1921-the kidney in lupus lfgw june08 handout

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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