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Define Systemic Lupus Erythematosis
- autoimmune disease
- generalized inflammation of connective tissue
- injury is pricipally a result of vasculitis.
- involves mulitple organs systems (skin, joints, kidneys)
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Most effected with SLE?
90% of patients are women in the childbearing years, ie 20-30
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What is the most common cause of death in SLE?
kidney failure.
glomerulonephritis--> hematuria, proteinuria, HT--> kidney failure
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Lesions of SLE are caused by?
lesions are caused by the deposition of immune complexes and fibrinoid material in tissue and by direct attack of autoantibodies.
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Joints: SLE
- up to 90% of pts have a non-erosive synovitis.
- joint pain is the CC in 40% of pts.
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polyarthralgias
cc joint pain
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Initial Signs and Symptoms of SLE
fever, malaise, anorexia, wt loss, rash( butterfly)
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SLE and arthritis
bilateral and symmetrical pain, swellign and sstiffness
arthiritis affects up to 90% of SLE pts
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X-ray and SLE
- normal joint space
- reversible deformities
- generalized osteporosis
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Lab presentation for SLE
- +FANA(flourescent antinucleur antibody) 99% of the time
- + LE(leukocyte) cells 70%
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Treatment for SLE
corticosteroid tratments
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Scleroderma aka is?
progressive systemic slerosis
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Define scleroderma
fenerilized infrlammation of connective tissue characterized by excesive fibrosis throughout the body.
SKIN, lungs GI trat, MS system, kidneys
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most commonly affected by scleroderma
- females 3x more than males
- 30-50yrs
- incidence increases with age
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CREST phenom. :
- 1. limited skin involvement
- 2. later visceral involvement
- 3. slower progrssion
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What does CREST stand for?
- c: calcinosis(deposit of calcium)
- r: raynaud's phenomenon( loss of vasculization @ distal extremities)
- e: esophageal dysfunction
- s:scleroderma
- t: telangiectasia-widening of the blood vessels
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diffuse scleroderma
- 1. Early, widespread skin involvemtn
- 2. early visceral involvement
- 3. rapid progression
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Skin and Scleroderma
- edema, progressive fibrosis of the dermis, thinning of epidermis, atrophy of dermal appendages, autoamputation.
- an increase in melanin
- subcutaneous calcium deposits in 20% of pts
- begins distally and progresses proximally in the upper extremitiy, neck, face
- bilateral and symmetrical
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scleroderma and lungs
- 70% of PSS pts have pumonary involvement
- impared gas exchanges due to fibrosing alveoli
- most common cuase of death with scleroderma pts.
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GI Tract and Scleroderma
- Atropy and replacement of teh muscularis layer with fibrotic tissue
- lower 2/3 of the esophagus become narow and rigid-->dysphagia
- small intestine and colon-->malabsorption
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MS Systems and scleroderma
- Inflammatory synovitis and fibrosis
- joint destruction is uncommmon
- fingers, knees
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Kidnesy and Scleroderma
- 2/3 of PSS pts have intimal thickening of vessel walls -->renal abn.
- 30% of pts develop HT
- formely the most common cause of death in PSS pts
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Clincal findings and scleroderma
- in most pts. exposure to cold or stress may initiate raynaud's phen.
- 50% of pts -- dysphasia
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xray finding of sclerodema
- resoprtion fo soft tissue of fingers with retraction of tips
- nomral joint space
- calcinosis of the soft tissue in 20% of pts
- may be disuse osteopenia
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Ankylosing Spondylitis x-ray findings:
- local osteoporosis
- erosions iwth reactive sclerosis
- bony ankylosis
usually bilateral and symmetrical
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SI joints and Ankylosing spondylitis
- lower 2/3 of joint
- iliac sde> scaral side
- loss of join definition (pseudowidening)
- erosions and reactive slcerosis
- bony ankylosis
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Spine and Ankylosing spondylitis
- 60% of the time, thoracolumbar jxn is first, then lumbosacral
- imflammation invloving the oter annular fibers
- vertical bridging syndesmophytes
- occuring over multiple segments (bamboo spine)
- erosions, sclerosis, decrease jt space at appophyseal and costovertebral jts.
- presevation of the disc space
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psoriatic arthritis definition:
- chronic skin disorder
- 5-8% of pts have an associated arthropathy
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Who is affected by Psoriatic arthritis?
- .2-3% of the pop.
- usually around 27 yrs old
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How does one get psoriatic arthritis
- family clustering suggest genetic factor
- some precipitation event are trauma, infecion, stress
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Clinical effects of psoriatic arthritis on skin:
- erythematos patches over covered with silvery scales
- auspitz sign- remove scales-->bleeding
- extensor surface- elboy, knee, scalp, back
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clinc of psoriatic arthritis on nails:
- thimble pitting(pathognomic). thickening, discoloration, separation
- found in up to 40% of psoriasis pt
- up to 80-905 of OsA pts
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athritis adn psoriatic arthritis
classic presentation- 1 larg jt. and 1-2 interphalangeal jts with dactylitis (sausage digits)
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Psoriatic arthritis and xray
- soft tissue swelling
- erosion and bone proliferation
- pencil in cup defomity
- "fluffy" new bone adjacent to marginal erosions doe to periositis
- SI joints 30-50% involvemtn
- erosions, reactive sclerosi
- bilateral, asymmetrical
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