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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)
Most effected with SLE?
90% of patients are women in the childbearing years, ie 20-30
What is the most common cause of death in SLE?
glomerulonephritis--> hematuria, proteinuria, HT--> kidney failure
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.
- up to 90% of pts have a non-erosive synovitis.
- joint pain is the CC in 40% of pts.
cc joint pain
Initial Signs and Symptoms of SLE
fever, malaise, anorexia, wt loss, rash( butterfly)
SLE and arthritis
bilateral and symmetrical pain, swellign and sstiffness
arthiritis affects up to 90% of SLE pts
X-ray and SLE
- normal joint space
- reversible deformities
- generalized osteporosis
Lab presentation for SLE
- +FANA(flourescent antinucleur antibody) 99% of the time
- + LE(leukocyte) cells 70%
Treatment for SLE
Scleroderma aka is?
progressive systemic slerosis
fenerilized infrlammation of connective tissue characterized by excesive fibrosis throughout the body.
SKIN, lungs GI trat, MS system, kidneys
most commonly affected by scleroderma
- females 3x more than males
- incidence increases with age
CREST phenom. :
- 1. limited skin involvement
- 2. later visceral involvement
- 3. slower progrssion
What does CREST stand for?
- c: calcinosis(deposit of calcium)
- r: raynaud's phenomenon( loss of vasculization @ distal extremities)
- e: esophageal dysfunction
- t: telangiectasia-widening of the blood vessels
- 1. Early, widespread skin involvemtn
- 2. early visceral involvement
- 3. rapid progression
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
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.
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
MS Systems and scleroderma
- Inflammatory synovitis and fibrosis
- joint destruction is uncommmon
- fingers, knees
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
Clincal findings and scleroderma
- in most pts. exposure to cold or stress may initiate raynaud's phen.
- 50% of pts -- dysphasia
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
Ankylosing Spondylitis x-ray findings:
- local osteoporosis
- erosions iwth reactive sclerosis
- bony ankylosis
usually bilateral and symmetrical
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
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
psoriatic arthritis definition:
- chronic skin disorder
- 5-8% of pts have an associated arthropathy
Who is affected by Psoriatic arthritis?
- .2-3% of the pop.
- usually around 27 yrs old
How does one get psoriatic arthritis
- family clustering suggest genetic factor
- some precipitation event are trauma, infecion, stress
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
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
athritis adn psoriatic arthritis
classic presentation- 1 larg jt. and 1-2 interphalangeal jts with dactylitis (sausage digits)
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