Scleroderma, RA, SLE Flashcards
scleroderma pathophysiology general
inflammation, vascular sclerosis, fibrosis of the skin and viscera (which is painful), injury to the vascular endothelium results in leakage of proteins into interstitial space (which results in non pitting edema).
collagen production is not slowed down and gets deposited throughout the body
scleroderma etiology, population affected
unknown etiology, collagen vascular disease and autoimmune characteristics
- onset 20-40 years old
- women
- accelerated by pregnancy
- crest syndrome
CREST syndrome
calcinoses related to calcium deposits
raynauds phenomenon
esophageal hypomotility
sclerodactyly-thickened/tight skin esp on hands
telangiectasia- dilated capillaries, causes red marks on skin surfaces
diffuse scleroderma syndrome
short interval (<1y) between onset of raynauds phenomenon and development of skin changes. truncal and peripheral skin involvement, tendon friction rubs, pulmonary fibrosis, renal failure, GI disease, MI involvement.
capillary drop out visible in skin folds
scl 70 antibody positive
anticentromere antibody negative
limited cutaneous scleroderma syndrome
long history of raynauds phenomenon, like this is their only symptom for years
-limited skin involvement
-calcification, telangiectasia, late onset of pulmonary HTN, cap dilation visible in nail fold
anticentromere antibody positive
over time lungs do change still, but long time.
scleroderma skin and MSK effects
skin thickened
diffuse edema
contractures, may have to go to OR for release
skeletal muscle myopathy
arthtirits and limited joint mobility
vascular necrosis, painful and can also go to OR for this
scleroderma nervous system effects
peripheral and cranial neuropathies due to nerve compression by thickened connective tissue
trigeminal neuralgia common (trigeminal nerve branches to forehead face and jaw so you’ll see pain there)
dry eyes
scleroderma and cardiovascular system effects
sclerosis of coronary arteries and conduction system r.t fibrosis and scared tissue
replacement of cardiac tissue with fibrous tissue
systemic and pulmonary HTN
pericarditis and pericardial effusion
peripherally-intermittent vasospasm
raynauds phenomenon
scleroderma and respiratory system effects
diffuse interstitial pulmonary fibrosis (up to 80% of patients)
arterial hypoxemia secondary to decreased diffusion capacity
decreased pulmonary compliance
thickened alveolar membrane
pCO2 not increased because of diffusibility of CO2
scleroderma and the kidneys
renal artery stenosis due to arteriolar intimal proliferation, decreased renal BF, HTN
scleroderma and GI
dry oral mucosa progressive fibrosis of GI tract dysphagia hypomotility decrease lower esophageal sphincter tone malabsorption- vitamin K deficiency
scleroderma and anesthesia case considerations
-fibrosis may lead to limited mouth opening and difficult intubation
-dermal thickening may lead to difficult IV access
pulmonary HTN
-decreased pulmonary compliance and decreased O2 diffusion
-chronic systemic HTN may have a contracted intravascular volume
-hypotonia of LES, at risk for aspiration
-sensitive to respiratory depressants
-regional anesthesia may be challenging with contractors and decreased joint mobility
-protect eyes from corneal abrasion
-renal dysfunction and drug elimination
-hypovolemia creates vasodilation when inducing and hypotension results
scleroderma and possible preop/intraop considerrations
preop: labs/PFT’s, echo
intraop: warm up OR, challenging IV access so get US, GA versus regional is hard, remember regional would be hard to place
lupus definition
complex multi systemic autoimmune disease characterized by presence of auto reactive B and T cells and the production of a broad, heterogenous group of autoantibodies
- nucleic acids, RBC’s, phospholipids, lymphocytes, platelets are impacted
- antinuclear (anti DNA) antibody production most characteristic
genetic predisposition and environmental exposures to lupus
women aged 15-44
AA, asian american, hispanic/latino, native american, pacific islanders.
if you’ve got family members with it, youre at increased risk as well
50 genes associated with/contribute to but dont directly cause SLE
UV light, infection, virus, stress are triggers
possibly estrogen since s/sx increase in pregnancy/menstruation
diagnosis of lupus
challenging because no 1 test. includes:
CBC
antibody tests (ANA 97% positive), also anti DNA antibody
complement test
blood clotting tests
urine tests
cascade of other tests to “paint the picture”
criteria for classification of lupus: have to have 4 of the following SIMULTANEOUSLY to officially be diagnosed with SLE
malar rash photosensitivity oral or nasopharyngeal ulcers discoid rash renal DO serositis (pleurisy, pericarditis) neurologic DOs hematologic DOs immunologic DOs non erosive arthritis of at least two peripheral joints presence of antinuclear antibody ANA
types of lupus (4)
SLE, drug induced, cutaneous, neonatal
