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
SLE:CNS
vasculitis, anxiety, depression, psychosis, seizures, stroke
SLE: blood sx
blood: thrombocytopenia, anemia, leukopenia, antiphospholipid sundrome (acquired hyper coagulability), embolism risk
SLE: cardiac sx
pericarditis, pericardial effusions, CHF, HTN
SLE: joints sx
arthritis (hands, wrists, elbows, knees), avascular necrosis due to increased use of steroids, may lead to joint replacements ex)femur head
SLE: kidneys sx
nephritis, proteinuria, hypoalbuminemia, hematuria, renal failure. may need HD/transplant
SLE: lungs sx
pleural effusions, restrictive disease, atelectasis
SLE: airway sx
mucosal ulceration, cricoaretynoid arthritis (seen mainly in RA), RLN palsy
drug induced lupus
lupus like disease that mimics lupus, s/sx usually disappear 6 months after drugs are stopped.
- doesnt mimic SLE with affecting major organs. will see more cutaneous manifestations
- men more than women due to using the 3 big culprit drugs more
- 3 big culprit drugs: hydralazine, procainamide, isonazid
cutaneous lupus
affected by sunlight and fluorescent
causes rashes and discount lesions on face arms, neck, shoulders, trunk. pigment change, hair loss due to lesions
-raynaud occurs in some individuals
-10% will develop SLE
neonatal lupus
affects infants in the womb in lupus women
caused by moms antibodies
born with skin rash, liver problems, low blood counts. disappear after some months
can be born with congenital heart block and need a pacer
2 antibodies you can test for
lupus moms and pregnancy:
at risk for pre eclampsia, flare, pre term delivery, miscarriages, intrauterine growth restrictions. recommended to have 6 month or longer remission period before trying to have better pregnancy outcomes
lupus treatment
a variation of these meds are prescribed
tylenol
NSAIDS
immunosuppressants: cyclophosphamide, methotrexate, azathioprine, mycophenolate mofetil
corticosteroid: prednisone (cushings syndrome)
antimalarial: hydroxychloroquine, chloroquine
anticoagulants: aspirin, heparin, warfarin
monoclonal antibodies: belumumab (only FDA tested and approved drug for lupus)
repository corticotropin injections: acthar
lupus and pre anesthesia evaluation
influenced by magnitude of organ system dysfunction.
preop testing: PFT’s, echo, EKG, renal function, labs
drugs theyre taking: may need to stress dose corticosteroids. be aware of immunosuppressants and antocoagulants
airway involvement: laryngeal function pre and post op, cricoaretynoid arthritis, hoarseness. monitor after extubation for postop obstruction
“what do they do to help while in a flare”
rheumatoid arthritis onset and prevalence
onset is 25-55years old
prevalence 2-3x more in women
RA etiology
environment (ex viral v bacterial) heredity viral/bacterial infection rheumatoid factors (not super hereditary, has to be activated)
big differences between osteoarthritis and RA
- OA is degenerative while RA is autoimmune
- OA is characterized by morning stiffness for less than 30 minutes while RA is characterized by morning stiffness lasting greater than 30 minutes
- OA is assymetrical in nature with loss of cartilage
- RA is symmetrical in nature with inflamed synovium
clinical manifestations of RA
joint involvement most often of hands, feet, wrists
1. inflammation of synovial joint membrane
2. rapid division and growth of cells in the joint
3. release of osteolytic enzymes, collagenases, and proteases
nerve entrapment that manifests as carpal tunnel syndrome
TMJ syndrome and synovitis
-early onset effects smaller joints first
RA and atlanto axial instability
C1 to C2 involvement
- atlanto odontoid separation, nerve involvement
- atlanto axial subluxation, pressure felt and arterial blood flow impaired
cricoaretynoid joint involvement and RA
vocal cord nodules/polyps, can be present without clinical symptoms
sx include hoarseness, pain with swallowing, stridor, dyspnea
CA joint dislocation may explain dyspnea/stridor
systemic involvement of organs: RA and pulmonary sx
pleural effusions
pneumonitis
pulmonary nodules
systemic involvement of organs: RA and cardiac sx
pericarditis
pericardial effusion
mitral/aortic regurgitation
conduction deficits
systemic involvement of organs: RA and eyes sx
destruction of lacrimal and salivary duct
systemic involvement of organs: RA and muscles sx
rheumatoid myositis
primary tx of RA
DMARDS: disease modifying anti rheumatic drugs
non biologic anti metabolite drugs for RA
methotrexate (impaired use of folate in body to use vitB. se: GI upset. can take folic acid supplements)
sulfasalazine
azathioprine
biologic TNF inhibitors for RA
etanercept (enbrel)
adalimumab (humira)
infiximab (remicade)
biologic interleukin 1 receptor agonists for RA
leflunomide (arava)
biologic anti CD20 monoclonal antibody drug for RA
rituximab
anesthetic considerations for RA
meds: NSAIDS (think of platelet dysfunction, anemia, renal and liver function), corticosteroids (think about hpa axis suppression and stress dosing)
airway: TMJ (think fiberoptic or awake intubation) cervical spine (think neck pain, avoid rotation, maybe do inline stability, mcgrath or video laryngoscopy), cricoarretynoid joint (hoarseness?)
narrow glottic opening means possibly smaller tube
positioning: padding, osteopenia from glucocorticoids possible. nerve palsies
spinal anesthesia: sensory: higher spread related to decreased subarachnoid space, decreased amount of CSF
