scleroderma Flashcards
what is scleraderma?
over production of collagen which leads to vascular damage and other organ damage
pathophysiology of scleraderma
increased cytokines which causes vascular damage and leads to increased fibrosis, leads to tightening of skin and can cause organ damage
morphea scleroderma
localized waxy patches varying in size shape and color under the skin and patches may thicken enlarge and disappear spontaneously appear between ages 20 and 50
linear scleroderma
starts as a line or streak a line of hard or waxy skin on arm or forehead
may form on a long crease referred as en coup de sabre
affect the motion of the joints which lie underneath
in childhood the growth of involved limbs may be affected
involves much deeper layers of skin
limited scleroderma
on hands and feet
internal problems are less common and less severe
also known as crest syndrome
CREST
calcinosis raynaud's phenomenon esophageal dysfunction (gerd) scerlodactylyl telangiectasias - dilitation of skin capillaries causing red marks on surface of the skin
diffuse systemic scleroderma
more ares of the skin are involved and thickened
mobility of the fingers hand and joints by the tightened skin
inflammation of the joints tendons and muscles
tight skin on the face can cause dental issues- good oral care!!
skin can lose or gain pigment
hair loss on the limbs
decreased sweat, develop dry skin involvement of GI tract, heart, lungs
Gi involvement of scleroderma
hypomotility leasts to retrosternal pain, reflux, stricture
delayed stomach emptying
small intestine= psuedo obstructions, pain, reflux, paralytic illeus, malabsorption
Large intestine= constipation, fecal impaction, diverticula
bacterial overgrowth in GI tract
nursing interventions for raynauds
keep warm dont smoke avoid caffeine cold medicines with psuedophedrine and beta-blockers, reduce stress calcium channel blockers
who else can get raynaud’s
vibrating power tools for several years artherosclerosis certain medications smoking frostbite
raynauds aggravating drugs
migrane medication decongestants beta blockers caffeine ADHD medications weight loss medications interferons cocaine
lung involvement with scleroderma
interstital lung disease
pulmonary hypertension
cardiac involvement with scleroderma
pericarditis
pericardial effusion
cardiomyopathy
MSK involvement with scleroderma
joint and muscle pain
tendon friction rub
osteomyelitis
diagnostics of scleroderma
ANA (screens for rhematic diseases)
high resolution ct scan- pulmonary htn
echocardiogram
esophageal studies
medical management of scleroderma
inflammation- nsaids or corticosteriods/joint stiffness
immunosuppresents- during flare ups
vascular diseases- Calcium channel blockers (nifedipine)
PPIS, ace inhibitors and low dose ASA
other treatments for scleroderma
immunosuppresives
phototherapy
vit d analogues
what does UV therapy do?
decreases collagen build up and improves the skin symptoms
teaching for a patient who has scleroderma
avoid high purine foods
strategies for good dental hygiene and mouth care
protect extremities from hot and cold temperatures
maintain joint function and preserve muscle strength
preform joint exercises and mouth excursion (yawning)
good pastures syndrome pathophysiology
auto immune syndrome that effects the kidneys and the lungs, the antibodies attack the kidneys causing nephritis
kidneys are shrunk to 1/5 their size and renal artery thickens
s/s of good pastures
hemoptysis cough shortness of breath
hematuria, burning with urinating, nausea and vomiting, swelling (edema, especially in the legs0
causes of good pastures
sniffing glue and siphoning gasoline with mouth (exposes lungs to hydrocarbons that cause the disease) tobacco smoke genetic mutations organic solvants viral infections metal dust inhalation sepsis high oxygen environment
assessment and diagnosis of goodpastures
ua specific gravity of 1.010 positive proteins in urine elevated K+ acid base imbalance anemia from lack of EPO Decreased albumin bc of protein loss decreased ca ams cardiac enlargement pulmonary edema abnormal ecg
treatments for good pastures
plasmapheresis- removes whole blood and replaces plasma with fluid, protein, and donated
corticosteroids
