scleroderma Flashcards

1
Q

what is scleraderma?

A

over production of collagen which leads to vascular damage and other organ damage

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
2
Q

pathophysiology of scleraderma

A

increased cytokines which causes vascular damage and leads to increased fibrosis, leads to tightening of skin and can cause organ damage

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
3
Q

morphea scleroderma

A
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
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
4
Q

linear scleroderma

A

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

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
5
Q

limited scleroderma

A

on hands and feet
internal problems are less common and less severe
also known as crest syndrome

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
6
Q

CREST

A
calcinosis 
raynaud's phenomenon 
esophageal dysfunction (gerd)
scerlodactylyl 
telangiectasias - dilitation of skin capillaries causing red marks on surface of the skin
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
7
Q

diffuse systemic scleroderma

A

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

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
8
Q

Gi involvement of scleroderma

A

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

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
9
Q

nursing interventions for raynauds

A
keep warm 
dont smoke 
avoid caffeine 
cold medicines with psuedophedrine and beta-blockers, reduce stress 
calcium channel blockers
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
10
Q

who else can get raynaud’s

A
vibrating power tools for several years 
artherosclerosis 
certain medications 
smoking 
frostbite
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
11
Q

raynauds aggravating drugs

A
migrane medication 
decongestants 
beta blockers 
caffeine 
ADHD medications 
weight loss medications 
interferons 
cocaine
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
12
Q

lung involvement with scleroderma

A

interstital lung disease

pulmonary hypertension

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
13
Q

cardiac involvement with scleroderma

A

pericarditis
pericardial effusion
cardiomyopathy

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
14
Q

MSK involvement with scleroderma

A

joint and muscle pain
tendon friction rub
osteomyelitis

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
15
Q

diagnostics of scleroderma

A

ANA (screens for rhematic diseases)
high resolution ct scan- pulmonary htn
echocardiogram
esophageal studies

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
16
Q

medical management of scleroderma

A

inflammation- nsaids or corticosteriods/joint stiffness
immunosuppresents- during flare ups
vascular diseases- Calcium channel blockers (nifedipine)
PPIS, ace inhibitors and low dose ASA

17
Q

other treatments for scleroderma

A

immunosuppresives
phototherapy
vit d analogues

18
Q

what does UV therapy do?

A

decreases collagen build up and improves the skin symptoms

19
Q

teaching for a patient who has scleroderma

A

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)

20
Q

good pastures syndrome pathophysiology

A

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

21
Q

s/s of good pastures

A

hemoptysis cough shortness of breath

hematuria, burning with urinating, nausea and vomiting, swelling (edema, especially in the legs0

22
Q

causes of good pastures

A
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
23
Q

assessment and diagnosis of goodpastures

A
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
24
Q

treatments for good pastures

A

plasmapheresis- removes whole blood and replaces plasma with fluid, protein, and donated
corticosteroids

25
Q

dietary changes for good pastures

A

limit intake of salt and fluids
low to moderate protein diet (dairy eggs meat)
severe kidney failure/ dialysis

26
Q

good pastures disease lung and kidney diseases

A

lungs will repair over time

kidney damage is permanent