Systemic sclerosis/ scleroderma Flashcards
1
Q
Definition
A
scleroderma (skin fibrosis) and vascular disease
2
Q
Types
A
- Limited - CREST syndrome
- Diffuse
3
Q
CREST syndrome
A
- Calcinonsis - Caclium deposits in any soft tissue
- Raynauds -Episodic event charactersised by digits tunring white and numb ten cyanosed then red and painful
- Esophageal dysmotility - Dysphagia, GORD
- sclerodactyl - swollen tight digits
- Telangestasia - FACE - localised colelcted of distended blood capillaries - spider, blanches
limited to hands, face , feet
4
Q
Antibody in crest syndrome
A
Anti-centromere AB
5
Q
Diffuse scleroderma
A
- whole body involvement in severe
- trincal and forearm
- early on fibrosis
6
Q
anti-bodies in diffuse scerloderma
A
anti-topoisomerase -1 - 40%
Anti-RNA polymerase - 20%
7
Q
Pathophysiology
A
mediators cause fibroblast activation - collagen accmulates - tightening of skin
- free radicals released
- enothelial cell injruy
- vasoactive mediators act to thicken SMC and cause hypertrophy
- worsening of raynauds
8
Q
Treatment
A
- no cure
- ace I in renal crisis
9
Q
A
