sclederma / systemic sclerosis Flashcards
Conditions CREST is made up of? (5)
Calcinosis
Raynauds Phenomenon
Esophageal dysmotility/strictures
Sclerodactyly - skin tightening
Telangiectasia - broken capillaries
Investigations to diagnose scleroderma (CREST)? (2)
Anti centromere antibodies (ACAs) - 70%
anti nuclear antibodies - ANA often +ve
Most common type of scleroderma?
CREST- limited cutaneous scleroderma
Treatment for scleroderma (CREST)? (4)
No Cure
Treat symptoms (e.g raynauds = handwarmers, GI Sx = PPI etc)
Annual echo’s and pulmonary function tests
Prevent renal crisis using ACEi
vasodilators for reynauds
- nifedipine
- iloprost
- sildenafil
what is Sclerodactyly and what does it lead to?
Local skin thickening or tightening on fingers/toes -> movement restriction - tearing of skin and hard to repair causing ulcers
What is Calcinosis and what does it lead to? (2)
Ca deposits in subcutaneous tissue -> renal failure
Look like bright white deposits on x-ray
What is scleroderma? (2)
- Also known as systemic sclerosis
- a chronic hardening and contraction of the skin and connective tissue, either locally or throughout the body due to excess collagen production
- damage to vessels
What is Raynaud’s? (2)
Digit ischemia due to sudden vasospasm, often precipitated by the cold and relieved with heat
Classic patient has white fingers
What is Telangiectasia and what does it lead to?
Spider veins -> risk of pulmonary htn
small dilated blood vessels that can occur near the surface of the skin or mucous membranes
what would you give to prevent renal failure because of the calcinosis
ace inhib
complications
pulm hypertension - telangiestasia
tearing of skin + skin ulcers - scleryodactyaly
digit ischaemia - raynauds
renal failure - chondrocalcinosis
what annual tests need to be conducted
annual echos
annual pulmonary function tests
