Systemic sclerosis Flashcards
Define systemic sclerosis
DEFINITION: rare connective tissue disease characterised by widespread small blood vessel damage and fibrosis in skin and internal organs
Also known as scleroderma
It is a spectrum of diseases.
What a re the 4 subtypes of systemic sclerosis
Pre-Scleroderma
Diffuse Cutaneous Systemic Sclerosis (40%)
Limited Cutaneous Systemic Sclerosis (60%)
Scleroderma sine Scleroderma
What 3 signs would you see in pre scleroderma?
Raynaud’s phenomenon
Nail-fold capillary changes
Antinuclear antibodies
What signs would you see in Diffuse CS scleroderma?
Raynaud’s phenomenon
Followed by skin changes with truncal involvement
Nail-fold capillary dilatation
Tendon friction
Joint contracture
Early lung disease
Heart, GI and renal disease
skin changes - shiny, taut, limts movement
lung: increased scar tissue = SOB & pulm htn
What signs would you see in Limited CS scleroderma?
What was another name for it?
Previously known as CREST Syndrome because of its
FIVE characteristic features:
Calcinosis - calcium deposition in body & skin
Raynaud’s phenomenon
Oesophageal dysmotility
Sclerodactyly - finger tautness and skin thickening
Telangiectasia - spider veins
Peripheral NOT truncal involvement
* The 5 characteristics of CREST are CROST
How is Scleroderma sine Scleroderma different for the other 2?
Internal organ disease with NO skin changes
Explain the aetiology/risk factors of systemic sclerosis
UNKNOWN
Genetic and environmental factors
Pathogenesis is unclear
Activated monocytes, macrophages and lymphocytes may interact with:
- Endothelial cells –> endothelial cell damage, platelet activation, narrowing of blood vessels
- Fibroblasts –> lay down collagen in the dermis
Summarise the epidemiology of systemic sclerosis
Age of onset: 30-60 yrs
3 x more common in FEMALES
Name some initial presenting symptoms for scleroderma
Initial symptoms are non-specific and include fatigue, vague musculoskeletal complaints, diffuse swelling of hands, and Raynaud’s phenomenon.
