Systemic Sclerosis Flashcards
How many more times common is systemic sclerosis in women than men?
4 times more common
What is the cause of systemic sclerosis?
It is uknown
What are the 3 patterns of disease in systemic sclerosis?
- Limited cutaneous systemic sclerosis
- Diffuse cutaneous systemic sclerosis
- Scleroderma (without internal organ involvement)
Describe limited cutaneous systemic sclerosis.
- Raynaud’s may be first sign
- Scleroderma affects face and distal limbs predominately
- A subtype of limited systemic sclerosis is
- CREST syndrome:
- Calcinosis of the skin - usually finger pulps,
- Raynaud’s phenomenon
- oEsophageal dysmotility
- Sclerodactyly
- Telangiectasia
What is limited cutaneous systemic sclerosis also known as?
CREST syndrome
What auto-antibody is associated with limited cutaneous systemic sclerosis?
Anti-centromere antibodies
What are the features of diffuse cutaneous systemic sclerosis?
- Scleroderma affects trunk and proximal limbs predominately
- Hypertension, lung fibrosis and renal involvement seen
- Poor prognosis
What auto-antibody is associated with diffuse cutaneous systemic sclerosis?
Associated with scl-70 antibodies
What are the features of Scleroderma (without internal organ involvement)?
- Tightening and fibrosis of skin
- May be manifest as plaques (morphoea) or linear
What autoantibodies are associated with scleroderma? (which one’s are associated with which subtypes aswell?)
- ANA positive in 90%
- RF positive in 30%
- Anti-scl-70 antibodies associated with diffuse cutaneous systemic sclerosis
- Anti-centromere antibodies associated with limited cutaneous systemic sclerosis
What is this?
Calcinosis of the finger pulps - pathognomonic for systemic sclerosis.
What is this?
Telangiectasia
What do you see?
Calcinosis of the backs of the hands and sclerodactyly.
