Systemic sclerosis Flashcards
1
Q
Systemic sclerosis
A
Sporadic and rapid onset
F:M 4:1
30 - 50 yrs old
Affects vasculature and causes fibrosis - organ failure
2
Q
Presentation of limited cutaneous systemic sclerosis
A
60% cases
- gradual onset
- CREST
3
Q
CREST syndrome
A
Calcinosis
Raynaud’s
Oesophageal and gut dysmotility → GORD (heartburn)
Sclerodactyly - swelling of hands
Telangiectasia - red spots on lips and skin
4
Q
Systemic sclerosis mx
A
No cure
Conservative
- Exercise and skin lubricants: ↓ contractures
- Hand warmers and nifedipine - Raynaud’s
- antacids - oesophageal reflux
Immunosuppression for lung fibrosis or extensive skin involvement
5
Q
Presentation of diffuse systemic sclerosis
A
- rapid onset
- associated with organ failure and digital ulceration
- skin involvement
- GORD, dysphagia and anal incontinence
- arrhythmias
6
Q
Ix
A
Bloods: FBC (anaemia), U+E (renal impairment)
Antibodies:
- Centromere - limited
- topoisomerase - diffuse
- RNA polymerase - diffuse
Urine: dipstick, PCR
Imaging
CXR: cardiomegaly, bibasal fibrosis
Hands: calcinosis
Ba swallow: impaired oesophageal motility
ECG + Echo: evidence of pulmonary HTN
