FN: Systemic Sclerosis Flashcards
1
Q
Epidemiology
A
F>M = 3:1
30-50 yrs
2
Q
Limited Systemic Sclerosis:
A
70%
CREST
3
Q
C
A
Calcinosis
4
Q
R
A
Raynauds
5
Q
E
A
esophageal and gut dysmotility
6
Q
S
A
Dclerodactyly
7
Q
T
A
Telangiectasia
8
Q
Skin involvement limitied to face, hands
A
Beak nose
Microstomia
Pulmonary HTN in 15%
9
Q
Diffuse Systemic Sclerosis:
A
30%
Diffuse skin involvement
Organ fibrosis
1. GI: GOR, aspiration, dysphagia, anal incontinence
2. Lung: fibrosis and PHT
3. Cardiac: arrythmias and conduction defects
4. Renal: acute hypertensive crisis (commonest cause of death)
10
Q
Investigations
A
Bloods Abs Urine imaging ECG and Echo
11
Q
Bloods show
A
Bloods: FBC (anaemia), U and E (renal impairment)
12
Q
Abs show
A
Centromere: limited
Scl70/toposomerase:diffuse
RNA pol 1,2,3: diffuse
13
Q
Urine
A
srix, PCR
14
Q
Imaging
A
CXR: cardiomegaly, bibasl fibrosis Hands: calcinosis BA swallow: impaired oesophageal motility HiRes CT Echo
15
Q
ECG and Echo
A
Evidence of pulmonary HTN
