FN: Systemic Sclerosis

Question 1

Epidemiology

Answer 1

F>M = 3:1

30-50 yrs

Question 2

Limited Systemic Sclerosis:

Answer 2

70%

CREST

Question 3

C

Answer 3

Calcinosis

Question 4

R

Answer 4

Raynauds

Question 5

E

Answer 5

esophageal and gut dysmotility

Question 6

S

Answer 6

Dclerodactyly

Question 7

T

Answer 7

Telangiectasia

Question 8

Skin involvement limitied to face, hands

Answer 8

Beak nose
Microstomia
Pulmonary HTN in 15%

Question 9

Diffuse Systemic Sclerosis:

Answer 9

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)

Question 10

Investigations

Answer 10

Bloods
Abs
Urine
imaging
ECG and Echo

Question 11

Bloods show

Answer 11

Bloods: FBC (anaemia), U and E (renal impairment)

Question 12

Abs show

Answer 12

Centromere: limited
Scl70/toposomerase:diffuse
RNA pol 1,2,3: diffuse

Question 13

Urine

Answer 13

srix, PCR

Question 14

Imaging

Answer 14

CXR: cardiomegaly, bibasl fibrosis
Hands: calcinosis
BA swallow: impaired oesophageal motility
HiRes CT
Echo

Question 15

ECG and Echo

Answer 15

Evidence of pulmonary HTN

Question 16

Management conservative

Answer 16

Exercise and skin lubricants: reduce contractures

Question 17

Medical

Answer 17

1. Immunosuppression
2. Raynauds: CCBs, ACEi, IV prostacyclin
3. RenalL intensive BP control ACEi 1st line
4. Oesophageal: PPis, prokinectics (metaclopramide)
5. PHT sildenafil, bosentan