FN: Systemic Sclerosis Flashcards Preview

Final Notes > FN: Systemic Sclerosis > Flashcards

Flashcards in FN: Systemic Sclerosis Deck (17):
1

Epidemiology

F>M = 3:1
30-50 yrs

2

Limited Systemic Sclerosis:

70%
CREST

3

C

Calcinosis

4

R

Raynauds

5

E

esophageal and gut dysmotility

6

S

Dclerodactyly

7

T

Telangiectasia

8

Skin involvement limitied to face, hands

Beak nose
Microstomia
Pulmonary HTN in 15%

9

Diffuse Systemic Sclerosis:

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

Investigations

Bloods
Abs
Urine
imaging
ECG and Echo

11

Bloods show

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

12

Abs show

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

13

Urine

srix, PCR

14

Imaging

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

15

ECG and Echo

Evidence of pulmonary HTN

16

Management conservative

Exercise and skin lubricants: reduce contractures

17

Medical

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

Decks in Final Notes Class (196):