scleroderma

Question 1

what does limited scleroderma spare?

Answer 1

kidneys

skin on trunk and proximal limbs

Question 2

typical group

Answer 2

women 30-50yrs

Question 3

underlying cause

Answer 3

increased collagen production in tissues

Question 4

antibodies

Answer 4

anti-centromere = limited
scl-70 = systemic

Question 5

CREST

Answer 5

calcinosis 
raynauds
esophageal dysmotility 
sclerodactyly
telangiectasia

Question 6

first noticeable symptoms

Answer 6

skin on fingers become tight
fingers stiff and inflexible
raynauds - cold and blue

Question 7

later features

Answer 7

tightening skin around lips 
joint and muscle pain 
ulceration 
vascular disease 
pulmonary fibrosis 
PAH
renal failure

Question 8

drugs to slow disease

Answer 8

cyclophosphamide

Question 9

management

Answer 9

physio, OT

analgesia, CCB, topical skin emollients, PPI and antacids

Question 10

treating pulmonary hypertension

Answer 10

bosentan
sildenafil
iloprost

Question 11

treating raynauds

Answer 11

nifedipine - CCB

Question 12

annual tests systemic sclerosis

Answer 12

echo and spirometry

Question 13

cure?

Answer 13

no