scleroderma Flashcards

1
Q

what does limited scleroderma spare?

A

kidneys

skin on trunk and proximal limbs

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2
Q

typical group

A

women 30-50yrs

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3
Q

underlying cause

A

increased collagen production in tissues

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4
Q

antibodies

A
anti-centromere = limited
scl-70 = systemic
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5
Q

CREST

A
calcinosis 
raynauds
esophageal dysmotility 
sclerodactyly
telangiectasia
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6
Q

first noticeable symptoms

A

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

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7
Q

later features

A
tightening skin around lips 
joint and muscle pain 
ulceration 
vascular disease 
pulmonary fibrosis 
PAH
renal failure
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8
Q

drugs to slow disease

A

cyclophosphamide

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9
Q

management

A

physio, OT

analgesia, CCB, topical skin emollients, PPI and antacids

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10
Q

treating pulmonary hypertension

A

bosentan
sildenafil
iloprost

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11
Q

treating raynauds

A

nifedipine - CCB

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12
Q

annual tests systemic sclerosis

A

echo and spirometry

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13
Q

cure?

A

no

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