systemic sclerosis Flashcards

1
Q

what is it?

A

autoimmune inflammation and skin thickening

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2
Q

what is it due to?

A

increased collagen deposition

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3
Q

what is the triad of skin thickening?

A

vascular changes
fibrosis
activation of immune system

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4
Q

what is limited systemic sclerosis?

A

above and below elbows and knees
Raynaud’s precedes onset of other symptoms

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5
Q

which antibody is associated with limited systemic sclerosis?

A

anti-centromere antibody

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6
Q

what is diffuse systemic sclerosis?

A

only below elbows and knees Raynaud’s coincides with or follows onset of other symptoms

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7
Q

which antibodies are associated with diffuse systemic sclerosis?

A

anti-SCL-50 antibody and anti-RNA polymerase III

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8
Q

what is the pneumonic for clinical presentation for limited systemic sclerosis?

A

Calcinosis
Raynaud’s phenomenon
Esophageal dysmotility (reflux)
Sclerodactyly
Telangiectasis

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9
Q

what is calcinosis?

A

calcium deposition in the skin

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10
Q

what is Raynaud’s phenomenon?

A

spasm of blood vessels in response to cold or stress

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11
Q

what is esophageal dysmotility?

A

acid reflux and decrease in motility of eosophagus

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12
Q

what is sclerodactyly?

A

thickening and tightening of the skin on the fingers and hands

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13
Q

what is telangiectasias?

A

dilation of capillaries causing red marks on surface of skin

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14
Q

what are the symptoms?

A

thickening and hardening of skin
painful ischaemic digital ulcers
fatigue
weakness
joint stiffness/pain

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15
Q

what are the signs?

A

calcinosis of fingertips

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16
Q

which antibody is present in limited systemic sclerosis?

A

anti-centromere antibody (ACA)

17
Q

which antibody is present in diffuse systemic sclerosis?

A

anti-topoisomerase

18
Q

what is present in most cases but not very specific?

A

ANA

19
Q

what is management?

A

tailored to specific symptoms

20
Q

what is treatment for Raynaud’s?

A

non-pharmacological - smoking cessation, warm gloves
CCB (nifedipine)
phosphodiesterase enzyme inhibitors

21
Q
A