scleroderma Flashcards

1
Q

definition

A

fibrosis/hardening of the skin

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2
Q

types

A

localized or generalized

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3
Q

localized

A

morphed, linear (coup de sabre), no systemic involvement, not big deal

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4
Q

generalized other names

A

cutaneous scleroderma, systemic sclerosis

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5
Q

path

A

fibrous tissue, inflammation/ autoimmunity» vascular damage

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6
Q

epi

A

RARE, but runs in rheum families, high ANA, D, chock tow Indians, twins, environ

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7
Q

ABs

A

ANA 1:160 SEN, NPV, anticentromere SPEC, PPV, anti scl70 SPEC, PPV

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8
Q

skin clinical

A

dermis fibrosis. collagen» reticular dermis, T cells infiltrate, surround blood vessels. peripheral» proximal

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9
Q

CREST

A

old, non applicable nemonic. does NOT equal limited

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10
Q

vascular clinical

A

ALL pts have raunauds

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11
Q

raynauds

A

digital intimal proliferation- collagen hyperplasia, no arterial lumen, BAD

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12
Q

pulm HTN

A

intimal fibrosis, late and limited

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13
Q

lung clinical

A

interstitial, ground glass» honeycomb

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14
Q

renal crisis triad

A

acute renal failure, abrupt HTN, micro angio apthic hemolytic anemia

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15
Q

renal crisis treatment

A

ACE inhibitor- deal with high renin

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16
Q

renal crisis path

A

collagen in vessels» narrowing, tricks body into thinking BP is LOW

17
Q

Nailed capillaroscopy

A

Distorted, dilated, contorted, drop out of capillaries

18
Q

What type of ace inhibitor used?

A

Short acting, like captor-is- don’t want hypotension

Renin stays high but effect avoided