Lec 10 Scleroderma Flashcards

1
Q

About 20% of patients with scleroderma have what antibody?

A

Anti endothelial cell cytotoxic factors

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2
Q

There is evidence scleroderma may be due to ____ that causes a graft vs host type disease

A

microchimerism

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3
Q

vaso_____ are important in the pathogenesis of scleroderma

A

constrictors

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4
Q

The ____ ____ may be a primary target tissue as evidenced by intimal sclerosis, fibrinoid change, and necrosis.

A

vascular endothelium

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5
Q

The is atrophy of skin ____ and loss of ____ ____ and ____ ____

A

appendages; hair follicles, sweat glands

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6
Q

Thinning of the ____ is also seen, with accumulation of excess ____ bundles and loss of ____ ____

A

epidermis; collagen; blood vessels

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7
Q

Changes in skin include puffy ____ and ____

A

face, hands

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8
Q

Skin changes over time:

____–>stiff with altered _____ –>atrophic

A

edematous; pigmentation

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9
Q

A characteristic skin finding is loss of _____

A

wrinkles

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10
Q

Raynouds phenomenon is often seen. In severe cases, these leads to ____ of the fingers and digital _____

A

infarction; pitting

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11
Q

____ dysmotility occurs in 90% of cases. Involvement of the ____ and ____ can lead to malabsorption

A

esophageal

duodenum, jejunum

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12
Q

In the colon, wide mouth ____ can occur

A

sacculations

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13
Q

In the kidney, slowly progressive ____ lesions with ____ proliferation may be seen

A

arterial; intimal

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14
Q

In the kidney, _____ ____ causes a gradual reduction in GFR

A

adventitial fibrosis

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15
Q

_____ ____ ____ is a sudden collapse of GFR that leads to severe volume depletion

A

scleroderma renal crisis

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16
Q

In contrast to SLE, renal damage is not ____ and there are no ____ ____

A

inflammatory; immune complexes

17
Q

What kind of ischemia can lead to cardiac damage

A

microvascular

18
Q

What acronym is used to describe symptoms of limited scleroderma?

A

CREST

19
Q

What do each of the letters in CREST stand for?

A

calcinosis, raynaud, esophageal dysmotility, sclerodactyly, telangiectasia

20
Q

Limited scleroderma is associated with ______ antibody.

A

anticentromere

21
Q

_____ scleroderma (en coup de sabre) is characterized by sclerotic induration and hyperpigmentation occuring on a single extremity or face

A

linear

22
Q

____ is a patch or patches of sclerodermatosis skin damage

A

morphea

23
Q

____ _____ syndrome is a syndrome of abrupt onset myalgia, fatigue, and peripheral eoisonphilia associated with ingestion of an L-tryptophan dietary supplement

A

eiosinophilia myalgia

24
Q

can silicon breast implants cause scleroderma?

A

no

25
Q

Diffuse scleroderma is associated with anti ___ antibody (anti-DNA topoisomerase 1)

A

Scl70

26
Q

Smoking cessatation is a mainstay because nicotine is a potent _____

A

vasoconstrictor

27
Q

a positive ____ is present in about 90% of cases. Anti-RNA _____ III may also be present

A

ANA; polymerase