40. Scleroderma (systemic sclerosis) Flashcards Preview

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Flashcards in 40. Scleroderma (systemic sclerosis) Deck (33)
1

scleroderma is a

autoimmune disease characterised by activation of fibroblasts and deposition of collagen
aka: systemic sclerosis

2

characteristics of scleroderma - triad

1. autoimmunity
2. noninflammatory vasculopathy
3. collagen deposition with fibrosis

3

scleroderma causes sclerosis of ( systems )

1. skin
2. renal
3. pulmonary
4. cardiovascular
5. GI system

4

scleroderma -sex

75% females

5

systemic scleroderma is divided into

1. diffuse scleroderma
2. limited scleroderma

6

localized scleroderma involves

only skin

7

systemic scleroderma involves

both skin and visceral organs

8

scleroderma - manifestation of skin ( apperance )

puffy, taut skin without wrinkles, fingertip pitting

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diffuse scleroderma - clinical characteristics

1. widespread skin involvement
2. rapid prograssion
3. eaarly visceral involvement

10

diffuse scleroderma is associated with

anti-scl 70 antibody ( anti DNA topoisomerase I antibody )

11

anti-Scl 70 antibody is

anti - DNA topoisomerase I antibody

12

Limited sclerodermia - clinical manifestation

- limited skin involment ( fingers and face)
- CREST : Calcinosis , Raynaud phenomenon, Esophangeal dysmotility, Sclerodactyly, Telangiectasia

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CREST manifestation

Calcinosis , Raynaud phenomenon, Esophangeal dysmotiity, Sclerodactily, Telangiectasia

14

Calcinosis is the

formation of calcium deposits in any soft tissue

15

telangiectasia is a condition characterized by

dilation of the capillaries causing them to appear as small red or purple clusters ( skin and organs)

16

limited sclerodermia is associated with

anti-centromere antibodies

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MCC of death in scleroderma

pulmonary involvement

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Limited vs systemic slerodermia according the course

Limited has more benign clinical course

19

localized sclerodema - labs

antibodies against to DNA topoisomerase II

20

Raynaud phenomemenon - mechanism

decreased blood flow to the skin due to arteriolar ( small vessel) vasospasm in response to cold or strell

21

Raynaud phenomenon - presentation

color change from white ( ischemia) to blue ( hypoxia) to red ( reperfusion)
MOST oFTEN IN THE FINGERS

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Raynaud phenomenon - most often in (area of the body )

fingers and toes

23

Raynaud phenomenon - raynaud disease vs syndrome

disease --> when 1ry (idiopathic) syndrome --> 2ry to a disease process such as mixed connective tissue disease, SLE, CREST --> digital ulceration

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Raynaud disease vs syndrome according to digital ulceration

RAynaud syndrome

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Raynaud phenomeno -tx

Ca2+ channel blockers

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• What condition involves excessive fibrosis and collagen deposition, most commonly under the skin? What other systems can be involved?

Scleroderma; it also can involve sclerosis of the gastrointestinal, renal, pulmonary, and cardiovascular systems

27

• What are the classic symptoms of diffuse scleroderma?

Widespread skin involvement, rapid progression, early visceral involvement

28

• What are the two types of scleroderma? Which has a more benign clinical course?

Diffuse scleroderma and CREST syndrome; CREST is more benign

29

• You suspect a 58-year-old woman has diffuse scleroderma. What antibody test can you order to help support this diagnosis?

Anti-Scl-70 (anti-DNA topoisomerase I) antibody titers

30

• What does CREST stand for? Is it a limited or diffuse form of scleroderma?

Calcinosis, Raynaud phenomenon, Esophageal dysmotility, Sclerodactyly, Telangiectasia; limited

31

• A patient with Raynaud phenomenon tests positive for anticentromere antibodies. In what two body areas is skin involvement mostly seen?

The fingers and face (this patient has limited scleroderma)

32

• A patient has skin telangiectasias, Raynaud phenomenon, heartburn, and calcinosis on x-ray. What test do you order to confirm the diagnosis?

Anticentromere antibody titers (C is for CREST)

33

• A patient has Raynaud phenomenon and heartburn. What serum marker would be positive if she had the diffuse form of this disease?

Anti-Scl-70 (anti-DNA topoisomerase I) antibodies are seen in diffuse scleroderma (anticentromere antibodies in limited scleroderma)

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