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AU'18 Oral Path II > Developmental and Immune-mediated Mucocutaneous Conditions - part IV > Flashcards

Developmental and Immune-mediated Mucocutaneous Conditions - part IV Flashcards

1
Q

T/F: systemic sclerosis is common

A

false, rare

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2
Q

systemic sclerosis

A

characterized by inappropriate deposition of dense collagen

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3
Q

etiology of systemic sclerosis

A

probably immunologically mediated

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4
Q

systemic sclerosis is also termed what?

A

scleroderma

sclero = hard
derma = skin
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5
Q

clinical features of systemic sclerosis

A
  1. diffuse, hard, smooth texture of skin
  2. Raynaud phenomenon
  3. sclerodactyly
  4. acro-osteolysis ± ulceration
  5. “mask-like” face
  6. atrophy of alae
  7. mouse facies
  8. microstomia “purse-string” appearance
  9. dysphagia with esophageal involvement
  10. pulmonary, renal, cardiac and GI fibrosis may be seen, with pulmonary hypertension
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6
Q

sclerodactyly

A

claw-like deformity

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7
Q

acro-osteolysis

A

resorption terminal phalanges…

destruction of digit tips, including bone

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8
Q

mouse facies

A

pinched appearance of nose

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9
Q

which sex is 3x more frequently affected by systemic sclerosis?

A

women 3x more than men

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10
Q

Raynaud’s phenomenon

A

discoloraton of fingers and/or toes after exposure to changes in temperature or emotional events causing spasm of blood vessels

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11
Q

T/F: Raynaud’s phenomenon is specific for systemic sclerosis

A

false, non specific

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12
Q

white discoloration of fingers and/or toes due to Raynaud’s phenomenon is caused by what?

A

spasm of blood vessels causes diminished blood supply

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13
Q

blue discoloration of fingers and/or toes due to Raynaud’s phenomenon is caused by what?

A

due to lack of oxygen

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14
Q

red discoloration of fingers and/or toes due to Raynaud’s phenomenon is caused by what?

A

as blood rushes back in

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15
Q

sclerodactyly is due to what?

A

collagen deposition, ulceration of fingertips

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16
Q

radiographic features of systemic sclerosis

A
  1. diffuse widening of periodontal ligament
  2. resorption of posterior ramus, condyle, coronoid process or chin
  3. may see root resorption
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17
Q

resorption of posterior ramus, condyle, coronoid process or chin is seen in what percent of systemic sclerosis pts?

A

10-20%

18
Q

histo features of systemic sclerosis

A

diffuse deposition of collagen throughout the superficial CT

19
Q

what is suggestive in dx’ing systemic sclerosis?

A

generally stiffened skin with development of Raynaud’s phenomenon suggestive of dx

20
Q

how is systemic sclerosis dx’ed?

A
  1. skin biopsy
  2. lab studies
  3. serologic studies
21
Q

serologic studies show that pts with systemic sclerosis have autoantibodies directed against what?

A

Scl-70

22
Q

serologic studies show what for pts with limited cutaneous systemic sclerosis?

A

anticentromere antibodies

23
Q

T/F: tx for systemic sclerosis is difficult and mainly symptomatic

A

true

24
Q

several drugs are used to do what in tx’ing symptoms of Raynaud’s phenomenon?

A
  1. used to inhibit collagen production

2. increase peripheral blood flow

25
Q

T/F: steroids are indicated for tx’ing Raynaud’s phenomenon

A

false, doesn’t seem to help

26
Q

what is used to tx hypertension especially renal in pts with systemic sclerosis?

A

ACE inhibitors

27
Q

tx for systemic sclerosis

A
  1. esophageal dilatation
  2. oral hygiene instruction
  3. fabricate prostheses with design to accommodate microstomia
28
Q

death from systemic sclerosis is due to what?

A

internal organ deposition of collagen

29
Q

T/F: cardiac involvement in pts with systemic sclerosis has a worse prognosis

A

true

30
Q

most deaths from systemic sclerosis is due to what involvement?

A

pulmonary

31
Q

what is the 10-year survival rate for limited cutaneous involvement in systemic sclerosis pts?

A

75-80%

32
Q

what is the 10-year survival rate for diffuse cutaneous involvement in systemic sclerosis pts?

A

55-60%

33
Q

CREST syndrome

A 
Calcinosis cutis
Raynaud's phenomenon
Esophageal dysfunction
Sclerodactyly
Telangiectasia
34
Q

what is a milder variant of systemic sclerosis?

A

CREST syndrome

35
Q

who is affected by CREST syndrome?

A

women, 6-7th decade

36
Q

calcinotis cutis

A

subcutaneous nodules are deposition of calcium salts

37
Q

tx of CREST syndrome if symptomatic

A

similar to systemic sclerosis

38
Q

T/F: px of CREST syndrome is worse than systemic sclerosis

A

false, better than systemic sclerosis

39
Q

what is the 6-year survival rate for pts with CREST syndrome?

A

80%

40
Q

what is the 12-year survival rate for pts with CREST syndrome?

A

50%

AU'18 Oral Path II (31 decks)

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