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D2 Fall > Gen Path Exam 3 - Autoimmune Disease - Systemic Sclerosis > Flashcards

Gen Path Exam 3 - Autoimmune Disease - Systemic Sclerosis Flashcards

1
Q

Another name for systemic sclerosis

A

Scleroderma

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2
Q

Chronic autoimmune disease of unknown etiology, characterized by diffuse fibrosis, degenerative changes, and vascular abnormalities in the skin, articular structures and other organs (kidneys, lung, heart, gastrointestinal and skeletal
muscles)

A

Systemic sclerosis

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3
Q

T/F: Systemic sclerosis can range from a mild disease, affecting only the skin, to a systemic disease that can cause death in a few months

A

True

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4
Q

What age range does systemic sclerosis effect?

A

16-40 yrs
40-75 yrs

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5
Q

When do symptoms of systemic sclerosis appear?

A

30s to 50s

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6
Q

Does systemic sclerosis affect men or women more frequently?

A

Women

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7
Q

Is there a genetic component to systemic sclerosis?

A

Yes, but it is rare

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8
Q

2 major forms of systemic sclerosis

A

Limited (localized)
Diffuse (progressive)

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9
Q

Limited (localized) or diffuse (progressive)?

Affects 80% of pts with systemic sclerosis

A

Limited (localized)

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10
Q

Limited (localized) or diffuse (progressive)?

Involves skin on fingers, forearms, and face

A

Limited (localized)

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11
Q

Limited (localized) or diffuse (progressive)?

If there is visceral/systemic involvement, it is mild and occurs late in disease process

A

Limited (localized)

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12
Q

Limited (localized) or diffuse (progressive)?

Clinical course is benign with minimal systemic involvement

A

Limited (localized)

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13
Q

Limited (localized) or diffuse (progressive)?

CREST syndrome

A

Limited (localized)

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14
Q

What antibodies is CREST syndrome associated with?

A

Anticentromere antibodies

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15
Q

What does CREST syndrome stand for?

A

subcutaneous Calcinosis
Raynaud’s phenomenon
Esophageal dysfunction
Sclerodactyly
Telangiectasia (spider veins)

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16
Q

Common form of limited (localized) systemic sclerosis

A

CREST syndrome

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17
Q

Limited (localized) or diffuse (progressive)?

Multisystem disease characterized by inflammation + fibrosis of many organs

A

Diffuse (progressive)

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18
Q

Limited (localized) or diffuse (progressive)?

Affects 20% of pts with systemic sclerosis

A

Diffuse (progressive)

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19
Q

Limited (localized) or diffuse (progressive)?

Constitutional symptoms, arthritis, carpal tunnel, swelling of hands and legs

A

Diffuse (progressive)

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20
Q

Limited (localized) or diffuse (progressive)?

Acute onset

A

Diffuse (progressive)

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21
Q

Limited (localized) or diffuse (progressive)?

Widespread skin thickening from fingers to trunk

A

Diffuse (progressive)

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22
Q

Limited (localized) or diffuse (progressive)?

Internal organ involvement including GI, pulmonary fibrosis, cardiac failure, renal failure

A

Diffuse (progressive)

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23
Q

Limited (localized) or diffuse (progressive)?

Overlapping with systemic lupus, Sjogren’s, RA, and dermatomyositis

A

Diffuse (progressive)

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24
Q

Describe the postulated sequence of events that may lead to systemic sclerosis

A
  1. Endothelial cell injury in small arteries
  2. T cells migrate to perivascular tissue
  3. CD4 cells respond to self antigen
  4. CD4 cells make cytokines: TGF-beta, IL-13, PDGF
  5. Repeated endothelial injury and platelet aggregation leads to proliferation and fibrosis
  6. Ischemic injury
  7. B cell activation: hypergammaglobulinemia + ANAs
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25
Q

What are the 8 clinical manifestations of systemic sclerosis?

A

Raynaud’s
Cutaneous
Oral
Musculoskeletal
GI
Cardiac
Pulmonary
Renal

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26
Q

Occurs in almost all pts with systemic sclerosis

A

Raynaud’s

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27
Q

Often the FIRST sign of systemic sclerosis

A

Raynaud’s

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28
Q

Some patients have recurrent episodes associated with digital pitting scars, nail fold infarcts, or digital ulcers

A

Raynaud’s

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29
Q

Diffuse, _________ __________ of the skin begins in fingers and upper extremities. Extends to involve upper arms, shoulders, neck, face

A

sclerotic atrophy

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30
Q

Early skin changes, starting with ____________ _________, often involve the whole hand and extremities

A

pitting edema

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31
Q

Edema is replaced by _____________ and ___________ of the skin, resulting in difficulty moving affected parts

A

tightening and hardening

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32
Q

Loss of blood supply leads to cutaneous ______________ and atrophic changes in fingers, including ________________

A

ulcerations; autoamputation

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33
Q

Hyperpigmentation, telangiectases, and subcutaneous ____________ may also occur, leading to _________ and severe cosmetic problems

A

calcifications; deformity

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34
Q

Sclerodactyly

A

Thick skin over fingers

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35
Q

En coup de sabre

A

Skin lesion on the middle forehead

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36
Q

Skin folds are lost around the mouth, giving a _____________ appearance to the face

A

mask-like

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37
Q

Lips become _________, and the oral aperture ______________

A

rigid; narrows

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38
Q

The ___________ can also become hard and rigid, making speaking and swallowing difficult

A

tongue

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39
Q

Involvement of the _____________ causes dysphagia

A

esophagus

40
Q

When the soft tissue and TMJ are affected, they restrict movement of the ____________, causing _____________

A

mandible; pseudoankylosis

41
Q

What is found on dental x-rays in pts with systemic sclerosis?

A

Uniform thickening of PDL
Calcinosis

42
Q

Where is uniform thickening of the PDL seen on dental x-rays?

A

Around posterior teeth

43
Q

Where is calcinosis seen on dental x-rays?

A

Soft tissue around jaws

44
Q

What happens to the mandible in pts with systemic sclerosis?

A

Pressure resorption

45
Q

Where is pressure resorption of the mandible particularly apparent?

A

Angle of the mandible, at the attachment of the masseter

46
Q

What occurs in ~70% of pts with systemic sclerosis?

A

Xerostomia

47
Q

What occurs in ~15% of pts with systemic sclerosis?

A

Secondary Sjogren’s

48
Q

What can occur at the corners of the mouth from riboflavin deficiency as a result of the GI malabsorption that can happen with systemic sclerosis?

A

Cheilosis

49
Q

What is a common finding in pts with systemic sclerosis due to narrowing of the oral aperture and rigidity of the tongue?

A

Microstomia

50
Q

What does microstomia complicate?

A

Dental tx
Oral hygiene

51
Q

What does “narrowing of the oral aperture” mean?

A

Reduced mouth opening

52
Q

What can a pt with narrowing of the oral aperture do to increase opening?

A

Stretching exercises
Bilateral commissurotomy

53
Q

What musculoskeletal manifestation is associated with synoviocyte hypertrophy and is common in early stages?

A

Inflammation of the synovium

54
Q

What musculoskeletal manifestations affect both small and large joints?

A

Polyarthralgias (pain in many joints)
Morning stiffness

55
Q

What do polyarthralgias and morning stiffness affect?

A

Small + large joints

56
Q

Inflammatory joint pain with _________ ________ often appears to be true _________ and can lead to premature/incorrect diagnosis of RA

A

swollen fingers; synovitis

57
Q

What can lead to a premature/incorrect diagnosis of RA in pts who actually have systemic sclerosis?

A

Inflammatory joint pain with swollen fingers

58
Q

What is affected in 90% of pts with systemic sclerosis?

A

GI tract

59
Q

In systemic sclerosis, progressive atrophy and fibrous replacement of the muscularis may develop at any level of the gut but are most severe in the ______________

A

esophagus

60
Q

What happens to the lower 2/3rds of the esophagus in pts with systemic sclerosis?

A

Becomes inflexible (like a rubber hose)

61
Q

What is the most common GI finding in pts with systemic sclerosis?

A

Distal esophageal motor dysfunction

62
Q

What results from weakness and incoordination of SM that leads to dysphagia and gastroesophageal reflux?

A

Distal esophageal motor dysfunction

63
Q

What does intestinal fibrosis lead to in pts with systemic sclerosis?

A

Severe intestinal malabsorption

64
Q

What leads to severe intestinal malabsorption in pts with systemic sclerosis?

A

Intestinal fibrosis

65
Q

What are the 3 clinical presentations of cardiac involvement in pts with systemic sclerosis?

A

Pericarditis
Conduction problems (arrhythmias)
Congestive heart failure

66
Q

Patchy ___________ of the myocardium and conduction system with ___________ tissue occurs in most pts with cardiac involvement

A

replacement; fibrous

67
Q

Why are the lungs affected in more than 50% of patients with systemic sclerosis?

A

Fibrosing alveolitis
Obliterative vasculopathy

68
Q

What is fibrosing alveolitis also referred to as?

A

Interstitial fibrosis

69
Q

What does fibrosing alveolitis lead to?

A

Restrictive lung disease
Obliterative vasculopathy

70
Q

What type of pulmonary vessels are affected by obliterative vasculopathy?

A

Small + medium pulmonary vessels

71
Q

What is obliterative vasculopathy associated with?

A

Pulmonary hypertension

72
Q

What is the MOST COMMON cause of death in patients with systemic sclerosis?

A

Pulmonary disease

73
Q

Is fibrosing alveolitis (interstitial fibrosis) more frequent and severe in pts with limited or diffuse systemic sclerosis?

A

Diffuse

74
Q

In pts with severe fibrosing alveolitis (interstitial fibrosis), the greatest damage occurs during the first ___________ of illness, before there are pulmonary symptoms

A

5 years

75
Q

What are the most prominent renal abnormalities in pts with systemic sclerosis?

A

Vascular lesions

76
Q

Where do the most prominent renal vascular lesions occur?

A

Walls of interlobular renal arteries

77
Q

What do the walls of interlobular renal arteries look like when there are vascular lesions?

A

Intimal thickening due to mucinous/collagenous material

78
Q

What occurs in 30% of pts with diffuse systemic sclerosis and can progress to renal crisis?

A

Hypertension

79
Q

What is hypertension secondary to in pts with systemic sclerosis?

A

Renal complications

80
Q

10% of pts with diffuse systemic sclerosis have a _______ ________ that mimics malignant __________, with rapidly progressive renal failure

A

renal crisis; hypertension

81
Q

In pts that have a renal crisis, renal vascular alterations are more pronounced and associated with fibrinoid necrosis of arterioles. What 3 things does this lead to?

A

Thrombosis
Infarction
Fatal renal failure

82
Q

What are present in 90-95% of pts with systemic sclerosis?

A

Antinuclear antibodies (ANAs)

83
Q

What are the specific antinuclear antibodies (ANAs) found in systemic sclerosis?

A

Anti-topoisomerase I
Anti-RNA polymerase I and III
Anti-centromere AB

84
Q

Which ANA is highly specific for diffuse systemic sclerosis?

A

Anti-topoisomerase I

85
Q

What is anti-topoisomerase I highly specific for?

A

Diffuse systemic sclerosis

86
Q

Which ANA is rarely found in limited systemic sclerosis?

A

Anti-topoisomerase I

87
Q

Which ANA correlates w/ development of more aggressive disease with pulmonary fibrosis and peripheral vascular changes?

A

Anti-topoisomerase I

88
Q

Which ANA correlates with rapid cutaneous involvement?

A

Anti-RNA polymerase I and III

89
Q

Which ANA correlates with high frequency of renal crisis?

A

Anti-RNA polymerase I and III

90
Q

Which ANA is found in 70% of pts with limited systemic sclerosis?

A

Anti-centromere AB

91
Q

Which ANA is found in 70% of pts with diffuse systemic sclerosis?

A

Anti-topoisomerase I

92
Q

Which ANA is found in 20% of pts with diffuse systemic sclerosis?

A

Anti-RNA polymerase I and III

93
Q

Which ANA is found in 90% of pts with CREST syndrome?

A

Anti-centromere AB

94
Q

Which ANA indicates a benign course?

A

Anti-centromere AB

95
Q

Which ANA is rarely found in pts with diffuse systemic sclerosis?

A

Anti-centromere AB

96
Q

Besides ANAs, what are other lab findings of pts with systemic sclerosis?

A

Anemia
Elevated erythrocyte sedimentation rate (ESR)
Hypergammaglobulinemia

97
Q

Where would you find the following from a pt with systemic sclerosis?

Compact collagen in reticular dermis
Hyalinization + fibrosis of arterioles
Thin epidermis
Loss of rete pegs
Atrophy of dermal appendages
Lots of mononuclear cells

A

Skin biopsy

D2 Fall (37 decks)

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