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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
What are the 8 clinical manifestations of systemic sclerosis?
Raynaud's Cutaneous Oral Musculoskeletal GI Cardiac Pulmonary Renal
26
Occurs in almost all pts with systemic sclerosis
Raynaud's
27
Often the FIRST sign of systemic sclerosis
Raynaud's
28
Some patients have recurrent episodes associated with digital pitting scars, nail fold infarcts, or digital ulcers
Raynaud's
29
Diffuse, _________ __________ of the skin begins in fingers and upper extremities. Extends to involve upper arms, shoulders, neck, face
sclerotic atrophy
30
Early skin changes, starting with ____________ _________, often involve the whole hand and extremities
pitting edema
31
Edema is replaced by _____________ and ___________ of the skin, resulting in difficulty moving affected parts
tightening and hardening
32
Loss of blood supply leads to cutaneous ______________ and atrophic changes in fingers, including ________________
ulcerations; autoamputation
33
Hyperpigmentation, telangiectases, and subcutaneous ____________ may also occur, leading to _________ and severe cosmetic problems
calcifications; deformity
34
Sclerodactyly
Thick skin over fingers
35
En coup de sabre
Skin lesion on the middle forehead
36
Skin folds are lost around the mouth, giving a _____________ appearance to the face
mask-like
37
Lips become _________, and the oral aperture ______________
rigid; narrows
38
The ___________ can also become hard and rigid, making speaking and swallowing difficult
tongue
39
Involvement of the _____________ causes dysphagia
esophagus
40
When the soft tissue and TMJ are affected, they restrict movement of the ____________, causing _____________
mandible; pseudoankylosis
41
What is found on dental x-rays in pts with systemic sclerosis?
Uniform thickening of PDL Calcinosis
42
Where is uniform thickening of the PDL seen on dental x-rays?
Around posterior teeth
43
Where is calcinosis seen on dental x-rays?
Soft tissue around jaws
44
What happens to the mandible in pts with systemic sclerosis?
Pressure resorption
45
Where is pressure resorption of the mandible particularly apparent?
Angle of the mandible, at the attachment of the masseter
46
What occurs in ~70% of pts with systemic sclerosis?
Xerostomia
47
What occurs in ~15% of pts with systemic sclerosis?
Secondary Sjogren's
48
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?
Cheilosis
49
What is a common finding in pts with systemic sclerosis due to narrowing of the oral aperture and rigidity of the tongue?
Microstomia
50
What does microstomia complicate?
Dental tx Oral hygiene
51
What does "narrowing of the oral aperture" mean?
Reduced mouth opening
52
What can a pt with narrowing of the oral aperture do to increase opening?
Stretching exercises Bilateral commissurotomy
53
What musculoskeletal manifestation is associated with synoviocyte hypertrophy and is common in early stages?
Inflammation of the synovium
54
What musculoskeletal manifestations affect both small and large joints?
Polyarthralgias (pain in many joints) Morning stiffness
55
What do polyarthralgias and morning stiffness affect?
Small + large joints
56
Inflammatory joint pain with _________ ________ often appears to be true _________ and can lead to premature/incorrect diagnosis of RA
swollen fingers; synovitis
57
What can lead to a premature/incorrect diagnosis of RA in pts who actually have systemic sclerosis?
Inflammatory joint pain with swollen fingers
58
What is affected in 90% of pts with systemic sclerosis?
GI tract
59
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 ______________
esophagus
60
What happens to the lower 2/3rds of the esophagus in pts with systemic sclerosis?
Becomes inflexible (like a rubber hose)
61
What is the most common GI finding in pts with systemic sclerosis?
Distal esophageal motor dysfunction
62
What results from weakness and incoordination of SM that leads to dysphagia and gastroesophageal reflux?
Distal esophageal motor dysfunction
63
What does intestinal fibrosis lead to in pts with systemic sclerosis?
Severe intestinal malabsorption
64
What leads to severe intestinal malabsorption in pts with systemic sclerosis?
Intestinal fibrosis
65
What are the 3 clinical presentations of cardiac involvement in pts with systemic sclerosis?
Pericarditis Conduction problems (arrhythmias) Congestive heart failure
66
Patchy ___________ of the myocardium and conduction system with ___________ tissue occurs in most pts with cardiac involvement
replacement; fibrous
67
Why are the lungs affected in more than 50% of patients with systemic sclerosis?
Fibrosing alveolitis Obliterative vasculopathy
68
What is fibrosing alveolitis also referred to as?
Interstitial fibrosis
69
What does fibrosing alveolitis lead to?
Restrictive lung disease Obliterative vasculopathy
70
What type of pulmonary vessels are affected by obliterative vasculopathy?
Small + medium pulmonary vessels
71
What is obliterative vasculopathy associated with?
Pulmonary hypertension
72
What is the MOST COMMON cause of death in patients with systemic sclerosis?
Pulmonary disease
73
Is fibrosing alveolitis (interstitial fibrosis) more frequent and severe in pts with limited or diffuse systemic sclerosis?
Diffuse
74
In pts with severe fibrosing alveolitis (interstitial fibrosis), the greatest damage occurs during the first ___________ of illness, before there are pulmonary symptoms
5 years
75
What are the most prominent renal abnormalities in pts with systemic sclerosis?
Vascular lesions
76
Where do the most prominent renal vascular lesions occur?
Walls of interlobular renal arteries
77
What do the walls of interlobular renal arteries look like when there are vascular lesions?
Intimal thickening due to mucinous/collagenous material
78
What occurs in 30% of pts with diffuse systemic sclerosis and can progress to renal crisis?
Hypertension
79
What is hypertension secondary to in pts with systemic sclerosis?
Renal complications
80
10% of pts with diffuse systemic sclerosis have a _______ ________ that mimics malignant __________, with rapidly progressive renal failure
renal crisis; hypertension
81
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?
Thrombosis Infarction Fatal renal failure
82
What are present in 90-95% of pts with systemic sclerosis?
Antinuclear antibodies (ANAs)
83
What are the specific antinuclear antibodies (ANAs) found in systemic sclerosis?
Anti-topoisomerase I Anti-RNA polymerase I and III Anti-centromere AB
84
Which ANA is highly specific for diffuse systemic sclerosis?
Anti-topoisomerase I
85
What is anti-topoisomerase I highly specific for?
Diffuse systemic sclerosis
86
Which ANA is rarely found in limited systemic sclerosis?
Anti-topoisomerase I
87
Which ANA correlates w/ development of more aggressive disease with pulmonary fibrosis and peripheral vascular changes?
Anti-topoisomerase I
88
Which ANA correlates with rapid cutaneous involvement?
Anti-RNA polymerase I and III
89
Which ANA correlates with high frequency of renal crisis?
Anti-RNA polymerase I and III
90
Which ANA is found in 70% of pts with limited systemic sclerosis?
Anti-centromere AB
91
Which ANA is found in 70% of pts with diffuse systemic sclerosis?
Anti-topoisomerase I
92
Which ANA is found in 20% of pts with diffuse systemic sclerosis?
Anti-RNA polymerase I and III
93
Which ANA is found in 90% of pts with CREST syndrome?
Anti-centromere AB
94
Which ANA indicates a benign course?
Anti-centromere AB
95
Which ANA is rarely found in pts with diffuse systemic sclerosis?
Anti-centromere AB
96
Besides ANAs, what are other lab findings of pts with systemic sclerosis?
Anemia Elevated erythrocyte sedimentation rate (ESR) Hypergammaglobulinemia
97
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
Skin biopsy

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