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NYCC MSP > Paget Disease > Flashcards

Paget Disease Flashcards

1
Q

What is another name for Paget disease?

A

Osteitis deformans

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2
Q

What is the most common metabolic disease worldwide?

A

osteoporosis

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3
Q

What is the 2nd most common metabolic disease worldwide?

A

Paget disease

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4
Q

What is Paget disease?

A

Bone remodeling disorder characterized by accelerated and abnormal remodeling:
- ^resorption of bone
- ^deposition of disorganized bone

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5
Q

What age group is generally effected by Paget disease?

A

> 50 years

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6
Q

What geographic areas have higher rates of Paget disease?

A

Northern European populations and areas with high immigration from Britain (eg. Australia, New Zealand, Canada, US)

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7
Q

What geographic areas have lower rates of Paget disease?

A
  • Asia
  • Middle East
  • Africa
  • South America
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8
Q

Paget disease is most likely to be found in which country?
A) Brazil
B) Turkey
C) Japan
D) Scotland

A

Scotland (northern europe)

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9
Q

What term implies that a bone disorder affects only one bone in the body?

A

monostotic

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10
Q

What term implies that a bone disorder affects multiple bones, but not all bones in the body?

A

polyostotic

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11
Q

What is the etiology of Paget disease?

A
  • mutations in genes encoding for proteins in RANK signaling pathway
  • infection with paramyxovirus (measles) activates genes
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12
Q

The RANK system induces ____ and ____

A

osteoclast activity
osteoclastogenensis

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13
Q

What changes to osteoclasts occur in Paget disease?

A
  • multinucleated (>100)
  • may contain inclusion bodies
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14
Q

What are inclusion bodies?

A

Virus-like particles that especially resemble paramyxoviruses (measles)

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15
Q

Most cases of Paget disease are (monostotic/polyostotic)

A

monostotic

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16
Q

Where is Paget disease commonly found in the body?

A

axial skeleton
- spine
- skull
- pelvis
- femur
- tibia
- humerus

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17
Q

Name the radiographic characteristics of Paget disease

A
  • bony expansion
  • cortical thickening
  • extensive trabecular patterns
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18
Q

How is Paget disease differentiated from metabolic diseases?

A

Paget disease is usually monostotic, unlike metabolic diseases which have generalized skeletal involvement

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19
Q

What diagnostic method would determine whether a case of Paget disease is monostotic or polyostotic?

A

bone scan (can look at all bones at once)

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20
Q

Name the stages of Paget disease?

A
  1. Lytic
  2. Mixed
  3. Blastic
  4. Malignant transformation (debated)
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21
Q

If Pagetic bone undergoes malignant transformation, it will become a(n) ____

A

osteosarcoma

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22
Q

Which stage of Paget disease is described as “hot” and why?

A

stage 1 - osteoclastic resorptive stage (lytic stage)

^vascularization of Pagetic bone

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23
Q

What occurs during stage 1 of Paget disease?

A
  • widespread osteolysis
  • marrow fibrosis (fibrous replacement of marrow)
  • dilation of marrow sinusoids
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24
Q

Marrow fibrosis in polyostotic Paget disease would increase the risk of ____

A

anemia (decreased blood cells)

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25
Q

How would stage 1 of Paget disease appear radiographically?

A

“blade of grass” or “candle flame”
(tapered appearance where there is less bone)

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26
Q

Bone scans show ____ activity, which occurs in reaction to ____ activity in Paget disease

A

blastic
lytic

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27
Q

How does stage 2 of Paget disease appear radiographically?

A
  • bones appear larger (bony expansion)
  • thickened cortex, accentuation of cancellous bone
  • “picture frame” appearance of vertebral bodies
  • protrusio acetabuli
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28
Q

What is protrusio acetabuli?

A

Acetabulum protrudes into pelvis, seen radiographically where the femoral head crosses the ilio-ischial line (Kohlers’s line)

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29
Q

In what stage of Paget disease is osteoclastic activity slowing down, while osteoblastic activity is catching up?

A

Stage 2: mixed osteoclastic & osteoblastic stage

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30
Q

What stage of Paget disease becomes a contraindication for adjusting and why?

A

Stage 2
bone softening –> deformities & ^Fx risk

31
Q

How can Paget disease be differentiated from metastasis?

A

metastasis doesn’t change size of bone, while Paget disease shows bony expansion

32
Q

In what stage(s) is Paget disease most commonly found?

A

Stages 2 & 3

33
Q

What stage of Paget disease is described as “cold” and why?

A

Stage 3: burnt-out (blastic) stage

Histologically, minimal osteoclastic activity

34
Q

What stage of Paget disease shows a mosaic pattern in bone with prominent and irregular basophilic-staining cement lines histologically?

A

Stage 3: burn-out (blastic) stage

35
Q

How does stage 3 of Paget disease appear radiographically?

A

thickened & disordered bone

36
Q

What stage of Paget disease is typically seen in severe polyostotic cases?

A

Stage 4: neoplastic transformation

37
Q

The lytic phase of Paget disease in the skull is called ____

A

osteoporosis circumscripta

38
Q

What is osteoporosis circumscripta? Where does it usually occur?

A

localized lysis in the skull (stage 1 pagets), usually frontal & parietal bones (calvarium)

39
Q

The mixed/blastic phases of Paget disease in the skull creates the appearance called ____

A

cotton wool skull

40
Q

Describe the appearance of the mixed/blastic stages of Paget disease in the skull.

A
  • thickening of inner & outer tables of calvarium
  • platybasia
  • basilar impression/invagination
  • misshapen jaw
  • tooth loss
  • Leontiasis Ossei
41
Q

What are some of the consequences of Paget disease in the skull?

A
  • increased cranial pressure –> headaches
  • basilar invagination/impression
  • platybasia –> can compress medulla/spinal cord
  • compress CN VIII –> hearing changes (tinitus)
  • cerebellar gait disturbances
  • “hat doesn’t fit”
42
Q

What is basilar invagination/impression?

A

Skull becomes too heavy and collapses on C1, so that the cervical spine is protruding into foramen magnum –> causing brain/spinal cord compression

43
Q

What is Leontiasis Ossei?

A

“lion-like face”
increase in facial bone size due to Paget disease in the skull

44
Q

What is platybasia?

A

flattening of the base of the skull

45
Q

How does Paget disease appear radiographically in the spine?

A
  • ivory vertebra
  • “picture frame” vertebrae (mixed/blastic stages)
46
Q

What is an ivory vertebra?

A

densely blastic vertebral body

47
Q

In Paget disease in the spine, bone gets bigger in what dimension?

A

laterally (A-P + M-L)
(bone softening, will compress bones, not allowing vertically)

48
Q

How would you form your differential diagnosis if you see “ivory vertebrae” on a radiograph?

A

IHOP:
- idiopathic (Dx of exclusion, must rule out the following)
- hodgkins lymphoma
- osteoblastic metastasis (no thickening/^size)
- Paget disease (picture frame vertebra)

49
Q

What is a possible consequence of Paget disease in the spine? How would this manifest clinically?

A
  • vertebral canal stenosis/narrowing (bone expands inward)
  • provoked in extension, feels better in flexion
50
Q

How does Paget disease appear in the tibia?

A
  • sabre shin (may affect gait)
  • pseudofractures
  • transverse fractures
51
Q

What term describes the course trabecular expansion creating the appearance of a curved anterior tibia?

A

sabre shin

52
Q

What term describes a “seam” of unmineralized osteoid?

A

pseudofracture

53
Q

Where do pseudofractures occur in Pagetic long bones?

A

convex side

54
Q

In what conditions might you see pseudofractures?

A

PROF:
- Paget disease
- Rickets
- Osteomalacia
- Fibrous dysplasia

55
Q

In a patient with pseudofractures, how is Rickets differentiated from Paget disease?

A

Rickets occurs in young population

56
Q

In a patient with pseudofractures, how is Osteomalacia differentiated from Paget disease?

A

osteopenic change in osteomalacia

57
Q

In a patient with pseudofractures, how is Fibrous dysplasia differentiated from Paget disease?

A

occurs on concave side in Fibrous dysplasia

58
Q

Transverse fractures are also called ____

A

banana fracture

59
Q

What are the general clinical manifestations of Paget disease?

A
  • frequently asymptomatic
  • bone pain in affected area
  • ^osteoarthritis when adjacent to joint
  • patho Fx
  • headaches
  • hearing loss
  • hat size changes
60
Q

What are the clinical manifestations of polyostotic Paget disease?

A
  • Pagetic steal
  • cardiomegaly
  • high output cardiac failure
61
Q

Why does Pagetic steal occur in Paget disease?

A

bone steals blood from rest of body –> can cause dizziness & gait disturbances

62
Q

Why does cardiomegaly occur in Paget disease?

A

bone increases demand for blood

63
Q

Why does high output cardiac failure occur in Paget disease?

A

heart can’t keep up with Pagetic bone demand

64
Q

What are the findings of diagnostic labs for Paget disease?

A
  • normal Ca and PO
  • ^alkaline phosphatase
  • ^serum & urinary hydroxyproline > hydroxylysine
65
Q

Hydroxyproline and hydroxylysine are large components of ____

A

collagen

66
Q

What levels of hydroxyproline and hydroxylysine would be found in diagnostic labs for Paget disease and why?

A
  • increased hydroxyproline > hydroxylysine
  • both are components of collagen
  • ^turnover of osteoid + destruction in Paget disease
67
Q

What is the treatment for Paget disease?

A
  • none, if asymptomatic
  • manage symptoms (Fx & osteoarthritis)
  • refer to rheumatology: osteoclast inhibitors (calcitonin & bisphosphonates)
68
Q

Pagetic steal is caused by the over proliferation of vasculature in dense, disorganized bone tissue causing patients to experience ____
A) orthostatic hypertension
B) hypercalcemia
C) orthostatic hypotension
D) spinal cord compression

A

orthostatic hypotension
(can cause dizziness & gait disturbances)

69
Q

Stage 1 of Paget disease is characterized by increased ____
A) osteoclastic activity
B) osteoclastic & osteoblastic activity
C) burn-out
D) necrosis

A

osteoclastic activity

70
Q

TRUE or FALSE:
Paget disease patients have an increased risk of developing a malignant bone tumor

A

true

71
Q

In what stage of Paget disease is “picture frame” vertebrae found?

A

mixed/blastic stages

72
Q

What % change is necessary to show on a Bone Scan?

A

3-5%

73
Q

What is an example of a bisphosphonate given for treatment of Paget disease?

A

Fosamax

NYCC MSP (28 decks)

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