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Musculoskeletal Physiopathology > Paget Disease > Flashcards

Paget Disease Flashcards

1
Q

Paget disease is also called ___

A

osteitis deformans

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2
Q

What is Paget disease?

A

Bone remodeling disorder characterized by accelerated and abnormal remodeling

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3
Q

Paget disease features ___ resorption of bone and ___ bone deposition

A

excessive resorption of bone
excessive, disorganized bone deposition

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4
Q

What demographic is most commonly affected by Paget disease?

A
  • Generally, individuals >50 years of age
  • Common in Northern European populations (and places they’ve immigrated to)
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5
Q

Paget disease affects ___% of the world population over 50

A

3%

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6
Q

Paget disease has a very low incidence in…

A

Asia, Middle East, Africa, and South America

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7
Q

Is Paget disease a monostotic or polyostotic?

A

Can be either

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8
Q

What does it mean if Paget disease is monostotic?

A

Affects one bone, active while child is growing, often inactive after puberty

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9
Q

What does it mean if Paget disease is polyostotic?

A

Affects multiple bones and affects individual throughout life

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10
Q

Paget disease involves mutations in genes encoding for…

A

proteins in the RANK signaling pathway

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11
Q

Paget disease involves mutations in genes encoding for proteins in the RANK signaling pathway
What does the RANK system induce?

A

Induces osteoclast activity and osteoclastogenesis

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12
Q

What are some osteoclast changes that occur with Paget disease?

A
  • Above normal numbers of nuclei (Pagets >100 nuclei vs normal <12 nuclei)
  • May contain virus-like particles
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13
Q

Which bones of the axial skeleton may be affected by Paget disease?

A
  • Spine
  • Skull
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14
Q

Which bones of the appendicular skeleton may be affected by Paget disease?

A
  • Pelvis
  • Femur
  • Tibia
  • Humerus
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15
Q

What are the 3-4 stages of Paget disease?

A
  1. Lytic
  2. Mixed
  3. Blastic
  4. Malignant transformation
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16
Q

About ___% of patients with Paget reach the malignant transformation stage

A

1%

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17
Q

Stage 1 of Paget, the lytic stage, is also called…

A

osteoclastic resorptive stage/hot stage

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18
Q

In which stage of Paget disease will the following present?

  • Widespread osteolysis
  • Marrow fibrosis
  • Dilation of marrow sinusoids
  • “Blade of grass”/”candle flame” radiography
A

Stage 1 - osteoclastic resorptive stage

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19
Q

In which stage of Paget disease will the following present?

  • Bones appear larger radiographically
  • Thickened cortex, accentuation of cancellous bone
A

Stage 2 - mixed osteoclastic and osteoblastic stage

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20
Q

In which stage of Paget disease will the following present?

  • Histologically, minimal activity
  • Radiographically, thickened and disordered bone
A

Stage 3 - burnt out/blastic stage

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21
Q

What are some presentations of stage 1 Paget disease?

A
  • Widespread osteolysis
  • Marrow fibrosis
  • Dilation of marrow sinusoids
  • “Blade of grass” or “candle flame” radiographically
22
Q

What are some presentations of stage 2 Paget disease?

A
  • Larger bones in radiographs
  • Thickened cortex, accentuation of cancellous bone
23
Q

Stage 3 of Paget disease, the blastic stage is also known as…

A

Burnt-out/cold stage

24
Q

What are the histological differences found in stage 3 Paget disease bones?

A

Mosaic pattern in bone with irregular cement lines

25
Q

What stage of Paget disease is present if bone histologically displays a mosaic pattern with irregular cement lines?

A

Stage 3 - burnt-out/cold stage

26
Q

Neoplastic transformation of Paget disease is typically seen in which cases?

A

Severe polyostotic cases

27
Q

Which types of neoplasm may occur in Paget disease?

A
  • Osteosarcoma
  • Chondrosarcoma
  • Fibrosarcoma
28
Q

What stage of Paget disease will present osteoporosis circumscripta in the skull?

A

Stage 1 (lytic phase)

29
Q

What is osteoporosis circumscripta?

A

Localized lysis in the skull, usually frontal and parietal bones
Occurs during phase 1 of Paget disease

30
Q

If the following has occurred to the skull, what phase of Paget disease is present?

  • Thickening of inner and outer tables
  • Platybasia
  • Skull is heavy and collapses on C1
A

Mixed/blastic phases: cotton wool skull

31
Q

If the following has occurred to the skull, what phase of Paget disease is present?

  • Misshapen jaw
  • Tooth loss
  • Facial bones may increase in size
A

Mixed/blastic phase: cotton wool skull

32
Q

Mixed/blastic phase of Paget disease presenting cotton wool skull may display platybasia
What is platybasia?

A

Flattening of the base of the skull; can compress medulla or spinal cord

33
Q

Mixed/blastic phase of Paget disease presenting cotton wool skull may make the skull too heavy and collapse on C1
What is a direct consequence of this?

A

Brain or spinal cord compression

34
Q

Mixed/blastic phase of Paget disease may present with leontiasis ossei
What is leontiasis ossei?

A

Lion face; facial bones increase in size

35
Q

What is the IHOP mnemonic for ivory vertebrae?

A

Idiopathic
Hodgkins lymphoma
Osteoblastic metastasis
Paget’s disease

36
Q

What are possible presentations of the spine with Paget disease?

A
  • Ivory vertebra
  • Picture frame vertebra
  • Possible spinal stenosis (narrowing)
37
Q

Paget disease can cause vertebral canal stenosis/narrowing
What is the direct result of this?

A

Spinal cord compression

38
Q

What is the deformity of tibia seen in Paget disease?

A

Sabre shin: coarse trabecular expansion at anterior tibia

39
Q

What kinds of fractures occur to a tibia with Paget disease?

A
  • Pseudofractures (convex side)
  • Transverse fractures (banana fracture)
40
Q

Pagetic bone is highly vascular bone
What are some results of this?

A
  • Pagetic steal (paraparesis or paraplegia)
  • Cardiomegaly
  • High output cardiac failure
41
Q

What are some clinical manifestations of Paget disease?

A
  • Frequently asymptomatic
  • Pain in the affected area
  • Increased osteoarthritis
  • Pathologic fracture
  • Headaches
  • Hearing loss
  • Hat size changes
42
Q

A patient complains of headaches, hearing loss, and mentions that their hat size has changed
What disease is suspected?

A

Paget disease

43
Q

With Paget disease, serum calcium and phosphate levels are:

A

normal

44
Q

With Paget disease, serum alkaline phosphatase levels are ___

A

elevated

45
Q

With Paget disease, ___ is elevated in urine

A

hydroxyproline

46
Q

With Paget disease, ___ is elevated in serum

A

hydroxylysine

47
Q

What symptoms are managed when a patient has Paget disease?

A

Fractures and osteoarthritis

48
Q

What are some pharmaceutical treatments for Paget disease?

A

Osteoclast inhibitors:

  • Calcitonin
  • Biphosphonates like Fosamax
49
Q

Pagetic steal is caused by the over proliferation of vasculature in dense disorganized bone tissue causing patients to experience ___

A

spinal cord compression

50
Q

Stage 1 of Paget disease is characterized by increased ___

A

osteoclastic activity

51
Q

True or False:
Paget disease patients have an increased risk of developing a malignant bone tumor

A

True, but rarely

Musculoskeletal Physiopathology (15 decks)

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