Paget Disease

Question 1

Paget disease is also called ___

Answer 1

osteitis deformans

Question 2

What is Paget disease?

Answer 2

Bone remodeling disorder characterized by accelerated and abnormal remodeling

Question 3

Paget disease features ___ resorption of bone and ___ bone deposition

Answer 3

excessive resorption of bone
excessive, disorganized bone deposition

Question 4

What demographic is most commonly affected by Paget disease?

Answer 4

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

Question 5

Paget disease affects ___% of the world population over 50

Answer 5

3%

Question 6

Paget disease has a very low incidence in ___

Answer 6

Asia, Middle East, Africa, and South America

Question 7

Is Paget disease monostotic or polyostotic?

Answer 7

Can be either

Question 8

What does it mean if Paget disease is monostotic?

Answer 8

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

Question 9

What does it mean if Paget disease is polyostotic?

Answer 9

Affects multiple bones and affects individual throughout life

Question 10

Paget disease involves mutations in genes encoding for ___

Answer 10

proteins in the RANK signaling pathway

Question 11

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

Answer 11

Induces osteoclastic activity and osteoclastogenesis

Question 12

What are some osteoclast changes that occur with Paget disease?

Answer 12

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

Question 13

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

Answer 13

• Spine
• Skull

Question 14

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

Answer 14

• Pelvis
• Femur
• Tibia
• Humerus

Question 15

What are the 3-4 stages of Paget disease?

Answer 15

1. Lytic
2. Mixed
3. Blastic
4. Malignant transformation

Question 16

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

Answer 16

1%

Question 17

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

Answer 17

osteoclastic resorptive stage/hot stage

Question 18

In which stage of Paget disease will the following present?

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

Answer 18

Stage 1 - osteoclastic resorptive stage

Question 19

In which stage of Paget disease will the following present?

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

Answer 19

Stage 2 - mixed osteoclastic and osteoblastic stage

Question 20

In which stage of Paget disease will the following present?

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

Answer 20

Stage 3 - burnt out/blastic stage

Question 21

What are some presentations of stage 1 Paget disease?

Answer 21

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

Question 22

What are some presentations of stage 2 Paget disease?

Answer 22

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

Question 23

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

Answer 23

Burnt-out/cold stage

Question 24

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

Answer 24

Mosaic pattern in bone with irregular cement lines

Question 25

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

Answer 25

Stage 3 - burnt-out/cold stage

Question 26

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

Answer 26

Severe polyostotic cases

Question 27

Which types of neoplasm may occur in Paget disease?

Answer 27

* Osteosarcoma * Chondrosarcoma * Fibrosarcoma

Question 28

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

Answer 28

Stage 1 (lytic phase)

Question 29

What is osteoporosis circumscripta?

Answer 29

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

Question 30

If the following has occurred to the skull, which phase of Paget disease is present? * Thickening of inner and outer tables * Platybasia * Skull is heavy and collapses on C1

Answer 30

Mixed/blastic phases: cotton wool skull

Question 31

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

Answer 31

Mixed/blastic phase: cotton wool skull

Question 32

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

Answer 32

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

Question 33

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?

Answer 33

Brain or spinal cord compression

Question 34

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

Answer 34

Lion face; facial bones increase in size

Question 35

What is the IHOP mnemonic for ivory vertebrae?

Answer 35

Idiopathic Hodgkins lymphoma Osteoblastic metastasis Paget's disease

Question 36

What are possible presentations of the spine with Paget disease?

Answer 36

* Ivory vertebra * Picture frame vertebra * Possible spinal stenosis (narrowing)

Question 37

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

Answer 37

Spinal cord compression

Question 38

What is the deformity of tibia seen in Paget disease?

Answer 38

Sabre shin: coarse trabecular expansion at anterior tibia

Question 39

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

Answer 39

* Pseudofractures (convex side) * Transverse fractures (banana fracture)

Question 40

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

Answer 40

* Pagetic steal (paraparesis or paraplegia) * Cardiomegaly * High output cardiac failure

Question 41

What are some clinical manifestations of Paget disease?

Answer 41

* Frequently asymptomatic * Pain in the affected area * Increased osteoarthritis * Pathologic fracture * Headaches * Hearing loss * Hat size changes

Question 42

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

Answer 42

Paget disease

Question 43

With Paget disease, serum calcium and phosphate levels are ___

Answer 43

normal

Question 44

With Paget disease, serum alkaline phosphatase levels are ___

Answer 44

elevated

Question 45

With Paget disease, ___ is elevated in urine

Answer 45

hydroxyproline

Question 46

With Paget disease, ___ is elevated in serum

Answer 46

hydroxylysine

Question 47

What symptoms are managed when a patient has Paget disease?

Answer 47

Fractures and osteoarthritis

Question 48

What are some pharmaceutical treatments for Paget disease?

Answer 48

Osteoclast inhibitors: * Calcitonin * Biphosphonates like Fosamax

Question 49

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

Answer 49

spinal cord compression

Question 50

Stage 1 of Paget disease is characterized by increased ___

Answer 50

osteoclastic activity

Question 51

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

Answer 51

True, but rarely