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Paget’s Disease of Bone
(Osteitis Deformans)
What is Paget’s disease?
Paget’s disease of bone is a
condition characterized by
abnormal and anarchic resorption
and deposition of bone, resulting in
distortion and weakening of the
affected bones
Bone consists of
Osteoblasts: Bone forming cells
Osteoclasts: Bone breaking cells
Osteocytes: Present in the matrix
Matrix: Collagen, Calcium, Osteocalcin
Bone remodeling
Bones commonly affected
Clinical & Radiographic Features
Pain in or over a bone
Joint pain and limited mobility
The shape of the bone may change
-the bone in the legs may bend or bow out
-the bone in the skull may get bigger
Maxillary disease is more common than mandibular
Lionlike facial deformity (leontiasis ossea)
Patchy sclerotic areas of Skull RG (cotton wool)
Bone Scan of Mandible may show Codylar to
condylar uptake(Black beard or Lincoln’s sign)
Clinical & Radiographic Features
Bone breaks more easily
In late stages hipjoint may damage
Hearing problems may occur because the bone
expands and heart problems may also occur
COTTON WOOL APPEARANCE
Histopathological Features
Uncontrolled alternating resorption and formation of bone
Basophilic reversal lines- indicate the junction between
alternating resorptive and formative phases result in a
jigsaw puzzle, or mosaic appearance of the bone
REVERSAL LINES
NORMAL SKULL DISEASED SKULL
SKULL SIZE IN PAGETS DISEASE
What causes Paget’s disease
Unknown; evidence favors an infection of
genetically predisposed individuals with a
measles virus of the paramyxovirus family
Studies have demonstrated that abnormal virus-
infected osteoclasts generate IL-6, a resorptive
cytokine from the bone marrow of patients with
Paget’s disease
How common is Paget’s disease
The exact number of Paget’s disease is not known
Men are more likely to be affected than woman
 It usually affects people over the age of 40 years
It’s estimated to affect 3% of people over the age of
40
Epidemiology
Prevalence
High: United Kingdom, United States, Australia,
New Zealand, Western Europe
Low: Scandinavia, China, Japan, India
Family History: positive in as many as 40% of patients
Treatment
At this time there is no cure for Paget’s
disease. Therefore treatment is designed to
control the symptoms and change the rate of
bone growth.
When symptomatic, bone pain is noted most
frequently and often may be controlled by
acetaminophen or nonsteroidal antiinfl
ammatory drugs (NSAIDs).
Treatment
Biphosphonates
heplfull in rebuilding bone
Inhibits bone resorption and mineralisation
by promoting apoptosis of osteoclasts
for example Didronel, Fosamex and
Actonel
Treatment
Calcitonin (Parathrmone Antogonist)
Slows bone loss and bone growth
Gives relieve of bone pain
Reduces skin temperature over affected bone
sites
For example acetaminophen (Tylenol, Panadol)
Treatment
Surgery
1. Fractures: to allow fractures to heal in better
position
2. Bone deformity: cutting and realigning of the
affected bones
3. Severe degenerative arthritis: joint replacement of
the hips, knees

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Pagets disease

  • 1. Paget’s Disease of Bone (Osteitis Deformans)
  • 2. What is Paget’s disease? Paget’s disease of bone is a condition characterized by abnormal and anarchic resorption and deposition of bone, resulting in distortion and weakening of the affected bones
  • 3. Bone consists of Osteoblasts: Bone forming cells Osteoclasts: Bone breaking cells Osteocytes: Present in the matrix Matrix: Collagen, Calcium, Osteocalcin
  • 6. Clinical & Radiographic Features Pain in or over a bone Joint pain and limited mobility The shape of the bone may change -the bone in the legs may bend or bow out -the bone in the skull may get bigger Maxillary disease is more common than mandibular Lionlike facial deformity (leontiasis ossea) Patchy sclerotic areas of Skull RG (cotton wool) Bone Scan of Mandible may show Codylar to condylar uptake(Black beard or Lincoln’s sign)
  • 7. Clinical & Radiographic Features Bone breaks more easily In late stages hipjoint may damage Hearing problems may occur because the bone expands and heart problems may also occur COTTON WOOL APPEARANCE
  • 8. Histopathological Features Uncontrolled alternating resorption and formation of bone Basophilic reversal lines- indicate the junction between alternating resorptive and formative phases result in a jigsaw puzzle, or mosaic appearance of the bone REVERSAL LINES
  • 9. NORMAL SKULL DISEASED SKULL SKULL SIZE IN PAGETS DISEASE
  • 10. What causes Paget’s disease Unknown; evidence favors an infection of genetically predisposed individuals with a measles virus of the paramyxovirus family Studies have demonstrated that abnormal virus- infected osteoclasts generate IL-6, a resorptive cytokine from the bone marrow of patients with Paget’s disease
  • 11. How common is Paget’s disease The exact number of Paget’s disease is not known Men are more likely to be affected than woman  It usually affects people over the age of 40 years It’s estimated to affect 3% of people over the age of 40
  • 12. Epidemiology Prevalence High: United Kingdom, United States, Australia, New Zealand, Western Europe Low: Scandinavia, China, Japan, India Family History: positive in as many as 40% of patients
  • 13. Treatment At this time there is no cure for Paget’s disease. Therefore treatment is designed to control the symptoms and change the rate of bone growth. When symptomatic, bone pain is noted most frequently and often may be controlled by acetaminophen or nonsteroidal antiinfl ammatory drugs (NSAIDs).
  • 14. Treatment Biphosphonates heplfull in rebuilding bone Inhibits bone resorption and mineralisation by promoting apoptosis of osteoclasts for example Didronel, Fosamex and Actonel
  • 15. Treatment Calcitonin (Parathrmone Antogonist) Slows bone loss and bone growth Gives relieve of bone pain Reduces skin temperature over affected bone sites For example acetaminophen (Tylenol, Panadol)
  • 16. Treatment Surgery 1. Fractures: to allow fractures to heal in better position 2. Bone deformity: cutting and realigning of the affected bones 3. Severe degenerative arthritis: joint replacement of the hips, knees