PAGETS DISEASE

OF BONE

By:
Dr.Shaheen Ahmed
INTRODUCTION:
Chronic, non-metabolic bone disorder.

Characterized by aggressive bone resorption, formation

and remodeling an unbalanced derangement of normal
processes.

Results in bone deformity, structural weakness, altered

joint biomechanics.
EPIDEMIOLOGY:
A disease of middle to advanced age.

Rare below age 40.

Men>Women (1.6 : 1).

ETIOLOGY:
Unknown.
Proposed Theories

1) Viral
-Paramyxovirus, measles virus, RSV, canine distemper virus
-Viral particles seen in pagetic osteoclasts

2)Genetic
- 5-40% have 1st degree relative with Pagets

3)Environmental
-Arsenic
-Animals: cattle, dogs, etc.
PATHOPHYSIOLOGY:
Abnormal osteoclasts:
quantity
size
activity
# of nuclei

Aggressive, focal bone resorption makes large cavities in bone.

Leads to osteoblast recruitment and overactive osteoblastic

activity.
 Rapid, disorganized bone formation.

New bone is:

Coarse, disorganized, irregular, woven
Less resistant to forces
Prone to deformity and fracture
PHASES OF DISEASE:
1.) Osteoclastic/Resorptive Phase:

Bursts of osteoclastic activity causing bone resorption.

Lytic lesions, trabecular and cortical thinning.

Osteolytic fronts advance longitudinally from bone end

toward middle ( V or flameshaped).
2.) Osteoblastic/Sclerotic or Mixed Phase:

Mixed osteoclastic and osteoblastic activity.

Net activity is osteoblastic with deposition of structurally

abnormal bone.

Bone expansion, hyperostosis, osteosclerosis, heterogeneous

ossification.
3.) Late Burn Out Phase:

Activity.

End result: widened, heterogeneously ossified, but

generally sclerotic bones with irregular, thickened
trabeculae.
DISEASE LOCATION:
Monostotic (25%) or polyostotic (75%)

Typically affects
Pelvis (70%)
Femur (55%)
Lumbosacral spine (53%)
Skull (42%)
Tibia (32%)
Rarely in hands, feet

No bone-to-bone spread.
CLINICAL FEATURES:
Usually asymptomatic

Incidental finding.
Abnormal radiograph or other imaging.
Abnormal labs (AlkPhos)

Bone pain

Constant
Poorly localized
Present at rest
Worse on weight bearing
 Bone/limb deformity.

Fracture.

Arthropathy.

Malignant transformation.

Hypercalcemia.

Progressive enlargement of the maxilla or, much less frequently,

the mandible.

Spaced teeth.
 Skin temperature

Hypervascularity due to bone turnover activity.

bone turnover causes cardiac demands and may lead to high-
output heart failure.

Neurological complaints

Hearing loss/vertigo
Cranial nerve palsies
Spine involvement
HISTOPATHOLOGY:
Disorganized, immature trabecular architecture with irregular
cement lines (mosaic pattern).

Rimming osteoblasts and/or multinucleated osteoclasts.

DIAGNOSIS:
Clinical assessment.

Characteristic radiographic appearance.

Labs (Alk Phos).

Bone Scan.
MANAGEMENT:
(May require no treatment if asymptomatic)
SUPPORTIVE CARE:

Counseling for fall+fracture prevention.

Physical therapy.
Analgesics.
Weight control.
PHARMACOLOGICAL:

Bisphosphonates
Inhibits bone resorption and mineralisation by promoting
apoptosis of osteoclasts.

Calcitonin
Slows bone loss and bone growth.
Gives relieve of bone pain.
SURGICAL:

Few require surgery.

Corrective osteotomy of long bone deformity.
Arthroplasty (hip, knee).
Combination arthroplasty with osteotomy.
Fracture fixation
THANKYOU!