PAGET’S DISEASE

Osteitis Deformans
Paget’s disease

Is a state of increase metabolic activity in

the bone characterized by excessive
bone remodeling,
both resorption and formation.
• Named after Sir James Paget (1814-1899)
• First described osteitis deformans in 1877
 Bone Remodeling Cycle

Bone Remodeling Cycle

Phase 1 (activation)
a stimulus activates the bone cells precursors in the
localized area of the bone to form osteoclasts.

Phase 2. (resorption)
osteoclast forms a “cutting cone” which gradually
resorb bone, leaving behind and elongated cavity.
Phase 3 (formation)
laying down of new bone by osteoblast lining the
walls of the resorptive cavity.
Etiology
The possible causes are many, but no exact
etiology has been defined.
– Genetic factor (Sequestrosome 1 gene on
chromosome 5)
– Virus infection may be necessary to trigger
– Chronic infection (Osteitis Deformans)
– Hormonal dysfunction
– Autoimmune states
Histological features
• Osteoclasts are enlarged
• Increased bone turnover produces
a mosaic pattern of lamellar bone
• Three phases recognised -
osteolytic, mixed and sclerotic
• Osteolytic or Destructive Phase
– Initial phase marked by extensive
resorption of existing bone

• Mixed or Active Phase

– Osteoclasts destroy the ordered lamellar
bone and osteoblast respond to the
destruction by rapid disposition of vascular
connective tissue and remodeled lamellar
bone

• Sclerotic or Osteoblastic Phase

– last phase wherein bone formation outstrips
resorption
 Paget’s disease
Pathophysiology
Etiology
Hormonal Hyperactivity of
Dysfunction osteoclasts
↑Ca ECF
Autoimmune
States Osteoclast destroys
Viral Causation lamellar bone
Chronic
Infection Increase extensive
resorption
Genetic Factor Compensatory
(Sequestrosome Mechanism
1 gene on ↑ osteoblast response
chromosome 5)
 Rapid disposition of
vascular connective
tissue

Increase bone formation

Increase bone size and

thickness
 Poorly Compressionof Overgrown
mineralized nerves bone
bones

• Bowing of legs • Bone pain •Skull

• Curve • Spinal stenosis enlargement
backbone • Numbness bitemporally
• Weakness and frontally
• (Skull) •Enlargemen
t of pelvis
headaches,
dizziness,
facial droop
with vision or
hearing
impairment
Signs & Symptoms
• There are usually no symptoms for a
prolonged period.
– If symptoms occur, they develop insidiously,
with pain, stiffness, fatigue, and bone
deformity.
– Bone pain is aching, deep, and occasionally
severe, sometimes worse at night.
– Pain also may arise from compression
neuropathy or osteoarthritis.
• Signs may include:

– Spine: curve backbone nerves can become

damaged and cause leg pain, numbness,
weakness, or cauda equina syndrome (an
emergency condition with symptoms that
include loss of feeling in the pelvic area and
legs)
• Skull: skull
enlargement
bitemporally and
frontally (frontal
“bossing”); dilated
scalp veins;
headaches,
dizziness, loss of
muscle strength in
the face (facial
droop), or problems
with vision or
hearing.
• Bowed legs, Enlarged
pelvis
Radiological features
• Osteolytic phase can produce osteoporosis
circumscripta (localized cranial
osteoporosis)
• Bone softening can produce bowing,
platybasia, protrusion acetabuli or greenstick
fractures
• Mixed phase shows generalized bone
enlargement
• Sclerotic phase shows increased density,
trabeculae and cortical thickening
Complications
• Pathological fractures - complete or
incomplete
• Neurological effects
– Cranial nerve lesions
– Spinal cord lesions

• Osteoarthritis
• Sarcomas
Biochemistry

• Serum calcium and phosphate are

usually normal
• Serum alkaline phosphatase is
increased
• Uric acid increased in about 30%
of patients
Diagnosis
• Plain x-rays and radioisotopes bone scan
– Increased bone sclerosis
– Abnormal architecture with coarse cortical
trabeculation or cortical thickening
– Bowing
– Bony enlargement
• Serum alkaline phosphatase, Ca, and PO4
• Bone scan after diagnosis established
Nursing Diagnosis
1. Acute pain related to impingement of abnormal
bone on spinal cord
2. Bathing/hygiene, dressing/grooming, toileting self-
care deficit related to musculoskeletal impairment
3. Disturbed body image related to bowing of legs.
4. Impaired physical mobility related to asymmetrical
bowing of tibia and femur