What is Paget¶s Disease

‡ Paget¶s disease of bone is a chronic disorder of

the skeleton in which areas of bone undergo
abnormal turnover , resulting in areas of enlarged
and softened bone.
‡ or
‡ It is a disorder that involves abnormal destruction
and regrowth, which results in deformity.

‡ The disease named after SIR JAMES PAGET , the

British surgeon who first described in 1877.

‡ Previously called ?

 ? now

preferred term is
   
 °AUSES
The cause of Paget's disease is unknown. The disorder
tends to run in families, and recent information suggest a
possible contribution of group of genetic defects. Also, some
evidence suggests that a virus is involved. Even if a virus is
involved, there is no evidence that the disorder is contagious.
‡ u
Paget disease may be caused by a Õ

Õ

½paramyxoviruses) present for
many yrs before symptoms appear.

‡  

There is also a hereditary factor.
 Pathogenesis ½cont)
‡ The pathogenesis of Paget's disease is
described in 3 stages which are:

‡ i. Osteoclastic or osteolytic stage.

‡ ii. Mixed osteoclastic-osteoblastic stage.

‡ iii. Exhaustive ½burnt out) stage.

‡ Normally, cells that break down old bone ½osteoclasts) and cells that
form new bone ½osteoblasts) work in balance to maintain bone
structure and integrity.

‡
 
Osteoclastic activity increases results in bone destruction.

‡
 
Both osteoclasts and osteoblasts become overactive in some
areas of bone, and the rate at which bone is broken down and
rebuilt increases tremendously in involved areas. The overactive
areas enlarge but, despite being large, are structurally abnormal and
weak.

‡
 
Both osteoclastic and osteoblastic activity ceases the bone is
sclerotic , weak and brittle
 ŒISTOPATŒOLOGY
‡ In this picture the old bone and new bone
are deposited and junction of both new
and old bone show prominent cement
lines , appearing as typicall mosaic pattern
pathognomonic of disease , the areas
between the bones are fibrous from which
bone has been resorbed.
 Symptoms
‡ Many patients do not know they have Paget's disease because they
have a mild case with no symptoms. Sometimes, symptoms may be
confused with those of arthritis or other disorders. In other cases,
the diagnosis is made only after complications have developed.
Symptoms can include:

‡ Bone pain is the most common symptom. Bone pain can occur in
any bone affected by Paget's disease. It often localizes to areas
adjacent to the joints.

‡ Œeadaches and hearing loss may occur when Paget's disease

affects the skull.

‡ Pressure on nerves may occur when Paget's disease affects the

skull or spine.
‡ Increased head size, bowing of limb, or curvature of
spine may occur in advanced cases.

‡ Œip pain may occur when Paget's disease affects

the pelvis or thighbone.

‡ Damage to joint cartilage may lead to osteoarthritis.

‡ °halkstick fractures.

‡ Œypercementosis in teeth may occur.

AP radiograph of the hip in a patient with paget disease
demonstrates dense sclerosis involving the femoral head
and neck. This is a high risk area for insufficiency
fracture.
 £
 
 

 
 
  
 

Lateral radiograph of the calvarium in a patient with paget

disease reveals multiple patches of sclerotic bone in the
calvarium ½ cotton wool appearance)
 Diagnosis
‡ Paget's disease may be diagnosed using one or more of the
following tests:

‡ Paget bone has a characteristic appearance on X-rays. A skeletal

survey is therefore indicated.

‡ Bone scans are useful in determining the extent and activity of the
condition.
‡
‡ Bone biopsy.

‡ £   

Elevated urinary hydroxyproline

‡ Elevated levels of alkaline phosphatase
‡ °alcium and phosphorus usually normal
 TREATMENT / MANAGEMENT

‡ Therapy
‡ °alcitonin
‡ Oral biphosphonates

‡ Supportive braces

‡ Response to therapy
‡ Alkaline phosphatase and urinary hydroxyproline
determinations.
‡ Orthopedic surgery may be need to correct a
deformity in severe cases.
Oral & Facial
findings