Paget's disease

1. Paget's disease
INTRODUCTION
 Paget’s disease is a chronic condition of bone characterized by disorder of the normal
bone remodeling process.
 Characterized by excessive breakdown of bone tissue followed by abnormal bone
formation.
 Also known as Osteitis Deformans is a bone disease unknown cause.
 Affecting men twice as frequently as women.
 Named after the England Surgeons Sir James Paget.
DISEASE CLASSIFICATION
INFLAMMATORY DISEASE
• Multifocal chronic skeletal disease due to Chronic paramyxoviral Virus infection.
• As a result, the bone that is formed is abnormal.
CONGENITAL
• Certain of genes may Been associated with Paget’s disease, including the Sequestrosome
1 Gene On Chromosome 5.
ETIOLOGY
 The cause of Paget’s disease is unknown.
 Disease may be caused by a virus.
 Paramyxovirus infection of osteoblasts may activate of the c-fos proto-oncogene,
resulting in localized abnormal osteoclastic activity typical of paget’s disease.
 Also, a person’s genes may predispose them to developing paget’s disease.
 Paget’s disease affects older skeletal bone of adults.
 There is also an extremely rare form of paget’s disease in children, referred to as Juvenile
Paget’s disease.
PATHOGENESIS
MANIFESTATIONS
SYMPTOMS
 Paget’s disease is more commonly asymptomatic, but may exhibit a variety of non-
specific symptoms due to increased bone turnover.
Hyper vascular/
Osteoclytic
phase
Initial phase of
disorder involves
bone resorption
by osteoclasts
Abnormal matrix
persists but
cellular activity
is nearly absent
Paget’s
disease
Intermediate
phase
Exhaustive
(burn out)
stage
Osteoclytic activity
+
Osteoblastic activity

2.  The bones that are move commonly affected include the pelvis, spine, skull, and the long
bone.
 Individuals may experience any or a combination of the following symptoms;
 Bone pain
 Fractures
 Skeletal Deformities – bowed legs, Fronto-occipital skull enlargement.
 Warm sensation – due to increased blood supply to abnormal bones.
 Nerve damage – hearing loss, visual abnormalities.
 Cardiac abnormalities – shortness of breath.
SIGNS
 Signs may be bitemporal skull enlargement with frontal “bossing”, diluted scalp veins,
nerve deafness in one or both ears.
 Also angioid streaks in the fundus of the eyes and a short kyphotic trunk.
SIGNIFICANT LAB TESTS
 Blood chemistry (blood patient who have Paget’s disease) results indicate very high
alkaline phosphates levels with normal serum calcium and phosphorus.
 There is no known cure for this disease.
 Most cases are mild and asymptomatic no treatment is necessary in symptomatic cases
medications.
IMAGING CONSIDERATION
 X-ray of the skull, spine, pelvis and long bone.
 CT scan
 MRI (Magnetic Resonance Imaging)
 Radionuclide bone scan or RNI (Radionuclide Imaging).
RADIOLOGICAL APPEARANCE
 Radionuclide bone scans readily detect Paget’s disease even in its very early stage.
 Radiographically the affected bone typically demonstrate cortical thickening, with a
coarse, thickened trabecular pattern.
 Mixed areas of radiolucent Osteolysis and radiopaque Osteosclerosis may be seen.
 Radiograph of the fibula and tibia of the patient in figure demonstrating the effect of
advanced proliferative Paget’s disease on the fibula and tibia.
Paget’s disease on pelvis
 Paget’s disease involving the left hemi pelvis and right, there is severe osteoarthritis of
the left hip but a relatively normal joint space in the right hip.