PATHOLOGY..

Paget’s Disease

Maryam SharifianMSc student In Isfahan University Of medical Sciences

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 freaquently 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.

CONGENTIAL

•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: Paramyxo virus

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 skeletal older bone of

adults

There is also an extremely rare form of paget’s

disease in children, referred to as Juvenile Paget’s

disease

In two of every three cases of Paget's disease,

more than one bone is affected

Pathogenesis

Hyper vascular/

Osteolytic phase

Initial phase of

Disorder involves

Bone resorption by

osteoclasts

Intermediate

phase

Osteolytic activity

+

Osteoblastic activity

Exhaustive

(burn out)

stage

Abnormal matrix

Persists but cellular

Activity is nearly

Absent.Paget’s disease

MANIFESTATIONS - SYMPTOMS

Paget’s disease is more commonly asymptomatic, but

may exhibit a variety of non-specific symptoms due to

increased bone turnover.

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

symptoms …

Pain

Stiffness

Difficulty moving your affected joints

Nerve damage

Loss of balance

bone fractures

Other symptoms …

Hearing loss

Headaches

Osteoarthritis

visual abnormalities

Cardiac abnormalities

Skeletal Deformities

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.

Radio graphically of the affected bone typically

demonstrate cortical thickening, with a coarse,

thickened trabecular pattern.

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….

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.

Paget’s disease….

Treatment …

Not everyone with Paget's disease will

require treatment.

If treatment is needed, medication is most

often used.

Maryam sharifian