PATHOLOGY..

Paget’s Disease

INTRODUCTIONINTRODUCTION

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 CLASSIFICATIONDISEASE CLASSIFICATION

INFLAMMATORYDISEASE

•Multifocal chronic skeletal disease due toChronic paramyxoviral Virus infection.•As a result, the bone thatis formed is abnormal.

CONGENTIAL

•Certain of genes may Been associated withPaget’s disease, includingThe Sequestrosome 1 Gene On Chromosome 5.

ETIOLOGYETIOLOGY

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.

PathogenesisPathogenesis

Hyper vascular/Osteolytic phase

Initial phase of Disorder involves

Bone resorption byosteoclasts

Intermediate phase

Osteoclytic activity+

Osteoblastic activity

Exhaustive (burn out)

stage

Abnormal matrixPersists but cellularActivity is nearly

Absent.Paget’s disease

MANIFESTATIONS - SYMPTOMSMANIFESTATIONS - 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

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.

MANIFESTATIONS - SIGNMANIFESTATIONS - SIGN

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 TESTSSIGNIFICANT 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 CONSIDERATIONIMAGING 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 APPEARANCERADIOLOGICAL 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 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….Paget’s disease….

References…References…

http://www.nlm.nih.govhttp://www.medicinet.comhttp://www.uncomparehealthcare.comhttp://www.wheelessonline.comhttp://www.e-radiography.nethttp://www.radiographics.highwive.org