Paget’s Disease of Paget’s Disease of BoneBone

(Osteitis Deformans)

Hosain Bouallouch Shen Zhou University 10 maart 2006Hosain Bouallouch Shen Zhou University 10 maart 2006

What is Paget’s disease?What is Paget’s disease?

- It’s a chronic disease of the It’s a chronic disease of the skeletonskeleton

- Normal growth of bone is Normal growth of bone is changedchanged

*Bone breaks down more quickly *Bone breaks down more quickly

*Bone can grow larger than before *Bone can grow larger than before

Bone consists of:Bone consists of:

Osteoblasts: bone forming cells

Osteoclasts: Bone breaking cells

Osteocytes: present in the matrix

Matrix: collagen, calcium, osteocalcin

Bone remodelingBone remodeling

2 forms of Paget’s 2 forms of Paget’s diseasedisease

Too much bone is formed, so gets Too much bone is formed, so gets larger and breaks easierlarger and breaks easier

Bone that’s replaced is soft and Bone that’s replaced is soft and porousporous

Bones commonly affected by Bones commonly affected by Paget’s diseasePaget’s disease

SymptomsSymptoms

Pain in or over a bonePain in or over a bone

The affected area may feel extra warmThe affected area may feel extra warm

The shape of the bone may changeThe shape of the bone may change

-the bone in the legs may bend or -the bone in the legs may bend or bow outbow out

-the bone in the skull may get bigger-the bone in the skull may get bigger

Skull of patient with paget’s disease normal skull

Bone breaks more easilyBone breaks more easily

In late stages hipjoint may damageIn late stages hipjoint may damage

Hearing problems may occur Hearing problems may occur because the bone expands and heart because the bone expands and heart problems may also occurproblems may also occur

SymptomsSymptoms

What causes Paget’s What causes Paget’s diseasedisease

Unknown; evidence favors an infection of Unknown; evidence favors an infection of genetically predisposed individuals with genetically predisposed individuals with a measles virus of the paramyxovirus a measles virus of the paramyxovirus familyfamily

Studies have demonstrated that Studies have demonstrated that abnormal virus-infected osteoclasts abnormal virus-infected osteoclasts generate IL-6, a resorptive cytokine from generate IL-6, a resorptive cytokine from the bone marrow of patients with Paget’s the bone marrow of patients with Paget’s diseasedisease

How common is Paget’s How common is Paget’s diseasedisease

The exact number of Paget’s disease The exact number of Paget’s disease is not knownis not known

Men are more likely to be affected Men are more likely to be affected than womanthan woman

It usually affects people over the It usually affects people over the age of 40 yearsage of 40 years

It’s estimated to affect 3% of people It’s estimated to affect 3% of people over the age of 40over the age of 40

EpidemiologyEpidemiology

PrevalencePrevalence High:High: United Kingdom, United United Kingdom, United

States, Australia, New Zealand, States, Australia, New Zealand, Western EuropeWestern Europe

Low:Low: Scandinavia, China, Japan, Scandinavia, China, Japan, IndiaIndia

Family History: positive in as many Family History: positive in as many as 40% of patientsas 40% of patients

TreatmentTreatment

At this time there is no cure for At this time there is no cure for Paget’s disease. Therefore Paget’s disease. Therefore treatment is designed to control treatment is designed to control the symptoms and change the the symptoms and change the rate of bone growthrate of bone growth

Treatment Treatment

Biphosphonates heplfull in rebuilding boneheplfull in rebuilding bone Inhibits bone resorption and Inhibits bone resorption and

mineralisation by promoting mineralisation by promoting apoptosis of osteoclastsapoptosis of osteoclasts

for example Didronel, Fosamex and for example Didronel, Fosamex and ActonelActonel

TreatmentTreatment

CalcitoninCalcitonin Slows bone loss and bone growthSlows bone loss and bone growth Gives relieve of bone painGives relieve of bone pain Reduces skin temperature over Reduces skin temperature over

affected bone sitesaffected bone sites For example acetaminophen For example acetaminophen

(Tylenol, Panadol)(Tylenol, Panadol)

TreatmentTreatment

SurgerySurgery 1. fractures: to allow fractures to 1. fractures: to allow fractures to

heal in better positionheal in better position 2. bone deformity: cutting and 2. bone deformity: cutting and

realigning of the affected bonesrealigning of the affected bones 3. severe degenerative arthritis: 3. severe degenerative arthritis:

joint replacement of the hips, kneesjoint replacement of the hips, knees

Shall I tell you what true knowledge is?

If you knowKnowing that you knowAnd if you don’t know

Knowing that you don’t knowThat is true knowledge

Confucius

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