Paget’s Disease of Paget’s Disease of BoneBone

(Osteitis Deformans)

By By

Dr. Muhammmad ATHAR khanDr. Muhammmad ATHAR khan

PGR OMFSPGR OMFS

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

Paget’s Disease/osteitis Paget’s Disease/osteitis deformansdeformans

Slowly progressive metabolic bone Slowly progressive metabolic bone condition of undetermined causecondition of undetermined cause

Etiologic factors may be Etiologic factors may be Paramyxo virus Paramyxo virus Genetic mutationsGenetic mutations   Mutations in the Mutations in the SQSTM1SQSTM1 gene are  gene are

the most commonthe most common Altered osteoclast development and Altered osteoclast development and

functionsfunctions

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

Diagonosis?Diagonosis?

lion face disese lion face disese “Leontiasis ossea”“Leontiasis ossea”

Polyostotic severe Polyostotic severe form of Pagets form of Pagets diseasedisease

3 stages3 stages

1.Resorptive phase1.Resorptive phase 2.Vascular phase2.Vascular phase 3.Sclerosing phase3.Sclerosing phase

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

Clinical features:-Clinical features:- Above 5o years of age Above 5o years of age Commonly involve bones Commonly involve bones

Pelvis,Skull,Tibia,Vertebrae,humerus and Pelvis,Skull,Tibia,Vertebrae,humerus and sternumsternum

Jaw is affected in 20% of patientJaw is affected in 20% of patient Maxilla symmetrically enlargeMaxilla symmetrically enlarge Bone pain moderate to severe or bone Bone pain moderate to severe or bone

deformity deformity Alveolar ridge widens and flattening of the Alveolar ridge widens and flattening of the

palatal vaultpalatal vault Severe cases difficult lip closureSevere cases difficult lip closure HypercementosisHypercementosis

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

Patchy opacitiesPatchy opacities Simian stance (monkey like)Simian stance (monkey like) Nasal obstructionNasal obstruction Deviated nasal septumDeviated nasal septum Changes in skull Osteoprosis Changes in skull Osteoprosis

circumscriptacircumscripta Revesal linesRevesal lines

Increase in temperature of the Increase in temperature of the overlying skin due to underlying bone overlying skin due to underlying bone hypervascularityhypervascularity

Neurological disturbances like Neurological disturbances like headache,auditory,visual headache,auditory,visual disturbance,facial disturbance,facial paralysis,vertigo,weaknessparalysis,vertigo,weakness

Edentulus patient denture is not fitEdentulus patient denture is not fit Dentulus patient spacing in teeth and Dentulus patient spacing in teeth and

looseningloosening

Skull of patient with paget’s disease normal skull

InvestigationsInvestigations

Bone scanBone scan Bone x-rayBone x-ray Ca level normalCa level normal Phosphorus level normalPhosphorus level normal Alkaline phosphatase raisedAlkaline phosphatase raised Urinary hydroxyproline level raisedUrinary hydroxyproline level raised Elevated markers of bone Elevated markers of bone

breakdown (, N-telopeptide)breakdown (, N-telopeptide)

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

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

Treatment Treatment

NSAIDNSAID CalcitoninCalcitonin BisphosphonateBisphosphonate Alendronate,risedronate 200–400 Alendronate,risedronate 200–400

mg doses (oral 2-6 month)mg doses (oral 2-6 month) I/V pamidronate who can not I/V pamidronate who can not

tolerates oral formtolerates oral form Zolendronates  single doseZolendronates  single dose

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 sites.affected bone sites.

Possible ComplicationsPossible Complications

Bone fracturesBone fractures DeafnessDeafness DeformitiesDeformities Heart failureHeart failure ParaplegiaParaplegia Spinal stenosisSpinal stenosis Convesion into osteosarcomaConvesion into osteosarcoma Severe bone pain,pathological fractureSevere bone pain,pathological fracture