Pagets disease-of-bone-by dr.athar
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Transcript of Pagets disease-of-bone-by dr.athar
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