Pagets disease osteitis deformans Definition idiopathic Skeletal bone

Pagets disease (osteitis deformans)

� Definition - idiopathic Skeletal bone disorder in which there is excessive bone resorption followed by replacement of altered bone tissue � The new bone is larger, disorganized and structurally weaker � Common sites– pelvis , long bones, spine, ribs, sternum and cranium

Etiology � Exact cause is unknown � Followed by viral infection (measles virus, respiratory syncytial virus ) � Genetic (15%-30%) � Men to women ratio – 2: 1 � Seen in below 40 years

Pathophysiology � Due to unknown causes � Increase BONE RESORPTION � Altered BONE TURN OVER � As a compensatory mechanism - increase bone formation � Newly formed bones are large, unorganized and weak � Structural changes in weight bearing limbs and other manifestation

Signs and symptoms � Free of symptoms in the early stages � Bone pain – deep ache – progress to severe pain � Fatigue � Progressive development of waddling gait � Become shorter � Barrel chest

� Bowing of tibia or femur � Changes in skin temperature � Manifestations related to nerve compression

� Increase bone volume in the spine can cause spinal cord or cranial root compression leads to head ache, blindness, vertigo, hearing loss with tinnitus and dementia � Pathologic fracture , osteosarcoma, fibrosacroma and osteoclastoma tumors are complications � Osteoarthritis are common associated with pagets disease

Diagnostic investigation �X rays – shows increased bone expansion and density. � Bone scan � Serum alkaline phosphates levels increased � N-telopeptide and C-telopeptide they are biomarkers used to measure the rate of bone turnover, can be measure from urine or serum

Management � Limited to symptoms � Supportive care � Correction of secondary deformity � Calcitonin – decrease osteoclastic activity