Paget's disease of Bone Flashcards
What is Paget’s disease of bone?
> Localised disorder of bone turnover
> Increased bone resorption followed by increased bone formation
> Leads to disorganised bone: bigger, less compact, more vascular and more susceptible to deformity and fracture
Epidemiology of Paget’s disease of bone?
> 15-30% are familial
> Loci of SQSTMI
> Restricted geographic distribution: those of Anglo-Saxon origins
> Environmental trigger: Possibility of chronic viral infection within Osteoclast
Paget’s disease of bone - Symptoms?
Symptoms:
> Presents in a patient>40 years with bone pain
> Occasionally presents with bone deformity,
> Excessive heat over the Pagetic bone
> Neurological complications such as nerve deafness
Paget’s disease of bone - Presentation?
> Isolated elevation of serum alkaline phosphatase- the commonest presentation in the 21st centuary
> Bone pain and local heat
> Bone deformity or fracture (Lion like facies and increasing hat size)
> Hearing loss due to impingement of cranial nerves
> Rare development
of osteosarcoma
in affected bone
> Complication of spinal stenosis and spinal cord compression
Paget’s disease of bone - Treatment?
> No evidence to treat asymptomatic Paget’s unless in skull or in area requiring surgical intervention.
> Do not treat based on a raised alkaline phosphatase alone but can use this to monitor
> Intravenous Bisphosphonate therapy-One off IV zoledronic acid
> NSAIDs for pain
> Calcium and Vit D supplementation are recommended, especially when on bisphosphonates
Xray findings in Paget’s disease of bone?
1) Bone enlargement and deformity
2) Osteoporosis circumscripta
3) Cotton wool appearance of the skull due to sclerosis (increased density) and lysis (Decreased density)
4) V-shaped defects in the long bone
Biochemistry changes in Paget’s disease of bone?
1) Raised alkaline phosphatase
2) Normal calcium
3) Normal phosphate
