Sunday, 27 January 2019

Osteitis deformans

Disease class: Bone diseases

Also known as

  •  Paget's disease of bone

This is a disease of bone metabolism.
After osteoporosis, this is the second most common bone disorder.
There is a wide range of severity. Most cases are identified by incidental diagnosis while investigating an unrelated disease.

 

Pathophysiology

There is increased osteoclastic activity and dysfunctional bone remodelling.

 

Causes

  • Unknown

 

Risk factors

  • Genetic factors
  • Advanced age (10% of patients over 80 years old have Paget's disease of bone)

 

Symptoms

Paget's only causes symptoms in approximately 35% of patients at the point of first diagnosis.
  • Bone pain 
  • Headache
  • Frequent bone fractures
  • Leg bowing
  • Hearing loss

 

Signs

  • Deformity of the bones, especially the long leg bones, vertebral column (spine), pelvic bones, and skull bones
  • Increased hat size
  • Warmth of affected areas

 

Complications

  • Osteoarthritis
  • Heart failure (rarely seen in severe cases): occurs due to increasing systemic vascular resistance introduced by abnormal vascularity in the affected bones.

 

Epidemiology

  • More common in men than women (3:2)
  • Most common in patients with British ancestry
  • Prevalence: 1.5 to 8.0% of the population

 

Management

  • There is no curative treatment for this disease.
  • The symptoms can be reduced with bisphosphonates and analgesics.
  • Surgery may be helpful in some situations.

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