Pagets disease of the bone (osteitis deformans)

Question 1

Definition

Answer 1

A slowly progressive disorder. Increased turnover of bone with enlargement, remodelling, deformity and weakness

Over time, bones can become fragile and misshapen

Question 2

Areas that can be affected the most by Pagets disease

Answer 2

Pelvis, skull, spine, and legs

Question 3

Epidemiology

Answer 3

One of most common afflictions of the general skeleton

Incidence increases with age

Rare before 40

Commoner in Anglo-Saxons (increased incidence in Britain and Germany)

Rare in Africa and Middle East

Question 4

Aetiology

Answer 4

Cause generally unknown

Scientists suspect a combination of environmental and genetic factors contribute to the disease

Question 5

Pathogenesis

Answer 5

Abnormal hyper nucleated osteoclasts, giving rise to enlarged, spongy bone with abnormal architecture, initially spongy and easily deformable, later brittle and liable to fracture

Question 6

Clinical features

Answer 6

Asymptomatic

Incidental discovery

Pain (change in patter, Sarcoma?)

Thickening/deformity of bone

Skull (increased hat size, headaches)

Sabre tibia

Kyphosis

Nerve root pain (due to vertebral thickening, maybe leading to spinal stenosis)

Deafness due to osteosclerosis

Pathological symptoms

High output cardiac failure

Question 7

Investigations

Answer 7

Radiographs:
– patchy changes in bone density
– decrease in vascularity
– increase in sclerotic stage
– deformed bone with thickened trabecular, thickened cortex not sharply demarcated from medulla

Biochemistry:
– Raised ALP (alkaline phosphatase)

Question 8

Complications

Answer 8

Osteosarcoma (bone tumour, proliferation of osteoblasts)

fractures

nerve compression with spinal stenosis

hypercalcaemia from immobilisations (calcium levels in blood too high, can damage bones)

Bleeding during/post surgery

Question 9

Treatment

Answer 9

None

Treatment of complications can be done however

No direct treatment of pagets disease of the bone