Paget’s disease

In station 5 you may be asked to assess a patient with raised ALP, fracture, bone pain etc.


History (3 minutes):


  • Timing questions (when did symptoms start, sudden/gradual onset, intermittent/constant, worsening)
  • Bone
    • Has your head got bigger, increase in hat size
    • Bone pain/deformity/fracture. Is bone pain increasing/any swelling (osteosarcoma)
    • Joint pain, back pain
  • Neuro
    • Headache, tinnitus, vertigo, hearing, vision, weakness, sensory change, speech, swallow
    • Carpal tunnel symptoms
  • CVS
    • Chest pain, SOB, ankle swelling, orthopnoea, PND, palpitations
  • Renal
    • Kidney stones
  • PMH (heavy bleeding during orthopaedic surgery/following fracture), Fx, Dx, Sx



Examine (3 minutes): often spot diagnosis (elderly patient with large head and hearing aid and not acromegaly!)


  • Walking aid
  • Head: large especially frontal bossing, measure circumference, prominent scalp veins, look for hearing aid, enlarged maxilla, palpate (warmth)
  • Back: kyphosis, palpate (occasionally tender, warm)
  • Arms: bowing, palpate (occasionally tender, warm)
  • Legs: bowing (femur/sabre tibia), palpate (tender, warm), scars from joint replacements
  • Neuro exam including cranial nerves especially CN VIII (conductive if otosclerosis of ossicles versus sensori-neural if auditory nerve compression) and assess for carpal tunnel if positive history
  • CVS exam (heart failure)
  • Resp and Abdo exam (exclude skeletal mets due to cancer)
  • Fundoscopy: optic atrophy, angioid streaks


ICE+Explanation (2 minutes)





Paget’s Disease:


Metabolic disease characterised by accelerated bone turnover and abnormal bone remodelling causing deformity and enlargement of bones. Osteolytic phase then mixed phase (osteoblastic and osteoclastic) then burnt out quiescent osteosclerotic phase.

Axial skeleton preferentially affected as well as long bones and skull.

Males often older than 55. Fairly common finding. Often asymptomatic.




  1. Bony
    1. Pathological fractures
    2. Osteosarcoma (worsening bone pain)
    3. Secondary osteoarthritis
  2. Neurological
    1. Hydrocephalus
    2. CN palsies (hearing loss and tinnitus, optic atrophy)
    3. Spinal cord compression or nerve root compression or cauda equina or spinal stenosis
  3. High output cardiac failure (due to increased blood flow through affected bone)
  4. Metabolic
    1. Gout
    2. Hypercalcaemia from immobilisation
    3. Kidney stones secondary to above with hypercalciuria





ALP (raised) and raised bone-specific ALP, Ca/P/PTH all normal (note calcium can be high if prolonged immobilisation/malignancy/fracture/hyperparathyroidism). Vitamin D (osteomalacia is a differential and level should be normal pre-bisphosphonate therapy)

Other LFTs normal including GGT

Urate (can be raised)

Xray: mixture of lytic and sclerotic lesions

  • Skull: cotton wool appearance, moth eaten appearance
  • Pelvis: brim sign
  • Vertebrae: picture frame sign
  • Long bones: trabecular thickening

Raised urine hydroxyproline (fasting sample)

Bone scan (increased uptake, to evaluate extent of disease)

Urine dip (blood if renal stones)

Bone biopsy if sarcomatous change suspected/metastatic disease/multiple myeloma

CT if malignancy suspected





Conservative: physio/OT, walking aid, hearing aid

Medical: analgesia (paracetamol, NSAIDs), oral or iv bisphosphonate for bone pain (exclude hypocalcaemia/vitamin D deficiency first and give calcium and vitamin D supplements to avoid hypocalcaemia, warn about osteonecrosis of the jaw) or calcitonin if intolerant of bisphosphonates, manage heart failure. Monitor ALP

Surgical: joint replacement, fracture repair, decompressive laminectomy, excision of tumour


Differential Diagnoses of bowing:

Rickets (bilateral and symmetrical) NB: pagets is assymetrical





Other Differentials:

Skeletal metastases (calcium may be raised)


Osteoporosis (normal ALP)

Osteomalacia (low calcium, low phosphate, raised PTH)

Osteosarcoma and giant cell tumours of bone

Multiple myeloma (normal ALP, often raised calcium)




Written by Dr Sarah Kennedy


Resources used to write this document include those listed in the references section of this webpage and also: