Bone Pain - Pagets Flashcards
What is Paget’s disease?
Focal disease of bone remodelling.
Normal cycle of bone renewal causes bones to become weakened and possibly deformed.
What are the symptoms of Paget’s disease?
Constant, dull bone pain
Joint pain, stiffness and swelling
A shooting pain that travels along or across the body, numbness and tingling, or loss of movement in part of the body.
Often no symptoms no condition discovered due to tests carried out for another reason.
What is the epidemiology of Paget’s disease?
Second most common metabolic bone disease
More common in males
Unusual in under 40s
2% of over 55s in UK
Often asymptomatic
Incidence declined rapidly in recent years
What is the pathology of Paget’s disease?
Increased localised bone cell activity.
Osteoclasts are bigger = absorb more bone
Osteoblasts lay bone in haphazard way to keep up with osteoclasts activity - poor quality bone, bone expansion = more likely to fracture.
Marrow replaced with fibrous tissue and blood vessels.
Radiological symptoms of Paget’s disease?
Sclerosis - due to new bone formation
Lyric areas - where osteoclasts working
How does Paget’s disease affect risk of sarcoma?
Increases risk
As bone cells working more than usual
Also makes it more difficult to pick up sarcoma as bone looks abnormal anyway.
How does genetics influence Paget’s disease?
10-20% have family history of Paget’s
Related to defects in SQSTM1 gene - which codes SQSTM1/p62 protein which is a regulator of osteoclasts RANK-mediated NF-kappa B signalling.
Unclear trigger for this - possibly environmental as incidences falling.
Clinical MSK features of Paget’s
Bone expansion - monostotic (one bone or portion involved) vs polystotic (2 or more).
Acetabular protrusion
Bone deformity
Bone pain - 50%, poorly localised, deep seated gnawing pain, night pain, progressive pain.
Fractures - 10-30% fissure fracture in lower limb.
Spinal stenosis
OA in neighbouring joints - abnormal biomechanics.
Commonly affected bones of Paget’s
Femur
Spine
Skull
Sternum
Pelvis
Neuro presentations of Paget’s
Related to bony overgrowth (in skull) and compression of neurological structure:
Basilar invagination
Cerebellar dysfunction
Obstructive hydrocephalus
Cranial nerve palsies
Spina stenosis/cauda equina
Deafness
Tinnitus
Para or quadriplegia (if spinal cord depressed)
What what you notice upon examination of Paget’s?
Areas warm, tender or deformed
(Rare) cardio symptoms of Paget’s
Increase cardiac output - increase blood flow to bone
Heart failure
Aortic stenosis
Endocardial calcification
Atherosclerosis
(Rare) metabolic symptoms of Paget’s
Hypercalcaemia
Hyperuricemia (too much uric acid in blood)
Immobilisation hypercalciuria
Nephrolithiasis
(Rare) neoplasia symptoms of Paget’s
Osteosarcoma
Chondrsarcoma
Fibrosarcoma
Giant cell tumours
Investigations for Paget’s
Isolate alkaline phosphatase (bony)
Radiological - plain x-ray - bones expanded, cortical thickening, coarsened trabeculae ,lysis, sclerosis
CT, MRI, PET CT, isotope bone scan
