Paget's disease of bone
Disordered osteoclast-driven bone remodelling
Overview
Localised disordered bone turnover — excessive osteoclastic resorption followed by disorganised osteoblastic formation, producing enlarged, structurally weak, deformed bone. Often asymptomatic with an isolated raised ALP; can cause bone pain, deformity (bowed tibia, skull enlargement), fractures, deafness, and (rarely) osteosarcoma. Calcium is normal. Treated with a bisphosphonate if symptomatic.
Recognise
- Often asymptomatic — incidental ISOLATED raised alkaline phosphatase with normal calcium/phosphate
- Bone pain, deformity (bowing of long bones, enlarging skull/hat size), warmth over affected bone; pathological fracture
- Complications: deafness (skull/ossicles), high-output cardiac failure (extensive disease), spinal stenosis, rare osteosarcoma
Red flags
- New severe pain/soft-tissue mass in Pagetic bone → exclude osteosarcoma
- Pathological fracture; neurological compression (skull base/spine)
Differentials & how to tell them apart
Investigations
Isolated raised ALP with NORMAL calcium/phosphate; X-ray (lytic + sclerotic, cortical thickening, bowing); isotope bone scan (extent). Exclude other causes of raised ALP (liver — check GGT).
Management
Bisphosphonate (for bone pain/active symptomatic disease)
- 1Confirm isolated raised ALP with normal calcium (check GGT to exclude liver source) and X-ray changes. Treat SYMPTOMATIC disease (bone pain) with a bisphosphonate; analgesia.Gate: A new lytic area or soft-tissue mass with rapidly worsening pain in Pagetic bone raises OSTEOSARCOMA — investigate, don't just escalate analgesia
- 2Manage complications: hearing aids/ENT (deafness), orthopaedics (fracture/deformity/arthroplasty), spinal decompression; monitor ALP response.
Key points
Isolated raised ALP + normal calcium + bone deformity/deafness in an older patient = Paget's. Bisphosphonate for pain. The rare but feared complication is osteosarcoma.
Monitor & prognosis
ALP (response/relapse); complications.
Good; rare malignant transformation.
Source: Society for Endocrinology; Paget's Association