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Educational draft · awaiting clinical reviewUse Rapid for revision, not patient-care decisions. Check current national and local guidance and the BNF or BNFC before acting.

Paget disease of bone

Endocrinology and metabolism · UK scope · Sources checked 27 Aug 2026 · Clinical review pending

RapidFull
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Neurological compression or pathological fracture changes urgency

New weakness, sensory loss, sphincter disturbance, acute hearing or cranial nerve change, severe focal pain with fracture, or a rapidly enlarging painful bone lesion needs urgent imaging and specialist assessment. Rare sarcomatous transformation should be considered when established Paget disease develops rapidly progressive focal pain, swelling or destructive imaging.

Action: Assess ABCDE and neurovascular status, immobilise a suspected fracture, provide analgesia and obtain urgent targeted imaging. Discuss spinal compression, skull-base compromise, pathological fracture or possible malignancy with orthopaedics, spinal surgery, neurology or sarcoma services rather than waiting for routine alkaline phosphatase review.

Synopsis

Recognise focal disordered bone remodelling, define its skeletal extent, treat Paget-related pain and distinguish complications requiring surgical or urgent specialist care.

  • Paget disease is focal acceleration and disorganisation of bone remodelling, producing enlarged but mechanically abnormal bone in one or several sites.
It becomes more common with age and often affects pelvis, spine, skull, femur or tibia; many patients are detected through raised alkaline phosphatase or incidental imaging.
  • Use total alkaline phosphatase with liver tests as first-line biochemical screening, then targeted radiographs and a radionuclide bone scan to define active extent.
  • Paget-related bone pain is usually localised, persistent and sometimes worse at night, but coexisting osteoarthritis, spinal disease and fracture are common alternative pain generators.
  • Bisphosphonates are recommended for bone pain attributable to active Paget disease, with intravenous zoledronic acid most likely to provide a favourable and durable pain response.
  • Treat symptoms and complications rather than pursuing normal alkaline phosphatase as an isolated target; intensive biochemical suppression has not improved clinical outcomes.
  • Hearing loss, skull enlargement, deformity, secondary osteoarthritis, nerve compression and pathological fracture require organ-specific assessment beyond biochemical treatment.
  • Before bisphosphonate therapy, correct hypocalcaemia and important vitamin D deficiency, assess renal function and obtain dental or jaw-risk review where indicated.
  • Investigation priorities

    01
    Total alkaline phosphatase with liver testsFirst step

    Screen for increased bone turnover while distinguishing a likely hepatic source.

    Management branches

    ConfirmDiagnose and map disease

    Raised bone ALP, focal bone pain, deformity or an incidental radiograph suggests Paget disease.

    1. Separate bone from liver ALP, take a focused skeletal and neurological history and obtain targeted radiographs of suspicious sites.
    2. Use radionuclide bone scanning to map active distribution and image clinically important scan-positive sites when characterisation remains incomplete.
    3. Review calcium-vitamin D, renal function, pain source and complications with a metabolic bone specialist before deciding whether treatment will improve symptoms.

    Key medicines

    Zoledronic acid for Paget diseaseGive the licensed single intravenous Paget regimen through a specialist service after confirming hydration, calcium-vitamin D sufficiency and renal eligibility; repeat treatment is based on relapse and symptoms rather than scheduled annual infusion.Acute-phase reaction, hypocalcaemia and kidney injury occur. Check creatinine clearance, avoid in severe renal impairment, review dental and jaw risk, and distinguish this regimen from osteoporosis or oncology schedules.
    Risedronate for Paget diseaseUse the licensed daily oral Paget course for the defined treatment duration when intravenous zoledronate is unsuitable, following strict fasting and upright administration instructions.Avoid in significant oesophageal disease, inability to remain upright, hypocalcaemia or renal function below the product limit. Adherence to daily administration determines absorption, and response may be less durable than zoledronate.
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    Sources and review status4 sources · checked 27 Aug 2026 · clinical review pending+
    Sources

    Sources and review status

    National guidance is shown before implementation-dependent detail. Typical adult dose examples remain subject to patient factors, contraindications and the live BNF or specialist protocol. Source check completed 27 Aug 2026; clinical approval remains outstanding.

    • UK Paget disease clinical guidelineDiagnosis, imaging, symptom-directed treatment and key recommendations.
    • Paget Association clinical managementALP, bone scanning, zoledronate and complication management.
    • Paget Association professional resourcesFull endorsed guideline and UK professional implementation materials.
    • Paget Association treatment guidanceUK treatment choices and safety information grounded in the clinical guideline.

    Editorial contact: Doctor’s Passport editorial team. Source-check date is separate from clinical approval. Report a content problem · Content changes

    Authoring stateRapid draftClinical stateAwaiting reviewJurisdictionUnited Kingdom