Bone Diseases
Paget Disease of Bone
aka Osteitis Deformans
Chronic disorder of accelerated, disorganised bone remodelling; jaw involvement causes progressive symmetrical enlargement, hypercementosis and denture non-fit.
§ figuresFigures (5)
Fig. 1Fémures de um indivíduo com sexo e idade desconhecida. Fémur direito afetado pela doença de Paget. Fémur esquerdo saudável. A. vista anterior, B. vista posterior. Os ossos pertencem ao Museu de História Natural de Viena,
“Fémures de um indivíduo com sexo e idade desconhecida. Fémur direito afetado pela doença de Paget” · Valenzuela E. N., Pietschmann P · CC BY-SA 4.0 · Wikimedia Commons
Fig. 2Paget's disease (Edes), third image
“Paget's Disease (cropped)” · Richard C. Cabot, M.D. · Public domain · Wikimedia Commons
Fig. 3Paget's disease of right hip bone in an 80-year-old man. This is an edited version of the source image made for use in the "Anatomist" iOS and Android app and shared here under the terms of the source image's Share Alike
“Paget's disease of Right Hip Bone” · Jmarchn · CC BY-SA 4.0 · Wikimedia Commons
Verified open-access images only, reproduced for education under their stated licences · no AI-generated or illustrative artwork · Paget Disease of Bone figures pending faculty review.
§ overviewOverview
Focal or multifocal disorder of bone remodelling characterised by increased and disorganised osteoclastic resorption followed by exuberant, disorganised osteoblastic bone formation.
§ icdICD Classification
M88
§ etiologyEtiology
- 01Multifactorial: genetic (SQSTM1 mutation), possible paramyxovirus trigger
- 02First-degree relatives 7× risk
§ epidemiologyEpidemiology
Adults >55 y; slight male predominance; higher prevalence in UK, Australia, New Zealand.
§ pathogenesisPathogenesis
Three phases: lytic (osteoclast-mediated) → mixed → sclerotic; disorganised bone is mechanically weak but hypervascular.
§ clinicalClinical Features
- 01Progressive bone pain, deformity
- 02Enlarging cranial vault (hat size increases)
- 03Bilateral symmetrical jaw enlargement, spacing of teeth, denture becomes tight
- 04Deafness (cranial nerve VIII compression)
- 05High-output cardiac failure (rare)
§ radiographicRadiographic Features
- 01Skull: 'cotton wool' patchy sclerosis
- 02Jaw: hypercementosis, obliteration of PDL, loss of lamina dura
- 03Ground-glass and mixed radiolucent-radiopaque appearance
§ labsLaboratory Findings
- 01Markedly elevated serum alkaline phosphatase (isolated)
- 02Normal calcium, phosphate, PTH
- 03Increased urinary hydroxyproline and N-telopeptides
§ treatmentTreatment
- 01Asymptomatic: monitor
- 02Symptomatic: bisphosphonates (single-dose IV zoledronate preferred) — normalises ALP
- 03Calcitonin (second line)
- 04Analgesia, orthopaedic surgery for fractures
- 05Dental: extractions difficult (dense bone, hypercementosis, bleeding); avoid elective surgery in active phase
§ complicationsComplications
- 01Pathological fracture
- 02Osteosarcoma (<1% but grim prognosis)
- 03Cranial nerve palsies
- 04Post-extraction haemorrhage or osteomyelitis
§ prognosisPrognosis
Generally good with bisphosphonate therapy; new hearing loss or increased pain warrants imaging to exclude sarcoma.
§ examKey Examination Points
- 01Enlarging skull, ill-fitting dentures, isolated ALP rise
§ revisionQuick Revision Summary
- 01Cotton-wool skull · isolated ALP · zoledronate
§ vivaBDS Viva Questions
- 01Biochemical marker of Paget?
- 02Complications of extractions in Paget?
- 03Malignant transformation risk?
§ mcqsMCQs — Assessment (3)
Question 1
Classic biochemical finding:
Question 2
First-line pharmacotherapy:
Question 3
Malignant transformation to:
References
- Ralston SH. NEJM 2013
Draft — pending faculty review. Educational use only; verify against current guidelines and primary sources before clinical application.