Paget's Disease M88.9 731.0


synonyms: Paget's disease, osteitis deformans

Paget's Disease ICD-10

Paget's Disease ICD-9

  • 731.0 Osteitis deformans without mention of bone tumor (Paget's Disease)

Paget's Disease Etiology / Epidemiology / Natural History

  • Pagets disease = a chronic, nonmetabolic bone disorder characterized by increased bone resorption, bone formation, and remodeling which may lead to structural weakness, altered joint biomechanics, and degenerative joint disease.
  • 0.5% to 3.0% of people older than age 60 years. (Lyles KW, J Bone Miner Res 2001; 16: 1379).
  • Men > women (1.6:1)
  • Etiology = unknown. Proposed theories = viral ( Paramyxoviruses such as measles), genetic, p62/ZIPgene mutation, and environmental (arsenic, cattle).
  • Primary abnormality = intense focal resorption of normal bone by abnormal osteoclasts. (Demulder A, Endocrinology 1993; 133: 1978). Leads to rapid osteoblast production of woven bone with poor structural properties.
  • Phases: (1) bone resorption (osteoclasts); (2) mixed phase(osteoclastic and osteoblastic activity); (3)chronic sclerotic phase (osteoblasts).

Paget's Disease Anatomy

  • Can be monostotic (25%) or polyostotic (75%).
  • Common locations: pelvis, lumbosacral spine, femur, tibia.
  • Rare in: hands, feet, or face.

Paget's Disease Clinical Evaluation

  • Generally an incidental xray finding.
  • May present with bone pain (constant, poorly localized pain that is present at rest), bone deformity, fracture, arthropathy, skin temperature changes, or neurologic complications (deafness, cranial nerve palsies, spinal cord compression, vertigo).
  • Deafness results from involvement of the petrous temporal bone and compression of the auditory nerve.

Paget's Disease Xray / Diagnositc Tests

  • Diagnosis is generally made based on radiographic appearance and bone scan.
  • Phase (1): bone resorption (osteoclasts) results in lytic changes in involved bones. Most commonly seen in the skull as osteoporosis circumscripta cranii. Long bones may demonstrate an advancing V-shaped lytic lesion (flame edge or blade of grass appearance). (Whitehouse RW, Semin Musculoskelet Radiol 2002; 6: 313).
  • Phase (2) mixed phase(osteoclastic and osteoblastic activityresults in both lytic and sclerotic changes.
  • Phase (3)chronic sclerotic phase (osteoblasts) demonstates predominately sclerotic changes.
  • Hip: evaluate for coxa vara deformity of the femoral neck and acetabular protrusio (common in Paget's disease).
  • Bone scan: demonstrates increased uptake except in early phase (1).
  • ESR, CRP, serum calcium, serum alkaline phosphatase and serum phosphate levels, 25-hydroxyvitamin D concentration, and liver function tests indicated. Elevated ESR may indicate malignant transformation. 25-Hydroxyvitamin D is normal in Paget’s disease; low osteomalacia. Liver function tests rule out liver disease as the cause ofelevated serum alkaline phosphatase.
  • Serum alkaline phosphatase is an enzyme found on the plasma membrane of osteoblasts and indicates osteoblast activity. 15% of patients with Paget's will have normal alk phos levels.

Paget's Disease Classification / Treatment

  • alendronate 40mg PO QD for 6 months, may cause severe esophagitis; risedronate 30mg PO QD for 2 months;
  • Calcitonin 50-100 units SQ/IM daily.
  • Surgical patients with Patet's disease should be placed on bisphosphonates before surgery to decrease hypervascularity.
  • NSAIDs for pain management.
  • TKA in Paget's disease patients:  functional outcomes are largely similar to other patients; high risk for intra-op patellar tendon avulsion.  Exposure is generally more difficult.

Paget's Disease Associated Injuries / Differential Diagnosis

  • Osteoporosis

Paget's Disease Complications

  • Fracture
  • Pain

Paget's Disease Follow-up Care

  • Surgical patients with Patet's disease should be placed on bisphosphonates before surgery to decrease hypervascularity.

Paget's Disease Review References

  • Klein GR, JAAOS 2006;14:577
  • Delmas PD, N Engl J Med 1997;336:558)
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