Updated: 12/26/2021

Paget Disease of Bone (Osteitis Deformans)

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  • Snapshot
    • A 60-year-old man presents to his primary care physician with hip pain and hearing loss. He denies any trauma to the hip or any recent changes in his medications. He also notes his hat fits tighter than before. Physical examination is unremarkable. Laboratory testing is significant for an elevated alkaline phosphatase level in the setting of normal serum calcium, phosphate, and parathyroid hormone.
  • Introduction
    • Clinical definition
      • a progressive metabolic bone disorder of abnormal bone remodeling
        • excessive bone resorption and formation is present
  • Epidemiology
    • incidence
      • second most common metabolic bone disorder (osteoporosis is first)
    • age
      • > 55 years of age
  • Etiology
    • Unknown
    • Pathogenesis
      • increased multinucleated osteoclastic and osteoblastic activity leads to a high bone remodeling rate resulting in abnormal bone architecture (lytic and blastic lesions)
  • Presentation
    • Symptoms
      • usually asymptomatic
      • bone and/or joint pain
      • bone deformity
        • bowing deformity
      • hearing loss
        • with skull involvement that leads to narrowing of the auditory foramen
    • Physical exam
      • typically unremarkable
  • Imaging
    • Radiography
      • indication
        • to identify osteolytic, osteoblastic, and sclerotic bone changes
    • Bone scintigraphy
      • indication
        • to determine the extent of Paget disease of bone and to assess for other sites of involvement
  • Studies
    • Labs
      • normal serum phosphate, calcium, and parathyroid hormone
      • elevated alkaline phosphatase
    • Histology
      • mosaic pattern of lamellar bone with prominent cement lines
  • Differential
    • Laboratory Abnormalities in Select Bone Disorders
      Etiology
      Serum Phosphate
      Serum Calcium
      Serum Alkaline Phosphatase
      Parathyroid Hormone
      Osteomalacia/ricketsDecreasedDecreasedIncreasedIncreased
      OsteoporosisNormalNormalNormalNormal
      OsteopetrosisNormalNormal or decreasedNormalNormal
      Paget disease of the boneNormalNormalIncreasedNormal
      Osteitis fibrosa cysticaPrimary
      hyperparathyroidism
      • decreased
      Secondary
      hyperparathyroidism
      • increased

        Primary
        hyperparathyroidism
        • increased

        Secondary
        hyperparathyroidism
        • decreased

          Primary and secondary hyperparathyroidism
          • increased
            Primary and secondary hyperparathyroidism
            • increased
              Hypervitaminosis DIncreasedIncreasedNormalDecreased
          • Treatment
            • Medical
              • bisphosphonates
                • indication
                  • first-line treatment for Paget disease of bone
            • Surgical
              • orthopedic surgery (e.g., joint/knee arthroplasty)
                • indication
                  • to manage symptomatic complications of Paget disease of bone (e.g., osteoarthritis, pathologic and pseudofractures, and spinal stenosis)
          • Complications
            • Neurologic
              • nerve root compression
              • hearing loss
              • spinal stenosis
            • Orthopedic
              • pathologic fractures
              • secondary osteoarthritis
            • Oncologic
              • osteosarcoma
            • Cardiovascular
              • high-output heart failure
                • abnormal bone deposition leads to increased bony vascularization, which decreases systemic vascular resistance (afterload), leading to high-output heart failure
          • Prognosis
            • Expansion of untreated Paget lesions
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          (M2.OR.17.4867) A 52-year-old man presents to his primary care physician for generalized pain. The patient states that he feels like his muscles and bones are in constant pain. This has persisted for the past several weeks, and his symptoms have not improved with use of ibuprofen or acetaminophen. The patient has a past medical history of alcohol abuse, repeat episodes of pancreatitis, constipation, and anxiety. He has a 22 pack-year smoking history. His temperature is 99.5°F (37.5°C), blood pressure is 140/95 mmHg, pulse is 70/min, respirations are 15/min, and oxygen saturation is 99% on room air. On physical exam, you note generalized tenderness/pain of the patient's extremities. Abdominal exam reveals normoactive bowel sounds and is non-tender. Dermatologic exam is unremarkable. Laboratory values are ordered as seen below.

          Hemoglobin: 12 g/dL
          Hematocrit: 36%
          Leukocyte count: 7,500/mm^3 with normal differential
          Platelet count: 147,000/mm^3

          Serum:
          Na+: 138 mEq/L
          Cl-: 100 mEq/L
          K+: 4.2 mEq/L
          HCO3-: 24 mEq/L
          BUN: 20 mg/dL
          Glucose: 99 mg/dL
          Creatinine: 1.0 mg/dL
          Ca2+: 10.2 mg/dL
          Alkaline phosphatase: 252 U/L
          Lipase: 30 U/L
          AST: 12 U/L
          ALT: 10 U/L

          Which of the following is associated with this patient's condition?

          QID: 109612

          Hearing loss

          53%

          (30/57)

          Bence Jones proteins

          9%

          (5/57)

          Hypercalcemia

          12%

          (7/57)

          Adenocarcinoma of the gallbladder

          9%

          (5/57)

          Obstructive jaundice

          16%

          (9/57)

          M 8 D

          Select Answer to see Preferred Response

          (M3.OR.15.2) A 52-year-old man presents to the emergency department after a motor vehicle collision. The patient is complaining only of left hip pain which has been ongoing for the past 6-8 months. His temperature is 98.5°F (36.9°C), blood pressure is 137/88 mm Hg, pulse is 95/min, respirations are 17/min, and oxygen saturation is 98% on room air. Examination of the left hip reveals mild crepitus, appropriate range of motion, adequate distal sensation, and palpable posterior tibial and dorsalis pedis pulses. A CT scan is ordered as seen in Figure A. Labs demonstrate an alkaline phosphatase of 876 U/L with a normal vitamin D and parathyroid hormone level (PTH). Which of the following is the most likely underlying etiology of this patient's symptoms?

          QID: 102581
          FIGURES:

          Multiple myeloma

          12%

          (2/16)

          Osteoarthritis

          6%

          (1/16)

          Osteogenic sarcoma

          6%

          (1/16)

          Paget disease of the bone

          69%

          (11/16)

          Rheumatoid arthritis

          0%

          (0/16)

          M 10 E

          Select Answer to see Preferred Response

          (M2.OR.15.69) A 67-year-old male presents with left hip pain. Examination reveals mild effusions in both knees, with crepitus in both patellofemoral joints. He states his hearing has worsened recently and that he feels like his hats don't fit anymore. Bone scan reveals diffuse uptake in the calvarium, right proximal femur, and left ilium. Which of the following laboratory abnormalities would be expected in this patient?

          QID: 104394

          Decreased serum alkaline phosphatase

          0%

          (0/36)

          Increased serum alkaline phosphatase

          89%

          (32/36)

          Decreased serum parathyroid hormone

          0%

          (0/36)

          Increased serum parathyroid hormone

          3%

          (1/36)

          Increased serum calcium

          3%

          (1/36)

          M 6 D

          Select Answer to see Preferred Response

          (M2.OR.15.38) A 63-year-old male presents to his family physician with a chief complaint of right knee and hip pain as well as pain in his left upper arm. He also reports frequent headaches and notes that he has had to adjust the headband on his favorite baseball cap to a larger size after it began feeling tight earlier this year. His wife, who accompanied the patient to the visit, complains that his hearing has deteriorated noticeably over the past several months. Physical examination shows bilateral knee effusions as well as tenderness to palpation of the left shoulder as well as the right hip and lower leg. Serologic testing reveals an elevated alkaline phosphate level but normal vitamin D, calcium, and PTH. A bone scan is conducted which is demonstrated in Figure A. Which of the following is the first-line treatment of this patient's condition?

          QID: 104485
          FIGURES:

          Teriparatide

          6%

          (5/83)

          Estrogen

          0%

          (0/83)

          Zoledronate

          71%

          (59/83)

          Dexamethasone

          7%

          (6/83)

          Methotrexate

          13%

          (11/83)

          M 7 C

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          (M2.OR.15.4694) 69-year-old man with no significant past medical history except for hypertension receives an X-ray of his skull (Figure A) after his car was rear-ended in a parking lot resulting in neck soreness. No fracture but what is the likely diagnosis of this incidental finding?

          QID: 107772
          FIGURES:

          Bony metastases

          0%

          (0/5)

          Osteoarthritis

          0%

          (0/5)

          Paget’s disease (osteitis deformans)

          100%

          (5/5)

          Osteitis fibrosa cystica

          0%

          (0/5)

          Osteomalacia

          0%

          (0/5)

          M 6 E

          Select Answer to see Preferred Response

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