Updated: 11/30/2019

Paget Disease of Bone (Osteitis Deformans)

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Questions
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Evidence
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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) 
  • Prognosis
    • expansion of untreated Paget 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/rickets  Decreased Decreased Increased Increased
Osteoporosis   Normal Normal Normal Normal
Osteopetrosis  Normal Normal or decreased Normal Normal
Paget disease of the bone  Normal Normal Increased Normal
Osteitis fibrosa cystica  Primary
hyperparathyroidism
• decreased
Secondary
hyperparathyroidism
• increased

Primary
hyperparathyroidism
• increased

Secondary
hyperparathyroidism
• decreased

Primary and secondary hyperparathyroidism
• increased
Primary and secondary hyperparathyroidism
• increased
Hypervitaminosis D Increased Increased Normal Decreased
 
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
 

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Questions (8)
Lab Values
Blood, Plasma, Serum Reference Range
ALT 8-20 U/L
Amylase, serum 25-125 U/L
AST 8-20 U/L
Bilirubin, serum (adult) Total // Direct 0.1-1.0 mg/dL // 0.0-0.3 mg/dL
Calcium, serum (Ca2+) 8.4-10.2 mg/dL
Cholesterol, serum Rec: < 200 mg/dL
Cortisol, serum 0800 h: 5-23 μg/dL //1600 h:
3-15 μg/dL
2000 h: ≤ 50% of 0800 h
Creatine kinase, serum Male: 25-90 U/L
Female: 10-70 U/L
Creatinine, serum 0.6-1.2 mg/dL
Electrolytes, serum  
Sodium (Na+) 136-145 mEq/L
Chloride (Cl-) 95-105 mEq/L
Potassium (K+) 3.5-5.0 mEq/L
Bicarbonate (HCO3-) 22-28 mEq/L
Magnesium (Mg2+) 1.5-2.0 mEq/L
Estriol, total, serum (in pregnancy)  
24-28 wks // 32-36 wks 30-170 ng/mL // 60-280 ng/mL
28-32 wk // 36-40 wks 40-220 ng/mL // 80-350 ng/mL
Ferritin, serum Male: 15-200 ng/mL
Female: 12-150 ng/mL
Follicle-stimulating hormone, serum/plasma Male: 4-25 mIU/mL
Female: premenopause: 4-30 mIU/mL
midcycle peak: 10-90 mIU/mL
postmenopause: 40-250
pH 7.35-7.45
PCO2 33-45 mmHg
PO2 75-105 mmHg
Glucose, serum Fasting: 70-110 mg/dL
2-h postprandial:<120 mg/dL
Growth hormone - arginine stimulation Fasting: <5 ng/mL
Provocative stimuli: > 7ng/mL
Immunoglobulins, serum  
IgA 76-390 mg/dL
IgE 0-380 IU/mL
IgG 650-1500 mg/dL
IgM 40-345 mg/dL
Iron 50-170 μg/dL
Lactate dehydrogenase, serum 45-90 U/L
Luteinizing hormone, serum/plasma Male: 6-23 mIU/mL
Female: follicular phase: 5-30 mIU/mL
midcycle: 75-150 mIU/mL
postmenopause 30-200 mIU/mL
Osmolality, serum 275-295 mOsmol/kd H2O
Parathyroid hormone, serume, N-terminal 230-630 pg/mL
Phosphatase (alkaline), serum (p-NPP at 30° C) 20-70 U/L
Phosphorus (inorganic), serum 3.0-4.5 mg/dL
Prolactin, serum (hPRL) < 20 ng/mL
Proteins, serum  
Total (recumbent) 6.0-7.8 g/dL
Albumin 3.5-5.5 g/dL
Globulin 2.3-3.5 g/dL
Thyroid-stimulating hormone, serum or plasma .5-5.0 μU/mL
Thyroidal iodine (123I) uptake 8%-30% of administered dose/24h
Thyroxine (T4), serum 5-12 μg/dL
Triglycerides, serum 35-160 mg/dL
Triiodothyronine (T3), serum (RIA) 115-190 ng/dL
Triiodothyronine (T3) resin uptake 25%-35%
Urea nitrogen, serum 7-18 mg/dL
Uric acid, serum 3.0-8.2 mg/dL
Hematologic Reference Range
Bleeding time 2-7 minutes
Erythrocyte count Male: 4.3-5.9 million/mm3
Female: 3.5-5.5 million mm3
Erythrocyte sedimentation rate (Westergren) Male: 0-15 mm/h
Female: 0-20 mm/h
Hematocrit Male: 41%-53%
Female: 36%-46%
Hemoglobin A1c ≤ 6 %
Hemoglobin, blood Male: 13.5-17.5 g/dL
Female: 12.0-16.0 g/dL
Hemoglobin, plasma 1-4 mg/dL
Leukocyte count and differential  
Leukocyte count 4,500-11,000/mm3
Segmented neutrophils 54%-62%
Bands 3%-5%
Eosinophils 1%-3%
Basophils 0%-0.75%
Lymphocytes 25%-33%
Monocytes 3%-7%
Mean corpuscular hemoglobin 25.4-34.6 pg/cell
Mean corpuscular hemoglobin concentration 31%-36% Hb/cell
Mean corpuscular volume 80-100 μm3
Partial thromboplastin time (activated) 25-40 seconds
Platelet count 150,000-400,000/mm3
Prothrombin time 11-15 seconds
Reticulocyte count 0.5%-1.5% of red cells
Thrombin time < 2 seconds deviation from control
Volume  
Plasma Male: 25-43 mL/kg
Female: 28-45 mL/kg
Red cell Male: 20-36 mL/kg
Female: 19-31 mL/kg
Cerebrospinal Fluid Reference Range
Cell count 0-5/mm3
Chloride 118-132 mEq/L
Gamma globulin 3%-12% total proteins
Glucose 40-70 mg/dL
Pressure 70-180 mm H2O
Proteins, total < 40 mg/dL
Sweat Reference Range
Chloride 0-35 mmol/L
Urine  
Calcium 100-300 mg/24 h
Chloride Varies with intake
Creatinine clearance Male: 97-137 mL/min
Female: 88-128 mL/min
Estriol, total (in pregnancy)  
30 wks 6-18 mg/24 h
35 wks 9-28 mg/24 h
40 wks 13-42 mg/24 h
17-Hydroxycorticosteroids Male: 3.0-10.0 mg/24 h
Female: 2.0-8.0 mg/24 h
17-Ketosteroids, total Male: 8-20 mg/24 h
Female: 6-15 mg/24 h
Osmolality 50-1400 mOsmol/kg H2O
Oxalate 8-40 μg/mL
Potassium Varies with diet
Proteins, total < 150 mg/24 h
Sodium Varies with diet
Uric acid Varies with diet
Body Mass Index (BMI) Adult: 19-25 kg/m2
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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?
Review Topic | Tested Concept

QID: 109612
1

Hearing loss

56%

(28/50)

2

Bence Jones proteins

8%

(4/50)

3

Hypercalcemia

10%

(5/50)

4

Adenocarcinoma of the gallbladder

8%

(4/50)

5

Obstructive jaundice

16%

(8/50)

L 4 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? Review Topic | Tested Concept

QID: 107772
FIGURES:
1

Bony metastases

0%

(0/4)

2

Osteoarthritis

0%

(0/4)

3

Paget’s disease (osteitis deformans)

100%

(4/4)

4

Osteitis fibrosa cystica

0%

(0/4)

5

Osteomalacia

0%

(0/4)

L 2 E

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(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? Review Topic | Tested Concept

QID: 104394
1

Decreased serum alkaline phosphatase

0%

(0/31)

2

Increased serum alkaline phosphatase

90%

(28/31)

3

Decreased serum parathyroid hormone

0%

(0/31)

4

Increased serum parathyroid hormone

3%

(1/31)

5

Increased serum calcium

0%

(0/31)

L 2 C

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(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? Review Topic | Tested Concept

QID: 104485
FIGURES:
1

Teriparatide

6%

(5/79)

2

Estrogen

0%

(0/79)

3

Zoledronate

72%

(57/79)

4

Dexamethasone

6%

(5/79)

5

Methotrexate

13%

(10/79)

L 3 C

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(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? Review Topic | Tested Concept

QID: 102581
FIGURES:
1

Multiple myeloma

67%

(2/3)

2

Osteoarthritis

0%

(0/3)

3

Osteogenic sarcoma

0%

(0/3)

4

Paget disease of the bone

33%

(1/3)

5

Rheumatoid arthritis

0%

(0/3)

L 2 E

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