Term
| What is another name for Paget's disease? |
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Definition
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Term
| What is the second most common bone disease after osteoporosis? |
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Definition
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Term
| Paget's disease is uncommon before age ___. |
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Definition
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Term
| What demographic is Paget's disease most common in? |
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Definition
| Northern European descent |
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Term
| What type of inheritance does classical Paget's disease have? |
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Definition
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Term
| What type of skeletal disorder is Paget's? |
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Definition
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Term
| What causes the symptoms of Paget's? |
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Definition
| increased size and number of osteoclasts |
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Term
| What happens to bone in a pt. with Paget's? |
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Definition
localized areas of excessive bone resorption and formation
may have only one affected bone or have pagetic lesions in multiple bones
new lesions rarely develop in previously unaffected bone after diagnosis |
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Term
| Pagetic bone is _________ than normal bone. |
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Definition
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Term
| Describe the pathogenesis of Paget's. |
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Definition
large and numerous osteoclasts cause excessive bone resorption
subsequent bone formation is abnormally increased with osteoblasts that are hyperactive but look normal
osteoblasts rapidly deposit new bone in a chaotic fashion |
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Term
| What does pagetic bone look like? |
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Definition
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Term
| What causes the extensive pitting of bone in Paget's? |
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Definition
| increased osteoclastic bone resorption |
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Term
| How is diagnosis of Paget's often made? |
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Definition
incidentally
pt. is often asymptomatic or only mildly symptomatic |
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Term
| What incidental findings may lead to a diagnosis of Paget's? |
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Definition
elevated total or bone specific serum alkaline phosphatase
radiological findings |
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Term
| If a patient is symptomatic, what symptoms might they present with if they have Paget's? |
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Definition
pain, fracture, deformity, osteoarthritis, hearing loss
non-specific symptoms that may be suggestive of other conditions |
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Term
| What are the most commonly affected bones in Paget's? |
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Definition
skull
vertebrae
pelvis
femur
tibia |
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Term
| What lab test is a marker of bone formation? |
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Definition
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Term
| What level of alkaline phosphatase points to a diagnosis of Paget's disease? |
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Definition
| any level above normal, especially in the absence of elevated liver enzymes |
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Term
| What lab test might be most reliable in diagnosing Paget's? |
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Definition
| bone-specific alkaline phosphatase |
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Term
| What usually confirms the diagnosis of Paget's? |
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Definition
| characteristic appearance of radiographs |
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Term
| What diagnostic tool is used to assess the extent of disease in Paget's? |
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Definition
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Term
| What is early-stage Paget's disease cause? |
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Definition
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Term
| What type of bone lesion is common in long bones during the lytic phase of Paget's? |
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Definition
| V-shaped "blade of grass" lesion |
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Term
| An osteolytic lesion in a long bone advances up or down the bone about ____ per year. |
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Definition
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Term
| As Paget's disease advances, the cortices become coarsely trabeculated and thickened, and the bone _________ and ______. |
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Definition
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Term
| What happens with the advancement of Paget's? |
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Definition
sclerotic changes with enlargement and thickening of long bones
secondary osteolytic fronts
cortical thickening |
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Term
| What is a common lesion of the skull that occurs during the lytic phase of Paget's? |
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Definition
| osteoporosis circumscripta (lytic border) |
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Term
| What radiologic appearance is characteristic of advanced sclerotic Paget's disease in the skull? |
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Definition
"cotton wool" skull
diffuse sclerotic changes |
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Term
| In the late (sclerotic) stage of Paget's disease, what predominates? |
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Definition
| overgrowth of bone predominates even though bone resorption continues |
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Term
| What makes the long bones affected by Paget's disease more likely to deform and fracture? |
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Definition
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Term
| What is the characteristic radiologic appearance in the vertebrae of a pt. with Paget's? |
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Definition
"picture frame" lesion
thickened cortex frames a lytic lesion |
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Term
| What might you find on physical exam in a pt. with Paget's? |
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Definition
-skeletal deformity
-abnormal gait
-elevated temp, erythema, tenderness over affected bone
-hearing loss
-dilated scalp veins
-high-output cardiac failure
-neurological findings |
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Term
| What physical exam findings might you see with Paget's disease of the skull? |
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Definition
frontal bossing
knobby deformity
enlargement of maxilla or mandible |
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Term
| What are other complications associated with Paget's? |
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Definition
-osteoarthritis adjacent to affected bones, particularly in the hips
-fracture (complete, fissure, vertebral, compression)
-neurologic
-cardiac
-neoplastic (rare--osteosarcoma, benign giant cell tumors) |
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Term
| What neurologic conditions are associated with Paget's? |
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Definition
hearing deficit
cranial nerve deficits
mottled retinal degeneration
angioid streaks
basilar impression
hydrocephalus
myelopathy
radicular neuropathies
spinal stenosis
spinal vascular steal syndrome |
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Term
| Why can Paget's cause neurologic symptoms? |
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Definition
When pagetic bone is adjacent to neural structures, expanded bone may cause neurologic sequelae.
It is also possible that dysfunction may occur from a vascular steal syndrome. |
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Term
| What is a vascular steal syndrome? |
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Definition
| vascular blood around the spine steals blood to feed the lesions, causing pain and decreased circulation |
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Term
| What bone is affected by Paget's if the pt. has hearing loss? |
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Definition
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Term
| What type of hearing loss should you expect in a pt. with Paget's? |
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Definition
high-frequency sensory loss
low-frequency conductive loss
bilateral
progressive |
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Term
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Definition
| crack in Bruch's membrane in the eye |
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Term
| What rare disorder is angioid streak also found in? |
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Definition
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Term
| What causes the increased cardiac output associated with Paget's disease? |
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Definition
| increased vascularity of bone and of the overlying muscle and skin |
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Term
| What specific cardiovascular complications can occur with Paget's? |
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Definition
increased cardiac output
CHF (rare)
generalized atherosclerosis
aortic valve calcification
endocardial calcification |
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Term
| What is the most serious complication of Paget's but only occurs in <1% of patients? |
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Definition
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Term
| What do patients with osteosarcoma secondary to Paget's usually die from? |
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Definition
| local extension or pulmonary metastases |
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Term
| What are common and frequent comorbidities with Paget's? |
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Definition
arthritis/arthrosis
hypertension
heart problems
depression |
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Term
| What is the gold standard in treatment of Paget's? |
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Definition
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Term
| What other treatments, besides bisphosphonates, are used in the treatment of Paget's? |
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Definition
SQ calcitonin (rarely used)
pain management (NSAIDs, COX-2 inhibitors, analgesics, opioids)
surgery (fractures, bone deformities, osteoarthritis) |
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Term
| What are the indications for treatment of Paget's disease? |
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Definition
-bone pain
-preparation for orthopedic surgery
-fracture of pagetic bone
-hypercalcemia and/or hypercalciuria
-neurologic deficit associated with cranial or vertebral disease
-presence of high-output CHF
-prevention of future complications |
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Term
| What are the primary goals of treatment of Paget's disease? |
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Definition
relieve current symptoms
prevent progression
help avoid complications |
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Term
| Does zoledronic acid (Reclast) or risedronate (Actonel) seem to have better efficacy in lowering alkaline phosphatase levels and maintaining them? |
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Definition
| zoledronic acid (Reclast) |
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Term
| How does synthetic salmon calcitonin work in treating Paget's disease? |
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Definition
reduces osteoclastic activity which decreases bone resorption
also has significant analgesic effect on bone pain
get healing of osteolytic lesions |
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Term
| What should a pt. take after a zoledronic acid infusion? |
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Definition
| 1500 mg of calcium and 800 IU of vitamin D (preferably D3) daily during the 10 days after infusion |
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Term
| What follow up is needed in an untreated pt. with Paget's disease? |
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Definition
annual serum total or bone-specific alkaline phosphatase
periodic x-rays of osteolytic lesions |
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Term
| What follow up is needed in a pt. treated for Paget's disease? |
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Definition
serum total or bone-specific alkaline phosphatase measurement every 3-6 months
bone resorption markers are optional
periodic x-rays of osteolytic lesions |
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Term
| What test can be used as an alternative to serum alkaline phosphatase? |
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Definition
| urinary collagen cross-link measurements |
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