CC Jan 2016 Paget's Disease of Bone

echoecho's version from 2016-02-08 13:53


Question Answer
What is the story of how Paget's disease was discovered?In 1877, James Paget described a man with progressive bone deformities (while man was alive developed enlargment of the cranium, anterior spinal cervature, bowing of the legs) and at autopsy, the man's bones were so soft that it could be sliced with a razor. Dr. Paget termed this condition osteitis deformans (Paget's disease of the bone)
Define Paget's disease of bone?one or more areas of aggresive osteoclast-mediated bone resorption FOLLOWED by ineffective osteoblast-mediated bone repair
What is the exact etiology of Paget's disease of the bone?unclear (although environmental, genetic and viral causes are probably involved)
Paget's is a disease of ____ and ____ age?middle; advanced
What % of the US population > 40 years old are affected by Paget's?one
Gender that is slightly more often affected?male
Changes of Paget's disease expand from a _____ ______ to involve the entire bone?focal wedge
The most commonly involved areas of Paget's disease is what area? followed by what other areas?first the pelvis, then followed by the lumbar spine, femur, and thoracic spine
Is the process usually asymptomatic?yes
How is the process often discovered?incidentally discovered on radiograph for another reason
Describe the pain in Paget's disease?1) mild to moderate 2) deep 3) aching 4) persists throughout the day 5) often becomes worse at night
Distortion of what bones may be seen?1) skull 2) jaw 3) clavicle 4) long bones of the leg
What may appear on the affected bone?1) improperly healing fractures 2) fissures
Neurological symptoms due to the compression of adjacent structures can cause what symptoms?1) deafness 2) headache 3) dizziness
Less than 1% of patients with Paget's disease develop ______ (which is typically aggressive)?osteosarcoma
Is hypercalcemia common in Paget's disease?no, it is uncommon in Paget's disease, so if elevated serum calcium is present, this should prompt a search for other causes such as hyperparathyroidism
Alterations in mineral homeostasis produce elevated ______ when enough bone is involved?alkaline phosphatase
If there is only a small focus of disease or following treatment, serum alkaline phosphatase may ______?normalize
Does asymptomatic Paget's disease require treatment?no
Occasional bone pain is treated with what?Tylenol or NSAIDs
Fractures when present may be treated with what?standard surgical techniques
Prolonged immobilization may predispose to what and should be avoided?hypercalcemia
List the indications for pharmacotherapy?1) bone pain 2) skull deformity causing headaches 3) back pain due to bony deformity 4) fissure fractures 5) prior to elective surgery 6) when immobilization-induced hypercalcemia occurs
What is the pharmacological treatments of choice? bisphosphonates
Question Answer
List the 5 bisphosphonates currently FDA approved for Paget's disease?1) etidronate (Didronel) 2) alendronate (Fosamax) 3) Tiludronate (Skelid), 4) Risedronate (Actonel) 5) pamidronate (ARedia)
What are the dosages of the above?1) Oral tiludronate (400 mg/day x 12 weeks) 2) oral alendronate (40 mg daily x 6 months) 3) oral risdedronate (Actonel) (30 mg /day x 2 months) 4) IV pamidronate (Aredia) 3 infusions of 60 mg at 2 week intervals, or 6 infusions of 30 mg at weekly intervals
Bisphosphonates should be avoided in patients with what?1) significant renal impairment 2) those with difficulty swallowing
Bisphosphonates also increase what of the jaw?osteonecrosis
*** Among the oral bisphosphonates, what 2 medications are the most potent for inhibiting bone turnover?1) alendronate 2) risedronate
*** A single course of medical therapy for several months can produce remission up to _____ months?18
Patients receiving biphosphonates therapy should also receive supplemental calcium and vitamin D, what are the doses?1) calcium 1000-1500 mg 2) vitamin D 400 IU
What medication can patients who can't tolerate oral bisphosphonates take?IV zoledronic acid (Reclast)
Benefit of IV zoledronic acid (Reclast)?stronger and faster response than oral agents in addition to avoiding gastrointestinal side effects
What is the disadvantage of IV zoledronic acid (Reclast)?1)transient hypocalcemia (this occurs during administration, so oral calcium is given simultaneously) 2) mild flu-like illness that may persist for a few days
What lab level may take several months to normalize?serum alkaline phosphatase
*** Did a randomized trial comparing aggressive bisphosphonate treatment to maintain normal-range alkaline phosphatase levels vs. symptomatic treatment find any clinical advantage?no
While often used as a marker for recurrence, is there any data established that states a preferred alkaline phosphatase level with Paget's disease?no
If symptoms recur or there is a rapid risk in alkaline phosphatase, is retreatment warranted?yes
List emerging treatment strategies?1) denosumab (monoclonal antibody against the RANK ligand) 2) tocilzumab (an interleukin-6 receptor inhibitor) 3) odanacatib (a cathespin K inhibitor)
*** SUMMARY = Paget's is a disease of what age group?middle and advanced age
*** SUMMARY = What % of the US population has Paget's disease that are > 40 years of age?1%
*** SUMMARY = Does asymptomatic Paget's disease require treatment?no
*** SUMMARY = Patients with occasional bone pain are treated with what?1) Tylenol 2) NSAIDs
*** SUMMARY = List the indications for pharmacotherapy?1) bone pain 2) skull deformity causing headaches 3) back pain due to bony deformity 4) fissure fractures 5) prior to elective surgery 6) when immobilization-induced hypercalcemia occurs
*** SUMMARY = THe pharmacological treatments of choice are what classification of medications?biphosphonates