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Pagets disease of bone – quick review card

Pagets disease of the bone – Focal skeletal disorder of bone remodelling

Non-inflammatory ,metabolic disorder , Excessive localised bone resorption via osteoclasts , Increased compensatory osteoblastic activity to compensate for excessive localised bone loss , Leads to unstructured , fibroblastic , biomechanically unstable bone , deformity & enlargement , Poor quality bone with both sclerotic & lytic areas

 

How common – chronic focal high bone turnover disorder second most common metabolic bone disorder after osteoporosis affects about 1 % to 2 % of the population and mostly people > 55 more common in men often runs in families ( up to 40 % of cases ) with autosomal dominant pattern of inheritance seen more commonly in people of British descent it is thought that incidence of PDB has decreased in most regions over recent years

 

also known as osteitis deformans first described in 1876 by London physician J Paget it may affect a single bone , several or many bones but does not affect the whole skeleton cause remains unknown but environmental factors as viral infection has been implicated (likely multifactorial ) osteoclasts which are abundant in Paget’s lesion have been found to be different – ie they are larger , have more nuclei per osteoclast , are more efficient at bone resorption , have increased 1,25-dihydroxyvitamin D3 and RANKL responsivity and secrete higher most commonly affects the pelvis , femur , lumbar spine , skull and tibia but lesions can happen anywhere

 

Presentation – mostly discovered incidentally (blood tests or radiographs ) pain which is worse at rest
( dull , deep ) , at night or after waking and is relieved by movement can be asymptomatic in majority of patients.

 

Complications – pathological fractures ○ painful fissure fractures bone enlargement and deformity ( facial appearance can change if skull and facial bones are involved ) , bowing of bones , kyphosis , reduced ROM hearing loss ( involvement of otic capsule ) , headache syndromes of neurological compression ,obstructive hydrocephalus , spinal canal stenosis secondary osteoarthritis ( damage to cartilage ) increased risk of cancerous transformation – osteosarcoma & other sarcomas excessive bleeding due to increased vascularity during surgery and delayed healing.

 

Others – cardiological system 
( high output CCF , aortic stenosis , atherosclerosis , endocardial calcification )

 metabolic as hypercalcemia , hypercalciuria , nephrolithiasis , hyperuricaemia

Diagnosis – calcium & phosphorus are usually N alkaline phosphatase is likely to be elevated but can be normal if skeletal involvement is minimal disease , monostotic PDB , inactive disease or following antipaget Rx focal lytic lesions total body bone scan or radiological skeletal survey radionuclide bone scanning bone biopsy

Management – pain control ( NSAIDs, aspirin ) orthotic support in patients with deformity antipagetic drug treatment as calcitonin , bisphosphonates -> mainstay of management surgery – fracture stabilization , corrective osteotomy , total joint arthroplasty

REFERENCES

  1. Bolland MJCundyPaget’s disease of bone: clinical review and update 
  2. Nebot Valenzuela, Elena, and Peter Pietschmann. “Epidemiology and pathology of Paget’s disease of bone – a review.” “Epidemiologie und Pathologie des Morbus Paget – ein Überblick.” Wiener medizinische Wochenschrift (1946) vol. 167,1-2 (2017): 2-8. doi:10.1007/s10354-016-0496-4
  3. Shaker, Joseph L. “Paget’s Disease of Bone: A Review of Epidemiology, Pathophysiology and Management.” Therapeutic advances in musculoskeletal disease vol. 1,2 (2009): 107-25. doi:10.1177/1759720X09351779
  4. Paget’s disease – Rare Disease Database Paget’s Disease – NORD (National Organization for Rare Disorders) (rarediseases.org)
  5. Luigi Gennari, Domenico Rendina, Tommaso Picchioni, Simone Bianciardi, Maria Materozzi, Ranuccio Nuti & Daniela Merlotti (2018) Paget’s disease of bone: an update on epidemiology, pathogenesis and pharmacotherapy, Expert Opinion on Orphan Drugs, 6:8, 485-496, DOI: 10.1080/21678707.2018.1500691
  6. Luis Corral-Gudino, Maria Borao-Cengotita-Bengoa, Javier Del Pino-Montes, Stuart Ralston,
    Epidemiology of Paget’s disease of bone: A systematic review and meta-analysis of secular changes, Bone, Volume 55, Issue 2, 2013, Pages 347-352, ISSN 8756-3282,
    https://doi.org/10.1016/j.bone.2013.04.024. ( Abstract )
  7. Paget’s disease of the bone Tim Cundy *Paget’s disease of bone (metabolismjournal.com)

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