Paget's disease of bone

For other diseases named after Paget, see Paget's disease (disambiguation).
Paget's disease of bone
Other namesosteitis deformans, Paget's disease
"This 92 year-old male patient presented for assessment of sudden inability to move half his body. An incidental finding was marked thickening of the calvarium. The diploic space is widened and there are ill-defined sclerotic and lucent areas throughout. The cortex is thickened and irregular. The findings probably correspond to the 'cotton wool spots' seen on plain films in the later stages of Paget's disease."
Pronunciation
SpecialtyRheumatology Edit this on Wikidata
Named afterJames Paget

Paget's disease of bone (commonly known as Paget's disease or, historically, osteitis deformans) is a condition involving cellular remodeling and deformity of one or more bones. The affected bones show signs of dysregulated bone remodeling at the microscopic level, specifically excessive bone breakdown and subsequent disorganized new bone formation.[1] These structural changes cause the bone to weaken, which may result in deformity, pain, fracture or arthritis of associated joints.[1]

The exact cause is unknown, although leading theories indicate both genetic and acquired factors (see Causes). Paget's disease may affect any one or several bones of the body (most commonly pelvis, tibia, femur, lumbar vertebrae, and skull), but never the entire skeleton,[1][2][3] and does not spread from bone to bone.[4] Rarely, a bone affected by Paget's disease can transform into a malignant bone cancer.

As the disease often affects people differently, treatments of Paget's disease can vary. Although there is no cure for Paget's disease, medications (bisphosphonates and calcitonin) can help control the disorder and lessen pain and other symptoms. Medications are often successful in controlling the disorder, especially when started before complications begin.

Paget's disease affects from 1.5 to 8.0% of the population, and is most common in those of British descent followed by Northern European and Northern Americans.[5] [6] It is primarily diagnosed in older people and is rare in people less than 55 years of age.[7] Men are more commonly affected than women (3:2).[8] The disease is named after English surgeon Sir James Paget, who described it in 1877.

  1. ^ a b c Paul Tuck, Stephen; Layfield, Robert; Walker, Julie; Mekkayil, Babitha; Francis, Roger (December 2017). "Adult Paget's disease of bone: a review". Rheumatology. 56 (12): 2050–2059. doi:10.1093/rheumatology/kew430. PMID 28339664.
  2. ^ Daroszewska, A.; Ralston, S. H. (2006). "Mechanisms of Disease: genetics of Paget's disease of bone and related disorders". Nature Reviews Rheumatology. 2 (5): 270–277. doi:10.1038/ncprheum0172. PMID 16932700. S2CID 22051952.
  3. ^ Ralston, Stuart H.; Layfield, Rob (29 April 2012). "Pathogenesis of Paget Disease of Bone". Calcified Tissue International. 91 (2): 97–113. doi:10.1007/s00223-012-9599-0. PMID 22543925. S2CID 17244878.
  4. ^ Charles, Julia F.; Siris, Ethel S.; Roodman, G. David (2018). "Paget Disease of Bone". Primer on the Metabolic Bone Diseases and Disorders of Mineral Metabolism. John Wiley & Sons. pp. 713–720. ISBN 978-1-119-26656-3.
  5. ^ Greene, Walter B. (1 December 2005). Netter's Orthopaedics (1st ed.). Saunders. p. 38. ISBN 978-1929007028.{{cite book}}: CS1 maint: date and year (link)
  6. ^ Nebot Valenzuela, Elena; Pietschmann, Peter (6 September 2016). "Epidemiology and pathology of Paget's disease of bone – a review". Wiener Medizinische Wochenschrift. 167 (1–2): 2–8. doi:10.1007/s10354-016-0496-4. PMC 5266784. PMID 27600564.
  7. ^ Ralston, Stuart H. (14 February 2013). "Paget's Disease of Bone". New England Journal of Medicine. 368 (7): 644–650. doi:10.1056/NEJMcp1204713. PMID 23406029.
  8. ^ Kumar, Parveen; Clark, Micheal (2009). Welcome to Kumar and Clark's Clinical Medicine (7th ed.). Elsiver. p. 565. ISBN 978-0-7020-2993-6.