Pagets Disease

Pagets Disease

pagets-disease

Definition

  • Paget’s is caused by the excessive breakdown and formation of bone, followed by disorganized bone remodeling. This causes affected bone to weaken, resulting in pain, misshapen bones, fractures and arthritis in the joints near the affected bones.

Epidemiology and burden of Pagets Disease

  • Paget’s disease occurs in 1–2% of white adults over 50 and is more common in men. Prevalence increases substantially with age and by the eighth decade of life it may be present in approximately 8% of men and 5% of women. (https://www.nursingtimes.net)
  • The global prevalence varies from 1.5% to 8.3% depending on factors such as age, country of residence, and sex. Incidence increases after age 50 years. The prevalence is higher in the United Kingdom, France, Germany, Australia, New Zealand, and North America. It is rare in Asia, India, and Scandinavia. PDB is slightly more prevalent in men than in women. (http://www.clevelandclinicmeded.com/)
  • Affects more than 3% of population over 40 years of age around 2% of the population at age 40 to around 10% in the elderly (more than 80 years). Relatively common in Anglo-Saxons, Britain, Germany and Australia. Britain has the highest recorded prevalence. (https://www.flinders.edu.au)

Treatment for Pagets Disease

  • Imiquimod therapy is an effective possible treatment option for vulvar Paget’s disease, especially for women who have experienced recurrence after multiple surgical resections or who are with poor surgical candidates. (Gynecol Oncol.2015 Oct;139(1):165-71)
  • The 800-mg daily dose of tiludronate is effective for the treatment of Paget’s disease of bone. (Arthritis Rheum.1992 Aug;35(8):967-74)
  • 5% Imiquimod cream is a safe and effective therapeutic option for the treatment of primary vulvar Paget disease. (J Low Genit Tract Dis.2014 Oct;18(4):347-50)
  • The alendronate-treated group had significantly greater efficacy showing decreases in both serum alkaline phosphatase (79% vs. 44%) and urinary deoxypyridinoline (75% vs. 51%) than the etidronate-treated group and was well tolerated and had a safety profile similar to that of etidronate. (J Clin Endocrinol Metab.1996 Mar;81(3):961-7)

Complication of Pagets Disease

  • Hearing loss occurs in 61% of the patients suffering from paget’s disease. (J Bone Miner Res. 2008 Jun; 23(6): 819–825)
  • Paget’s disease of bone is associated with involvement of the central and peripheral nervous system. The brain, spinal cord, cauda equina, spinal roots, and cranial nerves can be affected in Paget’s disease due to their anatomic relationship to bone. (JBone Miner Res. 1999 Oct;14 Suppl 2:88-91)
  • Extramammary Paget disease (EMPD) is often associated with underlying or distant synchronous malignancies. Most patients (80.4%) had localized disease, while 17.1% had locoregional spread and 2.5% presented with distant disease. (Br J Dermatol.2014 Mar;170(3):661-71)

Risk factors for Pagets Disease

  • Bovine cattle [odds ratio (OR) = 2.14; 95% confidence interval (CI) = 1.16-3.94], consumption of meat traceable to sick livestock (OR = 2.70; 95% CI = 0.98-7.43), and frequent consumption during youth of brains (OR = 1.77; 95% CI = 1.05-2.98) and other viscera increased the risk for Paget’s disease of bone. (1997 May;8(3):247-51)
  • Arg194Trp polymorphism can be associated with nonmelanoma skin cancer and extramammary Paget’s disease risk in a Japanese population. (Arch Dermatol Res.2012 Jul;304(5):363-70)
  • Ownership of cats and birds excluding dog owners, increased the risk of Paget’s disease (OR = 2.5; 95% CI = 1.0-5.0; OR = 2.3; 95% CI = 1.0-5.3, respectively). (Bone.1996 Jul;19(1):47-50)
  • Degree of parental consanguinity (OR = 4.1; 95% CI = 2.1-1.8), and green or blue eye colour (OR = 1.5; 95% CI = 1.1-2.1) were significantly associated with PD. (Anthropol Anz.2012;69(4):417-22)

Systematic reviews

  • Epidemiology of Paget’s disease of bone: a systematic review..more
  • Polymorphisms in Autophagy Genes Are Associated with Paget Disease of Bone..more
  • Paget’s disease of bone: an endocrine society clinical practice guideline..more
  • Surgical treatment in spine Paget’s disease: a systematic review..more

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