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Paget disease of bone

Last updated: February 5, 2021

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Paget disease of bone (PDB, or osteitis deformans) is a slowly progressive monostotic or polyostotic skeletal disease. It is characterized by increased bone turnover, which causes normal lamellar bone to be replaced by weak woven bone. The cause of this common yet underdiagnosed skeletal disease is not known. It predominantly affects individuals over the age of 55 and is characterized by localized pain and bony deformities (such as bowing of long bones). Skeletal x-ray, bone scans, and serum alkaline phosphatase are important tests for diagnosing and monitoring the progression of PDB. Treatment is mainly supportive and involves the use of bisphosphonates to inhibit osteoclastic function.

Paget disease of the bone should not be confused with Paget disease of the nipple or Paget disease of the vulva, which are named after the same physician.

  • Prevalence: second most prevalent skeletal disease after osteoporosis in individuals > 50 years of age [1]
  • Sex: > (1.2:1)
  • Age of onset: > 55 years [1]

Epidemiological data refers to the US, unless otherwise specified.

Overview

Stages of Paget disease

Bone remodeling in Paget disease occurs in three phases, followed by a quiescent stage: [2]

Disease localization

The pelvis, skull, vertebral column, and long bones of the lower extremities are the most commonly affected sites.

  • Monostotic PDB: affects only one bone (∼ ⅓ of cases)
  • Polyostotic PDB: affects two or more bones (∼ ⅔ of cases)

Laboratory tests

Paget disease should be considered in an asymptomatic patient who presents with isolated ALP elevation that cannot be explained by any other means (e.g., cholestasis or bone metastases).

Imaging [4][5]

Pathology

Osteopetrosis (marble bone disease) [6]

Fibrous dysplasia [7]

McCune-Albright syndrome

The 3 P's of McCune-Albright syndrome: Polyostotic fibrous dysplasia, Pigmentation (cafe au lait spots), Precocious puberty

The differential diagnoses listed here are not exhaustive.

Medical therapy [9]

Medical therapy should be initiated among all patients with active disease (elevated ALP levels or active disease foci on skeletal scintigraphy).

Surgical therapy [9]

  • Osteoarthritis
  • Malignant degeneration into osteosarcoma (very rare: < 1% of cases)
  • High-output cardiac failure (due to the formation of arteriovenous shunts within the bone, which leads to an increased overall blood flow)

We list the most important complications. The selection is not exhaustive.

  1. Lalam RK, Cassar-pullicino VN, Winn N. Paget Disease of Bone. Semin Musculoskelet Radiol. 2016; 20 (3): p.287-299. doi: 10.1055/s-0036-1592368 . | Open in Read by QxMD
  2. Le T, Bhushan V,‎ Sochat M, Chavda Y, Abrams J, Kalani M, Kallianos K, Vaidyanathan V. First Aid for the USMLE Step 1 2019. McGraw-Hill Medical
  3. Alexandersen P, Peris P, Guañabens N, et al. Non-isomerized C-telopeptide fragments are highly sensitive markers for monitoring disease activity and treatment efficacy in Paget's disease of bone. J Bone Miner Res. 2004; 20 (4): p.588-595. doi: 10.1359/JBMR.041212 . | Open in Read by QxMD
  4. Whyte MP. Clinical practice. Paget's disease of bone. N Engl J Med. 2006; 355 (6): p.593-600. doi: 10.1056/NEJMcp060278 . | Open in Read by QxMD
  5. Sclerotic bone tumors and tumor-like lesions. http://www.radiologyassistant.nl/en/p4bc9a97980036/sclerotic-bone-tumors-and-tumor-like-lesions.html. Updated: November 1, 2013. Accessed: January 15, 2017.
  6. Gupta RK. Long bone fractures in osteopetrosis: awareness of primary pathology and appropriate pre-operative planning necessary to avoid pitfalls in fixation. Injury Extra. 2005; 36 (9): p.411. doi: 10.1016/j.injury.2004.05.032 . | Open in Read by QxMD
  7. ller L, Wood NH, Khammissa RA, Lemmer J, Raubenheimer EJ. The nature of fibrous dysplasia. Head Face Med. 2009; 5 : p.22. doi: 10.1186/1746-160X-5-22 . | Open in Read by QxMD
  8. Dumitrescu CE, Collins MT. McCune-Albright syndrome. Orphanet J Rare Dis. 2008; 3 (1). doi: 10.1186/1750-1172-3-12 . | Open in Read by QxMD
  9. Ralston SH, Langston AL, Reid IR. Pathogenesis and management of Paget's disease of bone. Lancet. 2008; 372 (9633): p.155-163. doi: 10.1016/S0140-6736(08)61035-1 . | Open in Read by QxMD
  10. Singer FR, Fredericks RS, Minkin C. Salmon calcitonin therapy for Paget's disease of bone: The problem of acquired clinical resistance. Arthritis Rheum. 1980; 23 (10): p.1148-1154.