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Published on: December 10, 2010
Paget disease of bone
G David Roodman1, Jolene J Windle
1Department of Medicine, Division of Hematology-Oncology, University of Pittsburgh Medical Center, Pittsburgh, Pennsylvania 15240, USA. roodmang@upmc.edu
The Journal of Clinical Investigation
|February 4, 2005
Summary
Paget disease of bone involves abnormal bone remodeling. Research suggests a potential genetic link and the presence of paramyxoviruses in affected bone cells, pointing to possible causes.
Area of Science:
- Bone biology and pathology
- Genetics and virology
Background:
- Paget disease of bone (PD) is a chronic disorder characterized by focal areas of excessive bone resorption and disorganized new bone formation.
- This process leads to the replacement of normal bone marrow with vascular and fibrous tissue, compromising bone integrity.
Purpose of the Study:
- To review the current understanding of Paget disease of bone pathophysiology.
- To discuss the evidence supporting both genetic and viral factors in the etiology of PD.
Main Methods:
- Literature review of studies on Paget disease of bone.
- Analysis of genetic linkage data and identification of susceptibility loci.
- Examination of evidence for viral presence in pagetic osteoclasts.
Main Results:
- One PD-linked gene and multiple susceptibility loci have been identified.
- Paramyxoviral gene products have been detected in osteoclasts from patients with Paget disease.
- The exact etiology remains incompletely understood, but genetic and viral factors are implicated.
Conclusions:
- Paget disease of bone pathophysiology involves dysregulated bone remodeling.
- Genetic predisposition and potential viral triggers, such as paramyxoviruses, are key areas of investigation for PD etiology.
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