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Published on: December 10, 2010
Paget's disease of bone
Mickaël Rousière1, Laëtitia Michou, François Cornélis
1Fédération de Rhumatologie, Centre Viggo Petersen, Hôpital Lariboisière, 2 rue Ambroise Paré, 75475 Paris 10, France.
Abstract:
Paget's disease of bone is characterized by an anarchic bone remodelling, associated with morphological and functional abnormalities of osteoclasts. Its prevalence and incidence rates decreased gradually over the past two decades; the reason for this remains unclear. The aetiology of the disease is still obscure, the paramyxoviral theory being very controversial. Recent advances in understanding of the disease come from genetic studies, with the identification of specific mutations in the p62-sequestosome gene, which could be involved in pathogenetic mechanisms leading to increased osteoclast activity. The disease affects one or several bone pieces, leading to bone pain, deformities, characteristic imaging features, and increased markers of bone remodelling. The long-lasting disease activity leads to complications, including arthropathies, neurological compressions, fissures or fractures and, rarely, osteosarcomatous transformation of a pagetic lesion. Potent bisphosphonates have proven their efficacy in reducing symptoms and disease activity. They are currently used as the first-line treatment with the goal of normalizing bone remodelling and, hopefully, preventing late complications.
Insights
Paget
Area of Science:
- Bone biology and skeletal disorders.
- Genetics and molecular mechanisms of bone diseases.
Background:
- Paget's disease of bone involves abnormal bone remodeling and osteoclast dysfunction.
- The disease's prevalence has declined, but its cause remains largely unknown.
- Genetic factors, such as p62-sequestosome gene mutations, are implicated in pathogenesis.
Purpose of the Study:
- To review current understanding of Paget's disease of bone.
- To highlight recent genetic discoveries and their implications.
- To discuss disease manifestations, complications, and treatment strategies.
Main Methods:
- Literature review of Paget's disease of bone.
- Analysis of genetic studies identifying key mutations.
- Synthesis of information on clinical presentation and therapeutic outcomes.
Main Results:
- Paget's disease is characterized by disordered bone remodeling and osteoclast abnormalities.
- Genetic studies point to mutations in the p62-sequestosome gene.
- Bisphosphonates are effective first-line treatments for symptom and disease activity reduction.
Conclusions:
- Paget's disease of bone involves complex genetic and cellular mechanisms.
- Effective treatments like bisphosphonates manage symptoms and aim to prevent complications.
- Further research is needed to fully elucidate the disease etiology and optimize management.
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