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Pathogenesis of Paget's disease based on viral etiology
Abstract:
It has been slightly over 100 years since Sir James Paget's classic descriptions of "osteitis deformans" first appeared. He had described the mid- to late stages of patients with the chronic, debilitating, rare, and polyostotic forms of the disease. It is now known that the milder forms of the disease are quite common particularly in those of Anglo-Saxon ancestry. He believed the condition to be a chronic inflammation of unknown etiology because of its asymmetrical skeletal distribution, chronicity, and the gross appearance of the bones. With regard to the possible etiology of Paget's disease of bone, nothing worthy of note had been discovered until 1974 when viral-like inclusions were reported within the osteoclasts of all Paget's disease patients. In the ensuing decade, a great deal more circumstantial evidence from electron microscopic and immunologic studies supports the view that Paget's disease represents a slow virus infection. This article deals with the possible to probable viral etiology of Paget's disease with respect to its pathogenesis and its potential for eventual eradication. For many years Paget's disease was considered a disease almost exclusively confined to adulthood. Evidence now suggests that "familial chronic hyperphosphatasemia" represents the childhood form of Paget's disease.
Insights
Paget's disease of bone may be caused by a slow virus infection, with evidence mounting since 1974. This research explores the viral etiology, pathogenesis, and potential eradication of this chronic bone disease.
Area of Science:
- Bone biology
- Virology
- Genetics
Background:
- Paget's disease of bone, historically described by Sir James Paget, was initially considered a chronic inflammation of unknown cause.
- While classic descriptions focused on severe, late-stage disease, milder forms are now recognized as common, particularly in individuals of Anglo-Saxon descent.
- The condition was long thought to affect only adults, but familial chronic hyperphosphatasemia is now considered its childhood manifestation.
Purpose of the Study:
- To review the evidence supporting a viral etiology for Paget's disease of bone.
- To discuss the pathogenesis of the disease in light of a potential viral cause.
- To explore the implications of a viral etiology for the future eradication of Paget's disease.
Main Methods:
- Review of historical descriptions and epidemiological data.
- Analysis of findings from electron microscopy and immunologic studies.
- Examination of viral-like inclusions within osteoclasts.
Main Results:
- Viral-like inclusions were reported in osteoclasts of Paget's disease patients starting in 1974.
- Subsequent studies have provided circumstantial evidence supporting a slow virus infection as the cause.
- Paget's disease exhibits asymmetrical skeletal distribution and chronicity, consistent with certain viral infections.
Conclusions:
- Paget's disease of bone is increasingly suspected to result from a slow virus infection.
- Understanding the viral etiology is crucial for elucidating disease pathogenesis.
- Identifying a viral cause offers potential pathways for the eventual eradication of Paget's disease.