Paget's Disease of Bone

What is Paget’s disease of bone?

Paget’s disease of the bone is a disease characterized by an abnormal resorption of bones affecting one or multiple bones. The disease is initiated by an intense wave of bone destructing activity (osteolysis) with resorption of normal bone resulting in irregularly shaped resorption pattern created on the affected bone, which is followed by vigorous bone deposition activity (osteoblastic activity) forming bone after a variable period. The affected bones in Paget’s disease becomes weakened and distorted and may undergo severe complications as the condition progresses.

Paget’s Disease Symptoms

Paget’s disease is a relatively common disorder; however the prevalence of Paget’s disease shows a marked geographic variance. The disease is seen in Britain more commonly then the US and is vey rare in Asian and African countries. The disease mainly affects older individuals and majority of the times encountered in patients above 40 years of age.

The commonly affected bones are skull, femur, sacrum and pelvis. Bony deformities, facial asymmetry, deafness and mental disturbances may be experienced depending on the type and site of affected area of the bone. In case of skull bone involvements the skull becomes enlarged and patients may complain of tightening of existing hats. The upper jaw is more commonly in involved in Paget’s disease of facial bones and it leads to movement and migration of affected teeth. Bone pain is constant at weight bearing areas and bowing of legs may be seen if femur is affected. All bones affected by Paget’s disease are at a risk of pathological fractures.

Causes of Paget’s Disease of Bone

The exact cause of Paget’s disease is not known but inflammatory, genetic and endocrine factors are perceived as the contributing agents.
Generally, up to 40% of affected patients of Paget’s disease show a positive family history of the disease. Genetically Paget’s disease can be passed on as an autosomal dominant trait. It is found in patients with a rare hereditary syndrome that includes; Paget’s disease of bone, inclusion body myopathy and fronto-temporal dementia.

Another suspected causative factor for Paget’s disease is circulatory disturbances .In an affected bone with Paget’s diseases excessive vascularity is observed and it has been suggested that vessels are similar to arteriovenous aneurysms. Paget’s disease resulting from an infection with a slow virus is also one of the theories undergoing research. Others associated risk factors with Paget’s disease include vasculitis, trauma, endocrine diseases and degenerative neurological disorders.

Paget’s Disease Treatment

The medicinal treatment includes supplement of calcitonin, a parathyroid hormone antagonist produced by thyroid gland suppress bone resorption. The hormone is useful in relieving pain and decreasing serum alkaline. Few other drugs such as sodium phosphatase can be used to retard bone resorption. Drugs like Diphosphanates inhibit both resorption and mineralization of the bone. Mithramycin is drug that destroys osteoclast cells, hence retarding the rate of bone resorption. The other treatment options include surgical intervention and radiation to the affected bone. Radiation therapy is the most successful and widely accepted treatment modality.

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