Any bone or bones can be affected, but Pagets disease occurs most frequently in the spine, skull, pelvis, femur, and lower legs. Osteogenic sarcoma, a form of bone cancer, is a rare complication of Pagets disease occurring in less than one percent of those affected. The development of osteosarcoma may be suggested by the sudden onset or worsening pain. Epidemiology
Pagets disease of bone is the second most common metabolic bone disorder, after osteoporosis. The overall prevalence and severity of Pagets disease are decreasing; the cause for these changes is unclear. Pagets disease is rare in people less than 55 years of age, and the prevalence increases with age. Evidence from studies of autopsy results have demonstrated Pagets disease in about 3 percent of people older than 40 years of age. Pagets disease is more common in males than females. Rates of Pagets disease are about 50 percent higher in men than in women. About 15 percent of people with Pagets disease also have a family member with the disease. In cases where the disease is familial, it is inherited in an autosomal dominant fashion, although not all people that inherit the affected version of the genes will express the disease (incomplete penetrance).The incidence of Pagets disease varies considerably with geographic location. Pagets predominantly affects people of European descent, whereas people of African, Asian, or Indian descent are less commonly affected. Pagets disease is less common in Switzerland and Scandinavia than in the rest of Western Europe.
Zoledronic acid is given as an intravenous infusion; a single dose is effective for two years. This is recommended for most people at high risk with active disease. Calcitonin
Salcatonin, also called calcitonin-salmon is a synthetic copy of a polypeptide hormone secreted by the ultimobranchial gland of salmon. Miacalcin is administered by injection, three times per week or daily, for 6–18 months. Repeat courses can be given after brief rest periods. Miacalcin may be appropriate for certain patients but is seldom used. Calcitonin was putatively linked to increased chance of cancer. The European Medicines Agency (EMA) recommended that calcitonin be used only on a short-term basis for 3 conditions for which it had previously been approved in the European Union: Pagets disease, acute bone loss resulting from sudden immobilization, and hypercalcemia caused by cancer. As a solution for injection or infusion, calcitonin should be administered for no more than 4 weeks to prevent acute bone loss resulting from sudden immobilization, and normally for no more than 3 months to treat Pagets disease, the EMA said. The agency did not specify a time frame for the short-term use of calcitonin for treating hypercalcemia caused by cancer. The EMA based its recommendations on a review of the benefits and risks of calcitonin-containing medicines.