Enfermedad ósea de Paget

Paget's disease of bone (PDB) is the second most common metabolic bone disease after osteoporosis. Its histology is characterised by an increased number and size of osteoclasts. This increase in bone activity gives rise to the formation of larger sized, poor quality, deformed bone. The aetiology of PDB is not known. A multifactorial origin is postulated, associated with genetic predisposition and the intervention of environmental factors. The sequestosome gene (SQSTM1) is the most frequently associated. A suspicion of PDB is based on the presence of pain, deformity or pathological fracture, although patients are often asymptomatic and PDB is suspected due to the isolated elevation of alkaline phosphatase. Plain x-ray or bone scan confirm the diagnosis. Bisphosphonates are the standard treatment for symptomatic patients, together with zoledronic acid as the first option.