Abstract

Paget's disease of bone (PDB) is a condition characterized by excessive and abnormal bone remodelling. Due to a high rate of bone remodelling, bisphosphonates, and especially pamidronate and the newer zolendronate, are indicated in the treatment of PDB. The presence of asymptomatic, but active PDB represents an indication for treatment aimed at preventing later complications. An additional indication for treatment is the involvement of skeletal segments that may give rise to severe complications. Pamidronate has a long history in the treatment of PDB. The more utilised regimen is 3 to 6 i.v. infusion of 60 mg of pamidronate at an infusion rate of 1 mg/min within 3-21 days. Zolendronate (5 mg once yearly) is the most powerful amino-bisphosphonate currently used. This primacy recognizes both the ability to inhibit the farnesyl-pyrophosphate synthetase and the higher affinity to hydroxyapatite crystals as a cause. Both pamidronate and zolendronate are effective in PDB, with an evidence-based superiority of the latter.

Highlights

  • Paget’s disease of bone (PDB) is a condition fully described for the first time by Sir James Paget in 1877 characterised by excessive and abnormal bone remodelling

  • The disease seldom appears before the age of 40, but its prevalence tends to double each decade from the age of 50 onwards

  • In many patients the complications of PDB require additional symptomatic treatment, including analgesics and selected orthopaedic and neurosurgical interventions. In this concise review we focus on the treatment of PDB with pamidronate and zoledronic acid

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Summary

Introduction

Paget’s disease of bone (PDB) is a condition fully described for the first time by Sir James Paget in 1877 characterised by excessive and abnormal bone remodelling. Pamidronate was initially used in oral formulation, but it resulted in being poorly tolerated.[19,20] several intravenous (i.v.) dosing regimens have been proposed.[21,22,23,24,25,26,27,28,29,30] The more utilized regimen is 3 to 6 i.v. infusion of 60 mg of pamidronate (Aredia®) at an infusion rate of 1 mg/min within 3–21 days This dosage seems to determine a reduction of the alkaline phosphatase of the 50%–80%, a clearly symptomatologic improvement[19,20] and sometimes improvements in radiological and/or scintigraphic pictures too.[21,31] Effectiveness lasts for long periods after a single cycle of therapy in relation to entity of abolition of the osseous turnover: the greater the abolition, especially if it is within the normal range, the longer the persistence of efficacy.[23,25,29,30,32] Noteworthy, clinical improvement can include the remission of neurological complications.[33]. Of the 80% with normalized AP levels, 72% remained normalized despite the

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