Diagnosis and treatment of Paget's disease of bone : Aclinical practice guideline.

Abstract

Paget's disease of bone (osteitis deformans) is abenign focal disorder of accelerated skeletal remodeling. Either a single bone (monostotic) or multiple bones (polyostotic) can be affected. In patients with suspected Paget's disease plain radiographs of the suspicious regions of the skeleton are recommended. The initial biochemical evaluation of apatient should be done using serum total ALP (alkaline phosphatase) or with the use of amore specific marker of bone formation: PINP (intact N-terminal type1 procollagen propeptide) or CTX (cross-linked C‑telopeptide). Treatment with abisphosphonate is recommended for most patients with active Paget's disease who are at risk for further skeletal and extraskeletal complications. Asingle dose of 5mg i.v. zoledronate as the treatment of choice in patients without contraindications is suggested. Oral bisphosphonates are less potent when compared to zoledronate. Treatment with an antiresorptive agent induces amore rapid decrease in resorption markers compared to formation marker. Measurement of total ALP or other baseline disease activity markers (e. g. CTX) at 6 to 12weeks, when bone turnover will have shown asubstantial decline, is an acceptable and cost-effective option. Maximum suppression of high bone turnover may require measurement at 6months after administration. In patients with increased bone turnover, biochemical follow-up is recommended to be used as amore objective indicator of relapse rather than symptoms. The prolonged response after zoledronate treatment should be assessed every 1-2years after normal bone turnover. With less potent drugs, every 6 to 12months is appropriate.