UK guidelines on management of Paget's disease of bone

A. Scarsbrook, M. Brown and D. Wilson

Nuffield Orthopaedic Centre, Windmill Road, Headington, Oxford OX3 7BN, UK

Correspondence to: A. Scarsbrook. E-mail: andyscarsbrook1{at}aol.com

SIR, Over the past few decades several different methods of treatment have become available for Paget's disease of bone. There is no direct evidence that aggressive treatment is associated with prevention of progression or reduction in risk of future complications. However, limited evidence suggests benefit in treatment of: symptomatic metabolically active Paget's; hypercalcaemia complicating Paget's disease; prior to elective joint replacement surgery to prevent excessive blood loss due to hypervascular Pagetic bone, and in patients with spinal cord dysfunction. Some investigators believe that treatment is indicated in asymptomatic individuals with metabolically active disease and/or certain sites of disease, which place them at increased risk of progression and complications. Indirect evidence suggests increased risk of deafness with skull involvement, higher risk of neurological complications with vertebral involvement, and greater risk of secondary arthritis if Paget's disease affects periarticular areas of major joints. The lack of an evidence base for the treatment of such cases has prompted the establishment of the PRISM study (ISRCTN 12989577) in the UK.

UK guidelines on the management of Paget's disease of bone were recently developed by a working party derived from the Bone and Tooth Society and the National Association for the Relief of Paget's Disease [1]. These guidelines recommend that all patients with Paget's disease should have scintigraphy performed to assess the extent of skeletal involvement, so that treatment of asymptomatic individuals with areas of involvement associated with an increased risk of complication could be offered treatment. This has major potential impacts, in terms of both expense and radiation exposure, which do not appear to have been considered in the formulation of these guidelines. It is estimated that 2% of the British population over 50 yr of age have Paget's disease [2]. A bone scintigram costs around £135 and involves 57 and 200 times greater radiation exposure than skull and chest X-ray respectively. Bone scintigraphy services are not universally available, and current services would be overwhelmed if this proposal was fully implemented.

Whilst bone scintigraphy is more sensitive than radiography in detecting sites of disease, the discrepancy between the two was overstated in the published guidelines. Wellman et al. [3] reported that 69% of Pagetic lesions were detected on both types of imaging and 31% were not detected on plain films, not 50% as quoted in the guidelines [1]. Furthermore, the guidelines did not mention other studies that also suggest that plain radiographs are more sensitive than the guidelines claim. Fogelman and Carr [4] studied 23 patients with symptomatic disease and compared radiographic skeletal survey and bone scans. Of the 127 Pagetic sites detected, 120 (95%) were demonstrated by bone scintigraphy and 94 (74%) by radiography, i.e. 21% of positive scintograms had negative radiographs. Vellenga et al. [5] looked at the X-rays of abnormal sites on bone scintigraphy of 107 patient's with Paget's disease and found that 59 of the 373 lesions examined (16%) did not show any radiographic abnormality. They also determined that there was a clear correlation between the amount of radioactivity in a lesion on bone scintigraphy and the grade of deformity on the radiography. They found that in the radiograph-negative cases there were no symptoms in nearly all cases (89%). Meunier et al. [6] reported a series of 170 cases of untreated Paget's disease and found bone scintigraphy to be more sensitive than routine radiography, but there was a difference of only 8% between the two. Importantly, the researchers in the above studies found that virtually all lesions detected on scintigraphy but not on radiographs showed low uptake and were almost always asymptomatic and thought to reflect low disease activity.

Where patients have a clear indication for treatment of Paget's disease, such as bone pain, hypercalcaemia or neural compression, the decision as to whether to treat or not will not be altered by scintigraphy. Deafness is a common complication of Paget's disease, and in the current UK guidelines medical treatment is recommended in patients with Paget's disease of the skull base. The guidelines recommend bone scintigraphy to determine whether or not the skull base is involved. We believe a skull radiograph may be an acceptable method of assessing skull base disease; it has a considerably lower radiation dose and should provide diagnostic information in all but very low-grade cases.

It is important to stress that we feel it is unnecessary to refer all newly diagnosed cases of Paget's disease for skeletal scintigraphy. However, based on the literature available it seems sensible to consider plain radiography of the skull in new cases to exclude skull base involvement. There may be a limited role for scintigraphy to determine the extent of disease in patients with known Paget's disease but in whom there is indecision regarding the need for treatment. It is also important that radiologists who note coincidental Paget's disease on plain films requested for other reasons than possible involvement of the spine, periarticular areas or skull base, that a note should be made in the report to the referring clinician to consider referral to a metabolic bone specialist/endocrinologist for consideration of treatment. It would be appropriate for specialists to request bone scintigraphy to further assess disease involvement, but the blanket use of bone scintigraphy should, in our view, be discouraged.

The authors have declared no conflicts of interest.

References

  1. Selby et al. Guidelines on the management of Paget's disease of bone. Bone, 2002;31:366–73.[CrossRef][ISI][Medline]
  2. Cooper et al. The epidemiology of Paget's disease in Britain: Is the prevalence decreasing. J Bone Miner Res 1999;14:192–7.[ISI][Medline]
  3. Wellman et al. Skeletal scintimaging and radiography in the diagnosis and management of Paget's disease. Clin Orthop Rel Res 1977;127:55–62.[Medline]
  4. Fogelman et al. A comparison of bone scanning and radiology in the assessment of patients with symptomatic Paget's disease. Eur J Nuclear Med 1980;5:417–21.[ISI][Medline]
  5. Vellenga et al. Untreated Paget disease of bone studied by scintigraphy. Radiology 1984;153:799–805.[Abstract]
  6. Meunier et al. Skeletal distribution and biochemical parameters of Paget's disease. Clin Orthop Related Res 1987;217:37–44.[Medline]
Accepted 19 August 2003





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