Semin Musculoskelet Radiol 2002; 06(4): 313-322
DOI: 10.1055/s-2002-36730
Copyright © 2002 by Thieme Medical Publishers, Inc., 333 Seventh Avenue, New York, NY 10001, USA. Tel.: +1(212) 584-4662

Paget's Disease of Bone

Richard W. Whitehouse
  • Manchester Royal Infirmary, Manchester, United Kingdom
Further Information

Publication History

Publication Date:
22 January 2003 (online)

ABSTRACT

Paget's disease of bone is one of the most common diseases to affect bone, yet it is rare before the age of 50. The etiology of the condition remains unproven. Paget's disease of bone has become less common, less severe, and less extensive in recent decades. Isotope bone scans and radiographs remain the most frequent radiological investigations, demonstrating the extent of the disease and characteristic appearances in most cases. Recent changes in the radiological investigation of Paget's disease include increasing use of computed tomography (CT) and magnetic resonance (MR) imaging for the evaluation of less typical disease or disease complications; the incidental finding of Paget's disease on CT or MR requires recognition to avoid inappropriate investigation. The presence of sclerotic Paget's disease in the lumbar spine or hip may elevate bone mineral density measurements at these sites, with consequent potential to underestimate fracture risk. Awareness of the normal level-to-level vertebral variation in bone density in the spine, and careful assessment of the images acquired on dual energy X ray absorptiometry or quantitative CT will help to avoid this pitfall. Examples of these investigations and the combination of Paget's disease with other conditions such as osteoarthritis, metastatic bone disease, and bone infection are illustrated.

REFERENCES

  • 1 McNairn J D, Damron T A, Landas S K, Ambrose J L, Shrimpton A E. Inheritance of osteosarcoma and Paget's disease of bone: a familial loss of heterozygosity study.  J Mol Diagn . 2001;  3 171-177
  • 2 Reddy S V, Kurihara N, Menaa C, Roodman G D. Paget's disease of bone: a disease of the osteoclast.  Rev Endocrinol Metabol Disord . 2001;  2 195-201
  • 3 Friedrichs W E, Reddy S V, Bruder J M. Sequence analysis of measles virus nucleocapsid transcripts in patients with Paget's disease.  J Bone Miner Res . 2002;  17 145-151
  • 4 Good D, Busfield F, Duffy D. Familial Paget's disease of bone: nonlinkage to the PDB1 and PDB2 loci on chromosomes 6p and 18q in a large pedigree.  J Bone Miner Res . 2001;  16 33-38
  • 5 Helfrich M H, Hobson R P, Grabowski P S. A negative search for a paramyxoviral etiology of Paget's disease of bone: molecular, immunological, and ultrastructural studies in UK patients.  J Bone Miner Res . 2000;  15 2315-2329
  • 6 Drake W M, Kendler D L, Brown J P. Consensus statement on the modern therapy of Paget's disease of bone from a Western Osteoporosis Alliance Symposium. Biannual Foothills Meeting on Osteoporosis, Calgary, Alberta, Canada, September 9-10, 2000.  Clin Ther . 2001;  23 620-626
  • 7 Rothschild B M. Paget's disease of the elderly.  Compr Ther . 2000;  26 251-254
  • 8 Cooper C, Schafheutle K, Dennison E. The epidemiology of Paget's disease in Britain: is the prevalence decreasing?.  J Bone Miner Res . 1999;  14 192-197
  • 9 Renier J C, Fanello S, Bos C, Audran M. An etiologic study of Paget's disease.  Rev Rhum Engl Ed . 1996;  63 606-611
  • 10 Morales-Piga A A, Bachiller-Corral F J, Abraira V, Beltran J, Rapado A. Is clinical expressiveness of Paget's disease of bone decreasing?.  Bone . 2002;  30 399-403
  • 11 Tiegs R D, Lohse C M, Wollan P C, Melton L J. Long term trends in the incidence of Paget's disease of bone.  Bone . 2000;  27 423-427
  • 12 Altman R D. Paget's disease of bone: rheumatologic complications.  Bone . 1999;  24(Suppl 5) 47S-48S
  • 13 Vande-Berg B C, Malghelm J, Lecouvet F E, Maldague B. Magnetic resonance appearance of uncomplicated Paget's disease of bone.  Semin Musculoskel Radiol . 2001;  5 69-77
  • 14 Rosen M A, Matasar K W, Irwin R B, Rosenberg B F, Herkowitz H N. Osteolytic monostotic Paget's disease of the fifth lumbar vertebra. A case report.  Clin Orthop . 1991;  262 119-123
  • 15 Steinbach L S, Johnston J O. Case report 777. Osteolytic Paget's disease of the fifth lumbar vertebra.  Skeletal Radiol . 1993;  22 203-205
  • 16 Anderson J T, Dehner L P. Osteolytic form of Paget's disease: differential diagnosis and pathogenesis.  J Bone Joint Surg Am . 1976;  58 994-1000
  • 17 Eisman J A, Martin T J. Osteolytic Paget's disease. Recognition and risks of biopsy.  J Bone Joint Surg Am . 1986;  68 112-117
  • 18 Kaufmann G A, Sundaram M, McDonald D J. Magnetic resonance imaging in symptomatic Paget's disease.  Skeletal Radiol . 1991;  20 413-418
  • 19 Donath J, Szilagyi M, Fornet B, Bely M, Poor G. Pseudosarcoma in Paget's disease.  Eur Radiol . 2000;  10 1664-1668
  • 20 McNairn J DK, Damron T A, Landas S K, Ambrose J L. Benign tumefactive soft tissue extension from Paget's disease of bone simulating malignancy.  Skeletal Radiol . 2001;  30 157-160
  • 21 Tins B J, Davies A M, Mangham D C. MR imaging of pseudosarcoma in Paget's disease of bone: a report of two cases.  Skeletal Radiol . 2001;  30 161-165
  • 22 Lamovec J, Rener M, Spiler M. Pseudosarcoma in Paget's disease of bone.  Ann Diagn Pathol . 1999;  3 99-103
  • 23 Sundaram M, Khanna G, El-Khoury G Y. TI-weighted MR imaging for distinguishing large osteolysis of Paget's disease from sarcomatous degeneration.  Skeletal Radiol . 2001;  30 378-383
  • 24 Helliwell P S, Porter G. Controlled study of the prevalence of radiological osteoarthritis in clinically unrecognised juxta-articular Paget's disease.  Ann Rheum Dis . 1999;  58 762-765
  • 25 Fenton P, Resnick D. Metastases to bone affected by Paget's disease. A report of three cases.  Int Orthop . 1991;  15 397-399
  • 26 Stephens J C, Lennington W J, Schwartz H S. Primary lymphoma and Paget's disease of the femur.  Am J Clin Pathol . 1994;  101 783-786
  • 27 Gebhart M, Bandeweyer E, Nemec E. Paget's disease of bone complicated by giant cell tumour.  Clin Orthop . 1998;  352 187-193
  • 28 Resnick D, Niwayama G. Paget's disease. In: Resnick D, ed. Diagnosis of Bone and Joint Disorders Philadelphi, PA: WB Saunders 1995: 1923-1968
  • 29 Cherian R A, Haddaway M J, Davie M W, McCall I W, Cassar-Pullicino V N. Effect of Paget's disease of bone on areal lumbar spine bone mineral density measured by DXA, and density of cortical and trabecular bone measured by quantitative CT.  Br J Radiol . 2000;  73 720-726
  • 30 Bone H G, Cody D D, Monsell E M. Application of quantitative computed tomography to Paget's disease of bone.  Semin Arthritis Rheum. 199;  23 244-247
  • 31 Vasireddy S, Halsey J P. Incidental detection of lumbar Paget's disease by bone densitometry.  Rheumatology . 2001;  40 1424-1425
  • 32 Laroche M, Delpech B, Bernard J, Constantin A, Mazieres B. Measurement of bone mineral density by dual x-ray absorptiometry in Paget's disease before and after pamidronate treatment.  Calcif Tissue Int . 1999;  65 188-191
    >