Journal of Bone and Mineral Metabolism

, Volume 24, Issue 5, pp 359–367 | Cite as

Guidelines for diagnosis and management of Paget's disease of bone in Japan

  • Shinjiro Takata
  • Jun Hashimoto
  • Kiyoshi Nakatsuka
  • Noriko Yoshimura
  • Kousei Yoh
  • Ikko Ohno
  • Hiroo Yabe
  • Satoshi Abe
  • Masao Fukunaga
  • Masaki Terada
  • Masaaki Zamma
  • Stuart H. Ralston
  • Hirotoshi Morii
  • Hideki Yoshikawa
SPECIAL REPORT

Abstract

We here propose guidelines for the diagnosis and management of Paget's disease of bone (PDB) in Japan. These guidelines provide basic information on the epidemiology, pathophysiology, clinical signs and symptoms, diagnosis, indications for treatment, and available therapy, including orthopedic surgery. PDB is a chronic disorder characterized by focal abnormalities of bone turnover. The characteristic feature of PDB is excessive osteoclastic bone resorption coupled to increased and disorganized bone formation. The most common symptom of PDB is pain in involved bones. The most serious complication of PDB is malignant bone or soft-tissue tumor. PDB is uncommon in Japan; our survey in 2003 found 169 patients with PDB. The prevalence of PDB in Japan is 0.15/100 000; in patients aged 55 years or more, the proportion reaches 0.41/100 000. A careful medical history and physical examination are essential for the diagnosis. The diagnosis of PDB is based on finding the typical features on radiographs. Bone scintigraphy and measurement of serum alkaline phosphatase are sensitive means of screening for PDB. Since PDB is a rare disease in Japan, bone biopsy is quite often used to exclude bone metastases. The only evidence-based indication for treatment of PDB is pain in involved bones. In Japan, etidronate and calcitonin are approved by the Ministry of Health, Labour and Welfare for treating PDB, but currently risedronate is also under development for treating PDB in Japan. Indications for surgical intervention in PDB include unstable fractures, osteoarthritis, malignant soft-tissue tumor, osteosarcoma, and bone deformity.

Key words

Paget's disease of bone guideline diagnosis treatment 

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Copyright information

© Springer-Verlag Tokyo 2006

Authors and Affiliations

  • Shinjiro Takata
    • 1
  • Jun Hashimoto
    • 2
  • Kiyoshi Nakatsuka
    • 3
  • Noriko Yoshimura
    • 4
  • Kousei Yoh
    • 5
  • Ikko Ohno
    • 2
  • Hiroo Yabe
    • 6
  • Satoshi Abe
    • 7
  • Masao Fukunaga
    • 8
  • Masaki Terada
    • 9
  • Masaaki Zamma
    • 10
  • Stuart H. Ralston
    • 11
  • Hirotoshi Morii
    • 12
  • Hideki Yoshikawa
    • 2
  1. 1.Department of Orthopedics, Institute of Health BiosciencesThe University of Tokushima Graduate SchoolTokushimaJapan
  2. 2.Department of OrthopaedicsOsaka University Medical SchoolOsakaJapan
  3. 3.Department of Metabolism, Endocrinology and Molecular MedicineOsaka City University Graduate School of MedicineOsakaJapan
  4. 4.Department of Joint Disease Research, Graduate School of MedicineThe University of TokyoTokyoJapan
  5. 5.Department of Orthopedic SurgeryHyogo College of MedicineNishinomiyaJapan
  6. 6.Department of Orthopedic Surgery, School of MedicineKeio UniversityTokyoJapan
  7. 7.Department of Orthopedic SurgeryTeikyo University School of MedicineTokyoJapan
  8. 8.Department of Nuclear MedicineKawasaki Medical SchoolKurashikiJapan
  9. 9.Department of Orthopaedic SurgeryWakayama Medical CollegeWakayamaJapan
  10. 10.Sumitomo PharmaceuticalsTokyoJapan
  11. 11.Rheumatic Diseases UnitUniversity of Edinburgh, Western General HospitalEdinburghUK
  12. 12.Japan Osteoporosis SocietyTokyoJapan

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