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

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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.

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Authors and Affiliations

  1. Department of Orthopedics, Institute of Health Biosciences, The University of Tokushima Graduate School, 3-18-15 Kuramoto-cho, Tokushima, 770-8503, Japan

    Shinjiro Takata

  2. Department of Orthopaedics, Osaka University Medical School, Osaka, Japan

    Jun Hashimoto, Ikko Ohno & Hideki Yoshikawa

  3. Department of Metabolism, Endocrinology and Molecular Medicine, Osaka City University Graduate School of Medicine, Osaka, Japan

    Kiyoshi Nakatsuka

  4. Department of Joint Disease Research, Graduate School of Medicine, The University of Tokyo, Tokyo, Japan

    Noriko Yoshimura

  5. Department of Orthopedic Surgery, Hyogo College of Medicine, Nishinomiya, Japan

    Kousei Yoh

  6. Department of Orthopedic Surgery, School of Medicine, Keio University, Tokyo, Japan

    Hiroo Yabe

  7. Department of Orthopedic Surgery, Teikyo University School of Medicine, Tokyo, Japan

    Satoshi Abe

  8. Department of Nuclear Medicine, Kawasaki Medical School, Kurashiki, Japan

    Masao Fukunaga

  9. Department of Orthopaedic Surgery, Wakayama Medical College, Wakayama, Japan

    Masaki Terada

  10. Sumitomo Pharmaceuticals, Tokyo, Japan

    Masaaki Zamma

  11. Rheumatic Diseases Unit, University of Edinburgh, Western General Hospital, Edinburgh, UK

    Stuart H. Ralston

  12. Japan Osteoporosis Society, Tokyo, Japan

    Hirotoshi Morii

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  1. Shinjiro Takata
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Correspondence to Shinjiro Takata.

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Takata, S., Hashimoto, J., Nakatsuka, K. et al. Guidelines for diagnosis and management of Paget's disease of bone in Japan. J Bone Miner Metab 24, 359–367 (2006). https://doi.org/10.1007/s00774-006-0696-x

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  • DOI: https://doi.org/10.1007/s00774-006-0696-x

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