Epidemiologie des Morbus Paget

 

 Buy Article Permissions and Reprints

Zusammenfassung

Der Morbus Paget des Knochens weist eine Reihe von epidemiologischen Besonderheiten auf, die bis heute viele Fragen aufwirft. Das Auftreten des Erkrankungsbildes vor dem 40. Lebensjahr ist sehr selten, erreicht aber in der Altersgruppe > 80 Jahre in einigen Populationen Prävalenzraten von 6,9 bis 10 %. Auffällig ist zudem eine sehr ausgeprägte Variation der Prävalenz mit besonders hohen Raten in bestimmten geografischen Regionen Nordenglands, Süditaliens und bestimmten Bezirken Spaniens. Demgegenüber steht das extrem seltene Auftreten in Skandinavien und Afrika. Aktuelle Untersuchungen weisen in einigen Regionen auf eine rückläufige Inzidenz, Prävalenz, Morbidität und Mortalität der Erkrankung hin. Neben dem sporadischen Auftreten der Erkrankung werden auch familiäre Häufungen mit früherem Krankheitsbeginn und einem anderen Befallsmuster beobachtet.

Summary

Paget’s disease of bone is a disorder with extraordinary epidemiological aspects, which are partly unresolved up to date. While the disease is very uncommon below the age of 40 years prevalence increases with age in some populations and reaches 6,9 to 10 % in the age group > 80 years. There is a marked geographic variation of age and genderrelated prevalence rates with for example regions of high prevalence in north-western England, southern Italy and several regions in Spain in contrast to the extreme rarity of the disease in Scandinavia and Africa. Some data show a decline in incidence, prevalence, morbidity and mortality due to Paget’s disease of bone. Additionally there are sporadic and familial forms of this localized bone disease with different clinical outcomes.

• Literatur

• 1 Altman RD, Bloch DA, Hochberg MC, Murphy WA. Prevalence of pelvic Paget’s disease of bone in the United States. J Bone Miner Res 2000; 15: 461-465.
  PubMedGoogle Scholar
• 2 Barker DJ. The epidemiology of Paget’s disease of bone. Br Med J 1984; 40: 396-400.
  Google Scholar
• 3 Barker DJ, Chamberlain AT, Guyer PB, Gardner MJ. Paget’s disease of bone: the Lancashire focus. Br Med J 1980; 280: 1105-1107.
  CrossrefPubMedGoogle Scholar
• 4 Barker DJ, Clough PW, Guyer PB, Gardner MJ. Paget’s disease of bone in 14 British towns. Br Med J 1977; 01: 1181-1183.
  CrossrefPubMedGoogle Scholar
• 5 Boyd JT, Doll R, Hill GB, Sissons HA. Mortality from primary tumours of bone in England and Wales. Br J Prev Soc Med 1969; 23: 12-22.
  PubMedGoogle Scholar
• 6 Cooper C, Schafheutle K, Dennison E. et al. The epidemiology of Paget’s disease in Britain: Is the prevalence decreasing?. J Bone Miner Res 1999; 14: 192-197.
  CrossrefPubMedGoogle Scholar
• 7 Cundy T. Is the prevalence of Paget’s disease of bone decreasing?. J Bone Miner Res 2006; 21 (Suppl. 02) 9-13.
  CrossrefPubMedGoogle Scholar
• 8 Cundy T, McAnulty K, Wattie D. et al. Evidence for secular change in Paget’s disease. Bone 1997; 20: 69-71.
  CrossrefPubMedGoogle Scholar
• 9 Detheridge FM, Guyer PB, Barker DJ. European distribution of Paget’s disease of bone. Br Med J (Clin Res Ed) 1982; 285: 1005-1008.
  CrossrefPubMedGoogle Scholar
• 10 Dickson DD, Camp JD, Ghormley RK. Osteitis deformans: Paget’s disease of bone. Radiology 1945; 44: 449-470.
  CrossrefGoogle Scholar
• 11 Eekhoff ME, van der Klift M, Kroon HM. et al. Paget’s disease of bone in The Netherlands: a population-based radiological and biochemical survey – the Rotterdam Study. J Bone Miner Res 2004; 19: 556-570.
  Google Scholar
• 12 Gardner MJ, Barker DJ. Mortality from malignant tumours of bone and Paget’s disease in the United States and in England and Wales. Int J Epidemiol 1978; 07: 121-130.
  CrossrefPubMedGoogle Scholar
• 13 Gardner JM, Guyer PB, Barker DJ. Radiological prevalence of Paget’s disease of bone in British migrants to Australia. Br Med J 1978; 01: 1655-1657.
  CrossrefPubMedGoogle Scholar
• 14 Gennari L, Merlotti D, Martini G, Nuti R. Paget’s disease of bone in Italy. J Bone Miner Res 2006; 21 (Suppl. 02) P14-P21.
  CrossrefPubMedGoogle Scholar
• 15 Guyer PB, Chamberlain AT. Paget’s disease of bone in two American cities. Br Med J 1980; 280: 985.
  Google Scholar
• 16 Hamdy RC. Paget’s disease of bone. Eastbourne, New York: Praeger Publishers; 1981
  Google Scholar
• 17 Haslam SI, Thompson JMG, Haites NES, Ralston SH. Genetic mapping in Paget’s disease of bone: Evidence for a susceptibility locus on chromosome 18q. J Bone Miner Res 1996; 11 (Suppl. 01) S369.
  Google Scholar
• 18 Hutchinson J. On Osteitis deformans. Illustrated Medical News 1889; 02: 169-179.
  Google Scholar
• 19 Jacobs TP, Michelsen J, Polay JS. et al. Giant cell tumor in Paget’s disease of bone: Familial and geographic clustering. Cancer 1979; 44: 742-747.
  CrossrefPubMedGoogle Scholar
• 20 Kanis JA. Pathophysiology and treatment of Paget’s disease of bone, 2. Auflage. London: Martin Dunitz; 1998
  Google Scholar
• 21 Lawrence JS. Paget’s disease in population samples. Ann Rheum Dis 1970; 29: 562.
  PubMedGoogle Scholar
• 22 Lopez-Abente G, Morales-Piga A, Bachiller-Corral FJ. et al. Identification of possible areas of high prevalence of Paget’s disease of bone in Spain. Clin Exp Rheumatol 2003; 21: 635-638.
  PubMedGoogle Scholar
• 23 Melton III LJ, Tiegs RD, Atkinson EJ, O’Fallon WM. Fracture risk among patients with Paget’s disease: A population-based cohort study. J Bone Miner Res 2000; 15: 2123-2128.
  CrossrefPubMedGoogle Scholar
• 24 Miron-Canelo JA, Del Pino-Montes J, Vicente-Arroyo M, Saenz-Gonzalez MC. Epidemiological study of Paget’s disease of bone in a zone of the Province of Salamanca (Spain). The Paget’s disease of the bone study group of Salamanca. Eur J Epidemiol 1997; 13: 801-805.
  CrossrefPubMedGoogle Scholar
• 25 Morales-Piga AA, Rey-Rey JS, Corres-González J. et al. Frequency and characteristics of familial aggregation of Paget’s disease of bone. J Bone Miner Res 1995; 10: 663-670.
  PubMedGoogle Scholar
• 26 Paget J. On a form of chronic inflammation of bone. Medico-chirurgical Transactions, London 1877; 60: 37-63.
  Google Scholar
• 27 Paget J. Additional cases of osteitis deformans. Medico-chirurgical Transactions, London 1882; 65: 225-236.
  Google Scholar
• 28 Paget J. Remarks on osteitis deformans. The Illustrated Medical News 1889; 02: 181-182.
  Google Scholar
• 29 Pales L. Maladie de Paget préhistorique. Anthropologie 1929; 39: 263-270.
  Google Scholar
• 30 Rendina D, Gennari L, De Filippo G. et al. Evidence for increased clinical severity of familial and sporadic Paget’s disease of bone in Campania, southern Italy. J Bone Miner Res 2006; 21: 1828-1835.
  CrossrefPubMedGoogle Scholar
• 31 Rendina D, Mossetti G, Soscia E. et al. Giant cell tumor and Paget’s disease of bone in one family: Geographic clustering. Clin Orthop 2004; 421: 218-224.
  CrossrefPubMedGoogle Scholar
• 32 Ringe JD, Jend HH, Becker H. Epidemiologie der Osteoitis deformans Paget. MMW 1984; 126: 683-686.
  Google Scholar
• 33 Robertson MM, Thomas AF. Osteitis deformans in the South African Negro. S Afr Med J 1978; 53: 183-185.
  PubMedGoogle Scholar
• 34 Rogers J, Jeffrey DR, Watt I. Paget’s disease in an archeological population. J Bone Miner Res 2002; 17: 1127-1134.
  CrossrefPubMedGoogle Scholar
• 35 Rosenkrantz JA, Wolf J, Kaicher JJ. Paget’s disease (osteitis deformans); review of one hundred eleven cases. AMA Arch Intern Med 1952; 90: 610-633.
  CrossrefPubMedGoogle Scholar
• 36 Schmorl G. Über Ostitis deformans Paget. Virchows Arch Path Anat 1932; 283: 694-751.
  CrossrefGoogle Scholar
• 37 Seton M, Choi HK, Hansen MF. et al. Analysis of environmental factors in familial versus sporadic Paget’s disease of bone – The New England Registry for Paget’s Disease of Bone. J Bone Miner Res 2003; 18: 1519-1524.
  CrossrefPubMedGoogle Scholar
• 38 Siris ES. Epidemiological aspects of Paget’s disease: Family history and relationship to other medical conditions. Semin Arthritis Rheum 1994; 23: 222-225.
  CrossrefPubMedGoogle Scholar
• 39 Siris ES, Canfield RE. Paget’s disease of bone. In: Becker KL. Hrsg. Principles and practice of endocrinology and metabolism. Philadelphia: JB Lippincott; 1990
  Google Scholar
• 40 Siris ES, Ottman R, Flaster E, Kelsey JL. Familial aggregation of Paget’s disease of bone. J Bone Miner Res 1991; 06: 495-500.
  Google Scholar
• 41 Sofaer JA, Holloway SM, Emery AE. A family study of Paget’s disease of bone. J Epidemiol Community Health 1983; 37: 226-231.
  CrossrefPubMedGoogle Scholar
• 42 van Staa TP, Selby P, Leufkens HGM. et al. Incidence and natural history of Paget’s disease of bone in England and Wales. J Bone Miner Res 2002; 17: 465-471.
  CrossrefPubMedGoogle Scholar
• 43 Wilks S. Case of osteoporosis or spongy hypertrophy of bones. Transactions of the Pathological Society of London 1869; 20: 273-277.
  Google Scholar
• 44 Wu RK, Trumble TE, Ruwe PA. Familial incidence of Paget’s disease and secondary osteogenic sarcoma: A report of three cases from a single family. Clin Orthop 1991; 265: 306-309.
  Google Scholar