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Chair of orthopedic

Paget´s Disease of Bone

Dr. med. Sascha Göbel, Dr. med. Lothar Seefried, Dr. med. Sabine Bau and Prof. Dr. Franz Jakob

Paget´s disease of bone has been described by Sir James Paget in 1876. It affects approx. 4.6 % of the population in Great Britain, but the incidence in different regions varies considerably and decreases in recent years. Outside Great Britain a lower incidence was found, e.g. 0.5 – 2.5 % in European countries. Recently some inheritable syndromes like early onset Paget’s disease and juvenile Paget’s disease have been associated with mutations of components of the RANK / NFkB signaling pathway and with components of the ubiquitination machinery, which also affect NFkB signaling. This pathway is essential for osteoclast differentiation and activation and its alteration renders Paget´s disease of bone an osteoclast associated disease. There is also evidence for the involvement of an exogenous agent that triggers the disease. Virus-like particles have been found in osteoclasts of pagetic bone. Various viruses have been hypothesized to be involved but inconsistent results have been achieved as to the demonstration of specific viral nucleic acids and proteins in pagetic bone. Thus the question of an exogenous trigger of the disease awaits further clarification. Osteoclasts are the primary targets of aminobisphosphonates. They specifically inhibit the enzyme farnesylsynthase thereby affecting osteoclast function and survival. These compounds have been proven to be the compounds of choice to treat Paget´s disease of bone. Our group took part in a recent multicentre trial of zoledronate and risedronate in the treatment of Paget´s disease of bone (Reid et al. 2005). Our objectives are to develop state of the art patient care for diagnosis and treatment in the context of an Osteology network. In cooperation with scientific societies (see DVO) we are working on the distribution of knowledge and guidelines for evidence based diagnosis and treatment.


Literature

  • Reid IR, Miller P, Lyles K, Fraser W, Brown JP, Saidi Y, Mesenbrink P, Su G,Pak J, Zelenakas K, Luchi M, Richardson P, Hosking D. Comparison of a single infusion of zoledronic acid with risedronate for Paget's disease. N Engl J Med. 2005 Sep 1;353(9):898-908.
  • Jakob F (Edit.) Morbus Paget. Unimed Verlag 2006


http://www.minne.de/pdf/paget.pdf
http://www.whonamedit.com/doctor.cfm/283.html

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