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Review
. 2021 Feb;80(1):48-53.
doi: 10.1007/s00393-020-00897-x. Epub 2020 Oct 1.

[Paget's disease of bone-a current review of clinical aspects, diagnostics and treatment]

[Article in German]
P Klemm  1 , G Dischereit  1   2 , S von Gerlach  3 , U Lange  4
Affiliations
Review

[Paget's disease of bone-a current review of clinical aspects, diagnostics and treatment]

[Article in German]
P Klemm et al. Z Rheumatol. 2021 Feb.

Abstract
in English, German

Paget's disease is a monostotic or polyostotic progressive skeletal disease with a genetic predisposition. The affected bone areas show osseous swelling and often grotesque deformation, chronic pain and fractures. Many cases are asymptomatic for a long time resulting in a late diagnosis. The pathogenesis is still unknown. In addition to a genetic predisposition, viral factors are also discussed. Laboratory tests and imaging are used for diagnosis. The effective principle of medicinal bisphosphonate treatment is to inhibit osteoclastic bone resorption and should be initiated early to prevent secondary complications. This article presents the current knowledge about this rare osteological disease.

Beim Morbus Paget liegt eine mon- oder polyostotische, progrediente Skeletterkrankung mit genetischer Prädisposition vor. In den betroffenen Knochenarealen kommt es zur ossären Auftreibung und oft grotesken Verformung, zu chronischen Schmerzen und Frakturen. Viele Fälle sind lange Zeit asymptomatisch bis oligosymptomatisch, sodass sie erst spät diagnostiziert werden. Die Pathogenese ist weiterhin ungeklärt, neben einer genetischen Prädisposition werden auch virale Faktoren diskutiert. Zur Diagnosestellung dienen Labor und Bildgebung. Das wirksame Prinzip der medikamentösen Bisphosphonat-Therapie liegt in der Hemmung der osteoklastären Knochenresorption und sollte frühzeitig erfolgen, um Sekundärkomplikationen zu verhindern. Der vorliegende Artikel präsentiert die aktuellen Erkenntnisse zu dieser seltenen osteologischen Erkrankung.

Keywords: Bone remodeling activity; Clinic; Diagnostics; Osteopathy; Paget’s disease; Therapy options.

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References

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