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Morbus Paget des Knochens – eine aktuelle Übersicht zu Klinik, Diagnostik und Therapie

Paget’s disease of bone—a current review of clinical aspects, diagnostics and treatment

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Zusammenfassung

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.

Abstract

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.

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Literatur

  1. Britton C, Brown S, Ward L et al (2017) The changing presentation of Paget’s disease of the bone in Australia, a high prevalence region. Calcif Tissue Int 101:564–569

    CAS  PubMed  Google Scholar 

  2. Corall-Gudino L, Tan AJH, Del Pino-Montes J et al (2017) Bisphosphonates for Paget’s disease of bone in adults. Cochrane Database Syst Rev 12:CD4956

    Google Scholar 

  3. Cundy T, Maslowski K, Grey A, Reid IR (2017) Durability of responses to zoiledronate and treatment and competing mortality in Paget’s disease of bone. J Bone Miner Res 32:753–756

    CAS  PubMed  Google Scholar 

  4. Eastell R (1999) Biochemical markers of bone turnover in Paget’s disease of the bone. Bone 24(5):49–50

    Google Scholar 

  5. Eekhoff ME, van der Klift M, Kroon HM et al (2004) Paget’s disease of bone in the Netherlands: a population-based radiological and biochemical survey—the Rotterdam study. J Bone Miner Res 19:566–570

    PubMed  Google Scholar 

  6. Eekhoff EW, Karperien M, Houtsma D et al (2004) Familial Paget’s disease in The Netherlands: occurence, identification of new mutations in the sequestosome 1 gene, and their clinical associations. Arthritis Rheum 50:1650–1654

    CAS  PubMed  Google Scholar 

  7. Emory H (2019) Paget’s disease of bone: update for clinicans. Curr Opin Endocrinol Diabetes Obes 26:329–334

    Google Scholar 

  8. Freyschmidt J (1990) Ostitis deformans Paget. In: Freyschmidt J (Hrsg) Röntgenatlas. Springer, Stuttgart, New York, S 387–422

    Google Scholar 

  9. Goncalves MJ, Romao VC (2014) Saber tibia in Paget’s disease of bone. N Engl J Med 371:2417

    PubMed  Google Scholar 

  10. Grauer A, Abendroth K, Heller M et al (1998) Der Morbus Paget des Knochens. Dtsch Arztebl 95:A–2021–2026

    Google Scholar 

  11. Harinck HIJ, Papapoulos SE, Blanksma HJ et al (1987) Paget’s disease of bone: early and late responses to three different modes of treatment with aminohydroxypropylidene bisphosphonates (APD). Br Med J (Clin Res Ed) 295:1301–1305

    CAS  Google Scholar 

  12. Joshi SR, Ambhore S, Butala N et al (2006) Paget’s disease from Western India. J Assoc Physicians India 54:535–538

    PubMed  Google Scholar 

  13. Joshua F, Epstein M, Major G (2003) Bisphosphonate resistance in Paget’s disease of bone. Arthritis Rheum 48:2321–2323

    CAS  PubMed  Google Scholar 

  14. Kanecki K, Nitsch-Osuch A, Gorynski P et al (2018) Paget disease of bone among hospitalized patients in Poland. Ann Agric Environ Med 25:182–185

    PubMed  Google Scholar 

  15. Kaplan FS, Singer FR (1995) Paget’s disease of bone: pathophysiology, diagnosis, and management. J Am Acad Orthop Surg 3:336–344

    CAS  PubMed  Google Scholar 

  16. Kaufman GA, Sundaram M, McDonald DJ (1991) Magnetic resonance imaging in symptomatic Paget’s disease. Skelet Radiol 20:413–418

    Google Scholar 

  17. Khairi MR, Robb JA, Wellman HN, Johnston CC (1974) Radiographs and scans in diagnosing symptomatic lesions of Paget’s disease of bone (osteitis deformans). Geriatrics 29:49–54

    CAS  PubMed  Google Scholar 

  18. Langston AL, Campbell MK, Fraser WD et al (2010) PRISM trial group. Randomized trial of intensive bisphosphonate treatment versus symptomatic management in Paget’s disease of bone. J Bone Miner Res 25:20–31

    CAS  PubMed  Google Scholar 

  19. Laurin N, Brown JP, Morissette J, Raymond V (2002) Recurrent mutation of the gene encoding sequestosome 1 (SQSTM1/p62) in Paget disease of the bone. Am J Hum Genet 70:1582–1588

    CAS  PubMed  PubMed Central  Google Scholar 

  20. Ludkowski P, Wilson-MacDonald J (1990) Total arthroplasty in Paget’s disease of the hip. A clinical review and review of the literature. Clin Orthop Relat Res 225:160–167

    Google Scholar 

  21. Mangham DC, Davie MW, Grimer RJ (2009) Sarcoma arising in Paget’s disease of bone: declining incidence and increasing age at presentation. Bone 44:431–436

    CAS  PubMed  Google Scholar 

  22. McDonald DJ, Sim FH (1987) Total hip arthroplasty in Paget’s disease. A follow-up note. J Bone Joint Surg Am 69:766–772

    CAS  PubMed  Google Scholar 

  23. Miller PD, Brown JP, Siris ES et al (1999) A randomized, double-blind comparison of risedronate and etidronate in the treatment of Paget’s disease of bone. Am J Med 106:513–520

    CAS  PubMed  Google Scholar 

  24. Morales-Piga AA, Rey-Rey JS, Corres-Gonzales J et al (1995) Frequency and characteristics of familial aggregation of Paget’s disease of bone. J Bone Miner Res 10:663–670

    CAS  PubMed  Google Scholar 

  25. Parvizi J, Schall DM, Lewallen DG, Sim FH (2002) Outcome of uncemented hip arthroplasty components in patients with Paget’s disease. Clin Orthop Relat Res 403:127–134

    Google Scholar 

  26. Parvizi J, Frankle MA, Tiegs RD, Sim FH (2003) Corrective osteotomy for deformity in Paget’s disease. J Bone Joint Surg Am 85:697–702

    PubMed  Google Scholar 

  27. Ralston SH (2013) Paget’s disease of bone. N Engl J Med 368:644–650

    CAS  PubMed  Google Scholar 

  28. Ralston SH, Corral-Gudino L, Cooper C et al (2019) Diagnosis and management of Paget’s disease of bone in adults: a clinical guideline. J Bone Miner Res 34:579–604

    PubMed  Google Scholar 

  29. Reid IR, Davidson JS, Wattie D et al (2004) Comparative responses of bone turnover markers to bisphosphonate therapy in Paget’s disease of bone. Bone 35:224–230

    CAS  PubMed  Google Scholar 

  30. Reid IR, Nicholson GC, Weinstein RS et al (1996) Biochemical and radiologic improvement in Paget’s disease of bone treated with alendronate: a randomized, placebo-controlled trial. Am J Med 101:341–348

    CAS  PubMed  Google Scholar 

  31. Reid IR, Miller P, Lyles K et al (2005) Comparison of a single infusion of zoledronic acid with risedronate for Paget’s disease. N Engl J Med 353:898–908

    CAS  PubMed  Google Scholar 

  32. Ringe JD, Jend HH, Becker H (1984) Epidemiologie der ostitis deformans Paget. Münch Med Wochenschr 126:683–686

    Google Scholar 

  33. Shawn N, Burrell CL, Green D et al (2019) Molecular insights into an ancient form of Paget’s disease of bone. Proc Natl Acad Sci USA 116:10463–10472

    Google Scholar 

  34. Schwarz P, Rasmussen AQ, Kvist TM (2012) Paget’s disease of the bone after treatment with denosumab: a case report. Bone 50:1023–1025

    PubMed  Google Scholar 

  35. Reid IR, Shamara S, Kalluru R et al (2016) Treatment of Paget’s disease of bone with denosumab: case report and literature review. Calcif Tissue Int 99:322–325

    CAS  PubMed  Google Scholar 

  36. Sochart DH, Porter ML (2000) Charnley low-friction arthroplasty for Paget’s disease of the hip. J Arthroplasty 15:210–219

    CAS  PubMed  Google Scholar 

  37. Staufer RN, Sim FH (1976) Total hip arthroplasty in Paget’s disease of the hip. J Bone Joint Surg Am 58:476–478

    Google Scholar 

  38. Takata S, Hashimoto J, Nakatsuka K et al (2006) Guidelines for diagnosis and management of Paget’s disease of bone in Japan. J Bone Miner Metab 24:359–367

    PubMed  Google Scholar 

  39. Tan A, Ralston SH (2014) Clinical presentation of Paget’s disease: evaluation of a contemporary cohort and systematic review. Calcif Tissue Int 95:385–392

    CAS  PubMed  Google Scholar 

  40. Tan A, Goodman K, Walker A et al (2017) Long-term randomized trial of intensive versus symptomatic management in Paget’s disease of bone: the PRISM-EZ-study. J Bone Miner Res 32:1165–1163

    CAS  PubMed  Google Scholar 

  41. Termachi J, Nagata Y, Mohammad K et al (2016) Measles virus nucleocapsid protein increases osteoblast differentiation in Paget’s disease. J Clin Invest 126:1012–1022

    Google Scholar 

  42. Tuk P, Layfield R, Walker J et al (2017) Adult Paget’s disease of bone: a review. Baillieres Clin Rheumatol 56:2050–2059

    Google Scholar 

  43. van Staa TP, Selby P, Leufkens HG et al (2002) Incidence and natural history of Paget’s disease of bone in England and Wales. J Bone Miner Res 17:465–471

    Google Scholar 

  44. Wellman HN, Schauwecker D, Robb JA et al (1977) Skeletal scintimaging and radiography in the diagnosis and management of Paget’s disease. Clin Orthop Relat Res 127:55–62

    Google Scholar 

  45. Ziegler R, Holz G, Rotzler B, Minne H (1985) Paget’s disease of bone in West Germany. Prevalence and distribution. Clin Orthop Relat Res 194:199–204

    Google Scholar 

  46. Zlatkin MB, Lander PH, Hadjipavlou AG et al (1986) Paget’s disease of the spine: CT with clinical correlations. Radiology 160:155–160

    CAS  PubMed  Google Scholar 

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Correspondence to U. Lange.

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P. Klemm, G. Dischereit, S. von Gerlach und U. Lange geben an, dass kein Interessenkonflikt besteht.

Für diesen Beitrag wurden von den Autoren keine Studien an Menschen oder Tieren durchgeführt. Für die aufgeführten Studien gelten die jeweils dort angegebenen ethischen Richtlinien.

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U. Müller-Ladner, Bad Nauheim

U. Lange, Bad Nauheim

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Klemm, P., Dischereit, G., von Gerlach, S. et al. Morbus Paget des Knochens – eine aktuelle Übersicht zu Klinik, Diagnostik und Therapie. Z Rheumatol 80, 48–53 (2021). https://doi.org/10.1007/s00393-020-00897-x

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