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Pagets sykdom

Sist revidert:
Sist revidert av:


Definisjon:
En fokal eller multifokal kronisk beinsykdom med økt osteoklastisk beinresorpsjon og sekundært økt beindannelse som medfører abnormt beinvev med nedsatt styrke
Forekomst:
Meget sjelden i Skandinavia
Symptomer:
Tilstanden er først asymptomatisk, etter hvert oppstår lokale symptomer i form av ubehag og lette smerter, oftest fra skjelettet, disse er ofte konstante og verst om natten. Senere kan smertene bli intense
Funn:
Kan være et svært mykt eller svært hardt beinvev. Økende deformering kan føre til stor skalle, bøyd tibia og økt kyfose
Diagnostikk:
Røntgen og scintigrafi brukes i kartleggingen av tilstanden
Behandling:
De fleste behøver ingen behandling. Ved behov for behandling brukes bisfosfonater
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  2. Tuck SP, Layfield R, Walker J, et al. Adult Paget´s disease of bone: a review. Rheumatology 2017; 56: 2050-9. Rheumatology  
  3. Rianon NJ, des Bordes JK. Paget Disease of Bone for Primary Care. Am Fam Physician. 2020 Aug 15;102(4):224-228. PMID: 32803929. PubMed  
  4. Lalam RK, Cassar-Pullicino VN, Winn N. Paget Disease of Bone. Semin Musculoskelet Radiol. 2016 Jul. 20 (3):287-299.
  5. Bolland MJ, Cundy T. Paget's disease of bone: clinical review and update. J Clin Pathol 2013; 66: 924-7. PubMed  
  6. Corral-Gudino L, Borao-Cengotita-Bengoa M, Del Pino-Montes J, et al. Epidemiology of Paget's disease of bone: a systematic review and meta-analysis of secular changes. Bone. 2013;55(2):347–352.
  7. Polyzos SA, Cundy T, Mantzoros CS. Juvenile Paget disease. Metabolism. 2017 Nov 22. PMID: 29080812. PubMed  
  8. Ralston SH. Pathogenesis of Paget's disease of bone. Bone. 2008;43(5):819–825. PMID: 18672105. PubMed  
  9. Cundy T. Paget's disease of bone. Metabolism. 2018;80:5–14. PMID: 28780255. PubMed  
  10. Vallet M, Ralston SH. Biology and treatment of Paget's disease of bone. J Cell Biochem. 2016;117(2):289–299. PMID: 26212817. PubMed  
  11. Ralston SH. Clinical practice. Paget's disease of bone. N Engl J Med. 2013;368(7):644–650.
  12. Tan A, Ralston SH. Clinical presentation of Paget's disease: evaluation of a contemporary cohort and systematic review. Calcif Tissue Int. 2014;95(5):385–392. PMID: 25160936. PubMed  
  13. Parvizi J, Restrepo C, Sim FH. Paget's disease of the hip: surgical management. Future Rheumatology 2006;1:373-377. PubMed  
  14. Ralston SH, Corral-Gudino L, Cooper C, et al. Diagnosis and management of Paget's disease of bone in adults: a clinical guideline. J Bone Miner Res. 2019;34(4):579–604. PMID: 30803025. PubMed  
  15. Rosen CJ, Brown S. Severe hypocalcemia after intravenous bisphosphonate therapy in occult vitamin D deficiency. N Engl J Med 2003; 348: 1503-4. New England Journal of Medicine  
  16. Singer F, Bone HG, Hosking DJ et al. Paget's disease of bone: an endocrine society clinical practice guideline. J Clin Endocrinol Metab 2014; 99: 4408–22.
  17. Corral-Gudino L, Tan AJ, Del Pino-Montes J, et al. Bisphosphonates for Paget's disease of bone in adults. Cochrane Database Syst Rev. 2017;(12):CD004956. Cochrane (DOI)  
  18. Bolland MJ, Avenell A, Baron JA, et al. Effect of calcium supplements on risk of myocardial infarction and cardiovascular events: meta-analysis. BMJ 2010;341:c3691. BMJ (DOI)  
  19. Wermers RA, Tiegs RD, Atkinson EJ, et al. Morbidity and mortality associated with Paget's disease of bone: a population-based study. J Bone Miner Res. 2008;23(6):819–825. PMID: 18269308. PubMed  
  • Terje Johannessen, professor i allmennmedisin, Trondheim

Tidligere fagmedarbeidere

  • Arild Aamodt, overlege/professor, Ortopedisk avdeling, Lovisenberg Sykehus, Oslo