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Diagnosis and treatment of Paget’s disease of bone

A clinical practice guideline

Diagnose und Behandlung der Knochenerkrankung Morbus Paget

Eine klinische Praxisstudie

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Summary

Paget’s disease of bone (osteitis deformans) is a benign focal disorder of accelerated skeletal remodeling. Either a single bone (monostotic) or multiple bones (polyostotic) can be affected. In patients with suspected Paget’s disease plain radiographs of the suspicious regions of the skeleton are recommended. The initial biochemical evaluation of a patient should be done using serum total ALP (alkaline phosphatase) or with the use of a more specific marker of bone formation: PINP (intact N-terminal type 1 procollagen propeptide) or CTX (cross-linked C‑telopeptide). Treatment with a bisphosphonate is recommended for most patients with active Paget’s disease who are at risk for further skeletal and extraskeletal complications. A single dose of 5 mg i.v. zoledronate as the treatment of choice in patients without contraindications is suggested. Oral bisphosphonates are less potent when compared to zoledronate. Treatment with an antiresorptive agent induces a more rapid decrease in resorption markers compared to formation marker. Measurement of total ALP or other baseline disease activity markers (e. g. CTX) at 6 to 12 weeks, when bone turnover will have shown a substantial decline, is an acceptable and cost-effective option. Maximum suppression of high bone turnover may require measurement at 6 months after administration. In patients with increased bone turnover, biochemical follow-up is recommended to be used as a more objective indicator of relapse rather than symptoms. The prolonged response after zoledronate treatment should be assessed every 1–2 years after normal bone turnover. With less potent drugs, every 6 to 12 months is appropriate.

Zusammenfassung

Morbus Paget (Osteitis deformans) ist eine fokale benigne Erkrankung des Knochens mit beschleunigtem Knochenumsatz. Betroffen sind entweder einzelne (monostotische Form) oder mehrere Knochen (polyostotische Form). Diagnostisch werden Röntgenaufnahmen der suszipierten Skelettregionen empfohlen. Zur weiteren serologischen Untersuchung wird die alkalische Phosphatase (AP) empfohlen. Zusätzlich können spezifische Serummarker der Knochenformation (PINP Prokollagen Typ 1 N-terminales Propeptid) oder der Knochenresorption (CTX Cross-linked C‑Telopeptid) bestimmt werden. Zur Behandlung von Patienten mit einem aktiven M. Paget und einem erhöhten Risiko für weitere skeletale oder extraskeletale Manifestationen werden Bisphosphonate empfohlen. Eine einmalige i.v.-Dosis von 5 mg Zoledronat ist Mittel der Wahl bei Patienten ohne Kontraindikationen. Orale Bisphosphonate sind im Vergleich zu i.v.-Applikationen weniger potent. Die Behandlung mit einem antiresorptiven Medikament induziert eine schnellere Reduktion der Resorptionsmarker im Vergleich zu den Formationsmarkern. Die Bestimmung der AP oder anderer Marker (z. B. CTX) 6 bis 12 Wochen nach der Applikation eines Bisphosphonats ist eine kostengünstige Methode zur Überprüfung des therapeutischen Erfolgs. Die maximale Suppression wird erst nach 6 Monaten erreicht. Bei Patienten mit erhöhtem Knochenumsatz ist die Bestimmung der biochemischen Marker ein objektiverer Indikator im Vergleich zur Symptomkontrolle. Das Ansprechen auf eine Therapie mit Zoledronat sollte 1 bis 2 Jahre nach Applikation serologisch kontrolliert werden; mit weniger potenten antiresorptiven Medikamenten alle 6 bis 12 Monate.

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Correspondence to Christian Muschitz MD.

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Conflict of interest

C. Muschitz has received speaker and/or consultancy honoraria from Amgen, Novartis, Servier, Eli Lilly, Nycomed Pharma/Takeda, Kwizda Pharma, Boehringer Ingelheim, Actavis, Alexion, and Daiichi-Sankyo. C. Muschitz has received educational grants/research support from the Austrian Society for Bone and Mineral Research, Roche Austria, Eli Lilly Austria, Eli Lilly International, and Amgen Austria. He has nothing to disclose concerning this manuscript. R. Kocijan has received speaker honoraria from Eli Lilly. He has nothing to disclose concerning this manuscript. X. Feichtinger and J. Haschka declare that they have no competing interests.

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This review article was not supported by any grant or pharmaceutical company.

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Muschitz, C., Feichtinger, X., Haschka, J. et al. Diagnosis and treatment of Paget’s disease of bone. Wien Med Wochenschr 167, 18–24 (2017). https://doi.org/10.1007/s10354-016-0502-x

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