Paget's disease of bone

Overview 
Theory 
Diagnosis 
Management 
Follow up 
Resources 

Last reviewed: 11 Sep 2025

Last updated: 01 Sep 2022

Paget’s disease of bone (PDB) is a chronic localized bone remodeling disorder characterized by increased bone resorption, bone formation, and remodeling, which may lead to major long bone and skull deformities.

Majority of patients are asymptomatic. Symptomatic patients typically experience pain localized to the bone or joint, either from the pagetic lesion, secondary osteoarthritis, deformity, or pathologic fracture.

Neurologic symptoms due to bone overgrowth with consequent nerve impingement may include hearing loss, chronic facial pain, hydrocephalus, peripheral nerve entrapment, and spinal stenosis. Very rarely, high-output cardiac failure caused by high blood flow to metabolically active bone sites may occur.

Diagnosis is incidental in the majority of patients, with an isolated elevated serum alkaline phosphatase raising suspicion for disease. Plain radiographs are diagnostic; in less clear cases, a computed tomography scan is indicated. Bone biopsy is the ultimate confirmatory test, but it is rarely necessary.

If treatment is indicated, bisphosphonates are the first-line therapy to retard excessive osteoclastic activity. Adjunctive therapy includes physical therapy, orthoses, and walking and hearing aids. Analgesics are indicated for pain control.

Definição

A chronic bone disorder that is characterized by focal areas of increased bone remodeling, resulting in overgrowth of poorly organized bone. This unbalanced process may lead to osseous deformities, altered joint biomechanics, nerve compressions, and pathologic fractures.

História e exame físico

Principais fatores diagnósticos

family history of PDB
asymptomatic

Detalhes completos

Outros fatores diagnósticos

femoral, pelvis, and/or skull involvement
long-bone or back pain
pathologic fracture
bony deformities (e.g., frontal bossing, prognathism, bone bowing)
increased local temperature
hearing loss
facial pain
loosening teeth or disturbance in chewing
isolated raised alkaline phosphatase

Detalhes completos

Fatores de risco

family history of PDB
age >50 years
male sex (45- to 74-years age group)

Detalhes completos

Investigações diagnósticas

Primeiras investigações a serem solicitadas

plain x-ray
bone scan
total serum alkaline phosphatase
bone-specific alkaline phosphatase
serum calcium
serum procollagen 1 N-terminal peptide (P1NP)
serum C-terminal propeptide of type 1 collagen (CTX)
liver function tests
serum 25-hydroxyvitamin D

Detalhes completos

Investigações a serem consideradas

CT scan or MRI
bone biopsy

Detalhes completos

Algoritmo de tratamento

CONTÍNUA

asymptomatic patients, incidental diagnosis

symptomatic patients

Colaboradores

Autores

Roberto Civitelli, MD

Sydney M. & Stella H. Schoenberg Professor of Medicine

Professor of Orthopaedic Surgery and Cell Biology and Physiology

Chief, Division of Bone and Mineral Diseases

Musculoskeletal Research Center

Washington University School of Medicine

St. Louis

Declarações

RC is Editor-in-Chief of the Journal of Bone and Mineral Research (position ending 31/12/2022).

Mahshid Mohseni, MD, MSci

Assistant Professor

Director, Clinical Research Unit

Co-Director, Clinical Operations

Division of Bone and Mineral Diseases

Department of Medicine

Washington University School of Medicine

St. Louis

Declarações

MM declares that he has no competing interests.

Agradecimentos

Dr Roberto Civitelli and Dr Mahshid Mohseni would like to gratefully acknowledge Dr Julia F. Charles, Dr Camilo Restrepo, and Dr Javad Parvizi, previous contributors to this topic.

Declarações

JFC has received royalties from Up To Date, Waltham, MA, for authorship on articles relating to PDB; received grant funding from the National Institues of Health, the Rheumatology Research Foundation, and Brigham and Women's Hospital for research into regulation of osteoclast function, but not specifically relating to PDB; and had a manuscript published from a collaboration with Dr Jae Shim describing a pagetic phenotype in mice with osteoclast-specific loss of Chmp5. CR and JP are authors of a reference cited in this topic.

Revisores

Frederick Singer, MD

Director

Endocrine/Bone Disease Program

John Wayne Cancer Institute

Santa Monica

Declarações

FS declares that he has no competing interests.

Joseph Lane, MD

Orthopaedic Surgeon

Hospital for Special Surgery

New York

Declarações

JL declares that he has no competing interests.

Peer reviewer acknowledgements

BMJ Best Practice topics are updated on a rolling basis in line with developments in evidence and guidance. The peer reviewers listed here have reviewed the content at least once during the history of the topic.

Disclosures

Peer reviewer affiliations and disclosures pertain to the time of the review.

Referências

Nossas equipes internas de editoria e de evidências trabalham em conjunto com colaboradores internacionais especializados e pares revisores para garantir que forneçamos acesso às informações o mais clinicamente relevantes possível.

Principais artigos

Tuck SP, Walker J. Adult Paget's disease of bone. Clin Med (Lond). 2020 Nov;20(6):568-71.Texto completo Resumo

Singer FR, Bone HG 3rd, Hosking DJ, et al. Paget's disease of bone: an Endocrine Society clinical practice guideline. J Clin Endocrinol Metab. 2014 Dec;99(12):4408-22.Texto completo Resumo

Whitehouse RW. Paget's disease of bone. Semin Musculoskelet Radiol. 2002 Dec;6(4):313-22. Resumo

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 Apr;34(4):579-604.Texto completo Resumo

Artigos de referência

Uma lista completa das fontes referenciadas neste tópico está disponível para os usuários com acesso total ao BMJ Best Practice.

Diagnósticos diferenciais
- Osteomalacia
- Fibrous dysplasia
Mais Diagnósticos diferenciais
Diretrizes
- Diagnosis and management of Paget's disease of bone in adults
- Paget’s disease of bone
Mais Diretrizes
Conectar-se ou assinar para acessar todo o BMJ Best Practice

O uso deste conteúdo está sujeito ao nosso aviso legal

Paget's disease of bone

Summary

Definição

História e exame físico

Principais fatores diagnósticos

Outros fatores diagnósticos

Fatores de risco

Investigações diagnósticas

Primeiras investigações a serem solicitadas

Investigações a serem consideradas

Algoritmo de tratamento

asymptomatic patients, incidental diagnosis

symptomatic patients

Colaboradores

Autores

Roberto Civitelli, MD

Declarações

Mahshid Mohseni, MD, MSci

Declarações

Agradecimentos

Declarações

Revisores

Frederick Singer, MD

Declarações

Joseph Lane, MD

Declarações

Peer reviewer acknowledgements

Disclosures

Referências

Principais artigos

Artigos de referência

Uma lista completa das fontes referenciadas neste tópico está disponível para os usuários com acesso total ao BMJ Best Practice.

Diagnósticos diferenciais

Diretrizes

Conectar-se ou assinar para acessar todo o BMJ Best Practice

Conectar-se ou assinar para acessar todo o BMJ Best Practice

Conectar-se para acessar todo o BMJ Best Practice