Diagnosis and treatment of Paget's disease of bone: a mini-review

Abstract

Paget's disease of bone (PDB) is a chronic progressive disorder of bone metabolism that may go undetected for many years, and endocrinologists should be alert to its clinical signs and promptly diagnose and treat PDB before it results in irreversible complications, such as deformity, fracture or neurological sequelae. Most commonly, PDB is suspected upon the incidental finding of elevated serum alkaline phosphatase levels or a radiographic abnormality in an otherwise healthy individual above 55 years of age. Some of these individuals may have symptoms such as bone pain or enlargement with increased warmth. In general, a basic laboratory evaluation of bone metabolism, plain radiographies of affected bones and bone scintigraphy are sufficient to corroborate the diagnosis. Antiresorptive therapy with bisphosphonates is the mainstay of treatment of symptomatic PDB, and intravenous zoledronic acid has emerged as an effective and safe treatment option, leading to sustained remission and improved quality of life. It is extremely important, though, to ensure calcium and vitamin D sufficiency before and during treatment in order to prevent hypocalcemia. The benefit of treating all asymptomatic patients is not clear, but treatment is warranted if the pagetic lesion is located in a site where progression to fracture, deformity, or compression would significantly impair the patient quality of life. This mini-review focuses on important aspects of the diagnosis and treatment of PDB. Arq Bras Endocrinol Metab. 2013;57(8):577-82 Keywords Paget's disease of bone; bisphosphonates; zoledronic acid; osteoclast; antiresorptive treatment RESUMO A doença de Paget dos ossos (PDB) é uma doença progressiva e crônica do metabolismo ósseo que pode passar despercebida por muitos anos. Os endocrinologistas devem ficar alertas aos seus sinais clínicos e diagnosticar e tratar a PDB imediatamente, antes que ela gere complicações irreversíveis, como deformidade, fratura ou sequelas neurológicas. Mais comumente, suspeita-se da PBD após o achado incidental de níveis elevados de fosfatase alcalina no soro, ou anormalidades radiográficas em indivíduos aparentemente saudáveis com mais de 55 anos de idade. Alguns desses indivíduos podem apresentar sintomas, como a dor ou aumento ósseo com temperatura aumentada. Em geral, a avaliação laboratorial básica de metabolismo ósseo, radiografias simples dos ossos afetados e cintilografia óssea são suficientes para corroborar o diagnóstico. O tratamento antirreabsortivo com bifosfonatos é o principal tratamento da PDB sintomática, e o ácido zoledrônico intravenoso passou a ser uma opção de tratamento segura e eficiente, levando à manutenção da remissão e à melhora da qualidade de vida. É extremamente importante, entretanto, garantir níveis adequados de cálcio e vitamina D antes e durante o tratamento para se evitar a hipocalcemia. O benefício de se tratar todos os pacientes assintomáticos não está claro, mas o tratamento é recomendado se a localização da lesão pagética sugerir progressão para fratura, ...

Difficulties in diagnosis and treatment of Paget’s disease

“…The reason for this is increased bone resorption as compared to the impaired ossification in situations of no mechanical stimulation [8].…”

“…sites of increased uptake indicate increased bone turnover and increased vascularity. This examination is also particularly useful in assessing the effectiveness of treatment [8,9].…”

“…Computed tomography and magnetic resonance imaging may be useful in case of symptoms of spinal stenosis, compression of the nerve roots, vertebral compression fractures and suspected malignant lesions [3,8]. in the latter The efficacy of the treatment is assessed after 3 and 6 months after treatment with risedronate and alendronate, after 6 months of treatment with pamidronate and after one year of using zolendronic acid.…”

“…in the latter The efficacy of the treatment is assessed after 3 and 6 months after treatment with risedronate and alendronate, after 6 months of treatment with pamidronate and after one year of using zolendronic acid. if the symptoms return or the concentration of alkaline phosphatase increases by 75% above the upper limit of the normal level, the treatment should be repeated [8]. Bisphosphonates reduce bone turnover, pain intensity, decrease concentration of alkaline phosphatase and decrease radiological losses, but they do not prevent the progression of deformity and do not restore normal bone structure [3].…”

“…Bisphosphonates reduce bone turnover, pain intensity, decrease concentration of alkaline phosphatase and decrease radiological losses, but they do not prevent the progression of deformity and do not restore normal bone structure [3]. it was demonstrated that after a single dose of zolendronic acid, the therapeutic effect, in the form of normal bone turnover marker activity, persists for at least 2 years [5,8]. data on the efficacy of bisphosphonate treatment is not consistent.…”