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Authors
Advisor(s)
Abstract(s)
Introdução: A Doença Óssea de Paget (DOP) ou Osteíte Deformante é uma
doença óssea crónica focal, caracterizada por uma taxa de remodelação óssea acelerada
que, por sua vez, leva à formação de um novo osso desorganizado, aumentado e com
estrutura enfraquecida, propenso a deformidades e fraturas. Tanto a prevalência como
a apresentação clínica e demográfica desta condição clínica podem variar consoante a
população. Desta forma, face à morbilidade que esta doença pode causar, é importante
conhecer as suas características.
Objetivo: Este estudo tem como objetivo a avaliação e caracterização dos
parâmetros demográficos, clínicos, bioquímicos, imagiológicos e tratamento de
pacientes com o diagnóstico de DOP seguidos na Unidade de Reumatologia da Unidade
Local de Saúde Castelo Branco (ULSCB).
Metodologia: Para a concretização desta investigação, foi realizado um estudo
observacional, retrospetivo e transversal dos utentes com DOP desde março de 2011 até
setembro de 2021, na Unidade de Reumatologia da ULSCB. Foram recolhidos dados
demográficos, ano de apresentação, sintomas, existência ou não de complicações à data
de apresentação da doença e durante o período de follow-up. Também foi feita análise
dos exames imagiológicos efetuados em todos os pacientes. Para a recolha de dados
procedeu-se à consulta do processo clínico eletrónico e, posteriormente, o tratamento
de dados foi realizado no programa Microsoft Excel®.
Resultados: Este estudo sobre a DOP teve predominância masculina (90%) com
uma idade média ao diagnóstico de 66.1 anos. Em nenhum dos casos foi reportada a
existência de história familiar, bem como nenhum paciente se apresentou
assintomático ao diagnóstico, tendo todos exibido um certo grau de dor óssea ou dor
lombar. Não houve ainda nenhum registo da ocorrência de alguma fratura patológica. A
forma monostótica da doença foi a mais prevalente, sendo observada em 80% dos
pacientes. Os ossos afetados foram o sacro (30%), o ilíaco (50%) e o fémur (30%).
Relativamente à Fosfatase Alcalina (FA) sérica, 50% dos doentes apresentaram este
parâmetro aumentado ao diagnóstico, com uma média de 150.2 IU/L. Importante
acrescentar que todos os casos foram sujeitos a uma avaliação por cintigrafia óssea para
estudo da extensão da doença. Em termos de tratamento, 80% dos pacientes foram
sujeitos ao tratamento com Ácido Zoledrónico com resultados muito satisfatórios tanto no controlo dos sintomas como na redução dos níveis de FA. Apenas em um caso foi
necessário um segundo ciclo de tratamento, após 5 anos, por nova elevação dos níveis
de FA.
Conclusão: O presente estudo demonstrou tanto a existência como as
manifestações clínicas da DOP na Unidade de Reumatologia de Castelo Branco,
revelando que esta é uma condição clínica muito rara entre a população portuguesa.
Dentro da amostra analisada há uma maioria masculina e da doença monostótica, sem
história familiar significativa. Tanto as características clínicas como bioquímicas
clássicas apresentaram uma boa resposta à terapêutica com bifosfonato. No entanto, as
características demográficas e de apresentação clínica diferem da maioria dos estudos
realizados em vários países, o que pode ter sido causado pelo reduzido tamanho da
amostra utilizada.
Introduction: The Paget’s Bone Disease (PBD) or osteitis deformans is a chronic focal bone disease, characterized by an accelerated rate of bone remodelling which in turn leads to the formation of a new disorganized and increased bone, with a weakened structure, prone to deformities and fractures. Both the prevalence and the clinical and demographic presentation of this condition may vary according to the population. Thus, given the morbidity that this disease can cause, it is important to know its characteristics. Objective: This study aims to evaluate and characterize the demographic, clinical, biochemical, imaging and treatment parameters of patients with the diagnosis of PBD followed at the Rheumatology department of the Unidade Local de Saúde de Castelo Branco (ULSCB). Methods: To carry out this investigation, an observational, retrospective and crosssectional study of patients with PBD was made from march 2011 until september 2021, at the ULSCB Rheumatology department. Demographic data, year of presentation, symptoms, existence or not of complications at the time of the disease presentation and during the follow-up were collected. Analysis of the imagiologic exams performed in all patients was also made. For data collection, the electronic clinical file was consulted and, subsequently, the data processing was carried out using the Microsoft Excel® program. Results: This study about PBD had a male predominance (90%) with a mean age at diagnosis of 66.1 years. No positive family history was reported nor was any patient asymptomatic at diagnosis, having everyone exhibited a certain degree of bone pain or low back pain. Still, there was no report of the occurrence of any pathologic fracture. The monostotic form of the disease was the most prevalent, being observed in 80% of the patients. The bones affected were the sacrum (30%), the iliac (50%) and the femur (30%). Regarding the serum alkaline phosphatase (AP), 50% of the patients had this parameter increased at diagnosis, with an average of 150.2 IU/L. It is also important to add that all cases were submitted to an evaluation by bone scintigraphy to study the extent of the disease. In relation to the treatment, 80% patients underwent treatment with zoledronic acid with very satisfactory results both in controlling the symptoms and reducing the levels of AP. There was only one case where a second cycle of treatment was necessary, after 5 years, due to a new increase in AP levels. Conclusion: This present study demonstrated both the existence and the clinical manifestations of PBD at the Castelo Branco Rheumatology Unit, revealing that this is a very rare clinical condition among the Portuguese population. Within the analysed sample there is a male majority as well as the monostotic form of the disease, without significant family history. Both clinical and classical biochemical features showed a good response to the therapy with biphosphonates. However, the demographic characteristics and clinical presentation differ from most studies carried out in several countries, which may have been caused by the small sample size used.
Introduction: The Paget’s Bone Disease (PBD) or osteitis deformans is a chronic focal bone disease, characterized by an accelerated rate of bone remodelling which in turn leads to the formation of a new disorganized and increased bone, with a weakened structure, prone to deformities and fractures. Both the prevalence and the clinical and demographic presentation of this condition may vary according to the population. Thus, given the morbidity that this disease can cause, it is important to know its characteristics. Objective: This study aims to evaluate and characterize the demographic, clinical, biochemical, imaging and treatment parameters of patients with the diagnosis of PBD followed at the Rheumatology department of the Unidade Local de Saúde de Castelo Branco (ULSCB). Methods: To carry out this investigation, an observational, retrospective and crosssectional study of patients with PBD was made from march 2011 until september 2021, at the ULSCB Rheumatology department. Demographic data, year of presentation, symptoms, existence or not of complications at the time of the disease presentation and during the follow-up were collected. Analysis of the imagiologic exams performed in all patients was also made. For data collection, the electronic clinical file was consulted and, subsequently, the data processing was carried out using the Microsoft Excel® program. Results: This study about PBD had a male predominance (90%) with a mean age at diagnosis of 66.1 years. No positive family history was reported nor was any patient asymptomatic at diagnosis, having everyone exhibited a certain degree of bone pain or low back pain. Still, there was no report of the occurrence of any pathologic fracture. The monostotic form of the disease was the most prevalent, being observed in 80% of the patients. The bones affected were the sacrum (30%), the iliac (50%) and the femur (30%). Regarding the serum alkaline phosphatase (AP), 50% of the patients had this parameter increased at diagnosis, with an average of 150.2 IU/L. It is also important to add that all cases were submitted to an evaluation by bone scintigraphy to study the extent of the disease. In relation to the treatment, 80% patients underwent treatment with zoledronic acid with very satisfactory results both in controlling the symptoms and reducing the levels of AP. There was only one case where a second cycle of treatment was necessary, after 5 years, due to a new increase in AP levels. Conclusion: This present study demonstrated both the existence and the clinical manifestations of PBD at the Castelo Branco Rheumatology Unit, revealing that this is a very rare clinical condition among the Portuguese population. Within the analysed sample there is a male majority as well as the monostotic form of the disease, without significant family history. Both clinical and classical biochemical features showed a good response to the therapy with biphosphonates. However, the demographic characteristics and clinical presentation differ from most studies carried out in several countries, which may have been caused by the small sample size used.
Description
Keywords
Ácido Zoledrónico Bifosfonatos Doença Óssea de Paget Osso Osteíte Deformante