Clinical features, diagnostic evaluation and responses to treatment in Paget's disease of bone in a center specialized in bone metabolism.

The epidemiology of Paget's disease of bone (PDB) has changed in the last years but there is no update data on its clinical presentation, diagnosis and management in Latin America. Our aim was to describe its clinical features, diagnostic evaluation and responses to treatment in a group of PDB patients treated between June 2012 and December 2019 in an institution specialized in bone diseases, in Buenos Aires, Argentina. The frequency of PDB (180/10 714) was 1.68%. Median age was 67 (range 39-97) years and 59.5% were women. Most patients were asymptomatic (58.6%) and had monostotic disease (54.3%). Favorable responses were obtained in all patients who were treated with zoledronate (n = 36), in 10 out of 14 treated with pamidronate, in 9 out of 10 who received intravenous ibandronate and in 12 out of 13 who received oral bisphosphonates. The response rates were not significantly different when we compared monostotic vs. polyostotic disease. Among the biochemical parameters, mean values of bone specific and total alkaline phosphatase, and C-terminal crosslinked telopeptide of type I collagen decreased significantly after treatment with bisphosphonates. It seems that our results reflect the change in PDB epidemiology towards a more indolent disease. In the future, this would probably allow physicians to use lower doses of bisphosphonates than the ones historically recommended for these patients.

La epidemiología de la enfermedad de Paget ósea (EPO) ha cambiado en los últimos años. Son necesarios datos actualizados sobre su forma de presentación clínica, diagnóstico y tratamiento en nuestra región. Nuestro objetivo fue describir las características clínicas, evaluación diagnóstica y respuestas al tratamiento de un grupo de pacientes con EPO en un centro especializado en salud ósea de Buenos Aires, Argentina. Se evaluaron todos los pacientes que fueron atendidos en nuestra institución por enfermedades óseas entre junio de 2012 y diciembre de 2019. La frecuencia de EPO (180/10 714) fue de 1.68%. La mediana de edad fue de 67 (rango 39-97) años. El 59.5% eran mujeres. La mayoría se encontraba asintomático (58.6%) y tenían enfermedad monostótica (54.3%). Se objetivaron respuestas favorables en todos los que recibieron zoledronato (n = 36), en 10 de 14 pacientes que recibieron pamidronato, en 9 de 10 que utilizaron ibandronato endovenoso y en 12 de 13 con bifosfonatos orales. Los porcentajes de respuesta no variaron significativamente entre pacientes con formas monostóticas y poliostóticas. Entre los parámetros bioquímicos, los valores de fosfatasa alcalina total y ósea y de Β cross-laps disminuyeron significativamente luego del tratamiento con bifosfonatos. Nuestros resultados reflejarían un cambio en la epidemiología de la EPO hacia una forma de presentación más indolente. Esto permitiría probablemente el uso de dosis más bajas de bifosfonatos que las históricamente recomendadas para estos pacientes.

Misdiagnosis of Paget's Disease of Bone in a Congenital Generalized Lipodystrophy Patient: Case Report.

Paget's disease of bone (PDB) is a common skeleton disorder in which the diagnosis is suggested by radiological analyses. Congenital generalized lipodystrophy (CGL) is a rare, but a radiologic differential diagnosis of Paget's disease. Patients present total or almost total lack of subcutaneous adipose tissue, leptin deficiency, and precocious ectopic lipid accumulation, which lead to intense insulin resistance, poorly controlled diabetes mellitus, and hypertriglyceridemia. CGL subtypes 1 and 2 present sclerosis and osteolytic lesions that can resemble "pagetic" lesions. The clinical correlation is, therefore, essential. We report a CGL patient with bone lesions in which the radiographic findings led to a misdiagnosis of PDB. This case report brings awareness to CGL, a life-threating condition. Its early recognition is essential to avoid clinical complications and premature death. Therefore, it is important to consider CGL as PDB's differential diagnosis, especially in countries with high prevalence of this rare disease, such as Brazil.

Distrofia ósea esclerosante mixta / Mixed-sclerosing-bone-dystrophy

El término "distrofia ósea esclerosante mixta" describe la combinación de las características radiológicas correspondientes a melorreostosis, osteopoiquilosis y osteopatía estriada, como entidades individuales, que ocurren en un mismo paciente. El objetivo de esta comunicación es presentar el caso clínico de una paciente con diagnóstico de distrofia ósea esclerosante mixta y, a partir de este caso, realizar una revisión sobre el tema. (AU)

The term "mixed-sclerosing-bone-dystrophy" describes the combination of the radiological characteristics corresponding to melorheostosis, osteopoikilosis and osteopathia striata, as individual conditions, ocurring in the same patient. The aim of this communication is to present the clinical case of a patient diagnosed with mixed-sclerosing-bone-dystrophy and, based on this case, to undertake a review of this condition. (AU)

Diagnosis and management of Paget's disease of bone.

OBJECTIVE: To conduct a literature review on the diagnosis and management of Paget's disease of bone. MATERIALS AND METHODS: This scientific statement was generated by a request from the Brazilian Medical Association (AMB) to the Brazilian Society of Endocrinology and Metabolism (SBEM) as part of its Clinical Practice Guidelines program. Articles were identified by searching in PubMed and Cochrane databases as well as abstracts presented at the Endocrine Society, Brazilian Society for Endocrinology Annual Meetings and the American Society for Bone and Mineral Research Annual Meeting during the last 5 years. Grading quality of evidence and strength of recommendation were adapted from the first report of the Oxford Centre for Evidence-based Medicine. All grades of recommendation, including "D", are based on scientific evidence. The differences between A, B, C and D, are due exclusively to the methods employed in generating evidence. CONCLUSION: We present a scientific statement on Paget's disease of bone providing the level of evidence and the degree of recommendation regarding causes, clinical presentation as well as surgical and medical treatment.

Diagnosis and management of Paget?s disease of bone / Diagnóstico e tratamento da doença de Paget óssea

Objective: To conduct a literature review on the diagnosis and management of Paget’s disease of bone. Materials and methods: This scientific statement was generated by a request from the Brazilian Medical Association (AMB) to the Brazilian Society of Endocrinology and Metabolism (SBEM) as part of its Clinical Practice Guidelines program. Articles were identified by searching in PubMed and Cochrane databases as well as abstracts presented at the Endocrine Society, Brazilian Society for Endocrinology Annual Meetings and the American Society for Bone and Mineral Research Annual Meeting during the last 5 years. Grading quality of evidence and strength of recommendation were adapted from the first report of the Oxford Centre for Evidence-based Medicine. All grades of recommendation, including “D”, are based on scientific evidence. The differences between A, B, C and D, are due exclusively to the methods employed in generating evidence. Conclusion: We present a scientific statement on Paget’s disease of bone providing the level of evidence and the degree of recommendation regarding causes, clinical presentation as well as surgical and medical treatment. Arq Bras Endocrinol Metab. 2014;58(6):587-99 .

Objetivo: Conduzir uma atualização das últimas evidências científicas a respeito da apresentação, diagnóstico e manejo clínico da doença de Paget óssea. Materiais e métodos: Este documento foi concebido pelo Departamento de Metabolismo Ósseo da Sociedade Brasileira de Endocrinologia e Metabologia (SBEM) a partir daquele oriundo do Programa de Diretrizes da Associação Médica Brasileira (AMB). Realizamos uma revisão dos artigos mais relevantes obtidos nos bancos de dados PubMed e Cochrane, além de abstracts apresentados nos encontros anuais da Endocrine Society, Sociedade Brasileira de Endocrinologia e da American Society for Bone and Mineral Research dos últimos cinco anos e classificamos as evidências em níveis de recomendações de acordo com a força científica por tipo de estudo, adaptando o primeiro relato do “Oxford Centre for Evidence-based Medicine”. Todos os graus de recomendação, incluindo-se o “D”, foram baseados em evidência científica, sendo as diferenças entre o A, B, C e D devidas exclusivamente ao desenho empregado na geração da evidência. Conclusão: Apresentamos uma atualização científica a respeito da doença de Paget óssea, classificando e graduando em níveis de recomendações as principais evidências científicas sobre as suas causas, as variadas formas de apresentação, seu diagnóstico e tratamento. .

Paget's disease of bone: analysis of 134 cases from an island in Southern Brazil: another cluster of Paget's disease of bone in South America.

Paget's disease of bone (PDB) exhibits a marked geographic variation. In Brazil, the prevalence of PDB is unknown and only a few clinical data are available. The aim is to determine clinical, laboratory, imaging and response to treatment data in a large PDB case series in the city of Florianopolis, Brazil. We have performed a retrospective study based on charts reviews of all patients with PDB followed at the University Hospital of the Federal University of Santa Catarina and at five different private rheumatology outpatient offices in Florianopolis, between 1995 and 2009. One hundred and thirty-four patients with PDB were identified. Mean age at diagnosis was 63.2 ± 10.5 years, 67.2% were women, and 91.1% were Caucasian. Positive family history was reported in only 8.2%. Polyostotic disease was found in 75.0% of the cases, bone pain in 77.9%, and bone deformities in 15.9%. Higher levels of AP were significantly associated with polyostotic disease and skull involvement. Pelvic bones were the most frequently affected (53.7%). Complications included deafness in 8.2%, bone fractures in 3.0%, hydrocephalus in 2.2%, and cauda equina syndrome in 0.7% of the cases. Treatment with zoledronic acid achieved the best response with only 2.9% failing to respond adequately. According to literature data, PDB in South America seems to be characterized by an overall low prevalence, but with localized clusters with higher prevalence. The authors have described a cluster of PDB in Florianopolis, in Southern Brazil. Further properly designed studies are necessary to clarify the PDB epidemiology in South America.

Epidemiology of Paget's disease of bone in the city of Recife, Brazil.

Epidemiological studies of Paget's disease of bone (PDB) have shown a remarkable geographical variation in the prevalence rates of the disease. In South America, the frequency is considered to be low, despite scant epidemiological data on this continent regarding the disease. The aim of this study was to evaluate the prevalence and incidence of PDB in one institution in the city of Recife, Northeast Brazil. All patients aged 45 years and over attending the Pernambuco Osteoporosis Centre at the Endocrine and Diabetes Department between January 2006 and December 2009 were assessed. Period prevalence and incidence density were calculated for each year for males and females separately, with confidence intervals and hypothesis test for difference between proportions. A total of 7,752 patients were assessed of which 53 presented with PDB. The total prevalence was 6.8 per 1,000 patients, and the incidence density for PDB was 50.3 per 10,000 person-years. Both prevalence and incidence increased during the period. Our data show that prevalence and incidence rates of PDB in Recife are comparable to those from Southern Europe. To our knowledge, this is the first epidemiological study of PDB in South America.

Paget's disease of bone and its complications due to delay in diagnosis.

Paget's disease of bone is an osteometabolic focal disease characterized by defects in bone remodeling. It may be asymptomatic, but often is associated with bone pain, deformity, pathological fracture, secondary osteoarthrosis and deafness. The diagnosis is usually made by radiological and laboratory findings. This report describes a male patient, 74 years old, native of Amazon, without European ancestry, with polyostotic Paget's disease, with clinical, radiological and laboratory diagnosis after 30 years of disease. The authors emphasize several complications of Paget's disease due to delayed diagnosis and the rarity of the disease in this population group.

Doença de Paget com acometimento sacral: relato de caso / Paget's disease with sacral involvement: a case report

Os autores relatam o caso de um paciente do sexo masculino, 71 anos de idade, com diagnóstico de doença de Paget óssea sacral. Foi realizado estudo com radiografia, cintilografia, tomografia computadorizada e ressonância magnética, e o diagnóstico foi confirmado por análise histopatológica. O paciente evoluiu com boa resposta ao uso de ibandronato 150 mg, mensalmente, com redução significativa dos marcadores bioquímicos da doença.

The authors report a case of a 71-year-old male patient diagnosed with Paget's disease of sacrum. Imaging study was performed with radiography, scintigraphy, computed tomography and magnetic resonance imaging, and the diagnosis was confirmed by biopsy. The patient progressed with a good response to monthly treatment with ibandronate 150 mg, presenting a significant reduction in biochemical markers of disease.

[Monostotic patellar Paget's disease]. / Enfermedad de Paget monostótica rotuliana.

Paget's disease or osteitis deformans is a bone metabolic disease characterized by an increased bone resorption followed by an increased but irregular bone formation. This results in a weakened, deformed bone and an increase bone mass with collagen fibers forming an irregular and pathologic mosaic instead of the parallel symmetry that characterizes healthy bone. It is considered as a non-neoplastic disorder that mimics a bone tumor. Its origin is still uncertain; however, it has been postulated that genetic or environmental factors are involved in its etiology. It rarely occurs in young patients and its prevalence increases with age. It usually affects both genders with a slight predominance of males. It may be symptomatic or asymptomatic depending on the bones involved, with the most common clinical manifestation being pain of the affected bone. Lesion distribution varies from monostotic involvement (25%) to generalized disease (75%). The most frequent complication is a fracture in a pathological area, while the most serious one is sarcomatous degeneration (approximately 1%; higher in the generalized form). The diagnosis is usually radiologic, by means of plain X-rays and biochemical markers such as alkaline phosphatase, among others, which is elevated in 85% of patients. Treatment of this disease consists of bisphosphonates, which have a proven efficacy and high remission rates. They are indicated in patients with clinical manifestations and in asymptomatic patients with evidence of disease activity. We report the case of a patient with Paget's disease in an infrequent location as is the patella.

Characteristics of Paget's disease of bone in the city of Recife, Brazil.

In South America the incidence of Paget's disease of bone (PDB) is low and more than half of the cases published in the last 30 years come from Brazil and Argentina. The aim of this study was to describe the clinical and epidemiological characteristics of PDB in two institutions in Pernambuco. PDB patients in Recife, Brazil were studied retrospectively. A total of 108 cases were diagnosed from 1984 to 2005. Over 90% of the patients were of European descent. The average age was 66.2 years and 49.1% were male. The polyostotic form was the more common. Pain and bone deformity were significantly more frequent in this group and in the total group. Clear eyes were observed in 22.2% of the patients and 23.1% had a family history record of PDB. The most frequently affected bones were the pelvis, lumbar vertebrae, femurs, and skull. Zoledronate was the most effective drug in reducing the alkaline phosphatase. Our data show that PDB in Recife occurs predominantly among those of European descents, usually in the polyostotic presentation, thus providing evidence of genetic and historical factors in the prevalence of PDB in that region.

[Development of primary hyperparathyroidism in the follow-up of a patient with Paget's disease]. / Desarrollo de hiperparatiroidismo primario en el seguimiento de un paciente con enfermedad de Paget.

According to the medical literature, the association of primary hyperparathyroidism and Paget's disease varies from 2.2 to 6%. Up to the year 2006, a total of 73 cases had been described, where both diagnoses occurred simultaneously. However, no manifestation of primary hyperparathyroidism during the follow-up of Paget's disease has been reported in the revised literature. We report the case of a well-controlled patient, who developed primary hyperparathyroidism during the 10-year follow-up of Paget's disease. A 68-year-old male patient with active polyostotic Paget's disease was successfully treated with bisphosphonates for ten years. During follow-up, increased levels of calcemia, ionic calcium, alkaline phosphatase, bone alkaline phosphatase and intact parathyroid hormone values were registered. The patient was diagnosed with primary hyperparathyroidism. As a result of his unstable general health condition, surgery was postponed and intravenous zoledronic acid was prescribed, with a favorable outcome.

Desarrollo de hiperparatiroidismo primario en el seguimiento de un paciente con enfermedad de Paget / Development of primary hyperparathyroidism in the follow-up of a patient with Paget´s disease

La asociación de hiperparatiroidismo primario y enfermedad de Paget varía entre 2.2 y 6%. Hasta el año 2006 se habían descripto 73 casos con simultaneidad de ambos diagnósticos. Se presenta el caso de un paciente varón de 68 años con un Paget poliostótico activo medicado durante 10 años con bisfosfonatos, con buena evolución. A los 10 años de seguimiento se observan elevados niveles de calcemia, calcio iónico, la fosfatasa alcalina (FAL), su isoenzima ósea (FAIO), e intactos los valores de parathormona (PTHi). Se establece el diagnóstico de hiperparatiroidismo primario. Dado el inestable estado general se decide postergar la cirugía y tratarlo con zoledronato IV con buena respuesta.

According to the medical literature, the association of primary hyperparathyroidism and Paget's disease varies from 2.2 to 6%. Up to the year 2006, a total of 73 cases had been described, where both diagnoses occurred simultaneously. However, no manifestation of primary hyperparathyroidism during the follow- up of Paget's disease has been reported in the revised literature. We report the case of a well-controlled patient, who developed primary hyperparathyroidism during the 10-year follow-up of Paget's disease. A 68-yearold male patient with active polyostotic Paget's disease was successfully treated with bisphosphonates for ten years. During follow-up, increased levels of calcemia, ionic calcium, alkaline phosphatase, bone alkaline phosphatase and intact parathyroid hormone values were registered. The patient was diagnosed with primary hyperparathyroidism. As a result of his unstable general health condition, surgery was postponed and intravenous zoledronic acid was prescribed, with a favorable outcome.

Doença de Paget - enfoque atualizado com ênfase na elaboração de um correto diagnóstico / Paget's disese - updated focus emphasizing the development of a correct dignosis

Objetivo: a Doença de Paget, uma desordem focal da remodelação óssea. é caracterizada por uma excessiva reabsorção seguida de intensa neoformação do osso afetado, apresentando um importante componente genético com evidências de heterogeneidade. De achado geralmente ocasional, típica da idade adulta e de tratamento variável. a doença é capaz de afetar vários ossos, simultaneamente. e provocar profundas deformidades, necessitando, portanto, de novos estudos com o intuito de esclarecer o seu diagnóstico, uma vez que os sintomas nem sempre são elucidativos. Assim, o presente trabalhC? objetivou realizar um estudo de atualização da etiologia e dos aspectos clínicos da doença. com afinalidade defomecerdados que subsidiem a escolha dos métodos adequados para a realização de um correto diagnóstico. Método: procedeu-se a uma extensa busca na Intemet e na base de dados intemacionais Medline e LlLACS, abrangendo os periódicos científicos publicados no período 2000 a 2008. dos quais foram selecionados, para revisão bibliográfica, aqueles mais importantes e de maior rigor científico acerca desta doença. Livros publicados no mesmo período também foram consultados. Conclusões: o diagnóstico, geralmente, encontra-se alicerçado nos aspectos clínicos, radiográficos, bioquímicos, genéticos e histológicos, sendo os métodos por imagem os de mais fácil obtenção, com ênfase para a radiografia convencional, em razão do baixo custo e de sua especificidade, e para a cintilografia de corpo inteiro como método de escolha para rastreamento de lesões suspeitas. devido a sI/a alta sensibilidade.

Objective: The Paget's disease, a disorder of focal bone remodeling is characterized by an excessive absorption followed by intense neoformation of the affected bone, presenting an important genetic component wifh evidence of heterogeneity. Usually found occasionally, typical of adulthood and variable treatment, the disease is able to affect several bones, simultaneously, causing deep deformities, requiring, therefore, further studies in order to clarify the diagnosis, since the symptoms are not always clear Therefore, this study aimed to update the etiology and clinical aspects of the disease, in order to provide data that subsidize the choice of methods for the establishment of a correct diagnosis. Method: There was research on the World Wide Web and the online data bases Medline and LILACS, coverillg journi published from 2000 to 2008, of which were selected for review the ones of most importance arigorous about this disease. Books published in the same period were also consulted. Conclusions: The diagnosis, generally, is based on clinical, radiographic, biochemical, genetic and histological aspects and the methods of image are easier to obtain, with emphasis on conventional radiography because low cost and its specificity, and the whole body scintigraphy as a method of choice for tracking suspicious lesions, due to its high sensitivity.

Doença de Paget do osso / Paget’s bone disease

A doença de Paget óssea é uma doença osteometabólica focal comforte componente genético caracterizada por aumento da remodelaçãoóssea que afeta um ou mais sítios do esqueleto. A doença de Pagetóssea pode ser assintomática, porém, freqüentemente, se associa ador óssea, deformidades, fratura patológica, osteoartrite secundária esurdez. A prevalência parece estar diminuindo em algumas regiões domundo. O diagnóstico é realizado habitualmente por meio de achadosradiológicos e laboratoriais. No tratamento medicamentoso, é feitauma terapia padrão utilizando bifosfonatos de última geração, como,por exemplo, o ácido zoledrônico; porém, na avaliação rotineira destespacientes, freqüentemente, não são contemplados itens de qualidadede vida e, além disso, não há reversão de complicações.

Paget's disease of bone.

Paget's disease of bone is a focal disorder of bone remodeling accompanied initially by an increase in bone resorption, followed by a disorganized and excessive formation of bone, leading to pain, fractures and deformities. It exhibits a marked geographical variation in its prevalence. In Brazil it predominantly affects persons of European descent. The majority of the reported cases of the disease in Brazil are from Recife, owing to its peculiar mixed European colonization over approximately four centuries. The etiology is complex and involves both genetic and environmental factors. The disease is often asymptomatic and diagnosis is usually based on biochemical markers of bone turnover, radionuclide bone scan and radiological examination. Bisphosphonates, in particular zoledronic acid, are regarded as the treatment of choice for Paget's disease of bone.

Paget's disease of bone / Doença de Paget óssea

Paget's disease of bone is a focal disorder of bone remodeling accompanied initially by an increase in bone resorption, followed by a disorganized and excessive formation of bone, leading to pain, fractures and deformities. It exhibits a marked geographical variation in its prevalence. In Brazil it predominantly affects persons of European descent. The majority of the reported cases of the disease in Brazil are from Recife, owing to its peculiar mixed European colonization over approximately four centuries. The etiology is complex and involves both genetic and environmental factors. The disease is often asymptomatic and diagnosis is usually based on biochemical markers of bone turnover, radionuclide bone scan and radiological examination. Bisphosphonates, in particular zoledronic acid, are regarded as the treatment of choice for Paget's disease of bone.

Doença de Paget óssea é uma desordem focal da remodelação óssea, inicialmente acompanhada de um aumento da reabsorção óssea, seguida de desorganizada e excessiva formação óssea, levando a dor, deformidades e fraturas. Exibe uma variável distribuição geográfica em sua prevalência. No Brasil acomete predominantemente pacientes de descendência européia. Recife, devido à sua peculiar colonização mista européia por cerca de 4 séculos, tem a maioria dos casos relatados no Brasil. A etiologia é complexa e envolve fatores ambientais e genéticos. A doença é freqüentemente assintomática e o diagnóstico é feito usualmente através dos marcadores bioquímicos do turnover ósseo associado a cintilografia óssea e dos sinais típicos do exame radiológico. Os bisfosfonatos representam o tratamento de escolha na doença de Paget óssea, particularmente o ácido zolidrônico.

Paget disease of bone in Colombia and Latin America.

BACKGROUND: Paget disease of bone has an unknown etiology, having complex pathogenesis leading to increased bone resorption in the first phase and an excess of bone formation with more advanced disease. The disease has been associated to white ancestry in Europe and other countries, being less common in people without European origin. OBJECTIVES: The objectives of this study were to describe the Colombian cases of Paget disease and search the published literature for more Latin American cases and their characteristics. METHODS: Electronic databases were searched up to August 2004: MEDLINE, PUBMED, BIREME LILAC, and MEDCARIB, evaluating the entire bibliography regarding reports of Paget disease in Latin America during the last 30 years. Additionally, we searched the medical databases of local reference centers to describe new cases from Colombia. RESULTS: We found 14 cases of Paget disease from Colombia; 12 of them were previously reported elsewhere and 2 additional cases were found in the medical database of a local reference center. We describe the main clinic characteristics, including age, symptoms, type and stage of involved bone (monostotic or polyostotic), and treatment, which generally are similar to cases from Europe or the United States. The literature search showed that a total of 1149 cases of Paget disease have been previously published from Latin America in the last 30 years, more than half of them coming from Argentina and Brazil with predominant white ancestry. CONCLUSIONS: We emphasize the presence of white European origin or ancestry in the great majority of reported cases of Paget disease in Latin America and Colombia. Studies of factors that influence the etiology in cases of non-European ancestry would be of interest.

Quão acurado é o diagnóstico em Doença de Paget? / How sensitive is our diagnosis in Paget's Disease?

Este estudo visaa estabelecer quão acurado está o diagnóstico clínico na Doença de Paget (DP), através da análise comparativa retrospectiva dos dados de todos os pacientes atendidos de janeiro de 1981 a janeiro de 2004, com este diagnóstico inicial, no Serviço de Reumatologia do Hospital de Clínicas da Universidade Federal do Paraná - HC/UFPR. De cada paciente obteve-se: idade, sexo, duração das manifestações clínicas, sinais laboratoriais e radiológicos, complicações, incidência familiar e tratamento. Os pacientes foram, então, divididos em portadores ou não de DP. Um total de 15 pacientes foram avaliados: cinco com DP e 10 com outras doenças osteomusculares, como Displasia Fibrosa, Osteomielite Crônica, Artrose e Carcinossarcoma. A idade dos pacientes com DP foi maior (59 anos versus 34.1 anos daqueles do outro grupo). Dor e doença em coluna lombar foram, respectivamente, a principal manifestação clínica e alteração óssea em ambos os grupos. Apenas um paciente possuía história familiar de DP. Artrose e fraturas patológicas foram complicações na DP. Todos os pacientes foram inicialmente tratados com AINEs e, após o diagnóstico correto, os pacientes com DP receberam bisfosfonatos e calcitonina. Muitos pacientes referidos ao serviço não preenchiam as características clínicas, laboratoriais e radiológicas de DP. Estratégias para disseminar o conhecimento médico desta importante doença reduzirá manejo terapêutico errôneo e o próprio stress gerado ao paciente