i n f o a r t i c l e Historique de l'article : Accepté le 3 février 2010 Disponible sur Internet... more i n f o a r t i c l e Historique de l'article : Accepté le 3 février 2010 Disponible sur Internet le 15 mai 2010 Mots clés : Profil lipoprotéique Inflammation Syndrome de Sjögren r é s u m é Objectifs. -Étudier le profil lipoprotéique de patients atteints du syndrome de Sjögren primaire (SSp) et son association avec des paramètres biologiques, y compris les marqueurs de l'inflammation. Méthodes. -Nous avons réalisé une étude transversale chez des patients atteints de SSp et de témoins sains. Nous avons analysé le profil lipoprotéique de 73 patients atteints de SSp et les avons comparé à celui de 65 témoins sains. Nous avons évalué les associations éventuelles entre une dyslipidémie chez les patients atteints de SSp et les paramètres biologiques, y compris : hypergammaglobulinémie, autoanticorps (anticorps antinucléaires [ANA], facteur rhumatoïde [FR], anti-Ro, anti-La) et réactifs de la phase aiguë (protéine C-réactive [CRP] et vitesse de sédimentation [VS]).
Endereço para correspondência: Fabrício Souza Neves. Av. Rio Branco 633. CEP: 88015-203. Florianó... more Endereço para correspondência: Fabrício Souza Neves. Av. Rio Branco 633. CEP: 88015-203. Florianópolis, SC, Brasil. Tel: 55 (48) 3025-7222.
O angioedema adquirido é causado por diferentes medicamentos e doenças linfoproliferativas, e tem... more O angioedema adquirido é causado por diferentes medicamentos e doenças linfoproliferativas, e tem sido raramente relacionado com a presença de doenças autoimunes. Descrevemos aqui uma paciente de 47 anos com lúpus eritematoso sistêmico (LES) com envolvimento cutâneo importante que desenvolveu angioedema recorrente localizado em face incluindo lábios e pálpebras, membros superiores e tórax, não acompanhado de urticária e com dosagem do inibidor de C1 esterase reduzida. A utilização de antimaláricos, glicocorticoides e pulsoterapia com metilprednisolona associada ao uso de azatioprina não determinou melhora. A paciente utilizou também danazol sem sucesso, e apresentou resposta clínica somente após ter sido submetida a múltiplas sessões de plasmaferese, ocorrendo inclusive resolução de extenso angioedema na mucosa do trato gastrointestinal.
RBRE-135; No. of Pages 7 r e v b r a s r e u m a t o l . 2 0 1 4;x x x(x x):xxx-xxx REVISTA BRASI... more RBRE-135; No. of Pages 7 r e v b r a s r e u m a t o l . 2 0 1 4;x x x(x x):xxx-xxx REVISTA BRASILEIRA DE REUMATOLOGIA w w w . r e u m a t o l o g i a . c o m . b r
Revista Brasileira de Reumatologia (English Edition), 2014
r e v b r a s r e u m a t o l . 2 0 1 4;5 4(6):424-430 REVISTA BRASILEIRA DE REUMATOLOGIA w w w .... more r e v b r a s r e u m a t o l . 2 0 1 4;5 4(6):424-430 REVISTA BRASILEIRA DE REUMATOLOGIA w w w . r e u m a t o l o g i a . c o m . b r r e v b r a s r e u m a t o l . 2 0 1 4;5 4(6):424-430 425 : r e v b r a s r e u m a t o l . 2 0 1 4;5 4(6):424-430
Revista Brasileira de Reumatologia (English Edition), 2014
Palavras-chave: Espondiloartrites Tratamento Drogas modifi cadoras de doença Metotrexato Sulfasal... more Palavras-chave: Espondiloartrites Tratamento Drogas modifi cadoras de doença Metotrexato Sulfasalazina r e s u m o Introdução: Poucos estudos avaliaram o perfi l do uso de drogas modifi cadoras de doença (DMD) em pacientes brasileiros com diagnóstico de espondiloartrite (EpA). Métodos: Um protocolo comum de investigação foi prospectivamente aplicado em 1505 pacientes classifi cados como EpA pelos critérios do Grupo Europeu de Estudo das Espondiloartrites (ESSG), acompanhados em 29 centros de referência em Reumatologia no Brasil. Variáveis clínicas e demográfi cas foram obtidas e avaliadas, analisando-se suas correlações com o uso das DMD metotrexato (MTX) e sulfasalazina (SSZ). Resultados: Pelo menos uma DMD foi utilizada por 73,6% dos pacientes, sendo MTX por 29,2% e SSZ por 21,7%, enquanto 22,7% utilizaram ambas as drogas. O uso do MTX foi signifi cativamente associado ao acometimento periférico, e a SSZ foi associada ao comprometimento axial, sendo que as duas drogas foram mais utilizadas, isoladas ou combinadas, no comprometimento misto (p < 0,001). O uso de uma DMD esteve signifi cativamente associado à etnia branca (MTX; p = 0,014), lombalgia infl amatória (SSZ; p = 0,002), dor em nádegas (SSZ; p = 0,030), cervicalgia (MTX; p = 0,042), artrite de membros inferiores (MTX; p < 0,001), artrite de membros superiores (MTX; p < 0,001), entesite (p = 0,007), dactilite (MTX; p < 0,001), doença infl amatória intestinal (SSZ; p < 0,001) e acometimento ungueal (MTX; p < 0,001).
Page 1. 346 Rev Bras Reumatol 2009;49(4):346-61 ARTIGO ORIGINAL Recomendações nacionais baseadas ... more Page 1. 346 Rev Bras Reumatol 2009;49(4):346-61 ARTIGO ORIGINAL Recomendações nacionais baseadas em evidências científicas e opiniões dos especialistas sobre o uso do metotrexato nas doenças reumáticas, especialmente na artrite reumatoide. ...
RBRE-124; No. of Pages 29 r e v b r a s r e u m a t o l . 2 0 1 4;x x x(x x):xxx-xxx REVISTA BRAS... more RBRE-124; No. of Pages 29 r e v b r a s r e u m a t o l . 2 0 1 4;x x x(x x):xxx-xxx REVISTA BRASILEIRA DE REUMATOLOGIA w w w . r e u m a t o l o g i a . c o m . b r Available online xxx a b s t r a c t The treatment of autoimmune rheumatic diseases has gradually improved over the last half century, which has been expanded with the contribution of biological therapies or immunobiopharmaceuticals. However, we must be alert to the possibilities of undesirable effects from the use of this class of medications. The Brazilian Society of Rheumatology (Sociedade Brasileira de Reumatologia) produced a document based on a comprehensive literature review on the safety aspects of this class of drugs, specifically with regard to the RBRE-124; No. of Pages 29 2 r e v b r a s r e u m a t o l . 2 0 1 4;x x x(x x):xxx-xxx
Tumoral necrosis factor alpha blockers are very efficient in the treatment of many inflammatory s... more Tumoral necrosis factor alpha blockers are very efficient in the treatment of many inflammatory systemic diseases, including rheumatoid arthritis and psoriasis. However, a paradoxical arouse of psoriasiform lesions may occur in a few patients taking anti-TNFa. The etiology of this rare side effect is still a mystery, and its treatment may be difficult. The authors report the resolution of adalimumab-induced psoriasis in a woman with rheumatoid arthritis after the use of high vitamin D 3 doses for the treatment of vitamin D deficiency. This is the first report of resolution of anti-TNFa-induced psoriasiform lesions by high doses of vitamin D 3 in a patient with rheumatoid arthritis and vitamin D deficiency. This case raises interesting questions on the role of vitamin D deficiency in the pathogenesis of this side effect and on the possible usefulness of high-dose vitamin D 3 in its treatment.
Zoledronic acid is effective for osteoporosis at a single annual intravenous dose. It usually cau... more Zoledronic acid is effective for osteoporosis at a single annual intravenous dose. It usually causes few adverse effects; the most common are related to acute phase reactions. We reported the case of a 64-year-old woman who presented flare-up of hand osteoarthritis after zoledronic acid infusions. Despite the fact that arthralgia is a common side effect of intravenous bisphosphonates, development of inflammatory signs in osteoarthritic joints is a rare event. We hypothesized that this side effect is caused by a release of cytokines secondary to activation of gamma-delta T lymphocytes.
Hypertrophic osteoarthropathy (HOA) is characterized by periostitis of tubular bones, thickened s... more Hypertrophic osteoarthropathy (HOA) is characterized by periostitis of tubular bones, thickened skin, and digital clubbing. Its pathogenesis is unknown but an inflammatory factor and increased bone remodeling have been implicated. It is a very rare disease, usually diagnosed late with few therapeutic options. Bone and joint pains are secondary to periostitis and are usually difficult to control. Tumor necrosis factor-alpha is a cytokine that induces other inflammatory cytokine production, has an osteoclastogenic effect in different rheumatic diseases and probably also has an important role in periostitis and the systemic inflammatory manifestations in HOA. We describe the case of a patient with the primary form of HOA, who had refractory bone pain and arthritis that responded partially to infliximab treatment.
Background: Many questions remain unanswered about premature atherosclerosis in rheumatoid arthri... more Background: Many questions remain unanswered about premature atherosclerosis in rheumatoid arthritis (RA). Besides inflammation, some studies have suggested the role of autoantibodies on its pathogenesis. Objective: The aim of this study was to investigate the presence of antibodies against phospholipids, beta2-glycoprotein1 (beta2-gp1), lipoprotein lipase, and heat shock proteins (Hsp) in RA patients and to evaluate their possible association with subclinical carotid atherosclerosis. Methods: Seventy-one RA patients and 53 age-and sex-matched controls were selected to perform anticardiolipin antibodies (aCL) (IgG and IgM), anti-beta2-gp1 (IgG, IgM, and IgA), anti-lipoprotein lipase (anti-LPL), anti-Hsp 60, and anti-Hsp 65 by ELISA tests. Intima-medial thickness (IMT) of common carotid and presence of plaques were assessed by high-resolution B-mode ultrasonography. Exclusion criteria were smoking, diabetes, and arterial hypertension. Lipoproteins, erythrocyte sedimentation rate (ESR), C-reactive protein (CRP), and fibrinogen levels, as well as health assessment questionnaire (HAQ) and disease activity score (DAS) 28 were also evaluated. Results: Age (48.93 AE 12.31 vs. 45.37 AE 9.37 years; p ¼ 0.20) and body mass index (BMI) ( p ¼ 0.69) were similar in RA and controls, as well as female gender ( p ¼ 0.56). The mean IMT was similar between RA and controls (0.721 AE 0.16 vs. 0.667 AE 0.14 mm, p ¼ 0.07) but the frequency of plaques was higher in RA (14.1% vs. 1.9%; p ¼ 0.02). In RA patients, IMT measurements did not differ according to the presence or absence of these antibodies: IgG aCL 0.62 AE 0.64 vs. 0.72 AE 0.17 mm, p ¼ 0.24), IgM aCL (0.65 AE 0.79 vs. 0.73 AE 0.17 mm, p ¼ 0.33), anti-Hsp 60 (0.78 AE 0.20 vs. 0.71 AE 0.16 mm, p ¼ 0.27), anti-Hsp 65 (0.73 AE 0.16 vs. 0.72 AE 0.17 mm, p ¼ 0.77), IgG anti-beta2-gp1 (0.73 AE 0.16 vs. 0.71 AE 0.17 mm, p ¼ 0.72), and anti-CCP (0.71 AE 0.16 vs. 0.76 AE 0.20 mm, p ¼ 0.36).
To investigate the lipoprotein profile of patients with primary Sjögren's syndrome (pSS) and... more To investigate the lipoprotein profile of patients with primary Sjögren's syndrome (pSS) and its association with laboratory tests, including markers of inflammation.
Paget's disease of bone (PDB) exhibits a marked geographic variation. In Brazil, the prevalence o... more 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.
i n f o a r t i c l e Historique de l'article : Accepté le 3 février 2010 Disponible sur Internet... more i n f o a r t i c l e Historique de l'article : Accepté le 3 février 2010 Disponible sur Internet le 15 mai 2010 Mots clés : Profil lipoprotéique Inflammation Syndrome de Sjögren r é s u m é Objectifs. -Étudier le profil lipoprotéique de patients atteints du syndrome de Sjögren primaire (SSp) et son association avec des paramètres biologiques, y compris les marqueurs de l'inflammation. Méthodes. -Nous avons réalisé une étude transversale chez des patients atteints de SSp et de témoins sains. Nous avons analysé le profil lipoprotéique de 73 patients atteints de SSp et les avons comparé à celui de 65 témoins sains. Nous avons évalué les associations éventuelles entre une dyslipidémie chez les patients atteints de SSp et les paramètres biologiques, y compris : hypergammaglobulinémie, autoanticorps (anticorps antinucléaires [ANA], facteur rhumatoïde [FR], anti-Ro, anti-La) et réactifs de la phase aiguë (protéine C-réactive [CRP] et vitesse de sédimentation [VS]).
Endereço para correspondência: Fabrício Souza Neves. Av. Rio Branco 633. CEP: 88015-203. Florianó... more Endereço para correspondência: Fabrício Souza Neves. Av. Rio Branco 633. CEP: 88015-203. Florianópolis, SC, Brasil. Tel: 55 (48) 3025-7222.
O angioedema adquirido é causado por diferentes medicamentos e doenças linfoproliferativas, e tem... more O angioedema adquirido é causado por diferentes medicamentos e doenças linfoproliferativas, e tem sido raramente relacionado com a presença de doenças autoimunes. Descrevemos aqui uma paciente de 47 anos com lúpus eritematoso sistêmico (LES) com envolvimento cutâneo importante que desenvolveu angioedema recorrente localizado em face incluindo lábios e pálpebras, membros superiores e tórax, não acompanhado de urticária e com dosagem do inibidor de C1 esterase reduzida. A utilização de antimaláricos, glicocorticoides e pulsoterapia com metilprednisolona associada ao uso de azatioprina não determinou melhora. A paciente utilizou também danazol sem sucesso, e apresentou resposta clínica somente após ter sido submetida a múltiplas sessões de plasmaferese, ocorrendo inclusive resolução de extenso angioedema na mucosa do trato gastrointestinal.
RBRE-135; No. of Pages 7 r e v b r a s r e u m a t o l . 2 0 1 4;x x x(x x):xxx-xxx REVISTA BRASI... more RBRE-135; No. of Pages 7 r e v b r a s r e u m a t o l . 2 0 1 4;x x x(x x):xxx-xxx REVISTA BRASILEIRA DE REUMATOLOGIA w w w . r e u m a t o l o g i a . c o m . b r
Revista Brasileira de Reumatologia (English Edition), 2014
r e v b r a s r e u m a t o l . 2 0 1 4;5 4(6):424-430 REVISTA BRASILEIRA DE REUMATOLOGIA w w w .... more r e v b r a s r e u m a t o l . 2 0 1 4;5 4(6):424-430 REVISTA BRASILEIRA DE REUMATOLOGIA w w w . r e u m a t o l o g i a . c o m . b r r e v b r a s r e u m a t o l . 2 0 1 4;5 4(6):424-430 425 : r e v b r a s r e u m a t o l . 2 0 1 4;5 4(6):424-430
Revista Brasileira de Reumatologia (English Edition), 2014
Palavras-chave: Espondiloartrites Tratamento Drogas modifi cadoras de doença Metotrexato Sulfasal... more Palavras-chave: Espondiloartrites Tratamento Drogas modifi cadoras de doença Metotrexato Sulfasalazina r e s u m o Introdução: Poucos estudos avaliaram o perfi l do uso de drogas modifi cadoras de doença (DMD) em pacientes brasileiros com diagnóstico de espondiloartrite (EpA). Métodos: Um protocolo comum de investigação foi prospectivamente aplicado em 1505 pacientes classifi cados como EpA pelos critérios do Grupo Europeu de Estudo das Espondiloartrites (ESSG), acompanhados em 29 centros de referência em Reumatologia no Brasil. Variáveis clínicas e demográfi cas foram obtidas e avaliadas, analisando-se suas correlações com o uso das DMD metotrexato (MTX) e sulfasalazina (SSZ). Resultados: Pelo menos uma DMD foi utilizada por 73,6% dos pacientes, sendo MTX por 29,2% e SSZ por 21,7%, enquanto 22,7% utilizaram ambas as drogas. O uso do MTX foi signifi cativamente associado ao acometimento periférico, e a SSZ foi associada ao comprometimento axial, sendo que as duas drogas foram mais utilizadas, isoladas ou combinadas, no comprometimento misto (p < 0,001). O uso de uma DMD esteve signifi cativamente associado à etnia branca (MTX; p = 0,014), lombalgia infl amatória (SSZ; p = 0,002), dor em nádegas (SSZ; p = 0,030), cervicalgia (MTX; p = 0,042), artrite de membros inferiores (MTX; p < 0,001), artrite de membros superiores (MTX; p < 0,001), entesite (p = 0,007), dactilite (MTX; p < 0,001), doença infl amatória intestinal (SSZ; p < 0,001) e acometimento ungueal (MTX; p < 0,001).
Page 1. 346 Rev Bras Reumatol 2009;49(4):346-61 ARTIGO ORIGINAL Recomendações nacionais baseadas ... more Page 1. 346 Rev Bras Reumatol 2009;49(4):346-61 ARTIGO ORIGINAL Recomendações nacionais baseadas em evidências científicas e opiniões dos especialistas sobre o uso do metotrexato nas doenças reumáticas, especialmente na artrite reumatoide. ...
RBRE-124; No. of Pages 29 r e v b r a s r e u m a t o l . 2 0 1 4;x x x(x x):xxx-xxx REVISTA BRAS... more RBRE-124; No. of Pages 29 r e v b r a s r e u m a t o l . 2 0 1 4;x x x(x x):xxx-xxx REVISTA BRASILEIRA DE REUMATOLOGIA w w w . r e u m a t o l o g i a . c o m . b r Available online xxx a b s t r a c t The treatment of autoimmune rheumatic diseases has gradually improved over the last half century, which has been expanded with the contribution of biological therapies or immunobiopharmaceuticals. However, we must be alert to the possibilities of undesirable effects from the use of this class of medications. The Brazilian Society of Rheumatology (Sociedade Brasileira de Reumatologia) produced a document based on a comprehensive literature review on the safety aspects of this class of drugs, specifically with regard to the RBRE-124; No. of Pages 29 2 r e v b r a s r e u m a t o l . 2 0 1 4;x x x(x x):xxx-xxx
Tumoral necrosis factor alpha blockers are very efficient in the treatment of many inflammatory s... more Tumoral necrosis factor alpha blockers are very efficient in the treatment of many inflammatory systemic diseases, including rheumatoid arthritis and psoriasis. However, a paradoxical arouse of psoriasiform lesions may occur in a few patients taking anti-TNFa. The etiology of this rare side effect is still a mystery, and its treatment may be difficult. The authors report the resolution of adalimumab-induced psoriasis in a woman with rheumatoid arthritis after the use of high vitamin D 3 doses for the treatment of vitamin D deficiency. This is the first report of resolution of anti-TNFa-induced psoriasiform lesions by high doses of vitamin D 3 in a patient with rheumatoid arthritis and vitamin D deficiency. This case raises interesting questions on the role of vitamin D deficiency in the pathogenesis of this side effect and on the possible usefulness of high-dose vitamin D 3 in its treatment.
Zoledronic acid is effective for osteoporosis at a single annual intravenous dose. It usually cau... more Zoledronic acid is effective for osteoporosis at a single annual intravenous dose. It usually causes few adverse effects; the most common are related to acute phase reactions. We reported the case of a 64-year-old woman who presented flare-up of hand osteoarthritis after zoledronic acid infusions. Despite the fact that arthralgia is a common side effect of intravenous bisphosphonates, development of inflammatory signs in osteoarthritic joints is a rare event. We hypothesized that this side effect is caused by a release of cytokines secondary to activation of gamma-delta T lymphocytes.
Hypertrophic osteoarthropathy (HOA) is characterized by periostitis of tubular bones, thickened s... more Hypertrophic osteoarthropathy (HOA) is characterized by periostitis of tubular bones, thickened skin, and digital clubbing. Its pathogenesis is unknown but an inflammatory factor and increased bone remodeling have been implicated. It is a very rare disease, usually diagnosed late with few therapeutic options. Bone and joint pains are secondary to periostitis and are usually difficult to control. Tumor necrosis factor-alpha is a cytokine that induces other inflammatory cytokine production, has an osteoclastogenic effect in different rheumatic diseases and probably also has an important role in periostitis and the systemic inflammatory manifestations in HOA. We describe the case of a patient with the primary form of HOA, who had refractory bone pain and arthritis that responded partially to infliximab treatment.
Background: Many questions remain unanswered about premature atherosclerosis in rheumatoid arthri... more Background: Many questions remain unanswered about premature atherosclerosis in rheumatoid arthritis (RA). Besides inflammation, some studies have suggested the role of autoantibodies on its pathogenesis. Objective: The aim of this study was to investigate the presence of antibodies against phospholipids, beta2-glycoprotein1 (beta2-gp1), lipoprotein lipase, and heat shock proteins (Hsp) in RA patients and to evaluate their possible association with subclinical carotid atherosclerosis. Methods: Seventy-one RA patients and 53 age-and sex-matched controls were selected to perform anticardiolipin antibodies (aCL) (IgG and IgM), anti-beta2-gp1 (IgG, IgM, and IgA), anti-lipoprotein lipase (anti-LPL), anti-Hsp 60, and anti-Hsp 65 by ELISA tests. Intima-medial thickness (IMT) of common carotid and presence of plaques were assessed by high-resolution B-mode ultrasonography. Exclusion criteria were smoking, diabetes, and arterial hypertension. Lipoproteins, erythrocyte sedimentation rate (ESR), C-reactive protein (CRP), and fibrinogen levels, as well as health assessment questionnaire (HAQ) and disease activity score (DAS) 28 were also evaluated. Results: Age (48.93 AE 12.31 vs. 45.37 AE 9.37 years; p ¼ 0.20) and body mass index (BMI) ( p ¼ 0.69) were similar in RA and controls, as well as female gender ( p ¼ 0.56). The mean IMT was similar between RA and controls (0.721 AE 0.16 vs. 0.667 AE 0.14 mm, p ¼ 0.07) but the frequency of plaques was higher in RA (14.1% vs. 1.9%; p ¼ 0.02). In RA patients, IMT measurements did not differ according to the presence or absence of these antibodies: IgG aCL 0.62 AE 0.64 vs. 0.72 AE 0.17 mm, p ¼ 0.24), IgM aCL (0.65 AE 0.79 vs. 0.73 AE 0.17 mm, p ¼ 0.33), anti-Hsp 60 (0.78 AE 0.20 vs. 0.71 AE 0.16 mm, p ¼ 0.27), anti-Hsp 65 (0.73 AE 0.16 vs. 0.72 AE 0.17 mm, p ¼ 0.77), IgG anti-beta2-gp1 (0.73 AE 0.16 vs. 0.71 AE 0.17 mm, p ¼ 0.72), and anti-CCP (0.71 AE 0.16 vs. 0.76 AE 0.20 mm, p ¼ 0.36).
To investigate the lipoprotein profile of patients with primary Sjögren's syndrome (pSS) and... more To investigate the lipoprotein profile of patients with primary Sjögren's syndrome (pSS) and its association with laboratory tests, including markers of inflammation.
Paget's disease of bone (PDB) exhibits a marked geographic variation. In Brazil, the prevalence o... more 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.
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