Incidence of Infectious Adverse Events in Patients With Rheumatoid Arthritis and Spondyloarthritis on Biologic Drugs-Data From the Brazilian Registry for Biologics Monitoring.

BACKGROUND: The safety profile of biologic drugs might present substantial regional differences. Since 2009, the Brazilian Society of Rheumatology has maintained BIOBADABRASIL (Brazilian Registry for Biologic Drugs), a registry for monitoring of biologic therapies in rheumatic diseases. OBJECTIVES: The aim of this study was to verify the incidence rate (IR) of serious infections in rheumatoid arthritis (RA) and spondyloarthritis (SpA) patients on biologic drugs. METHODS: BIOBADABRASIL prospectively included patients with rheumatic diseases who started the first biologic drug or a synthetic disease-modifying antirheumatic drug as a parallel control group. This study focuses on serious infectious adverse events (SIAEs) in RA and SpA patients on biologic drugs compared with controls, from January 2009 to June 2015. Time of exposure was set from initiation of the drug to the date of last administration or censorship. Serious infectious adverse events IR was calculated per 1000 patient/years with 95% confidence interval (CI). RESULTS: A total of 1698 patients (RA, 1121; SpA, 577) were included, 7119 patient/years. Serious infectious adverse events were more common among patients on tumor necrosis factor inhibitors (TNFi's) than controls (adjusted IR ratio, 2.96 [95% CI, 2.01-4.36]; p < 0.001). Subsequent TNFi was associated with a higher SIAEs incidence when compared with first TNFI (adjusted IR ratio, 1.55 [95% CI, 1.15-2.08]; p = 0.004). Serious infectious adverse events were associated with age and corticosteroids intake. Serious infectious adverse events were more frequent in the respiratory tract in all subgroups. CONCLUSIONS: In BIOBADABRASIL, biologic drugs, especially the subsequent TNFi, were associated with a higher risk of serious infections compared with synthetic DMARDs. Corticosteroid intake and age represented risk factors for SIAEs. Constant monitoring is required to follow the safety profile of drugs in the clinical setting of rheumatic conditions in Brazil.

Ultrastructural deposits appearing as "zebra bodies" in renal biopsy: Fabry disease?- comparative case reports.

BACKGROUND: Fabry Disease (FD) is a genetic disorder caused by alpha-galactosidase A deficiency. Certain drugs, such as hydroxychloroquine, can produce renal deposits that mimic morphological findings seen in FD, characterizing a type of drug-induced renal phospholipidosis. CASE PRESENTATION: Case 1: A 28-year-old female patient with systemic lupus erythematosus who had been using hydroxychloroquine for 14 months presented subnephrotic proteinuria. Renal biopsy showed deposits compatible with FD. Neither activity analysis of alpha-galactosidase A nor genetic analysis were available and were not performed. These deposits were not detected in a subsequent renal biopsy three years after withdrawal of the medication, characterizing a possible hydroxychloroquine-induced renal phospholipidosis. Case 2: A 29-year-old male patient presented with acroparesthesia, angiokeratomas, cornea verticillata and subnephrotic proteinuria. Deposits compatible with FD were detected upon renal biopsy. The evaluation of alpha-galactosidase A showed no activity in both blood and leukocytes. Genetic analysis identified an M284 T mutation in exon 6, and such mutation was also found in other family members. CONCLUSION: Clinical investigation is necessary in suspected cases of Fabry Disease upon renal biopsy in order to confirm diagnosis. Drug-induced renal phospholipidosis should be considered in differential diagnosis in cases with intracellular osmiophilic, lamellar inclusions in electron microscopy.

Incidence of tuberculosis among patients with rheumatoid arthritis using TNF blockers in Brazil: data from the Brazilian Registry of Biological Therapies in Rheumatic Diseases (Registro Brasileiro de Monitoração de Terapias Biológicas - BiobadaBrasil).

OBJECTIVES: To assess the incidence of tuberculosis and to screen for latent tuberculosis infection among Brazilians with rheumatoid arthritis using biologics in clinical practice. PATIENTS AND METHODS: This cohort study used data from the Brazilian Registry of Biological Therapies in Rheumatic Diseases (Registro Brasileiro de Monitoração de Terapias Biológicas - BiobadaBrasil), from 01/2009 to 05/2013, encompassing 1552 treatments, including 415 with only synthetic disease-modifying anti-rheumatic drugs, 942 synthetic DMARDs combined with anti-tumor necrosis factor (etanercept, infliximab, adalimumab) and 195 synthetic DMARDs combined with other biologics (abatacept, rituximab and tocilizumab). The occurrence of tuberculosis and the drug exposure time were assessed, and screening for tuberculosis was performed. STATISTICAL ANALYSIS: Unpaired t-test and Fisher's two-tailed test; p<0.05. RESULTS: The exposure times were 981 patient-years in the controls, 1744 patient-years in the anti-TNF group (adalimumab=676, infliximab=547 and etanercept=521 patient-years) and 336 patient-years in the other biologics group. The incidence rates of tuberculosis were 1.01/1000 patient-years in the controls and 2.87 patient-years among anti-TNF users (adalimumab=4.43/1000 patient-years; etanercept=1.92/1000 patient-years and infliximab=1.82/1000 patient-years). No cases of tuberculosis occurred in the other biologics group. The mean drug exposure time until the occurrence of tuberculosis was 27(11) months for the anti-TNF group. CONCLUSIONS: The incidence of tuberculosis was higher among users of synthetic DMARDs and anti-TNF than among users of synthetic DMARDs and synthetic DMARDs and non-anti-TNF biologics and also occurred later, suggesting infection during treatment and no screening failure.

Difficulties in the differential diagnosis between Takayasu arteritis and rheumatic fever: case report.

In this article, we present the case of a patient with heart failure with biological aortic valve prosthesis and multiple vascular changes consistent with Takayasu arteritis (TA) who was seen in our department receiving corticosteroids and secondary prevention of rheumatic fever (RF); it was not possible to exclude the association between both diseases.

Characterization of inflammatory markers associated with systemic lupus erythematosus patients undergoing treatment.

OBJECTIVE: To characterize the inflammatory profiles of patients with systemic lupus erythematosus receiving standard treatment compared to healthy controls. PATIENTS AND METHODS: Peripheral venous blood was collected from systemic lupus erythematosus patients (n=14) and controls (n=18) at enrollment. Blood samples were used for quantification, by flow cytometry, of CD11b (integrin) and Chemokine receptor CXCR2 expression surface antigen in neutrophils and lymphocytes, while cytokines were assayed in serum samples. Purified neutrophils were assayed by their ability to phagocytize human plasma-opsonized zymosan. RESULTS: Patients had a median (interquartile range) disease activity index of 1.0 (0-2.0) characteristic of patients in remission. Interleukin-6 and interleukin-10 serum concentrations were significantly higher in the patient group compared to controls and the phagocytic index of circulating neutrophils was significantly reduced in patients compared to controls. The levels of interleukin-2, interleukin-5, interleukin-8 and tumor necrosis factor alpha did not significantly differ between patients and controls. Flow cytometric analysis revealed that the integrin expression levels were reduced in lymphocytes (but not in neutrophils) obtained from systemic lupus erythematosus patients, while surface expression of the chemokine receptor 2 was similar in both neutrophils and lymphocytes. CONCLUSION: Systemic lupus erythematosus patients receiving standard treatment presented with elevated systemic levels of interleukin-6 and interleukin-10, reduced neutrophil phagocytic capacity, and reduced lymphocyte expression of integrin even when symptoms were in remission. These alterations to innate immune components may put these individuals at a greater risk for acquiring infections.

Drug survival and causes of discontinuation of the first anti-TNF in ankylosing spondylitis compared with rheumatoid arthritis: analysis from BIOBADABRASIL.

Treatment survival with biological therapy may be influenced by many factors, and it seems to be different among various rheumatic diseases and biological agents. The goal of the study was to compare the drug survival and the causes of discontinuation of anti-tumoral necrosis factor (anti-TNF) therapy in ankylosing spondylitis (AS) with rheumatoid arthritis (RA). Study participants were a cohort from the Brazilian Registry of Biological Therapies in Rheumatic Diseases (BIOBADABRASIL) between 2008 and 2012. The observation time was up to 4 years following the introduction of the first treatment. Gender, age, disease duration, disease activity, comorbidities, and concomitant therapies were assessed. A total of 1303 patients were included: 372 had AS and 931 had RA in which 38.7 % (n = 504) used infliximab (IFX), 34.9 % (n = 455) used adalimumab (ADA), and 26.4 % (n = 344) used etanercept (ETA). The anti-TNF drug survival of patients with AS was 63.08 months (confidence interval (CI) 60.24, 65.92) and patients with RA was 47.5 months (CI 45.65, 49.36). It was significant higher in AS (log-rank; p ≤ 0.001). Patients with RA discontinued anti-TNF more than patients with AS when adjusted to gender and corticosteroid. The adjHR (95 % CI) was 1.6 (1.14, 2.31). Female patients who were also corticosteroid users, but not of advanced age, have shown lower survival for both diseases (log-rank, p ≤ 0.001). The discontinuation rate of IFX, but not of ADA or ETA, was significantly higher in RA than in SA; HR (95 % CI) was 2.49 (1.46, 4.24). The main causes of discontinuation were ineffectiveness and adverse event in both diseases. AS patients have better drug survival adjusted to gender, age, and corticosteroid. This results appear to be related to the disease mechanism.

Incidence of tuberculosis among patients with rheumatoid arthritis using TNF blockers in Brazil: data from the Brazilian Registry of Biological Therapies in Rheumatic Diseases (Registro Brasileiro de Monitoração de Terapias Biológicas - BiobadaBrasil) / Incidência de tuberculose em pacientes com artrite reumatoide em uso de bloqueadores do TNF no Brasil: dados do Registro Brasileiro de Monitoração de Terapias Biológicas BiobadaBrasil

Abstract Objectives To assess the incidence of tuberculosis and to screen for latent tuberculosis infection among Brazilians with rheumatoid arthritis using biologics in clinical practice. Patients and methods This cohort study used data from the Brazilian Registry of Biological Therapies in Rheumatic Diseases (Registro Brasileiro de Monitoração de Terapias Biológicas - BiobadaBrasil), from 01/2009 to 05/2013, encompassing 1552 treatments, including 415 with only synthetic disease-modifying anti-rheumatic drugs, 942 synthetic DMARDs combined with anti-tumor necrosis factor (etanercept, infliximab, adalimumab) and 195 synthetic DMARDs combined with other biologics (abatacept, rituximab and tocilizumab). The occurrence of tuberculosis and the drug exposure time were assessed, and screening for tuberculosis was performed. Statistical analysis: Unpaired t-test and Fisher's two-tailed test; p < 0.05. Results The exposure times were 981 patient-years in the controls, 1744 patient-years in the anti-TNF group (adalimumab = 676, infliximab = 547 and etanercept = 521 patient-years) and 336 patient-years in the other biologics group. The incidence rates of tuberculosis were 1.01/1000 patient-years in the controls and 2.87 patient-years among anti-TNF users (adalimumab = 4.43/1000 patient-years; etanercept = 1.92/1000 patient-years and infliximab = 1.82/1000 patient-years). No cases of tuberculosis occurred in the other biologics group. The mean drug exposure time until the occurrence of tuberculosis was 27(11) months for the anti-TNF group. Conclusions The incidence of tuberculosis was higher among users of synthetic DMARDs and anti-TNF than among users of synthetic DMARDs and synthetic DMARDs and non-anti-TNF biologics and also occurred later, suggesting infection during treatment and no screening failure.

Resumo Objetivos Avaliar incidência de tuberculose e triagem para tuberculose latente em brasileiros com artrite reumatoide em uso de agentes biológicos na prática clinica. Pacientes e métodos Estudo de coorte com dados do Registro Brasileiro de Monitoração de Terapias Biológicas (BiobadaBrasil), de 01/2009 a 05/2013, abrangeu 1.552 tratamentos, 415 somente com drogas modificadoras do curso da doença (MMCDs) sintéticas, 942 MMCDs sintéticas em associação com anti-TNF (etanercepte, infliximabe, adalimumabe) e 195 MMCDs sintéticas em associação com outros biológicos (abatacepte, rituximabe e tocilizumabe). Avaliaram-se ocorrência de tuberculose, tempo de exposição às drogas e triagem para TB. Análise estatística: teste t não pareado e teste de Fisher bicaudal; p < 0,05. Resultados O tempo de exposição dos controles foi de 981 pacientes-ano, do grupo de anti-TNF foi de 1.744 pacientes-ano (adalimumabe = 676, infliximabe = 547 e etanercepte = 521 pacientes-ano) e o de outros biológicos de 336 pacientes-ano. A incidência de TB foi de 1,01/1.000 pacientes-ano nos controles e de 2,87 pacientes-ano nos usuários de anti-TNF (adalimumabe = 4,43/1.000 pacientes-ano; etanercepte = 1,92/1.000 pacientes-ano e infliximabe = 1,82/1.000 pacientes-ano). Não houve casos de tuberculose no grupo de outros biológicos. O tempo médio de exposição até a ocorrência de tuberculose foi de 27(11) meses para o grupo anti-TNF. Conclusões A incidência de tuberculose foi maior nos usuários de MMCDs sintéticas e anti-TNF do que nos usuários de MMCDs sintéticas e de MMCDs sintéticas e biológicos não anti-TNF, e também mais tardia, sugerindo infecção durante o tratamento, e não falha na triagem.

Dificuldades no diagnóstico diferencial entre arterite de Takayasu e febre reumática: relato de caso / Difficulties in the differential diagnosis between Takayasu arteritis and rheumatic fever: case report

Resumo Apresentamos o caso de uma paciente portadora de insuficiência cardíaca com prótese valvar aórtica biológica e alterações vasculares compatíveis com arterite de Takayasu (AT) que chegou ao serviço em uso de corticoides e em profilaxia para febre reumática (FR). Não foi possível afastar a associação entre ambas as enfermidades.

Abstract In this article, we present the case of a patient with heart failure with biological aortic valve prosthesis and multiple vascular changes consistent with Takayasu arteritis (TA) who was seen in our department receiving corticosteroids and secondary prevention of rheumatic fever (RF); it was not possible to exclude the association between both diseases.

Characterization of inflammatory markers associated with systemic lupus erythematosus patients undergoing treatment / Caracterização de marcadores inflamatórios associados a pacientes com lúpus eritematoso sistêmico em tratamento

ABSTRACT Objective: To characterize the inflammatory profiles of patients with systemic lupus erythematosus receiving standard treatment compared to healthy controls. Patients and methods: Peripheral venous blood was collected from systemic lupus erythematosus patients (n = 14) and controls (n = 18) at enrollment. Blood samples were used for quantification, by flow cytometry, of CD11b (integrin) and Chemokine receptor CXCR2 expression surface antigen in neutrophils and lymphocytes, while cytokines were assayed in serum samples. Purified neutrophils were assayed by their ability to phagocytize human plasma-opsonized zymosan. Results: Patients had a median (interquartile range) disease activity index of 1.0 (0-2.0) characteristic of patients in remission. Interleukin-6 and interleukin-10 serum concentrations were significantly higher in the patient group compared to controls and the phagocytic index of circulating neutrophils was significantly reduced in patients compared to controls. The levels of interleukin-2, interleukin-5, interleukin-8 and tumor necrosis factor alpha did not significantly differ between patients and controls. Flow cytometric analysis revealed that the integrin expression levels were reduced in lymphocytes (but not in neutrophils) obtained from systemic lupus erythematosus patients, while surface expression of the chemokine receptor 2 was similar in both neutrophils and lymphocytes. Conclusion: Systemic lupus erythematosus patients receiving standard treatment presented with elevated systemic levels of interleukin-6 and interleukin-10, reduced neutrophil phagocytic capacity, and reduced lymphocyte expression of integrin even when symptoms were in remission. These alterations to innate immune components may put these individuals at a greater risk for acquiring infections.

RESUMO Objetivo: Caracterizar os perfis inflamatórios de pacientes com lúpus eritematoso sistêmico (LES) que recebiam o tratamento padrão em comparação com controles saudáveis. Pacientes e métodos: Coletou-se o sangue venoso periférico de pacientes com LES (n = 14) e controles (n = 18) no momento da entrada no estudo. As amostras de sangue foram usadas para quantificação, por citometria de fluxo, da expressão dos antígenos de superfície CD11b (integrina) e CXCR2 em neutrófilos e linfócitos, enquanto as citocinas foram avaliadas em amostras de soro. Avaliou-se a capacidade dos neutrófilos purificados de fagocitar zimosan opsonizado com plasma humano. Resultados: Os pacientes apresentavam uma pontuação mediana (intervalo interquartil) no Sledai de 1 (0-2), característica de pacientes em remissão. As concentrações séricas de IL-6 e IL-10 foram significativamente maiores no grupo de pacientes em comparação com os controles; o índice de fagocitose de neutrófilos circulantes estava significativamente reduzido nos pacientes em comparação com os controles. Os níveis de IL-2, IL-5, IL-8 e TNF-α não diferiram significativamente entre pacientes e controles. A análise da citometria de fluxo revelou que os níveis de expressão de CD11b estavam reduzidos nos linfócitos (mas não nos neutrófilos) obtidos de pacientes com LES, enquanto a expressão do receptor de superfície CXCR2 foi semelhante em neutrófilos e linfócitos. Conclusão: Os pacientes com LES que recebiam tratamento padrão apresentaram níveis sistêmicos elevados de IL-6 e IL-10, redução na capacidade fagocítica dos neutrófilos e redução da expressão de CD11b em linfócitos, mesmo quando os sintomas estavam em remissão. Essas alterações nos componentes da imunidade inata podem colocar esses indivíduos em maior risco de adquirir infecções.

Brazilian biologic registry: BiobadaBrasil implementation process and preliminary results.

OBJECTIVES: The present study aimed at describing the implementation process of a national registry in a developing country (Brazil) and at reporting the main preliminary results of the BiobadaBrasil registry. MATERIAL AND METHODS: Through a PANLAR agreement, the Biobadaser protocol was used as a model for implementing the new registry in our country. During the first two years of this effort, the original protocol was adapted, translated, and presented to all Brazilian rheumatologists. For ten months, data of 1,037 patients (750 subjects treated with biological drugs and 287 control subjects) from 15 centers were collected. RESULTS: Most patients had rheumatoid arthritis (RA) (n = 723). Infliximab was the most frequently used anti-TNF agent, and the total exposure to biologic drugs was 2,101 patient-years. The most common reason for interrupting drug use was lack or loss of efficacy (50%), while 30% withdrew from the treatment arm due to adverse events. Three cases of tuberculosis were observed in the biologic group, with an incidence higher than that of the general Brazilian population. Infections were observed in 23% of the biologic group, and the upper respiratory tract was the most commonly affected site. Only one case of tuberculoid leprosy was observed. No deaths or malignancies attributed to drug effects were observed as of February 2010. CONCLUSIONS: The implementation of the BiobadaBrasil registry was successful, and, although recent, the registry has provided important data.

Registro brasileiro de biológicos: processo de implementação e resultados preliminares do BiobadaBrasil / Brazilian biologic registry: BiobadaBrasil implementation process and preliminary results

OBJETIVOS: O presente estudo teve por objetivo descrever o processo de implementação de um registro nacional em um país em desenvolvimento (Brasil) e relatar os principais resultados preliminares do registro BiobadaBrasil. MATERAL E MÉTODOS: Através de um acordo com a PANLAR, o protocolo Biobadaser foi utilizado como modelo para a implementação de um novo registro no nosso país. Durante os dois primeiros anos desse esforço, o protocolo original foi adaptado, traduzido e apresentado a todos os reumatologistas brasileiros. Durante dez meses, dados de 1.037 pacientes (750 tratados com biológicos e 287 controles) de 15 centros foram coletados. RESULTADOS: A maioria dos pacientes tinha artrite reumatoide (AR) (n = 723). Infliximabe foi o agente anti-TNF mais usado, e a exposição total a biológicos foi 2.101 pacientes-ano. A razão mais comum para suspensão da droga foi ineficiência ou perda de efetividade (50 por cento), e 30 por cento dos pacientes interromperam o tratamento devido a eventos adversos. Três casos de tuberculose foram observados no grupo biológico, representando maior incidência do que aquela da população brasileira geral. Infecções foram observadas em 23 por cento dos pacientes do grupo biológico, sendo o trato respiratório superior o local mais comumente afetado. Apenas um caso de hanseníase tuberculoide foi observado. Nenhuma morte nem malignidade atribuível ao efeito dos medicamentos foi observada até fevereiro de 2010. CONCLUSÕES: A implementação do registro foi bem sucedida. Embora recente, o registro BiobadaBrasil já forneceu importantes dados.

OBJECTIVES: The present study aimed at describing the implementation process of a national registry in a developing country (Brazil) and at reporting the main preliminary results of the BiobadaBrasil registry. MATERIAL AND METHODS: Through a PANLAR agreement, the Biobadaser protocol was used as a model for implementing the new registry in our country. During the first two years of this effort, the original protocol was adapted, translated, and presented to all Brazilian rheumatologists. For ten months, data of 1,037 patients (750 subjects treated with biological drugs and 287 control subjects) from 15 centers were collected. RESULTS: Most patients had rheumatoid arthritis (RA) (n = 723). Infliximab was the most frequently used anti-TNF agent, and the total exposure to biologic drugs was 2,101 patient-years. The most common reason for interrupting drug use was lack or loss of efficacy (50 percent), while 30 percent withdrew from the treatment arm due to adverse events. Three cases of tuberculosis were observed in the biologic group, with an incidence higher than that of the general Brazilian population. Infections were observed in 23 percent of the biologic group, and the upper respiratory tract was the most commonly affected site. Only one case of tuberculoid leprosy was observed. No deaths or malignancies attributed to drug effects were observed as of February 2010. CONCLUSIONS: The implementation of the BiobadaBrasil registry was successful, and, although recent, the registry has provided important data.

Acquired hemophilia associated with rheumatoid arthritis / Hemofilia A adquirida associada à artrite reumatoide

O aparecimento do anticorpo contra fator VIII é um fenômeno bem estabelecido na hemofilia A, ocorrendo em 5 a 15 por cento dos pacientes hemofílicos dos Estados Unidos, Inglaterra, Suécia e França. Nos pacientes não-hemofílicos o seu aparecimento é raro, podendo ocorrer em indivíduos saudáveis, principalmente idosos e mulheres no puerpério, pacientes com neoplasia maligna ou doenças autoimunes, como lúpus eritematoso sistêmico, artrite reumatoide e síndrome de Sjõgren. Descrevemos o caso de uma paciente de 64 anos de idade, portadora de artrite reumatoide soropositiva há 23 anos, que há 10 dias desenvolveu equimoses e hematomas progressivos cuja investigação foi compatível com a presença de anticorpo contra fator VIII. Foi instituída terapia com metilprednisolona, ciclofosfamida endovenosa, imunoglobulina e reposição de complexo protrombínico, com remissão do quadro hemorrágico e negativação do anticorpo contra o fator VIII. Concluímos com esse caso que, apesar de ser rara, a presença de inibidores adquiridos do fator VIII deve ser pesquisada quando pacientes portadores de doença autoimune desenvolvem manifestações hemorrágicas associadas ao prolongamento do TTPA com TAP e contagem plaquetária normais.

The occurrence of the antibody against factor VIII is a well-known phenomenon in hemophilia A, occuring in 5 to 15 percent of the hemophilic patients in the United States, England, Sweden and France. The development of factor VIII in non-hemophilic patients is rare and may occur in healthy individuals, mostly elderly and women in postpartum period, and in patients with malignant neoplasia or autoimmune diseases, such as systemic lupus erythematosus, rheumatoid arthritis and Sjõgren's syndrome. We described the case of a 64-year-old female patient who has had seropositive rheumatoid arthritis for 23 years and presented with a ten-day history of progressive ecchymosis. Therapy with methylprednisolone, intravenous cyclophosphamide, immunoglobulin and factor VIII reposition was instituted, resulting in a remission of the bleeding and negativity for antibodies against factor VIII titers. We concluded that, despite its rarity, the presence of acquired factor VIII inhibitors should be investigated when patients with autoimmune diseases develop bleeding manifestations.

Concomitância de fibromialgia em pacientes portadores de hipotireoidismo e de alterações tireoideanas em pacientes com fibromialgia / Fibromyalgia concomitance in patients with hypothyroidism and thyroid disorders in patients with fibromyalgia

INTRODUÇAO: a fibromialgia (FM) pode ser definida como uma síndrome dolorosa musculoesquelética não-inflamatória de caráter crônico, tendo como sintomas mais comuns a fadiga e o sono não-reparador. Tais manifestações podem não resultar da FM, mas de outras doenças associadas, como o hipotireoidismo. OBJETIVO: descrever a ocorrência de FM em pacientes com hipotireoidismo, e os casos de hipotireoidismo em pacientes com FM, além de determinar os sintomas associados a essas condições clínicas. MÉTODOS: foram avaliados 166 pacientes portadores de FM e 56 com hipotireoidismo. Os voluntários responderam a um questionário para detecção dos principais sinais e sintomas dessas doenças em estudo e foram submetidos às dosagens séricas de hormônio estimulador da tireóide (TSH), tiroxina livre (T4 livre) e anticorpo antitireoperoxidase (a-TPO). RESULTADOS: nos 166 pacientes com FM, foram diagnosticados hipotireoidismo clínico ou subclínico em 35 (21,1 por cento). Dos 56 pacientes portadores de hipotireoidismo, em 36 (64,3 por cento) foi confirmado o diagnóstico de FM pelos critérios do American College of Rheumatology (ACR). Nos pacientes com hipotireoidismo previamente diagnosticado, a fadiga, o sono não-restaurador e a cefaléia crônica foram estatisticamente mais freqüentes naqueles que apresentavam diagnóstico de FM. A rigidez matinal foi mais prevalente nos pacientes com hipotireoidismo sem FM. CONCLUSAO: há que se considerar a presença de manifestações clínicas ou laboratoriais de disfunção tireoideana nos pacientes portadores de FM e de FM em pacientes portadores de hipotireoidismo, para uma melhor abordagem diagnóstica e posterior tratamento dos mesmos.

Osteólise carpo-tarsal: relato de caso / Carpal-tarsal osteolysis: a case report

Os autores relatam um caso de paciente do sexo masculino, com 42 anos de idade, com queixa de poliartralgia de caráter progressivo, iniciada na infância. O quadro evoluiu com deformidades de mãos e pés, impedindo-o de deambular. Não havia alterações associadas em outros sistemas. As radiografias das mãos e pés mostraram lise dos ossos do carpo, do tarso, metacarpais e metatarsais. A osteólise carpo-tarsal é uma síndrome rara, que necessita de uma melhor abordagem clínica, diagnóstica e terapêutica diante de novos casos.

Polimiosite associada a câncer de mama: relato de caso e revisão da literatura / Polymiositis associated with breast cancer: case report and literature review

A polimiosite está classificada dentro do grupo das miopatias inflamatórias idiopáticas. Segundo dados da literatura há uma associação de dermatomiosite e polimiosite com neoplasias. Apresentamos, nesse relato, o caso de uma paciente que 30 dias após ter notado a presença de um nódulo na mama esquerda, desenvolveu um quadro de fraqueza muscular proximal da cintura pélvica e escapular, disfagia e rouquidão. Foi feito o diagnóstico da associação de polimiosite e câncer de mama, sendo essa uma relação de baixa prevalência na população geral. Os autores discutem o tempo de aparecimento do tumor e da miopatia e as prováveis causas de morte.

Dermatomiosite amiopática associada à necrose digital: relato de caso e revisão da literatura / Amyopathic dermatomyositis associated with digital necrosis: case report and literature review

A dermatomiosite amiopática é uma miopatia inflamatória idiopática, que se apresenta com mínimas alterações ou, às vezes, com nenhum comprometimento muscular, apesar das manifestações cutâneas típicas da dermatomiosite. Sua etiologia ainda não está bem esclarecida, embora, na literatura, haja referências sobre uma freqüente associação a doenças neoplásicas e drogas. Por se tratar de uma patologia rara, constituindo apenas 10% dos casos do grupo das miopatias inflamatórias idiopáticas e que também apresenta baixa prevalência na população geral (representando 0,5 a 8,4 casos por milhão), os autores relatam o caso de um paciente com quadro clínico laboratorial característico de dermatomiosite amiopática, associado ao aparecimento de necrose digital e fenômeno de Raynaud, com comprovação histológica.

Lúpus neonatal e lúpus eritematoso cutâneo subagudo na infância / Neonatal lupus and cutaneous subacute lupus erythematosus in childhood

O lúpus eritematoso (LE) raramente ocorre na infância e, quando aparece, manifesta-se, mais freqüentemente, sob a forma de lúpus eritematoso sistêmico. A forma restrita à pele é extremamente incomum e pode apresentar-se nas variantes lúpus eritematoso discóide, lúpus eritematoso cutâneo subagudo (LECS) e lúpus neonatal (LN). Em razão dessa raridade e com vistas aos dados reportados na literatura, apresentamos dois casos de lúpus cutâneo em crianças. O caso um refere-se a um paciente masculino, branco, cinco anos, com lesões de pele eritematoso-descamativas, alopecia, artralgia e dificuldade para deambular. O exame neurológico evidenciou paraparesia espástica com sinais de liberação piramidal. O caso dois refere-se a um lactente de um mês, masculino, filho de mãe com LECS, com lesões de pele eritematoso-ovalares presentes ao nascimento, além de anemia, plaquetopenia e evidência ecocardiográfica de canal arterial pérvio. O diagnóstico de LN foi baseado nos dados clínicos, laboratoriais, histopatológico e antecedentes familiares. Concluímos com a apresentação desses casos que, em razão da raridade do lúpus cutâneo na infância, diante de casos suspeitos, deve-se estar atento para o diagnóstico precoce e acompanhamento dos pacientes, de modo a prevenir possíveis complicações e sistematização da doença.

Osteoartropatia hipertrófica primária: relato de caso e revisão da literatura / Primary hypertrophic osteoarthropathy: a case report and review of the literature

Os autores relatam um caso de osteoartropatia hipertrófica do tipo primária em paciente de 29 anos de idade, com dores articulares e aumento de volume das extremidades há 15 anos. Ao exame físico apresentava baqueteamento dos dedos e unhas em "vidro de relógio". A osteoartropatia hipertrófica foi considerada como primária porque foram excluídas todas as causas da forma secundária. As principais alterações radiológicas foram: espessamento das camadas corticais dos ossos tubulares e reação periosteal contínua do tipo lamelar. Não foram notadas alterações dos espaços articulares

The authors report a case of primary hypertrophic osteoarthropathy in a 29-year-old male with swollen joints. Physical examination revealed also digital clubbing and convexity of the nails. All other causes of secondary forms of hypertrophic osteoarthropathy were excluded. The main radiological abnormalities found were bone cortical hypertrophy and periosteal proliferation. Abnormalities of the joint spaces were not observed.

Síndrome de Evans na evolução da doença de Still / Evans syndrome occurring in Still's disease

Objetivo: Mostrar a importância do diagnóstico precoce e tratamento da síndrome de Evans, ocorrendo como complicação de uma doença sistêmica, a doença de Still. Descrição: Relato do caso da paciente A.L.F.T., 4 anos e 1 mês, com diagnóstico de doença de Still há um ano e cuja evolução, durante sua hospitalização, complicou-se com a síndrome de Evans. Comentário: A síndrome de Evans, ocorrendo no curso da doença de Still, é uma entidade clínica infreqüente, mas importante em termos do prognóstico do paciente. É difícil a distinção dos sintomas dessas enfermidades e a estratégia de tratamento constitui uma urgência.

Artropatia de Jaccoud: revisäo da literatura com apresentaçäo de caso / Jaccoud's arthropathy: review of the literature and report of a case

Os autores apresentam um caso de artropatia de Jaccoud e chamam a atençäo para o fato de que esta síndrome pode ser confundida freqüentemente com outras doenças reumáticas. Em razäo de seu grande potencial de agressäo às estruturas valvares cardíacas, seu diagnóstico precoce se impöe. Para muitos autores, esta síndrome seria uma forma crônica de febre reumática acompanhada de deformidades nas articulaçöes periféricas, causadas mais especificamente por distensäo da cápsula articular e dos tendöes das mäos do que por lesäo das membranas sinoviais. Esse fato é o motivo pelo qual, em alguns casos, pode ser dificil distingui-la da artrite reumatóide, responsável pela maioria dos casos de deformidade nas doenças reumáticas