RESUMO

Amyloidosis is a localized or systemic disease caused by deposition of proteins in the extracellular space of various organs and tissues. As part of the disease, proteins that were originally soluble misfold and acquire a fibrillar conformation that renders them insoluble and resistant to proteolysis. Systemic amyloidosis is a rare, often underdiagnosed condition. In recent years, the incidence of newly diagnosed cases of amyloidosis has been increasing in association with the aging of the population and greater access to diagnostic tests. From a clinical perspective, systemic amyloidosis is frequently associated with involvement of the kidneys (causing nephrotic syndrome), heart (cardiac failure and arrhythmia), and peripheral nervous system (sensorimotor polyneuropathy and autonomic dysfunction). This condition is important to the rheumatologist for several reasons, such as its systemic involvement that mimics autoimmune rheumatic diseases, its musculoskeletal manifestations, which when recognized can allow the diagnosis of amyloidosis, and also because reactive or secondary AA amyloidosis is a complication of rheumatic inflammatory diseases. The treatment of amyloidosis depends on the type of amyloid protein involved. Early recognition of this rare disease is fundamental for improved clinical outcomes.

Assuntos

RESUMO

Este artigo descreve a frequência e o perfil de suscetibilidade a antimicrobianos dos microrganismos isolados em amostras de urina analisadas pelo laboratório de análises clínicas do Hospital Universitário Prof. Polydoro Ernani de São Thiago, localizado em Florianópolis ­ SC, Brasil. Os dados foram obtidos de exames de culturas de urina de origem comunitária, submetidas a testes de suscetibilidade antimicrobiana automatizada, realizadas no período de janeiro de 2013 a dezembro de 2016. Foram analisadas 7964 uroculturas. O microrganismo mais frequente foi Escherichia coli (65,7%), seguido por Klebsiella pneumoniae (8,5%), Proteus mirabilis (6,8%), e Enterococcus faecalis (4,9%). Destaca-se que na faixa etária <15 anos a prevalência do P. mirabilis foi de 13,5%, para o sexo feminino, e 37,7%, para o sexo masculino. Nos idosos (≥60 anos) a prevalência de E. faecalis foi de 6,0% para o sexo feminino e 13,8% para o sexo masculino. A suscetibilidade de E. coli ao ciprofloxacino na população foi de 83,9%, variando de 72,5% em homens a 85,4% em mulheres e de 97,7% em meninos entre 0-14 anos a 58,9% em homens com 60 anos ou mais. O modelo de regressão logística demonstrou que, para os isolados de E coli, o sexo masculino e o aumento da idade estão significantemente ligados a maior resistência a vários antimicrobianos, entre os quais o ciprofloxaxino (P<0,001). Concluiu-se que a frequência dos microorganismos identificados e seu perfil de sensibilidade variam conforme o sexo e a idade dos pacientes. Recomenda-se considerar tais fatores durante a escolha de antibioticoterapia empírica para infecções do trato urinário.

This article describes the frequency and susceptibility profile of microbial isolates from urine cultures samples analyzed by the Hospital Universitário Prof. Polydoro Ernani de São Thiago's clinical analysis laboratory, located in Florianópolis ­ SC, Brasil. Community-acquired isolates were analyzed between January, 2013 and December, 2016. A total of 7964 urine culture isolates were analyzed. Escherichia coli (65,7%), Klebsiella pneumoniae (8,5%), Proteus mirabilis (6,8%) and Enterococcus faecalis (4,9%) were the most frequent isolates. Among the age <15 years, P. mirabilis accounted for 13,5% and 37,7% of female and male respectively isolates. Within the elderly group (aged 60 years and over), E. faecalis was found in 6,0% and 13,8% of female and male respectively isolates. Ciprofloxacin resistance rates in E. coli was 83,9%, ranging from 72,5% in men to 85,4% in women, and from 97,7% in boys between 0-14 years old to 58,9% in men aged 60 year and over. A logistic regression model demonstrated significant relationship of higher antimicrobials resistance rates with increasing age and the male gender for several antimicrobials including ciprofloxacin (P<0,001). In conclusion, microbial profile and susceptibility rates may vary significantly according to gender and age. These factors should be considered when choosing an antimicrobial agent for empiric treatment of urinary tract infections.

RESUMO

Mesial temporal lobe epilepsy with hippocampal sclerosis (MTLE-HS) is characterized by its well-defined clinical profile. Limbic encephalitis is increasingly recognized as a possible etiology of adult-onset MTLE-HS, and neuronal autoantibodies have been detected in patients even without previous signs of encephalitis. The aim of this study is to analyze the frequency of specific autoantibodies in patients with MTLE-HS. A case-control study was carried out with 100 patients with MTLE-HS and 50 healthy controls. Sera samples from subjects were tested by indirect immunofluorescence assay for detection of anti-N-methyl-d-aspartate receptor (NMDA-R), anti-contactin-associated protein-like 2 (CASPR2), anti-leucine-rich glioma inactivated 1 (LGI1), anti-gamma aminobutyric acid B receptor (GABA-B-R), anti-alpha-amino-3-hydroxy-5-methyl-4-isoxazolepropionic acid 1 and 2 receptors (AMPA-1-R and AMPA-2-R), and enzyme-linked immunosorbent assay for detection of anti-glutamic acid decarboxylase 65 (GAD65). Mean age of patients and controls was 41.2 vs 42 years, and 55% vs 56% were female. Mean duration of epilepsy was 27.2 years. No neuronal autoantibodies were found in either group, except for anti-GAD65 in 3 patients and 2 controls. This study adds to the mounting evidence that, in Brazilian patients, MTLE-HS without signs and symptoms of autoimmune encephalitis may be infrequently associated with these autoantibodies. Differences regarding accuracy of used methodologies for autoantibody detection and genetic and environmental characteristics are discussed. Further works with different methodologies tested simultaneously in different populations may help clarify the incongruent study results about autoantibodies in MTLE-HS.

Assuntos

RESUMO

Objectives: To evaluate whether the use of a support tool for shared decision-making in the choice of feeding method for patients with severe dementia can benefit the patient/caregiver dyad, and to evaluate the quality of the decision-making process. Method: A search was performed in the Medline, LILACS, IBECS, SciELO, WHOLIS databases for randomized controlled trials, whether double-blind or otherwise, and quasi-experimental, cohort, case-control, or cross-sectional observational studies in Portuguese, Spanish, English and French. Results: Eight articles were found that showed that the use of a decision support tool, as an aid for the shared decision-making method, is beneficial as it reduces decisional conflict and increases the knowledge of caregivers about the subject. The quality of the decision-making process is unsatisfactory due to the low frequency of discussions between caregivers and the health team and the poor evaluation of caregivers about the participation of the team in the decision-making process. Conclusion: Decision support tools provide benefits for caregivers/patients undergoing the difficult task of deciding about feeding methods. Findings suggest that the current quality of decision-making is inadequate.

Objetivos: Avaliar se o uso de instrumento de apoio para decisão compartilhada na escolha da via de alimentação em pacientes com demência em estágio grave traz benefício para a díade paciente/cuidador e avaliar a qualidade do processo decisório. Método: Busca nas bases de dados Medline, LILACS, IBECS, SciELO, WHOLIS por ensaios clínicos randomizados duplo cegos ou não, estudos quase-experimentais, observacionais de coorte, caso-controle ou transversais nos idiomas português, espanhol, inglês e francês. Resultados: Obtidos oito artigos que mostraram que o uso de apoio à decisão como método auxiliar de decisão compartilhada traz benefício, pois reduz o conflito decisional e aumenta o conhecimento dos cuidadores sobre o assunto. A qualidade do processo decisório é insatisfatória devido à baixa frequência das conversas entre cuidadores e equipe de saúde e a má avaliação dos cuidadores sobre a participação da equipe no processo decisório Conclusão: O instrumento de apoio à decisão compartilhada traz benefícios para cuidadores/pacientes submetidos à difícil tarefa de decidir a via de alimentação. Os achados sugerem que a atual qualidade do processo decisório é inadequada.

Assuntos

RESUMO

Acquired angioedema is caused by different drugs and lymphoproliferative diseases, and rarely it has also been related to the presence of auto-immune disorders. We report the case of a 47 year old female with systemic lupus erythematosus (SLE) and severe cutaneous involvement who developed recurrent localized angioedema of the face, including lips and eye lids, upper limbs, and thorax, not associated with urticaria, and with reduced levels of C1 esterase inhibitor. Treatment with antimalarials, glucocorticoids, and pulse therapy with methylprednisolone associated with azathioprine did not improve her condition. The patient was also unsuccessfully treated with danazol, and she only showed clinical response after several sessions of plasmapheresis, including resolution of the extensive edema of the gastrointestinal tract.

Assuntos

RESUMO

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.

Acquired angioedema is caused by different drugs and lymphoproliferative diseases, and rarely it has also been related to the presence of auto-immune disorders. We report the case of a 47 year old female with systemic lupus erythematosus (SLE) and severe cutaneous involvement who developed recurrent localized angioedema of the face, including lips and eye lids, upper limbs, and thorax, not associated with urticaria, and with reduced levels of C1 esterase inhibitor. Treatment with antimalarials, glucocorticoids, and pulse therapy with methylprednisolone associated with azathioprine did not improve her condition. The patient was also unsuccessfully treated with danazol, and she only showed clinical response after several sessions of plasmapheresis, including resolution of the extensive edema of the gastrointestinal tract.

Assuntos

RESUMO

OBJETIVO: Um instrumento de avaliação da atividade da Doença de Behçet (DB) denominado Behçet's Disease Current Activity Form (BDCAF) foi originalmente elaborado em língua inglesa e adaptado para a língua portuguesa do Brasil na versão BR-BDCAF. Recentemente, um modelo simplificado desse protocolo (com 12 itens) foi apresentado pela Sociedade Internacional para a Doença de Behçet (International Society for Behçet's Disease, ISBD) para produzir um índice denominado BDAI (Behçet's Disease Activity Index). Por esse motivo, neste trabalho produzimos o modelo simplificado da versão brasileira, medimos sua confiabilidade e avaliamos a validade dos resultados de ambos os instrumentos, comparando seus desempenhos. MÉTODO: O modelo simplificado da versão brasileira BR-BDCAF, adaptada transculturalmente, foi denominado BR-BDCAF(s). Ambos os protocolos foram aplicados em 25 pacientes com DB. A confiabilidade do BR-BDCAF(s) foi avaliada através das reprodutibilidades intra e interobservadores pela estatística kappa. A validade e as propriedades diagnósticas sensibilidade(S), especificidade(E) e acurácia dos dois protocolos na definição de casos ativos da doença foram avaliadas pela comparação ao julgamento clínico de um reumatologista perito em DB, e os melhores pontos de corte foram estabelecidos para cada instrumento através da curva ROC (receive-operator characteristic). RESULTADOS E CONCLUSÕES: BR-BDCAF(s) apresentou boa confiabilidade nas questões sobre manifestações mucocutâneas e articulares da DB. Os melhores pontos de corte para a definição de casos ativos foram índices maiores que quatro no BR-BDCAF (S = 80,0 por cento, E = 86,7 por cento) e maiores que um no BR-BDCAF(s) (S = 70,0 por cento, E = 86,7 por cento). Suas acurácias foram semelhantes, sugerindo que ambos podem ser utilizados como instrumentos de medida de atividade da DB.

OBJECTIVE: Behçet's Disease Current Activity Form (BDCAF), an instrument for assessing activity of Behçet's Disease (BD), was originally produced in English. Its cross-cultural adaptation to Brazilian Portuguese language generated a version called BR-BDCAF. Recently, a simplified model of this protocol (with 12 items) was developed by the International Society for Behçet's Disease (ISBD) to generate an index called BDAI (Behçet's Disease Activity Index). For this reason, in this work we intended to create the simplified model of the Brazilian version, measure their reliability and evaluate the validity of the results of both instruments, comparing their performances. METHODS: The simplified version of BR-BDCAF was called BR-BDCAF(s). Both protocols were applied in 25 BD patients. Reliability of BR-BDCAF(s) was evaluated through intra- and interobserver agreement according to kappa statistic. Sensitivity (Se), specificity (Sp) and accuracy of both protocols on defining BD active cases were evaluated by comparing the rheumatologist expert clinical judgement to each protocol, and the best cut-off points were established for each instrument by the ROC curve (receive-operator characteristic). RESULTS AND CONCLUSIONS: BR-BDCAF(s) showed good reliability on items relating to mucocutaneous and articular manifestations of BD. The best cut-off points for the definition of active cases were: higher than four with BR-BDCAF (Se = 80.0 percent, Sp = 86.7 percent) and higher than one with BR-BDCAF( s) (Se = 70.0 percent, Sp = 86.7 percent). Accuracy of both protocols was similar, suggesting that both can be used as tools to assess activity of DB.

RESUMO

A doença de Behçet tem sua fisiopatologia caracterizada por hiperatividade neutrofílica, particularmente em relação à quimiotaxia, e períodos de atividade da doença podem ser desencadeados por exposição a estreptococos. Uma vez que células do sistema imune inato são ativadas pelo ácido lipoteicoico (LTA) de bactérias gram-positivas via receptor toll-like (TLR) 2 e CD14, cujas expressões são reguladas pelos fatores estimulantes de colônias de granulócitos (G-CSF) e granulócitos-macrófagos (GM-CSF), o objetivo principal deste estudo foi determinar se há hiperexpressão de TLR2 em neutrófilos de DB ativa e se a quimiotaxia de polimorfonucleares (PMN) neutrófilos na DB poderia ser hiperestimulada pelo LTA. Além do TLR2, foram medidas as expressões de TLR4, CD14, CD114 (receptor de G-CSF) e CD116 (receptor de GM-CSF) nos neutrófilos e nos monócitos de pacientes com doença de Behçet (DB), as concentrações séricas de CD14 solúvel (CD14s) e as respostas quimiotáxicas dos PMNs de DB sob diferentes estímulos. A expressão dos receptores foi medida pela citometria de fluxo, as concentrações séricas por ELISA e as respostas quimiotáxicas foram avaliadas em câmara de Boyden. Nos PMNs, os receptores foram igualmente expressos nos dois grupos e, estimulados com LTA, suas respostas quimiotáxicas também foram similares. Somente à incubação com plasma os PMNs de DB desenvolveram hiperquimiotaxia em relação aos PMNs controles. A expressão do TLR2 foi maior em monócitos de DB em relação aos controles, e a concentração de CD14s sérica, de origem monocitária, foi maior nos pacientes com DB ativa. Em conjunto, os resultados demonstram que PMNs de DB, isoladamente, não reagem exacerbadamente ao LTA, e suas respostas migratórias são estritamente dependentes de fatores estimulantes solúveis.

Expressions of toll-like receptor (TLR) 2, TLR4, CD14, CD114 and CD116 were assessed on polymorphonuclear (PMN) neutrophils and monocytes of patients with Behçets disease (BD). PMN chemotactic responses under different stimulations were also measured. The objective was to determine if BD PMN chemotaxis may be overstimulated by lipoteichoic acid (LTA) from gram-positive bacteria. Receptor expressions were measured by flow cytometry and PMN chemotaxis was assessed in a Boyden chamber. Only TLR2 expression was higher on monocytes of the BD group than in control group. On PMNs, however, TLR2 expression was similar in both groups and, when stimulated with LTA, BD PMN cells showed chemotactic responses similar to the controls. These cells only exhibited increased chemotaxis when incubated with plasma. In conclusion, isolated BD PMN did not overreact to LTA, and its hyperchemotaxis is strictly dependent on soluble stimulating factors.

Assuntos

RESUMO

One patient with dermatomyositis (DM) who developed spontaneous pneumomediastinum (SPnM) is described. A review of 15 previously reported cases shows that this is a complication of DM but not of polymyositis. It occurs in young patients with recent onset of disease characterized by cutaneous vasculopathy, and patients treated with immunosuppressive drugs have, in general, better outcomes. We suggest that the onset of SPnM in DM patients must alert to possible vasculitic activity and that immunosuppressive agents with progressive tapering of corticosteroids are part of the most reasonable approach in these cases.

Assuntos

RESUMO

Two patients with neuropathic arthropathy (NA) of hands and wrists due to cervical syringomyelia are described. Articular deformities resembling rheumatoid arthritis (RA) were present in both cases. RA was misdiagnosed in the first case, leading to incorrect treatment with iatrogenic consequences, and there was real coexistence of RA and NA in the second one. Physicians must be aware of this rare condition in the differential diagnosis of wrist and hand deformities.

Assuntos

RESUMO

Relatamos aqui o caso de um paciente masculino de 30 anos de idade, sexo masculino, com achados clínicos típicos da síndrome de Behçet (SB) e da policondrite recidivante (PR) sem, no entanto, preencher completamente os critérios de classificação para nenhuma das entidades isoladamente. O termo síndrome MAGIC (Mouth And Genital ulcers with Inflamed Cartilage) tem sido usado para classificar pacientes semelhantes. Uma breve revisão dos casos previamente relatados também é apresentada.

Assuntos