Sjögren: unique surname, two men, four syndromes and one disease.

Henrik and Torsten Sjögren (/'ʃoʊɡrən/ or SHOH-grən) were two Swedish physicians living in the same period, but completely unrelated, except for their notable contributions to Medicine. The first one described keratoconjunctivitis sicca, afterward called Sjögren's syndrome, and a fishing net aspect retinal pigmentation affecting visual acuity, nowadays known as Sjögren reticular dystrophy. The last one contributed to the understanding of Spielmeyer-Sjögren disease, Marinesco-Sjögren, and Sjögren-Larsson syndromes, all related to genetic disorders and neurological symptoms. In this paper, we aim to describe each disorder, in order to avoid any misunderstanding in diagnosis and for historical record.

Henrik e Torsten Sjögren (/ˈʃoʊɡrən/ ou SHOH-grən) foram dois médicos suecos que viveram na mesma época, mas não tinham nenhuma relação entre si, exceto por suas notáveis contribuições à medicina. O primeiro descreveu a ceratoconjuntivite sicca, posteriormente chamada de síndrome de Sjögren, e uma pigmentação da retina com aspecto de rede de pesca que afeta a acuidade visual, hoje conhecida como distrofia reticular de Sjögren. O último contribuiu para a compreensão da doença de Spielmeyer-Sjögren, das síndromes de Marinesco-Sjögren e Sjögren-Larsson, todas relacionadas a distúrbios genéticos e sintomas neurológicos. Neste artigo, pretendemos descrever cada desordem, a fim de evitar qualquer mal-entendido no diagnóstico e para registro histórico.

Sjögren: unique surname, two men, four syndromes and one disease / Sjögren: sobrenome único, dois homens, quatro síndromes e uma doença

Abstract Henrik and Torsten Sjögren (/'ogrƏn/ or SHOH-grƏn) were two Swedish physicians living in the same period, but completely unrelated, except for their notable contributions to Medicine. The first one described keratoconjunctivitis sicca, afterward called Sjögren's syndrome, and a fishing net aspect retinal pigmentation affecting visual acuity, nowadays known as Sjögren reticular dystrophy. The last one contributed to the understanding of Spielmeyer-Sjögren disease, Marinesco-Sjögren, and Sjögren-Larsson syndromes, all related to genetic disorders and neurological symptoms. In this paper, we aim to describe each disorder, in order to avoid any misunderstanding in diagnosis and for historical record.

Resumo Henrik e Torsten Sjögren (/'ogrƏn/ or SHOH-grƏn) foram dois médicos suecos que viveram na mesma época, mas não tinham nenhuma relação entre si, exceto por suas notáveis contribuições à medicina. O primeiro descreveu a ceratoconjuntivite sicca, posteriormente chamada de síndrome de Sjögren, e uma pigmentação da retina com aspecto de rede de pesca que afeta a acuidade visual, hoje conhecida como distrofia reticular de Sjögren. O último contribuiu para a compreensão da doença de Spielmeyer-Sjögren, das síndromes de Marinesco-Sjögren e Sjögren-Larsson, todas relacionadas a distúrbios genéticos e sintomas neurológicos. Neste artigo, pretendemos descrever cada desordem, a fim de evitar qualquer mal-entendido no diagnóstico e para registro histórico.

Guidelines on COVID-19 vaccination in patients with immune-mediated rheumatic diseases: a Brazilian Society of Rheumatology task force

Abstract Objective: To provide guidelines on the coronavirus disease 2019 (COVID-19) vaccination in patients with immune-mediated rheumatic diseases (IMRD) to rheumatologists considering specific scenarios of the daily practice based on the shared-making decision (SMD) process. Methods: A task force was constituted by 24 rheumatologists (panel members), with clinical and research expertise in immunizations and infectious diseases in immunocompromised patients, endorsed by the Brazilian Society of Rheumatology (BSR), to develop guidelines for COVID-19 vaccination in patients with IMRD. A consensus was built through the Delphi method and involved four rounds of anonymous voting, where five options were used to determine the level of agreement (LOA), based on the Likert Scale: (1) strongly disagree; (2) disagree, (3) neither agree nor disagree (neutral); (4) agree; and (5) strongly agree. Nineteen questions were addressed and discussed via teleconference to formulate the answers. In order to identify the relevant data on COVID-19 vaccines, a search with standardized descriptors and synonyms was performed on September 10th, 2021, of the MEDLINE, EMBASE, Cochrane Central Register of Controlled Trials, ClinicalTrials.gov, and LILACS to identify studies of interest. We used the Newcastle-Ottawa Scale to assess the quality of nonrandomized studies. Results: All the nineteen questions-answers (Q&A) were approved by the BSR Task Force with more than 80% of panelists voting options 4—agree—and 5—strongly agree—, and a consensus was reached. These Guidelines were focused in SMD on the most appropriate timing for IMRD patients to get vaccinated to reach the adequate covid-19 vaccination response. Conclusion: These guidelines were developed by a BSR Task Force with a high LOA among panelists, based on the literature review of published studies and expert opinion for COVID-19 vaccination in IMRD patients. Noteworthy, in the pandemic period, up to the time of the review and the consensus process for this document, high-quality evidence was scarce. Thus, it is not a substitute for clinical judgment.

Cardiovascular Risk Factors and Risk Measurement in Patients with Psoriatic Arthritis in a University Hospital

Abstract Background: Psoriatic Arthritis is the spondyloarthritis associated with psoriasis, which is often related to high mortality due to cardiovascular causes. Objectives: To quantify cardiovascular risk factors (hypertension, diabetes, dyslipidemia, obesity and smoking) and to measure risk by the Global Cardiovascular Risk Score in patients with psoriatic arthritis. Methods: Patients with psoriatic arthritis according to the Classification Criteria for Psoriatic Arthritis, aged between 30 and 74 years and without any other clinically manifest chronic inflammatory disease, atherosclerotic disease or heart failure were included. After an interview, clinical examination and data extraction from medical records, risk stratification was performed using a calculator available on the online platform of the Framingham Heart Study. We considered p < 0.05 as significant. Chi-square test and Fisher's exact test were used to compare frequencies, as well as correlation measurements. Results: 45 patients were included, 68,9% of which were women and the mean age was 53,94 years. Dyslipidemia was confirmed in approximately 93%, hypertension in 46%, obesity in 40%, 33.3% were diabetics and, 13.3%, smokers; 95% had increased abdominal circumference. It was observed that 53% had high cardiovascular risk, 29% had intermediate risk and 18% had low risk. Individuals with altered C-reactive protein and erythrocyte sedimentation rate presented, respectively, higher levels of LDL-C and total cholesterol. Conclusions: There was a high occurrence of risk factors and the majority of the sample was stratified into high or intermediate cardiovascular risk.

Incidence of neoplasms in the most prevalent autoimmune rheumatic diseases: a systematic review.

This article is a systematic review of the literature about the coexistence of cancer and autoimmune rheumatic diseases, their main associations, cancers and possible risk factors associated, with emphasis on existing population-based studies, besides checking the relation of this occur with the use of the drugs used in the treatment of autoimmune diseases. A search was conducted of scientific articles indexed in the Cochrane / BVS, Pubmed / Medline and Scielo / Lilacs in the period from 2002 to 2012. Also consulted was the IB-ICT (Brazilian digital library of theses and Masters), with descriptors in Portuguese and English for "Systemic sclerosis", "Rheumatoid Arthritis", " Systemic Lupus Erythematosus" and "Sjögren's syndrome", correlating each one with the descriptor AND "neoplasms". The results showed that in the database IBICT a thesis and a dissertation for the descriptor SLE met the inclusion criteria, none met RA one thesis to SS. Lilacs in the database/Scielo found two articles on "Rheumatoid Arthritis" AND "neoplasms". In Pubmed/Medline the inicial search resulted in 118 articles, and 41 were selected. The review noted the relationship between cancer and autoimmune rheumatic diseases, as well as a risk factor for protection, although the pathophysiological mechanisms are not known.

Incidência de neoplasias nas doenças reumatológicas autoimunes mais prevalentes: uma revisão sistemática / Incidence of neoplasms in the most prevalent autoimmune rheumatic diseases: a systematic review

O presente artigo é uma revisão sistemática da literatura que aborda a coexistência de neoplasias e doenças reumatológicas autoimunes, suas principais associações, tipos de cânceres e os possíveis fatores de riscos associados, com ênfase nos estudos de base populacional existentes, além de verificar a relação dessa ocorrência com o uso dos fármacos utilizados no tratamento de doenças autoimunes. Foi realizada uma busca de artigos científicos indexados na Cochrane/BVS, Pubmed/Medline e Scielo/Lilacs no período de 2002 a 2012. Também foi consultada a IBICT (biblioteca digital brasileira de teses e mestrados), com os descritores em português e inglês para as palavras: "Esclerose sistêmica", "Artrite reumatoide", "Lúpus Eritematoso Sistêmico" e "Síndrome de Sjögren", correlacionando cada um com o descritor AND "neoplasias". Os resultados mostraram que, na base de dados IBICT, preencheram os critérios de inclusão uma tese e uma dissertação para o descritor LES, nenhuma para AR e uma tese para SS. Na base de dados Lilacs/Scielo foram encontrados dois artigos sobre "Artrite Reumatoide" AND "neoplasias". No Pubmed/Medline, a busca inicial resultou em 118 artigos; destes, preencheram os critérios e foram secionados 41 artigos. Esta revisão observou relação entre neoplasias e as doenças reumatológicas autoimunes, tanto como fator de risco quanto de proteção, embora os mecanismos fisiopatológicos não estejam totalmente elucidados.

This article is a systematic review of the literature about the coexistence of cancer and autoimmune rheumatic diseases, their main associations, cancers and possible risk factors associated, with emphasis on existing population-based studies, besides checking the relation of this occur with the use of the drugs used in the treatment of autoimmune diseases. A search was conducted of scientific articles indexed in the Cochrane / BVS, Pubmed / Medline and Scielo / Lilacs in the period from 2002 to 2012. Also consulted was the IB-ICT (Brazilian digital library of theses and Masters), with descriptors in Portuguese and English for "Systemic sclerosis", "Rheumatoid Arthritis", " Systemic Lupus Erythematosus" and "Sjögren's syndrome", correlating each one with the descriptor AND "neoplasms". The results showed that in the database IBICT a thesis and a dissertation for the descriptor SLE met the inclusion criteria, none met RA one thesis to SS. Lilacs in the database/Scielo found two articles on "Rheumatoid Arthritis" AND "neoplasms". In Pubmed/Medline the inicial search resulted in 118 articles, and 41 were selected. The review noted the relationship between cancer and autoimmune rheumatic diseases, as well as a risk factor for protection, although the pathophysiological mechanisms are not known.

Non-pharmacological therapy and complementary and alternative medicine in fibromyalgia.

Fibromyalgia is a chronic painful syndrome that affects up to 5% of the world population. It is associated with sleep and mood disorders, fatigue, and functional disability. Its pathogenesis involves a disorder of the central modulation of pain, impairment of the descending inhibitory system, and hyperactivity of substance P. Because of the extensive symptomatology of patients with fibromyalgia and its multifactorial pathogenesis, its ideal treatment requires a multidisciplinary approach including the association of pharmacological and non-pharmacological therapies. The pharmacological therapy currently recommended for the syndrome includes antidepressants, calcium-channel modulators, muscle relaxants, and analgesics. In most cases, the non-pharmacological treatment consists of patient education, supervised aerobic physical activity, and cognitive-behavioral therapy. However, many patients do not respond satisfactorily, or have side effects associated with the long-term use of drugs, in addition to reporting difficulties in adhering to a therapy based on exercises and physical medicine. Thus, physicians and patients are increasingly interested in an alternative and complementary therapy for fibromyalgia. This review approaches the different therapeutic modalities used in fibromyalgia, emphasizing the evidence of non-pharmacological therapy and use of alternative and complementary medicine for these patients.

Uso da terapia não farmacológica, medicina alternativa e complementar na fibromialgia / Non-pharmacological therapy and complementary and alternative medicine in fibromyalgia

A fibromialgia é uma síndrome dolorosa crônica que afeta até 5 por cento da população mundial. Pode associar-se com distúrbios do sono, do humor e fadiga, e cursar com incapacidade funcional. Sua patogênese envolve distúrbio de modulação central da dor, comprometimento do sistema inibitório descendente e hiperatividade da substância P. Em função da vasta sintomatologia apresentada pelos pacientes e da natureza multifatorial da sua patogênese, seu tratamento ideal requer uma abordagem multidisciplinar incluindo a associação de terapia farmacológica e não farmacológica. A terapia farmacológica atualmente preconizada nessa síndrome inclui, entre outros compostos, antidepressivos, moduladores dos canais de cálcio, relaxantes musculares e analgésicos. O tratamento não farmacológico é realizado, na maioria dos casos, por meio de educação do paciente, atividade física aeróbica supervisionada e terapia cognitivo-comportamental. No entanto, muitos pacientes não apresentam respostas satisfatórias ou apresentam efeitos colaterais associados ao uso dos fármacos a longo prazo, além de referirem dificuldades em permanecer em uma terapia baseada em exercícios e medicina física. Há, portanto, um crescente interesse por parte dos médicos e pacientes por uma terapia alternativa e complementar na fibromialgia. Nesta revisão, os autores discorrem sobre as diversas modalidades terapêuticas empregadas nessa doença, enfatizando as evidências da terapia não farmacológica e do uso de medicina alternativa e complementar nesses pacientes.

Fibromyalgia is a chronic painful syndrome that affects up to 5 percent of the world population. It is associated with sleep and mood disorders, fatigue, and functional disability. Its pathogenesis involves a disorder of the central modulation of pain, impairment of the descending inhibitory system, and hyperactivity of substance P. Because of the extensive symptomatology of patients with fibromyalgia and its multifactorial pathogenesis, its ideal treatment requires a multidisciplinary approach including the association of pharmacological and non-pharmacological therapies. The pharmacological therapy currently recommended for the syndrome includes antidepressants, calcium-channel modulators, muscle relaxants, and analgesics. In most cases, the non-pharmacological treatment consists of patient education, supervised aerobic physical activity, and cognitive-behavioral therapy. However, many patients do not respond satisfactorily, or have side effects associated with the long-term use of drugs, in addition to reporting difficulties in adhering to a therapy based on exercises and physical medicine. Thus, physicians and patients are increasingly interested in an alternative and complementary therapy for fibromyalgia. This review approaches the different therapeutic modalities used in fibromyalgia, emphasizing the evidence of non-pharmacological therapy and use of alternative and complementary medicine for these patients.

Manifestações musculoesqueléticas nos pacientes em programa de hemodiálise / Musculoskeletal manifestations in hemodialysis patients

Uma diversidade de alterações osteoarticulares tem sido descrita em pacientes em hemodiálise crônica. Objetivo: Verificar a proporção e o tipo de manifestação musculoesquelética (MME) nos pacientes em programa de hemodiálise, em três centros da região metropolitana do Recife, e relacioná-las com as variáveis sexo, etnia, idade atual do paciente e, ao iniciar da diálise, tempo de tratamento dialítico. Métodos: Inicialmente foram aplicados questinários em 197 pacientes distribuídos nos três centros no período de março de 2001 a janeiro de 2002. Após exluir 35 pacientes com diagnóstico prévio de doença reumatológica, investigou-se a presença de sinais e/ou sintomas de MME em 162 pacientes. A média de idade foi de 47,3 anos, a média da idade no início do tratamento foi de 43,8 anos, 94 pacientes (58 por cento) eram do sexo masculino e 120 (74,1 por cento) não-caucasóides. O tempo médio do tratamento dialítico foi de 44,1 meses, sendo utilizada a membrana de polissulfona em todos. Resultados: MME foram observadas em 55 (34 por cento) dos 162 pacientes do estudo. Destes, 38 apresentaram um único tipo de manifestações e 17 pacientes, mais de um tipo (16 apresentaram dois e um, três tipos), perfazendo um total de 73 manifestações distribuídas entre articulações (44), ossos (18), estruturas neuromusculares (seis) e periarticulares (cinco). A artralgia foi responsável por 46,6 por cento de todas as MME, e o joelho foi a articulação mais acometida (52,9 por cento dos casos). A dor óssea foi a segunda queixa mais comum (21,9 por cento), as alterações periarticulares corresponderam a 6,8 por cento das MME; e síndrome do túnel do carpo, deformidades ósseas e tumorações articulares ocorreram em 4,1 por cento, 2,7 por cento e 2,7 por cento, respectivamente, do total de MME. Entre os 55 pacientes, alterações articulares foram encontradas em 72,7 por cento deles, ósseas em 32,7 por cento, neuromusculares em 10,8 por cento e periarticulares em 9,1 por cento dos pacientes. Neste estudo, observou-se a relação entre o tempo médio de tratamento dialítico (59,8 meses) e o desenvolvimento de MME (p<0,001). Conclusões: a partir destes dados, conclui-se que a proporção de MME nos nossos pacientes foi de 34 por cento; as manifestações articulares foram as mais observadas e a artralgia foi a queixa mais comum. No nosso estudo, com exceção do tempo de tratamento dialítico, as demais variáveis não mostraram associação com o aparecimento de MME