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. /ogrƏn/ ogrƏn / ogrƏn/ (/'ogrƏn SHOH–grƏn SHOHgrƏn SHOH grƏn period unrelated Medicine sicca Sjögrens s syndrome acuity dystrophy SpielmeyerSjögren Spielmeyer disease MarinescoSjögren, MarinescoSjögren Marinesco Sjögren, Marinesco-Sjögren SjögrenLarsson Larsson syndromes symptoms paper disorder record /ogrƏn

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. /ogrƏn/ ogrƏn / ogrƏn/ (/'ogrƏn SHOH–grƏn SHOHgrƏn SHOH grƏn época si medicina sicca visual SpielmeyerSjögren, SpielmeyerSjögren Spielmeyer Spielmeyer-Sjögren MarinescoSjögren Marinesco SjögrenLarsson, SjögrenLarsson Larsson, Larsson Sjögren-Larsson neurológicos artigo desordem malentendido mal entendido histórico /ogrƏn

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.

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.

Resumo O lúpus eritematoso sistêmico (LES) é uma doença autoimune multissistêmica que tem como uma das manifestações mais marcantes a nefrite. Apesar de poder coexistir com outras doenças autoimunes e determinar a predisposição a diversas complicações infecciosas, o LES raramente é descrito em associação a nefropatias de etiologia não lúpica. Relatamos o caso da rara associação entre LES e nefropatia por IgA (NIgA) primária, a glomerulopatia primária mais frequente na população mundial. A paciente foi diagnosticada com LES pela ocorrência de eritema malar, alopecia, derrame pleural, proteinúria, pancitopenia, FAN 1:1.280 padrão nuclear pontilhado fino e anticardiolipina IgM 280 U/mL. A biópsia renal revelou hipercelularidade mesangial com depósitos isolados de IgA, compatível com NIgA primária. Foi tratada com antimalárico, prednisona e inibidor da enzima conversora de angiotensina e apresentou boa evolução. Por consistirem em doenças relativamente frequentes, a coexistência de LES e NIgA pode ser de fato um achado incomum por motivos desconhecidos ou uma condição subdiagnosticada. Este relato atenta para a importância da distinção entre a atividade de doença renal do LES e nefropatias não lúpicas, em especial a NIgA, definição que tem implicações importantes sobre o prognóstico renal e regimes terapêuticos a serem adotados em curto e longo prazo.

Abstract Systemic erythematosus lupus (SLE) is a multisystemic autoimmune disease which has nephritis as one of the most striking manifestations. Although it can coexist with other autoimmune diseases, and determine the predisposition to various infectious complications, SLE is rarely described in association with non‐lupus nephropathies etiologies. We report the rare association of SLE and primary IgA nephropathy (IgAN), the most frequent primary glomerulopathy in the world population. The patient was diagnosed with SLE due to the occurrence of malar rash, alopecia, pleural effusion, proteinuria, ANA 1: 1,280, nuclear fine speckled pattern, and anticardiolipin IgM and 280 U/mL. Renal biopsy revealed mesangial hypercellularity with isolated IgA deposits, consistent with primary IgAN. It was treated with antimalarial drug, prednisone and inhibitor of angiotensin converting enzyme, showing good progress. Since they are relatively common diseases, the coexistence of SLE and IgAN may in fact be an uncommon finding for unknown reasons or an underdiagnosed condition. This report focus on the importance of the distinction between the activity of renal disease in SLE and non‐SLE nephropathy, especially IgAN, a definition that has important implications on renal prognosis and therapeutic regimens to be adopted in the short and long term.

Resumo O maior entendimento das bases fisiopatológicas e do comportamento das vasculites sistêmicas, aliado ao desenvolvimento de regimes terapêuticos com perfil de segurança e eficácia cada vezes melhores, modificou drasticamente o prognóstico dos pacientes diagnosticados com essas entidades clínicas. Recentemente, o emprego do rituximabe no tratamento de pacientes com vasculites ANCA associadas em ensaios clínicos randomizados se mostrou uma opção importante em casos selecionados, especialmente pacientes refratários ou intolerantes à terapia-padrão com ciclofosfamida e corticosteroides. O presente artigo traz o relato de sete casos de vasculites sistêmicas com tratamento bem-sucedido com rituximabe.

Abstract The greater understanding of pathophysiology and behavior of systemic vasculitis, together with the development of therapeutic regimens with increasingly better safety and efficacy profiles, dramatically changed the prognosis of patients diagnosed with these clinical entities. Recently, the use of rituximab in the treatment of patients with ANCA-associated vasculitis in randomized clinical trials showed an important alternative in selected cases, especially patients refractory or intolerant to standard therapy with cyclophosphamide and corticosteroids. This article presents the report of seven cases of systemic vasculitis successfully treated with rituximab.

RESUMO As parasitoses intestinais – helmintíases e protozooses – são doenças cosmopolitas com maior prevalência em regiões tropicais. Pacientes com diagnóstico de doenças reumáticas autoimunes apresentam, em função da própria doença de base ou de seu tratamento, um maior risco de ocorrência de manifestações graves das parasitoses intestinais. Embora a prevalência dessas parasitoses seja bastante elevada em nosso meio, nem sempre o reumatologista está atento à necessidade de investigação e tratamento das helmintíases e protozooses antes do uso de terapias imunomoduladoras, imunossupressoras e dos medicamentos biológicos modificadores do curso da doença. Neste documento, a Sociedade Brasileira de Reumatologia estabelece recomendações gerais sobre o diagnóstico e tratamento das parasitoses intestinais no Brasil em pacientes com doenças reumáticas autoimunes, com destaque para a artrite reumatoide, o lúpus eritematoso sistêmico e as espondiloartrites.

ABSTRACT Intestinal parasites – helminths and protozoa – are cosmopolitan diseases which are most prevalent in tropical regions. Patients with diagnoses of autoimmune rheumatic diseases have, due to the underlying disease or its treatment, an increased risk of occurrence of severe manifestations of intestinal parasites. Although the prevalence of these parasitic infections is very high in our environment, not always is the rheumatologist attentive to the need for investigation and treatment of helminthiasis and protozooses before the use of immunomodulatory, immunosuppressive therapies, and of biological drugs that are modifiers of the course of the disease. In this document, the Brazilian Society of Rheumatology establishes general recommendations on the diagnosis and treatment of intestinal parasitic infections in Brazil in patients with autoimmune rheumatic diseases, highlighting rheumatoid arthritis, systemic lupus erythematosus and spondyloarthritis.

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.

A fibromialgia é uma síndrome dolorosa crônica que afeta até 5% 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% 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.

Este estudo foi conduzido com o objetivo de avaliar a atividade repelente do N,N-diethyl-3-methylbenzamide (DEET) sobre ninfas de Amblyomma cajennense. Dois bioensaios para a avaliação de repelência foram comparados e cálculos da concentração eficaz e do tempo de repelência foram realizados. Foram empregados o bioensaio da ponta do dedo, para avaliação in vivo de quatro concentações do químico (0,200; 0,100; 0,050 e 0,025 mg.cm-2) e o bioensaio do papel filtro, para a avaliação in vitro das duas concentrações mais altas. O composto conferiu mais de 90% de repelência em todas as concentrações utilizadas e 95% de repelência por mais de cinco horas na maior concentração. A concentração do composto efetiva contra 50% das ninfas testadas (CE50) foi de 0,006 mg.cm-2 e a CE99 foi de 0,036 mg.cm-2. Estas concentrações são mais baixas do que as observadas em outras espécies de carrapatos, denotando a efetividade do princípio contra A. cajennense. O tempo de repelência de 50% dos carrapatos (TR50) foi de 4,8 horas e o TR90 de 2,7 horas. Os dois bioensaios avaliados permitiram a observação de percentuais de repelência igualmente altos e se mostraram adequados para tal avaliação, sendo que o teste in vivo é mais indicado para cálculo da concentração eficaz e da duração da repelência.

This study was developed to evaluate the repellent activity of N,N-diethyl-3-methylbenzamide (DEET) against Amblyomma cajennense nymphs. Two repellent bioassays were compared and the effective concentration and repellent time were calculated. The fingertip test was accomplished to evaluate in vivo four concentrations of the compound (0.200; 0.100; 0.050 and 0.025 mg.cm-2) and the filter-paper bioassay to evaluate in vitro the two highest concentrations. The compound provided repellence higher than 90% in all concentrations and at least 95% repellency in the highest concentration over 5 hours. The effective concentration against 50% of tested nymphs (EC50) was 0.006 mg.cm-2 and the EC99 was 0.036 mg.cm-2. Those concentrations were lower than the ones obtained against other tick species, denoting the effectiveness of DEET against A. cajennense. The repellency time against 50% of the ticks (RT50) was 4.8 hours and the RT90 was 2.7 hours. Both bioassays were adequate to evaluate A. cajennense repellency and provided similar results; however the in vivo test is more appropriate to estimate the effective concentration and repellency time.

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 relacionálas com as variáveis sexo, etnia, idade atual do paciente e, ao iniciar a diálise, tempo de tratamento dialítico. MÉTODOS: Inicialmente, foram aplicados questionários em 197 pacientes distribuídos nos três centros no período de março de 2001 a janeiro de 2002. Após excluir 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%) eram do sexo masculino e 120 (74,1%), 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%) dos 162 pacientes do estudo. Destes, 38 apresentaram um único tipo de manifestação 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% de todas as MME, e o joelho foi a articulação mais acometida (52,9% dos casos). A dor óssea foi a segunda queixa mais comum (21,9%), as alterações periarticulares corresponderam a 6,8% das MME; e síndrome do túnel do carpo, deformidades ósseas e tumorações articulares ocorreram em 4,1%, 2,7% e 2,7%, respectivamente, do total de MME. Entre os 55 pacientes, alterações articulares foram encontradas em 72,7% deles, ósseas em 32,7%, neuromusculares em 10,8% e periarticulares em 9,1% 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%; 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.

A large diversity of ostearticular complaints has been described in patients in long-term hemodialysis. OBJECTIVE: To verify the proportion and the type of musculoskeletal complaints in patients in maintenance hemodialysis, at three centers of metropolitan Recife, and to relate these complains to the following variables: sex, ethnic group, patient's current age and when the dialysis began, and dialitic treatment time. METHODS: Firstly, questionnaires were applied to 197 patients, distributed at the three centers from March 2001 to January 2002. After excluding 35 patients with previous diagnosis of rheumatic disease, we investigatied the presence of musculoskeletal signs and/or symptoms in 162 patients. The average age was 47,3 years old, and 43,8 years old was the average age at the beginning of the treatment; 94 patients (58%) were men and 120 patients (74.1%) were not Caucasian. The average time of the dialitic treatment was 44,1 months, and polysulphone membrane was used in all of them. RESULTS: Musculoskeletal manifestations were observed in 55 (34%) of the patients on this research. One type of manifestation was presented by 38 of these patients, and 17 patients presented more than one type (16 presented two and 1 presented three types), making up a total of 73 manifestations, distributed among joints (44), bones (18), neuromuscular (6) and periarticular (5) structures. Arthralgia was responsible for 46.6% of all musculoskeletal complaints and the knee was the joint most injured (52.9% of all cases). Bone pain was the second common complaint (21.9%), periarticular changes were responsible for 6.8% of musculoskeletal manifestations, and carpal tunnel syndrome, bone deformities and joint tumours occurred in 4.1%, 2.7% and 2.7% of all manifestations, respectively. Among 55 patients, joint changes were found in 72.7% of them, bone changes in 32.7%, neuromuscular complaints in 10.8% and periarticular in 9.1% of the patients. The relation between the dialitic treatment time (59,8 months) and the development of the musculoskeletal manifestations (p < 0.001) was observed in this research. CONCLUSIONS: According to this information, we can conclude that the proportion of musculoskeletal complaints on our patients was 34%; the joint problems were the most frequently observed; and arthralgia was the most common complaint. Except for the dialitic treatment time, all the other variables were not associated with the musculoskeletal manifestations.