Abstract Background: The Functional Index for Hand Osteoarthritis (FIHOA) is a simple, reliable, and reproducible specific instrument to evaluate hand OA that can be applied both in clinical practice and research protocols. In order to be used in Brazil, FIHOA has to be translated into Portuguese, culturally adapted and have the reliability of the translated FIHOA version tested, which is the purpose of this study. Methods: The FIHOA was translated into Brazilian Portuguese and administered to 68 patients with hand OA recruited between May 2019 and February 2020. The test-retest was applied to 32 patients and the reliability was assessed using Spearman’s correlation coefficient and intraclass correlation coefficient (ICC). The internal consistency reliability was evaluated using Cronbach’s alpha. External construction validity was assessed using the Spearman’s correlation test between FIHOA and pain, assessed with a Visual Analogue Scale (VAS), the Cochin Hand Functional Scale (CHFS) and Health Assessment Questionnaire (HAQ). Results: The 30 participants that initially answered the translated version of the FiHOA did not report difficulties in understanding or interpreting the translated version. The test-retest reliability for the total score was strong ( r =0.86; ICC = 0.89). Mean differences (1.37 ± 0.68) using Bland Altman’s analysis did not significantly differ from zero and no systematic bias was observed. Cronbach’s alpha was also high (0.89) suggesting a strong internal coherence in the test items. There were also correlations between FIHOA and the CHFS ( r =0.88), HAQ ( r = 0.64) and pain in the hands both at rest ( r =0.55) and in motion ( r =0.44). Conclusion: The translation of the FIHOA into Brazilian Portuguese proved a valid instrument for measuring the functional capacity of patients with hand OA who understand Brazilian Portuguese.

Resumo Neurotecomas, também conhecidos como mixomas da bainha neural, são tumores benignos raros da bainha neural afetando mais comumente a cabeça, braços e ombros de mulheres entre 20 e 40 anos de idade. Devido à baixa prevalência e quadro clínico mal definido, essas lesões são raramente consideradas no diagnóstico diferencial de tumores cutâneos. Relatamos o caso de uma mulher de 24 anos de idade que procurou atendimento em 2016 relatando dor moderada por mais de um ano e limitação dos movimentos do ombro esquerdo. Ao exame, foi constatada restrição da mobilidade dessa articulação e uma ressonância magnética revelou imagem multilobular com aumento de sinal em T2 na região quadrilateral, aparentando invasão da região cortical do úmero subjacente. A histopatologia de uma biópsia incisional mostrou lesão composta por tecido conjuntivo frouxo, sem sinais de invasão, figuras de mitose ou atipias. Foi realizada excisão completa da lesão e o diagnóstico de neurotecoma foi confirmado após análise histopatológica que incluiu painel imunohistoquímico. À revisão de 18 meses, a paciente estava assintomática com recuperação completa do movimento e sem evidência de recidiva da lesão.

Abstract Neurothekeomas, also known as neural sheath myxomas, are rare benign tumors of the neural sheath affecting most commonly the head, arms and shoulder of women in their 2nd and 3rd decades of life. Due to the low prevalence and undefined clinical picture, they are hardly considered in the initial differential diagnosis of skin tumors. We report the case of a 24 year-old woman who was seen in 2016 reporting > 1 year of moderate pain and limited mobility of her left shoulder. Clinical evaluation revealed restricted mobility of the affected shoulder and nuclear magnetic resonance imaging showed a T2-weighted contrast-enhanced multilobular mass in the quadrilateral area apparently invading the adjacent humeral cortical region. Histopathology of a needle sample material revealed loose fibroconnective tissue with no signs of invasion, mitosis or atypical figures. Successful surgical excision was performed and the diagnosis of neurothekeoma was confirmed after detailed histopathology, including immunohistochemistry. The patient was asymptomatic at 18 months of follow-up, with full recovery of shoulder movement and no signs of relapse.

Abstract Background: Injection of Hylan G-F20 (HY) into joints may provoke local flares, which mechanisms may involve reaction to protein contaminants. We have previously developed a protein-free saline-soluble galactomannan derived from guar gum (GM) that displays both analgesia and chondroprotection in experimental osteoarthritis (OA). We now demonstrate that both GM and Hylan G-F20 (HY) promote mild synovitis with cytokine release after intra-articular injection. Methods: Mice received 100 μg/25 μL GM or HY or saline into the knees. Joint pain was evaluated using von Frey test; cell influx, interleukin (IL)-1, IL-6, and CXCL-1 (pg/mL) levels were assessed in joint lavage at 6 h. Synovia were excised for histopathology. Results: Neither GM nor HY after being given into mice knee joints induced pain albeit promoting mild cell influx into joint washings as well as mild synovitis at histology, with no damage to the underlying cartilage. HY but not GM promoted IL-1 release into mice joints. Both compounds induced IL-6 and CXCL-1 release. Conclusion: Intra-articular injection of HY or GM promote acute transient synovitis whilst not provoking detectable significant joint damage. Local administration of these polysaccharides induces acute intra-articular release of inflammatory cytokines, which may account for joint flares following viscosupplementation.

Resumo A artrite reumatoide (AR) é uma doença inflamatória autoimune, sistêmica, de curso progressivo, caracterizada por exuberante sinovite crônica, que pode gerar deformidades e incapacidade funcional, cujo tratamento precoce minimiza o dano às juntas. Sua etiopatogenia ainda não está completamente elucidada, mas compreende respostas imunológicas com a participação de células T auxiliares (Th1). Uma aparente menor gravidade da AR em pacientes de regiões com menor renda poderia estar associada a maior prevalência de parasitoses intestinais, especialmente as helmintíases. A rigor, um desvio na resposta imune para o predomínio de células T auxiliares (Th2), decorrente da exposição crônica a helmintos, modularia negativamente a inflamação em doentes com AR, e levaria a menor gravidade e dano articular. A revisão de aspectos da influência da reposta imunológica nas parasitoses intestinais, especialmente as helmintíases, em pacientes com artrite reumatoide é o objetivo desse trabalho.

Abstract Rheumatoid arthritis (RA) is a systemic autoimmune inflammatory disease, with a progressive course, characterized by chronic synovitis that may evolve with deformities and functional disability, and whose early treatment minimizes joint damage. Its etiopathogenesis is not fully elucidated but comprises immunologic responses mediated by T helper cells (Th1). An apparent minor severity of RA in patients from regions with lower income could be associated with a higher prevalence of gut parasites, especially helminths. Strictly, a shift in the immune response toward the predominance of T helper cells (Th2), due to the chronic exposure to helminths, could modulate negatively the inflammation in RA patients, resulting in lower severity/joint injury. The interaction between the immunological responses of parasitic helminths in rheumatoid arthritis patients is the purpose of this paper.

Resumo O padre Cícero Romão Batista é provavelmente o mais célebre personagem cearense de todos os tempos. Importante protagonista da região do Cariri, sul do Estado do Ceará, no fim do século XIX e primeiro terço do século XX, teve grande atuação política e religiosa, para além de outros feitos não tão conhecidos, como ensinamentos ecológicos, que o levaram ser agraciado com o título de Patrono das Florestas, bem como enormes esforços e sacrifício pessoal pela melhoria das condições de vida humana. Através da leitura de sua biografia, veio-nos à percepção a possibilidade de que o “Padim Ciço” tenha sido portador de espondiloartropatia inflamatória. A plausibilidade dessa hipótese diagnóstica é aqui apresentada. Busca-se enfatizar que o ouvido atento e a observação clínica, ainda que de forma indireta, sem o privilégio do contato com o paciente, constituem ferramentas incomparáveis na propositura de um diagnóstico.

Abstract Father Cicero Romao Batista is probably the most famous Ceará character of all time. An important protagonist of the Cariri region, situated in the south of Ceara State, in the late nineteenth century and the first third of the twentieth century, Father Cicero had great political and religious activity, as well as other less well-known achievements, for instance, his ecological teachings that led him to be awarded the title of “Patron of Forests”, besides an enormous effort and personal sacrifice for the improvement of the conditions of human life. Inspired by reading his biography, we find that the “Padim Ciço” could have inflammatory spondyloarthropathy. In this article, we present the plausibility of this diagnostic hypothesis, seeking to emphasize that an attentive ear and clinical observation, albeit indirectly and without the privilege of a personal contact with the patient, are unparalleled tools for bringing forth a diagnosis.

The osteoarticular involvement in sickle cell disease has been poorly studied and it is mainly characterized by osteonecrosis, osteomyelitis and arthritis. The most frequent complications and those that require hospital care in sickle cell disease patients are painful vaso-occlusive crises and osteomyelitis. The deoxygenation and polymerization of hemoglobin S, which results in sickling and vascular occlusion, occur more often in tissues with low blood flow, such as in the bones. Bone microcirculation is a common place for erythrocyte sickling, which leads to thrombosis, infarct and necrosis. The pathogenesis of microvascular occlusion, the key event in painful crises, is complex and involves activation of leukocytes, platelets and endothelial cells, as well as hemoglobin S-containing red blood cells. Osteonecrosis is a frequent complication in sickle cell disease, with a painful and debilitating pattern. It is generally insidious and progressive, affecting mainly the hips (femur head) and shoulders (humeral head). Dactylitis, also known as hand-foot syndrome, is an acute vaso-occlusive complication characterized by pain and edema in both hands and feet, frequently with increased local temperature and erythema. Osteomyelitis is the most common form of joint infection in sickle cell disease. The occurrence of connective tissue diseases, including rheumatoid arthritis and systemic lupus erythematosus, has rarely been reported in patients with sickle cell disease. The treatment of these complications is mainly symptomatic, and more detailed studies are required to understand the pathophysiological mechanisms involved in the complications and propose more adequate and specific therapies.

OBJETIVOS: A utilização do metotrexato (MTX) tem sido a base da terapia da artrite reumatoide (AR), porém ainda não temos uniformidade sobre as normas para seu uso clínico. O objetivo deste estudo foi criar recomendações baseadas em evidências científicas e opiniões de especialistas (experts) sobre o uso do MTX, as quais permitirão melhorar nossa prática clínica. MÉTODOS: O 3E (Evidence, Expertise, Exchange) Initiative in Rheumatology é um grupo multinacional de reumatologistas oriundos de 17 países, incluindo o Brasil. Após uma seleção de dez questões sobre o uso de MTX, feita pelo método Delphi, realizou-se uma revisão sistemática da literatura (RSL) (Medline, Pubmed, Embase, Cochrane, Abstracts EULAR 2005-2007 e ACR 2006-2007) por seis revisores bibliográficos internacionais escolhidos pelos mentores do estudo 3E. Duas diferentes questões nacionais do Brasil também foram incluídas e essa pesquisa foi realizada por um revisor bibliográfico nacional.** Os resultados da RSL foram apresentados por sete membros do comitê científico brasileiro do 3E*, em um encontro nacional de 48 reumatologistas, os quais discutiram as informações da RSL, votaram e elaboraram recomendações nacionais aqui apresentadas. Estas foram utilizadas posteriormente na criação de recomendações multinacionais. RESULTADOS E CONCLUSÕES: Formularam-se 21 recomendações acerca das dez questões internacionais e das duas questões nacionais, com um nível de concordância entre os participantes de 77% (63 a 100%). O MTX é indicado inicialmente por via oral, na dose mínima de 10 mg/sem e máxima de 25 mg/sem. A elevação de AST/ALT acima de 3 vezes o limite superior do valor normal, por pelo menos três vezes, justifica a suspensão temporária do MTX, podendo-se reinstituir com a normalização dos valores encontrados. MTX é seguro a longo prazo. O uso de álcool ( > 100 g/sem) deve ser evitado. Recomenda-se combinação do MTX com drogas antirreumáticas modificadoras da doença (DMARDs), embora haja risco de maior toxicidade. Ácido fólico em dose maior que 5 mg/sem deve ser associado. Devem-se solicitar hemograma, creatinina, AST/ALT, sorologia para vírus B e C da hepatite e raio X de tórax antes de iniciar o MTX, e deve-se inquirir sobre contracepção, comorbidades, uso de drogas ilícitas e álcool, hepatopatias e medicamentos hepatotóxicos. O MTX pode ser mantido durante cirurgias eletivas. Sugere-se a interrupção do MTX por, pelo menos, três meses antes do planejamento de gravidez, tanto em homens quanto em mulheres. Justifica-se a utilização de métodos de contracepção com o uso de MTX em idade reprodutiva. Pode-se usar MTX como poupador de corticoide em pacientes com arterite de células gigantes, polimialgia reumática (PMR), dermatomiosite juvenil e lúpus eritematoso sistêmico (LES) com envolvimento cutâneo e/ou articular.

OBJECTIVES: The use of methotrexate (MTX) has been the basis for rheumatoid arthritis (RA) therapy, but there is no uniformity on the guidelines for its clinical use. The objective of this study was to develop recommendations based on scientific evidence and opinions of experts on the use of MTX, which will allow the improvement of our clinical practice. METHODS: 3E (Evidence, Expertise, Exchange) Initiative in Rheumatology is a multinational group of rheumatologists from 17 countries, including Brazil. After a selection of 10 questions about the use of MTX, held by the Delphi method, a systematic literature review (SLR) was done (Medline, Pubmed, Embase, Cochrane, Abstracts EULAR 2005-2007 and ACR 2006-2007) by six international bibliographic reviewers chosen by the mentors of the 3E study. Two other different national questions from Brazil were also included, and the SLR was done by a national bibliographic reviewer.** The results of SLR were presented by 7 members of our Brazilian 3E scientific committee* at a meeting of 48 rheumatologists, which discussed RSL details, voted, and produced the national recommendations presented here. These recommendations were subsequently used in the creation of multinational recommendations. RESULTS AND CONCLUSIONS: 21 recommendations concerning the 10 international and the 2 national questions were formulated, with an agreement level of 77% among the participants (63-100%). Oral MTX should be started at a minimum dose of 10 mg/wk and a maximum dose of 25 mg/wk. Elevation of AST/ALT above 3x the upper limit, for at least 3 times consecutively, justifies the temporary suspension of MTX, which can be restored after normalization of serum liver enzyme levels; MTX is safe for long term use. The use of alcohol (100 g/wk) should be avoided. Combinations of MTX with disease modifying antirheumatic drugs are recommended, although there is risk of greater toxicity. Folic acid should be associated with MTX in dose higher than 5 mg/wk. Total blood cell count, creatinine, AST/ALT, serology for hepatitis B and C virus, and chest X-ray should be ordered before initiating MTX. Inquire about contraception methods, comorbidities, use of illicit drugs, alcohol, and liver diseases and hepatotoxic drugs should be performed. The MTX can be maintained during elective surgeries; discontinuation of MTX for at least 3 months before planning of pregnancy is suggested, for both men and women Use of contraception method is justified with the use of MTX in reproductive age. MTX can be used to reduce the cumulative dose of corticosteroid in patients with giant cell arthritis, rheumatic polymyalgia (RPM), juvenile dermatomyositis, and in systemic lupus erythematosus (SLE) with cutaneous or joint involvement.

INTRODUÇÃO: O uso de ciclofosfamida endovenosa é uma opção terapêutica nas formas graves de lúpus eritematoso sistêmico (LES). No entanto, a toxicidade e o perfil de risco ainda não estão adequadamente definidos. OBJETIVO: Avaliar sobre a ocorrência de alterações do sono em pacientes portadores de LES submetidos à terapia com ciclofosfamida endovenosa. MÉTODOS: Nós estudamos 30 casos consecutivos (27 do sexo feminino) com idade entre 14 e 53 anos (30,5 ± 10), em pulsoterapia com ciclofosfamida (mg) (média 948,27 ± 221,39). Os pacientes foram avaliados quanto à presença de sintomas depressivos, qualidade do sono e sonolência excessiva diurna (SED). A qualidade do sono foi estudada pelo índice de qualidade do sono de Pittsburgh (IQSP), a SED pela escala de sonolência de Epworth e os sintomas depressivos pelo Inventário de Depressão de Beck (21 itens). A gravidade da doença foi avaliada por intermédio do SLEDAI. RESULTADOS: O SLEDAI oscilou entre 2 e 46 (17 ± 11,4). Hipertensão (30%), anemia (23,3%), osteoporose (23,3%) e miocardiopatia (6,6%) foram as comorbidades mais observadas. Um paciente tinha história de convulsões (3,3%). Má qualidade do sono (PSQI e" 6) foi encontrada em 66,7% e SED (ESS > 10), em 30% dos pacientes. Sintomas de depressão (BDI > 19) estavam presentes em 40% dos casos. Os sintomas depressivos associaram-se à presença de má qualidade do sono (p = 0,03). CONCLUSÕES: Nosso estudo mostra que alterações do sono e sintomas depressivos são comuns em pacientes portadores de LES em pulsoterapia com ciclofosfamida. Esses achados são similares a outros estudos previamente relatados e são independentes do tipo de tratamento utilizado.

BACKGROUND: Pulse i.v. cyclophosphamide is a therapeutic option in severe forms of systemic lupus erythematosus (SLE). However, the overall toxicity and risk profile are yet to be adequately defined. OBJECTIVE: To evaluate the occurrence of sleep disturbances in SLE patients subjected to i.v. cyclophosphamide. METHODS: We studied thirty consecutive SLE patients (27 female) age range 14 to 53 years (mean 30.5 ± 10 years) that received i.v. cyclophosphamide (mg) (mean 948.27 ± 221.39). Depressive symptoms, quality of sleep, and the presence of excessive daytime sleepiness were evaluated. Disease severity was assessed by the SLEDAI. Quality of sleep was assessed by the Pittsburgh Sleep Quality Index (PSQI) and excessive daytime sleepiness (EDS) by the Epworth Sleepiness Scale (ESS). Depressive symptoms were evaluated using the 21-item Beck Depression Inventory (BDI). RESULTS: SLEDAI values ranged from 2 to 46 (mean 17 ± 11.4). The most common comorbidities were systemic arterial hypertension (30%), anemia (23.3%), osteoporosis (23.3%), and cardiomyopathy (6.6%). Seizures occurred in one patient (3.3%). Poor quality of sleep (PSQI e" 6) and EDS (ESS >10) were found in 66.7% and 30% of the patients, respectively. Depressive symptoms (BDI >19) were present in 40% of the patients and were associated with poor sleep quality (P = 0.03). CONCLUSIONS: Our findings show an increased prevalence of poor sleep quality and depressive symptoms in SLE patients receiving pulse i.v. cyclophosphamide. These findings were similar to other previously reported series of SLE patients regardless of the therapies used.