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.

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.

Os autores revisam a literatura sobre os possíveis efeitos da gravidez no desencadeamento de osteoporose, juntamente com uma breve discussão de aspectos da regulação do metabolismo do cálcio na mulher grávida. A carência de estudos a esse respeito é ressaltada. Os autores enfatizam a necessidade de estudos em nossa população, enfocando a possível associação entre paridade e fraturas decorrentes de osteoporose.

The authors review the literature approaching the possible effects of pregnancy in the development of osteoporosis, coupled to a brief discussion of aspects about the regulation of calcium metabolism in pregnant women. The lack of studies focusing this subject is remarked. The authors emphasize the need for studies based on our population, approaching the possible association of parity and osteoporosis related fractures.

OBJETIVO: fraturas do quadril em pacientes acima de 60 anos representam séria morbidade ligada à osteoporose. Influências genéticas e ambientais foram relatadas como associadas à epidemiologia da osteoporose. Sobral, localizada no nordeste do Brasil (3º41'10" S/40º20'59"E), tem 138.565 mil habitantes que são predominantemente descendentes de brancos portugueses e índios brasileiros. MÉTODO: avaliamos a ocorrência de fraturas do quadril em Sobral entre julho de 1996 e junho de 2000. Os dados foram obtidos dos prontuários médicos da Santa Casa de Sobral, que é o hospital de referência local. Todos os registros de pacientes maiores de 20 anos com diagnóstico de fratura do fêmur ou do quadril foram revisados. RESULTADOS: identificamos no total 79 fraturas. Dez (12,6%), que ocorreram em menores de 50 anos, foram excluídas da análise. Sessenta e nove (87,3%) ocorreram em maiores de 50 anos, compreendendo 19 (27,5%) homens e 50 (72,4%) mulheres, com uma incidência anual idadeajustada de 5,59/10.000/ano em homens e 12,4/10.000/ano em mulheres, respectivamente, para essa faixa etária. Dois pacientes, de 79 e 82 anos, morreram 9 e 6 dias após hospitalização, respectivamente. A média de tempo de hospitalização foi de 7,5 dias (variação de 4-19 dias). CONCLUSÕES: Houve tendência a acréscimo na incidência de fraturas de quadril em pessoas acima de 50 anos. A incidência de fraturas do quadril nessa população foi cerca de quatro vezes menor que a relatada em brancos caucasóides.

OBJECTIVE: Hip fractures in patients older than 60 years old represent a serious morbidity linked to osteoporosis. Both genetic and environmental influences have been reported as important factors related to the epidemiology of osteoporosis. Sobral is located in the northeast of Brazil (3º41'10" S/40º20'59"E) and has 138,565 inhabitants, which are mostly descendants from white Portuguese and native Brazilian. METHODS: We evaluated the occurrence of hip fractures in Sobral between July/1996 and June/2000. This was a retrospective cohort based study. Data were obtained from the medical records of the Santa Casa de Sobral that is the reference hospital in this area. All chart records of patients aged more than 20 years old with a diagnosis of femoral or hip fracture were revised. RESULTS: A total of 79 fractures were identified. Ten (12.6%) cases that occurred in patients less than 50 years old were excluded from further analysis. Sixty-nine cases (87.3%) occurred in patients aged more than 50 years old comprising 19 (27.5%) men and 50 (72.4%) women giving an age-adjusted annual incidence rate of 5.59/10,000/ year in men and 12.4/10,000/year in women, respectively for people in this age range. Two patients, aged 79 and 82, died 9 and 6 days after hospital admission, respectively. The average time of hospitalization was 7.5 days (range 4-19 days). CONCLUSIONS: There was a tendency to a rise in the incidence of hip fractures in people older than 50 years old. Interestingly, the hip fracture rate in this population is considerably lower (around four times less) than the reported rates in white Caucasians.

OBJETIVO: Investigar o papel do óxido nítrico (NO) no influxo celular (IC) e a incapacitação articular (IA) na artrite por zymosan (AZy). MÉTODOS: Ratos Wistar receberam injeção de Zy (1mg) no joelho direito. Grupos-controles receberam salina. A IA foi avaliada pelo Teste de incapacitação articular para ratos. Após sacrifício, o IC e a liberação de PGE2 foram avaliados no lavado articular. Avaliou-se o edema pela diferença peso úmido/peso seco do tecido articular. Grupos foram tratados i.p. com L-NAME - inibidor de sintases de NO (NOS) (LN=30-300mg/kg) ou aminoguanidina (AG= 30-300mg/kg) - inibidor seletivo de iNOS 30 min antes ou 2 h após o Zy. RESULTADOS: O pré-tratamento com L-NAME ou AG inibiu de forma dose-dependente a IA e o IC, mas não o edema ou a liberação de PGE2, sendo máximo para LN ou AG (100mg/kg). L-arginina (1g/kg) co-injetada com LN (100mg/kg) reverteu a inibição da IA e do IC. D-NAME (100mg/kg) não teve efeito. Co-injeção de naloxona (antagonista opióide) reverteu o efeito de LN e AG sobre a IA. A pressão arterial média elevou-se (p<0,01) após LN (100mg/kg), mas não com AG (100mg/kg). LN e AG (100mg/kg) dados 2 h após Zy não tiveram efeito. Azul de metileno (inibidor de GMPc), e não hemoglobina (scavenger de NO), reduziu IA e IC (p<0,01). Nitroprussiato de sódio (doador de NO) dado 2 h após Zy, reduziu a IA, mas não o IC (p<0,01). CONCLUSÕES: A antinocicepção na AZy por inibição da iNOS só ocorre como pré-tratamento, por efeito antiinflamatório via GMPc, independentemente de NO exógeno ou de PGE2, associada à liberação de opióides endógenos. Um doador de NO produz analgesia na AZy. Enfim, o trabalho demonstra efeito analgésico de um doador de NO em um modelo de periartrite, abrindo a perspectiva do uso dessas substâncias em humanos.

OBJECTIVE: To study the role of nitric oxide (NO) in the cell influx (CI) and the articular incapacitation (AI) in the zymosan induced-arthritis (ZyA). METHODS: Wistar rats received 1 mg of zymosan (Zy) into the right knee joint, and controls received saline. The AI was evaluated by the Test for AI in rats. After sacrifice, the CI and the release of prostaglandin E2 (PGE2) were assessed in the joint exudate. The joint edema was measured by the wet/dry weight ratio of the articular tissue. Groups were treated i.p. with L-NAME - NO synthase (NOS) inhibitor (LN = 30-300 mg/kg) or aminoguanidine (AG = 30-300 mg/kg) - selective iNOS inhibitor - 30 minutes before or 2 hours after the Zy. RESULTS: Pretreatment with L-NAME or AG dose-dependently inhibited the AI and the CI, but not altering edema or PGE2 release, being maximal for the 100 mg/kg doses of L-N and AG. Coinjection of L-arginine (1g/kg) and LN (100 mg/kg) reversed the inhibition of the AI and of the CI. D-NAME (100 mg/kg) had no effect. Coinjection of naloxone (opioid antagonist) reversed LN or AG effect on the AI. Mean arterial pressure increased (p<0,01) with LN (100 mg/kg), but not with AG (100 mg/ kg). LN and AG (100 mg/kg) given 2 hours after the Zy had no effect. Methylene blue (GMPc inhibitor), but not hemoglobin (NO scavenger) reversed AI and CI (p<0,01). Sodium nitroprusside (NO donor) given 2 hours after Zy reduced significantly AI, but not CI (p<0,01). CONCLUSIONS: The antinociceptive effect of iNOS inhibition in ZyA happens only with a prophylactic administration, due to an anti-inflammatory effect via GMPc, independently of exogenous NO or PGE2 release, but is associated to endogenous opioid release. The administration of an NO donor produces analgesia in ZyA.