The idiopathic inflammatory myopathies module of the Rheumatic Diseases Portuguese Register.

AIMS: To characterise the idiopathic inflammatory myopathies (IIM) module of the Rheumatic Diseases Portuguese Register (Reuma.pt/myositis) and the patients in its cohort. METHODS: Reuma.pt is a web-based system with standardised patient files gathered in a registry. This was a multicentre open cohort study, including patients registered in Reuma.pt/myositis up to January 2022. RESULTS: Reuma.pt/myositis was designed to record all relevant data in clinical practice and includes disease-specific diagnosis and classification criteria, clinical manifestations, immunological data, and disease activity scores. Two hundred eighty patients were included, 71.4% female, 89.4% Caucasian, with a median age at diagnosis and disease duration of 48.9 (33.6-59.3) and 5.3 (3.0-9.8) years. Patients were classified as having definite (N=57/118, 48.3%), likely (N=23/118, 19.5%), or possible (N=2/118, 1.7%) IIM by 2017 EULAR/ACR criteria. The most common disease subtypes were dermatomyositis (DM, N=122/280, 43.6%), polymyositis (N=59/280, 21.1%), and myositis in overlap syndromes (N=41/280, 14.6%). The most common symptoms were proximal muscle weakness (N=180/215, 83.7%) and arthralgia (N=127/249, 52.9%), and the most common clinical signs were Gottron's sign (N=75/184, 40.8%) and heliotrope rash (N=101/252, 40.1%). Organ involvement included lung (N=78/230, 33.9%) and heart (N=11/229, 4.8%) involvements. Most patients expressed myositis-specific (MSA, N=158/242, 65.3%) or myositis-associated (MAA, 112/242, 46.3%) antibodies. The most frequent were anti-SSA/SSB (N=70/231, 30.3%), anti-Jo1 (N=56/236, 23.7%), and anti-Mi2 (N=31/212, 14.6%). Most patients had a myopathic pattern on electromyogram (N=101/138, 73.2%), muscle oedema in magnetic resonance (N=33/62, 53.2%), and high CK (N=154/200, 55.0%) and aldolase levels (N=74/135, 54.8%). Cancer was found in 11/127 patients (8.7%), most commonly breast cancer (N=3/11, 27.3%). Most patients with cancer-associated myositis had DM (N=8/11, 72.7%) and expressed MSA (N=6/11) and/or MAA (N=3/11). The most used drugs were glucocorticoids (N=201/280, 71.8%), methotrexate (N=117/280, 41.8%), hydroxychloroquine (N=87/280, 31.1%), azathioprine (N=85/280, 30.4%), and mycophenolate mofetil (N=56/280, 20.0%). At the last follow-up, there was a median MMT8 of 150 (142-150), modified DAS skin of 0 (0-1), global VAS of 10 (0-50) mm, and HAQ of 0.125 (0.000-1.125). CONCLUSIONS: Reuma.pt/myositis adequately captures the main features of inflammatory myopathies' patients, depicting, in this first report, a heterogeneous population with frequent muscle, joint, skin, and lung involvements.

Assessment of calcinosis in Portuguese patients with systemic sclerosis - a multicenter study.

INTRODUCTION/OBJECTIVES: The study aims to define the clinical and subclinical calcinosis prevalence, the sensitivity of radiographed site and clinical method for its diagnosis, and the phenotype of Portuguese systemic sclerosis (SSc) patients with calcinosis. METHOD: A cross-sectional multicenter study was conducted with SSc patients fulfilling Leroy/Medsger 2001 or ACR/EULAR 2013 classification criteria, registered in the Reuma.pt. Calcinosis was assessed through clinical examination and radiographs of hands, elbows, knees, and feet. Independent parametric or non-parametric tests, multivariate logistic regression, and sensitivity calculation of radiographed site and clinical method for calcinosis detection were performed. RESULTS: We included 226 patients. Clinical calcinosis was described in 63 (28.1%) and radiological calcinosis in 91 (40.3%) patients, of which 37 (40.7%) were subclinical. The most sensitive location to detect calcinosis was the hand (74.7%). Sensitivity of the clinical method was 58.2%. Calcinosis patients were more often female (p = 0.008) and older (p < 0.001) and had more frequently longer disease duration (p < 0.001), limited SSc (p = 0.017), telangiectasia (p = 0.039), digital ulcers (p = 0.001), esophageal (p < 0.001) and intestinal (p = 0.003) involvements, osteoporosis (p = 0.028), and late capillaroscopic pattern (p < 0.001). In multivariate analysis, digital ulcers (OR 2.63, 95% CI 1.02-6.78, p = 0.045) predicted overall calcinosis, esophageal involvement (OR 3.52, 95% CI 1.28-9.67, p = 0.015) and osteoporosis (OR 4.1, 95% CI 1.2-14.2, p = 0.027) predicted hand calcinosis, and late capillaroscopic pattern (OR 7.6, 95% CI 1.7-34.9, p = 0.009) predicted knee calcinosis. Anti-nuclear antibody positivity was associated with less knee calcinosis (OR 0.021, 95% CI 0.001-0477, p = 0.015). CONCLUSIONS: Subclinical calcinosis high prevalence suggests that calcinosis is underdiagnosed and radiographic screening might be relevant. Multifactorial pathogenesis may explain calcinosis predictors' variability. Key Points • Prevalence of subclinical calcinosis in SSc patients is substantial. • Hand radiographs are more sensitive to detect calcinosis than other locations or clinical method. • Digital ulcers were associated with overall calcinosis, esophageal involvement and osteoporosis were associated with hand calcinosis, and late sclerodermic pattern in nailfold capillaroscopy was associated with knee calcinosis. • Anti-nuclear antibody positivity may be a protective factor for knee calcinosis.

Predictors of cardiac involvement in idiopathic inflammatory myopathies.

Objectives: Idiopathic inflammatory myopathies (IIM) are a group of rare disorders that can affect the heart. This work aimed to find predictors of cardiac involvement in IIM. Methods: Multicenter, open cohort study, including patients registered in the IIM module of the Rheumatic Diseases Portuguese Register (Reuma.pt/Myositis) until January 2022. Patients without cardiac involvement information were excluded. Myo(peri)carditis, dilated cardiomyopathy, conduction abnormalities, and/or premature coronary artery disease were considered. Results: 230 patients were included, 163 (70.9%) of whom were females. Thirteen patients (5.7%) had cardiac involvement. Compared with IIM patients without cardiac involvement, these patients had a lower bilateral manual muscle testing score (MMT) at the peak of muscle weakness [108.0 ± 55.0 vs 147.5 ± 22.0, p=0.008] and more frequently had oesophageal [6/12 (50.0%) vs 33/207 (15.9%), p=0.009] and lung [10/13 (76.9%) vs 68/216 (31.5%), p=0.001] involvements. Anti-SRP antibodies were more commonly identified in patients with cardiac involvement [3/11 (27.3%) vs 9/174 (5.2%), p=0.026]. In the multivariate analysis, positivity for anti-SRP antibodies (OR 104.3, 95% CI: 2.5-4277.8, p=0.014) was a predictor of cardiac involvement, regardless of sex, ethnicity, age at diagnosis, and lung involvement. Sensitivity analysis confirmed these results. Conclusion: Anti-SRP antibodies were predictors of cardiac involvement in our cohort of IIM patients, irrespective of demographical characteristics and lung involvement. We suggest considering frequent screening for heart involvement in anti-SRP-positive IIM patients.

CSN1S1 and CSN3 gene variants in female Murrah buffaloes in the Brazilian Amazon.

The characteristics of milk are controlled by several genes, with emphasis on the four genes from casein, CSN1S1; CSN1S2; CSN2 and CSN3, which are responsible encoding of fractions the milk protein. The study of genetic variants in these genes, seek to investigate alleles, insertions or deletions, that can directly reflect on productive characteristics, indicating differences in milk quality, composition and yield. The CSN1S1 and CSN3 genes were analyzed in lactating Murrah buffaloes using nucleotide sequencing. An SNP was found in the amplified fragment of the CSN1S1 gene, located in nucleotide number 2,123 of the promoter region in position nt-258 (A/G). As for the CSN3 gene, two SNPs of exon number 4 were identified in codons 33 (ACC/ATC) and 34 (ACC/ACT) of the analyzed fragment. This study contributes to important associations between genetic variants and the desired characteristics of milk and its derivatives in future studies, because the variants found may be associated with the quality of milk, enabling genetic selection to be assisted by molecular markers, indicating a major advance that makes it possible to select animals early.

Risk factors associated with the bovine subclinical mastitis in an Amazon micro-region.

In this study, we evaluated the main risk factors for the occurrence of bovine mastitis, in the southeastern of Pará, in the Brazilian Amazon. We surveyed 91 dairy farmers to identify management practices and bovine breed characteristics. From each farm, 50 mL of milk sample was collected for microbiological analysis and somatic cell count (SCC). Depending on the management practices and breed, a logit model was used to determine the odds ratio of subclinical mastitis (SCM) occurrence. In irrigated pastures, an SCM-associated risk factor, the occurrence of SCM was 5.03 times higher than that in the non-irrigated pastures. Similarly, in Girolando breed herds, the occurrence of SCM increased by 5.8 times compared to the crossbred herds. Moreover, the occurrence of mastitis was 33 times higher in farms using common cloths for drying teats than in farms using paper towels. Therefore, adoption of better management practices can lead to SCC reduction, milk quality improvement and a guarantee to contain SCC within prescribed Brazilian limits for the Amazon region.

Isolation and evaluation of quinoa (Chenopodium quinoa Willd.) protein fractions. A nutritional and bio-functional approach to the globulin fraction.

This study evaluated the solubility profiles of quinoa grain proteins and applied a complete process for the isolation of its main protein fractions, namely: albumins, globulins, prolamins and glutelins, which corresponded to 26.96%, 41.3%, 1.7% and 23.16% of the total protein content, respectively. When these fractions were digested with pepsin followed by pancreatin, the degrees of hydrolysis achieved varied between 26.62% (for unheated globulin fraction) and 38.97% (for unheated glutelin), with casein reached 33.73% hydrolysis. After heating, the globulin hydrolysis degree increased to 34.7%, not significantly differing from casein. These results reflect its good susceptibility to hydrolysis by digestive enzymes, and this observation is reinforced with assays with pepsin, trypsin and chymotrypsin tested separately. Globulins, the largest protein fraction, showed promising results in additional assays regarding the amino acid profile, with limitation only for lysine in relation to the FAO standard, and the potential for releasing bioactive peptides after digestion. Although pepsin-digested globulin inhibited only 5% of ACE activity under the conditions tested, after 24h with the addition of pancreatin, the inhibition was 100%. Antioxidant activity (DPPH assay) also indicated very similar results, when hydrolysis with pepsin was inefficient in releasing antioxidant peptides, while hydrolysis by pancreatin led to 35 times greater results.

Predictors of hospitalization in patients with systemic lupus erythematosus: a 10-year cohort study.

INTRODUCTION/OBJECTIVES: Recognising systemic lupus erythematosus (SLE) patients at higher risk for hospitalization, aiming at developing tailored management strategies, may help minimize admissions and improve long-term health outcomes. Our study aimed to identify predictors for hospitalization in patients with SLE. METHOD: Cohort study of SLE patients followed in a referral centre. All hospitalizations from study baseline up to 120 months were identified, and the primary indication for admission was categorized as follows: (1) SLE disease activity; (2); infection; and (3) other conditions. Demographic, clinical, and laboratory parameters at baseline were sought as predictors of hospitalization for (i) any cause, (ii) disease activity, and (iii) infection using survival analysis with Kaplan-Meier curves and log-rank tests. Potential predictors were further tested using multivariate Cox proportional hazards regression models. RESULTS: We included 398 patients (median follow-up: 120 months). The incidence rate of hospitalization was 17.7 per 100 patient-years. The most frequent indications for hospitalization were SLE disease activity (29.4%) and infection (23.4%). In multivariate analysis, male gender, age > 50 years, antiphospholipid antibodies positivity (aPL), SLEDAI-2 K > 5, organ damage, and prednisone daily dose (PDN) predicted hospitalization for any cause. SLEDAI-2 K > 5, aPL, PDN, and IS medication predicted hospitalization for active SLE. Male gender, prior biopsy-proven lupus nephritis, aPL, organ damage, and ongoing treatment with high-risk IS predicted hospitalization for infection. Treatment with antimalarials was associated with a lower risk of hospitalization for any cause and for infection. CONCLUSIONS: Positive aPL identifies SLE patients presenting a higher risk of hospitalization, while medication with antimalarials was associated with a lower risk. Key Points • Positive aPL is predictive of hospitalization for any medical condition, disease activity, and infection • Organ damage is predictive of hospitalization for any condition and infection • Antimalarials are predictive of a lower risk of hospitalization for any condition and infection.

Long-term Outcome of Children Born to Women with Autoimmune Rheumatic Diseases: A Multicentre, Nationwide Study on 299 Randomly Selected Individuals.

The concern about the offspring's health is one of the reasons for a reduced family size of women with rheumatic diseases (RD). Increased risk of autoimmune diseases (AD) and neurodevelopmental disorders (ND) has been reported in children born to patients with RD. Within a nationwide survey about reproductive issues of women with RD, we aimed at exploring the long-term outcome of their children. By surveying 398 patients who received their diagnosis of RD during childbearing age (before the age of 45), information about the offspring were obtained from 230 women who declared to have had children. A total of 148 (64.3%) patients were affected by connective tissue diseases (CTD) and 82 (35.7%) by chronic arthritis. Data on 299 children (156 males, 52.1%; mean age at the time of interview 17.1 ± 9.7 years) were collected. Twelve children (4.0%), who were born to patients with CTD in 75% of the cases, were affected by AD (8 cases of celiac disease). Eleven children had a certified diagnosis of ND (3.6%; 6 cases of learning disabilities); 9 of them were born to mothers with CTD (5 after maternal diagnosis). No association was found between ND and prenatal exposure to either maternal autoantibodies or anti-rheumatic drugs. Absolute numbers of offspring affected by AD and ND were low in a multicentre cohort of Italian women with RD. This information can be helpful for the counselling about reproductive issues, as the health outcomes of the offspring might not be an issue which discourage women with RD from having children.

Postpartum Ankle Pain: A Suspected Complex Regional Pain Syndrome or a Rare Cause of Septic Arthritis?

Ankle tuberculosis is a relatively rare condition and may develop after hematogenous dissemination from the pulmonary origin, particularly in cases of immunosuppression. Both pregnancy and delivery are relatively immunosuppressive states, and immune modulations during these periods can contribute to the pathogenesis of disseminated tuberculosis. A 26-year-old mother presented with severe, continuous, and debilitating pain in the left ankle, lasting for three months after delivery and associated with fever. Inspection demonstrated ankle swelling and redness, with a cold and cyanotic forefoot. Ankle radiograph and musculoskeletal ultrasound evaluation were obtained. Tibiotalar joint arthrocentesis revealed purulent liquid suggestive of septic arthritis and an emergent arthroscopic washout of the ankle was performed. The synovial mycobacterial culture was posteriorly positive and the diagnosis established was both pulmonary and osteoarticular tuberculosis. A comprehensive rehabilitation program was then implemented to achieve maximum functional gains. This report presents a rare case of ankle tuberculosis diagnosed in the postpartum period. Early evaluation, treatment, and adequate rehabilitation interventions can be crucial to promote functionality and enhance the quality of life.

Novel Microsatellite Markers Used for Determining Genetic Diversity and Tracing of Wild and Farmed Populations of the Amazonian Giant Fish Arapaima gigas.

The Amazonian symbol fish Arapaima gigas is the only living representative of the Arapamidae family. Environmental pressures and illegal fishing threaten the species' survival. To protect wild populations, a national regulation must be developed for the management of A. gigas throughout the Amazon basin. Moreover, the reproductive genetic management and recruitment of additional founders by aquaculture farms are needed to mitigate the damage caused by domestication. To contribute to the sustainable development, we investigated the genetic diversity of wild and cultivated populations of A. gigas and developed a panel composed by 12 microsatellite markers for individual and population genetic tracing. We analyzed 368 samples from three wild and four farmed populations. The results revealed low rates of genetic diversity in all populations, loss of genetic diversity and high inbreeding rates in farmed populations, and genetic structuring among wild and farmed populations. Genetic tracing using the 12 microsatellite markers was effective, and presented a better performance in identifying samples at the population level. The 12-microsatellite panel is appliable to the legal aspects of the trade of the A. gigas, such as origin discrimination, reproductive genetic management by DNA profiling, and evaluation and monitoring of genetic diversity.

Carcass and commercial cuts yields of caiman (Caiman crocodilus yacare) farmed in a ranching system in the Brazilian Pantanal.

Given the growing use of alternative sources of protein, studies on the commercial viability of amazonians wild species, based on the yield of their carcass, are still scarce in the literature. To evaluate yields of carcass and commercial cuts of caiman meat (Caiman crocodilus yacare), according to sex and weight categories, besides analyzing their revenue. Thirty animals in termination phase were selected, separated by sex and weighed. The cuts analyzed was of 59.7% in relation to total live weight, and of 70.7% in relation to the carcass. Regarding the yields of the cuts, the average weight difference in detriment to the sex of the animals was minimal, however, in the weight categories, animals classified as intermediate and heavy (5.9-9.1 kg) resulted in higher cut weights (p < 0.01) and consequently in revenue for most of the meat cuts (66.7%), especially for tail, back, and sirloin fillets. It can be concluded that the carcass yield of caiman is high, and that heavy animals resulted in higher yields of prime cuts, emphasizing the need to carry out studies that prioritize the cost/benefit ratio taking into account the age and weight of the animals.

Genomic approach for conservation and the sustainable management of endangered species of the Amazon.

A broad panel of potentially amplifiable microsatellite loci and a multiplex system were developed for the Amazonian symbol fish species Arapaima gigas, which is currently in high danger of extinction due to the disorderly fishing exploitation. Several factors have contributed to the increase of this threat, among which we highlight the lack of genetic information about the structure and taxonomic status of the species, as well as the lack of accurate tools for evaluation of the effectivity of current management programs. Based on Arapaima gigas' whole genome, available at the NCBI database (ID: 12404), a total of 95,098 unique perfect microsatellites were identified, including their proposed primers. From this panel, a multiplex system containing 12 tetranucleotide microsatellite markers was validated. These tools are valuable for research in as many areas as bioinformatics, ecology, genetics, evolution and comparative studies, since they are able to provide more accurate information for fishing management, conservation of wild populations and genetic management of aquaculture.

Avaliação da capacidade funcional, força muscular respiratória e pico de fluxo expiratório em pacientes com apneia obstrutiva do sono / Evaluation of functional capacity, respiratory muscular strength and expiratory flow peak in patients with obstructive sleep apnea

Introdução: A síndrome da apneia obstrutiva do sono (SAOS) é caracterizada pela obstrução das vias aéreas superiores durante o sono, o que causa efeitos sistêmicos durante a vigília. Objetivo: O objetivo deste estudo é avaliar a capacidade funcional, a força da musculatura respiratória e o pico de fluxo expiratório (PFE) de pacientes com SAOS. Métodos: Trata-se de um estudo transversal realizado na cidade de Patos de Minas/MG no ano de 2019, no qual foram incluídos 14 indivíduos que possuíam SAOS moderada e grave. Primeiramente os pacientes responderam a um questionário sociodemográfico elaborado pelos autores. Em seguida, foi realizada a manovacuometria, a mensuração do PFE e o teste de caminhada de seis minutos (TC6M). Resultados: A maioria dos pacientes eram homens (71,4%), obesos (78,6%) e hipertensos (57,1%). Como resultados dos testes obtivemos - PImáx obtida vs predita = 86,4 vs 102,8 (p = 0,004), PEmáx obtida vs predita = 98,5 vs 113,5 (p = 0,05), PFE obtido vs predito = 381,3 vs 495,8 (p < 0,001) e TC6M obtido vs predito = 485,3m vs 536,6m (p = 0,03). Conclusão: Nessa amostra de indivíduos com SAOS grave e moderada houve redução da força muscular respiratória, do PFE, e da capacidade funcional. (AU)

Introduction: Obstructive sleep apnea syndrome (OSAS) is characterized by upper airway obstruction during sleep, which causes systemic effects during wakefulness. Objective: The objective of this study is to assess functional capacity, respiratory muscle strength and peak expiratory flow (PEF) in patients with OSAS. Methods: This is a cross-sectional study held in the city of Patos de Minas/MG in the year of 2019, that included 14 individuals who had severe and moderate sleep apnea. First, the patients answered to a sociodemographic questionnaire prepared by the authors. Then, manovacuometry, PEF and six-minute walk test (6MWT) were performed. Results: Most patients were men (71.4%), obese (78.6%) and hypertensive (57.1%). As results of the tests we obtained - MIP obtained vs predicted = 86.4 vs 102.8 (p = 0.004), MPE obtained vs predicted = 98.5 vs 113.5 (p = 0.05), PEF obtained vs predicted = 381.3 vs 495.8 (p <0.001) and 6MWT obtained vs predicted = 485.3m vs 536.6m (p = 0.03). Conclusion: In this sample of individuals with severe and moderate OSAS, we observed a reduction in respiratory muscle strength, PEF, and functional capacity. (AU)

Tolerance to irrigation water salinity in physalis plants: productive aspects / Tolerância à salinidade da água de irrigação em plantas de fisális: aspectos produtivos

The cultivation of non-traditional fruits has gained ground in the horticulture sector, but which, in certain situations, are plants that require previous studies related to soil adaptability, climate, and irrigation water quality. In this sense, this work aimed to evaluate the effects of irrigation water salinity on Physalis peruviana L. (fisális) plants in the different growth phases. The experimental area was installed on the premises of the Federal University of Campina Grande, adopting a casualized block design, with four saline levels of irrigation water (0.3; 1.2; 2.1 and 3.0 dS m-1) and five repetitions per treatment. The variables analyzed were: stem diameter, plant height and number of leaves every 15 days, leaf area at 55 days after transplanting, number of side branches, number of flower buds, number of flowers, average fruit weight, polar diameter, and equatorial diameter of fruits, number of fruits per plant and productivity. According to the results, the plants were tolerant to saline levels of irrigation water of up to 3.0 dS m-1, without prejudice to the phenological and productive characteristics of the crop. The unitary increase in the salinity of the irrigation water did not result in damage to the physiological characteristics of the plants until the 60 days of transplanting. (AU)

O cultivo de frutas não tradicionais tem ganhado espaço no setor de hortifrútis, mas que, em determinadas situações, são plantas que requerem estudos prévios relacionados a adaptabilidade de solo, clima e qualidade da água de irrigação. Neste sentido, este trabalho teve como objetivo avaliar os efeitos da salinidade da água de irrigação em plantas de Physalis peruviana L. (fisális) nas distintas fases de crescimento. A área experimental foi instalada nas dependências da Universidade Federal de Campina Grande, adotando o delineamento em blocos casualizados, com quatro níveis salinos da água de irrigação (0,3; 1,2; 2,1 e 3,0 dS m-1) e cinco repetições por tratamento. As variáveis analisadas foram: diâmetro do caule, altura das plantas e número de folhas a cada 15 dias, área foliar aos 55 dias após o transplantio, número de ramos laterais, número de botões florais, número de flores, peso médio dos frutos, diâmetro polar e diâmetro equatorial dos frutos, número de frutos por planta e produtividade. Conforme os resultados, as plantas se mostraram tolerantes a níveis salinos da água de irrigação de até 3,0 dS m-1, sem prejuízos nas características fenológicas da cultura e produtivas. O aumento unitário da salinidade da água de irrigação não resultou em danos nas características fisiológicas das plantas até os 60 dias de transplantio. (AU)

Avaliação da qualidade de vida, capacidade funcional e força da musculatura respiratória em pacientes com insuficiência cardíaca / Evaluation of quality of life, functional capacity and strength of respiratory and muscles in patients with heart failure

A insuficiência cardíaca (IC) é uma síndrome clínica, via final comum de diversas doenças cardíacas. O estudo teve como objetivo avaliar a qualidade de vida, a capacidade funcional e a força muscular respiratória em pacientes com IC classe funcional I e II. Tratou-se de um estudo observacional transversal, com amostra composta por 20 indivíduos com faixa etária entre 58 e 82 anos, em acompanhamento clínico no Centro Clínico de Especialidades Médicas de Patos de Minas. Após a realização dos testes, foi observado em relação à qualidade de vida que o domínio mais comprometido foi o de limitação por aspectos físicos. Não houve limitação na capacidade funcional e na força muscular respiratória, devido aos indivíduos realizarem acompanhamento fisioterapêutico. A IC gera um impacto negativo sobre a qualidade de vida e que a fisioterapia é indispensável no acompanhamento e prognóstico desta população. (AU)

Heart failure (HF) is a clinical syndrome, the common end-stage of several heart diseases. The study aimed to evaluate the quality of life, functional capacity and respiratory muscle strength in patients with HF functional class I and II. This was a cross-sectional study, with a sample of 20 individuals aged between 58 and 82 years, under clinical follow-up at the Clinical Center of Medical Specialties of Patos de Minas. After performing the tests, it was observed in relation to the quality of life that the most compromised domain was that of limitation by physical aspects. There was no limitation in the functional capacity and in the respiratory muscle strength, due to the individuals performing physiotherapeutic follow-up. It is concluded that HF has a negative impact on the quality of life and that physical therapy is essential in the monitoring and prognosis of this population. (AU)

The role of opioid analgesics in rheumatic disorders: a position paper from the Portuguese Rheumatology Society.

Pain is a common feature of most rheumatic diseases and it is often the main reason for the patient to seek for a clinical appointment. Chronic pain has a major impact on patient's quality of life, being frequently associated with functional incapacity, sleep and mood disorders. This leads to absenteeism and heavy consumption of health resources, both representing huge burdens on national economy. Managing musculoskeletal pain is pivotal but can be challenging. The use of the available pharmaceutical armamentarium should be parsimonious. Opioids are strong analgesic drugs that mostly act through their agonist action on µ-receptors in the central nervous system. Opioid-related side effects are not negligible and are mediated through both central and peripheral opioid receptors. The use of opioids is well established in the treatment of oncologic pain but their role in the management of musculoskeletal pain is still controversial. Inflammatory rheumatic diseases, osteoarthritis, osteoporotic fractures, chronic low back pain and fibromyalgia represent diverse major rheumatic conditions that frequently lead to chronic pain. In order to standardize and optimize management of musculoskeletal chronic pain in these prevalent diseases, the Portuguese Rheumatology Society elaborated this position paper. The objectives were: a) to define the importance of pain assessment and classification; b) to guide patient selection, appropriate choice of opioids, their management, and raise awareness of their adverse effects; c) to review the existent data on possible indications of opioid therapy on rheumatic diseases.

Reuma.pt/vasculitis - the Portuguese vasculitis registry.

BACKGROUND: The vasculitides are a group of rare diseases with different manifestations and outcomes. New therapeutic options have led to the need for long-term registries. The Rheumatic Diseases Portuguese Register, Reuma.pt, is a web-based electronic clinical record, created in 2008, which currently includes specific modules for 12 diseases and > 20,000 patients registered from 79 rheumatology centres. On October 2014, a dedicated module for vasculitis was created as part of the European Vasculitis Society collaborative network, enabling prospective collection and central storage of encrypted data from patients with this condition. All Portuguese rheumatology centres were invited to participate. Data regarding demographics, diagnosis, classification criteria, assessment tools, and treatment were collected. We aim to describe the structure of Reuma.pt/vasculitis and characterize the patients registered since its development. RESULTS: A total of 687 patients, with 1945 visits, from 13 centres were registered; mean age was 53.4 ± 19.3 years at last visit and 68.7% were females. The most common diagnoses were Behçet's disease (BD) (42.5%) and giant cell arteritis (GCA) (17.8%). Patients with BD met the International Study Group criteria and the International Criteria for BD in 85.3 and 97.2% of cases, respectively. Within the most common small- and medium-vessel vasculitides registered, median [interquartile range] Birmingham Vasculitis Activity Score (BVAS) at first visit was highest in patients with ANCA-associated vasculitis (AAV) (17.0 [12.0]); there were no differences in the proportion of patients with AAV or polyarteritis nodosa who relapsed (BVAS≥1) or had a major relapse (≥1 major BVAS item) during prospective assessment (p = 1.00, p = 0.479). Biologic treatment was prescribed in 0.8% of patients with GCA, 26.7% of patients with AAV, and 7.6% of patients with BD. There were 34 (4.9%) deaths reported. CONCLUSIONS: Reuma.pt/vasculitis is a bespoke web-based registry adapted for routine care of patients with this form of rare and complex diseases, allowing an efficient data-repository at a national level with the potential to link with other international databases. It facilitates research, trials recruitment, service planning and benchmarking.