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.

Hypokalemic paralysis due to renal tubular acidosis: uncommon initial manifestation of primary Sjögren´s syndrome.

Primary Sjögren´s Syndrome is an immune-mediated disease characterized by exocrine glands dysfunction due to lymphoplasmacytic infiltration with sicca symptoms being one of its main features. The disease may, however, present as distal renal tubular acidosis due to renal involvement, which can range from asymptomatic to life-threatening. We describe the case of a 33-year-old woman with hypokalemic paralysis and metabolic acidosis secondary to distal renal tubular acidosis, leading to the diagnosis of primary Sjögren´s Syndrome. Although rare, recognizing primary Sjögren´s Syndrome as a possible cause of distal renal tubular acidosis may elicit an earlier diagnosis and treatment, improving the patient´s prognosis.

Effectiveness of biosimilar infliximab CT-P13 compared to originator infliximab in biological-naïve patients with rheumatoid arthritis and axial spondyloarthritis: data from the Portuguese Register.

OBJECTIVES: To compare the effectiveness of the infliximab biosimilar CT-P13 with originator infliximab over 24 months of follow-up in biological-naïve patients with rheumatoid arthritis (RA) and axial spondyloarthritis (axSpA). METHODS: Biological-naïve patients from the Rheumatic Diseases Portuguese Register (Reuma.pt), with a clinical diagnosis of RA or axSpA, who were starting either the infliximab biosimilar CT-P13 or the originator infliximab after 2014 (date of market entry of CT-P13 in Portugal), were included. Patients on biosimilar and originator were compared regarding different response outcomes at 3 and 6 months, adjusting for age, sex and baseline C-reactive protein (CRP). The main outcome was the change in DAS28-erytrocyte sedimentation rate (ESR) for RA and the ASDAS-CRP for axSpA. Additionally, the effect of infliximab biosimilar vs originator on different response outcomes over 24 months of follow-up was tested with longitudinal generalized estimating equations (GEE) models. RESULTS: In total, 140 patients were included, 66 (47%) of which with RA. The distribution of patients starting the infliximab biosimilar and the originator was the same between the two diseases (approximately 60% and 40%, respectively). From the 66 patients with RA, 82% were females, mean age was 56 years (SD 11) and mean DAS28-ESR 4.9 (1.3) at baseline. As for the patients with axSpA, 53% were males, mean age was 46 years (13) and mean ASDAS-CRP 3.7 (0.9) at baseline. There were no differences in efficacy between RA patients treated with the infliximab biosimilar and the originator, either at 3 months (∆DAS28-ESR: -0.6 (95% CI -1.3; 0.1) vs -1.2 (-2.0; -0.4)), or at 6 months (∆DAS28-ESR: -0.7 (-1.5; 0.0) vs -1.5 (-2.4; -0.7)). This was also true for patients with axSpA (∆ASDAS-CRP at 3 months: -1.6 (-2.0; -1.1) vs -1.4 (-1.8; -0.9) and at 6 months: -1.5 (-2.0; -1.1) vs -1.1 (-1.5; -0.7)). Results were similar with the longitudinal models over 24 months. CONCLUSION: There are no differences in effectiveness between the infliximab biosimilar CT-P13 and the infliximab originator in the treatment of biological-naïve patients with active RA and axSpA in clinical practice.

Does the halo count on temporal and axillary ultrasound predict time to relapse in giant cell arteritis?

OBJECTIVES: To determine whether the halo count (HC) on temporal and axillary artery US (TAUS) predicts time to relapse in giant cell arteritis (GCA). METHODS: We conducted a single-centre retrospective study of GCA patients. HC, the number of vessels with non-compressible halo on the TAUS at diagnosis, was determined by retrospective review of the US report and images. Relapse was defined as increase in GCA disease activity requiring treatment escalation. Cox proportional hazard regression was used to identify predictors of time to relapse. RESULTS: A total of 72 patients with confirmed GCA were followed up for a median of 20.9 months. Thirty-seven of 72 (51.4%) relapsed during follow-up, at a median prednisolone dose of 9 mg (range 0-40 mg). Large-vessel (axillary artery) involvement did not predict relapse. On univariable analysis, a higher HC was associated with shorter time to relapse (per-halo hazard ratio 1.15, 95% CI 1.02, 1.30; P = 0.028). However, statistical significance was lost when the 10 GCA patients with an HC of zero were excluded from analysis. CONCLUSION: In this real-world setting, relapse occurred at a wide range of glucocorticoid doses and was not predicted by axillary artery involvement. GCA patients with a higher HC at diagnosis were significantly more likely to relapse, but significance was lost on excluding those with HC of zero. HC is feasible in routine care and may be worth incorporating into future prognostic scores. Further research is required to determine whether confirmed GCA patients with negative TAUS represent a qualitatively different subphenotype within the GCA disease spectrum.

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.

Qualidade de vida no trabalho e work engagement em profissionais da Rede Nacional de Cuidados Continuados Integrados / Quality of life at work and work engagement in professionals from the National Network for Integrated Continuous Care

A Rede Nacional de Cuidados Continuados Integrados, é uma das mais recentes respostas do Sistema Nacional de Saúde Português. É por isso fulcral, a realização de estudos que avaliem esta resposta, mas que também incidam sobre os profissionais que diariamente asseguram a prestação de cuidados. O conhecimento sobre qualidade de vida no trabalho e o work engagement, dos profissionais é fundamental para melhorar a qualidade destas organizações. Surge desta forma a motivação para este estudo, que teve como objetivo conhecer os níveis de qualidade de vida no trabalho e o work engagement dos profissionais da Rede Nacional de Cuidados Integrados. Para o efeito, utilizaram-se 3 instrumentos, um questionário de caraterização sociodemográfico e profissional construído pelo autor, pelo Quality of Work Life Scale (QWLS) de Sirgy et al. (2001) traduzido e adaptado para a população portuguesa por Sinval et al. (2020) e pela Utrecht Work Engagement Scale short version (UWES-9) de Schaufeli e Bakker (2003), traduzida e adaptada para a população portuguesa por Sinval et al. (2018). Num universo de cerca de 1000 profissionais das Residências Montepio ­ serviços de saúde S.A., foram estudados 240 indivíduos. Os resultados obtidos demostram que os profissionais apresentam valores de qualidade de vida no trabalho em linha com outros estudos e que apresentam valores de work engagement considerados altos. Verificaram-se diferenças estatisticamente significativas nas médias de qualidade de vida no trabalho em função das variáveis sociodemográficas, sexo (t=-1,97; p=0,016) e nacionalidade (t=-2,041; p=0,042) e das variáveis socioprofissionais, classificação no nível remuneratório (t=--7,865; p<0,001), experiência profissional (t=-2,51; p=0,013), antiguidade na instituição (t=2,123; p=0,035), categoria profissional (F=3,483; p=0,033) e tipo de vinculo laboral (F=3,178; p=0,043). Também se verificaram diferenças nas médias de work engagement tendo em conta as variáveis sociodemográficas, estado civil (F=-4,28; p=0,006) e faixa etária (F=3,476; p=0,009) e socioprofissionais, classificação no nível remuneratório (t=--5,015; p<0,001), experiência profissional (t=-3,343; p<0,001) e categoria profissional (F=6,385; p=0,002). Verificou-se uma correlação positiva, moderada e altamente significativa entre a qualidade de vida no trabalho e o work engagement (r=0,658; p<0,001). Conclui-se que as avaliações e consequentemente as intervenções ao nível da qualidade de vida no trabalho e do work engagement dos profissionais da Rede Nacional de Cuidados Continuados integrados, tem de ter em conta a variabilidade sociodemográfica e profissional destes indivíduos e que ações ao nível da qualidade de vida no trabalho e especificamente ao nível das necessidades de atualização dos profissionais, podem ter efeitos positivos nos níveis de work engagement.

The national network of integrated continuity care is one of the most recent responses of the Portuguese National Health System. It is therefore crucial to carry out studies that assess this response, but also focus on the professionals who ensure the provision of care on a daily basis. Knowledge about the quality of work life and work engagement of professionals is essential to improve the quality of these organizations. In this way, the motivation for this study arises, which aimed to know the levels of quality of life at work and work engagement of professionals in the national network of integrated continuity care. The data collection instrument consisted of a sociodemographic and professional questionnaire, the Quality of Work Life Scale (QWLS) by Sirgy et al. (2001) translated and adapted for the Portuguese population by Sinval et al. (2020) and the Utrecht Work Engagement Scale short version (UWES-9) by Schaufeli and Bakker (2003), translated and adapted for the Portuguese population by Sinval et al. (2018). In a universe of around 1000 professionals from Residências Montepio ­ Serviços de Saúde S.A., 240 individuals were studied. The results obtained show that professionals have values of quality of work life in line with other studies and that they have values of work engagement considered high. There were statistically significant differences in the means of quality of work life according to sociodemographic variables, gender (t=-1.97; p=0.016) and nationality (t=-2.041; p=0.042) and socio-professional variables, classification in salary level (t=--7.865; p<0.001), professional experience (t=-2.51; p=0.013), seniority in the institution (t=2.123; p=0.035), professional category (F=3.483 ; p=0.033) and type of employment relationship (F=3.178; p=0.043). There were also differences in the averages of work engagement taking into account sociodemographic variables, marital status (F=-4.28; p=0.006) and age group (F=3.476; p=0.009) and socioprofessional variables, classification in salary level ( t=--5.015; p<0.001), professional experience (t=-3.343; p<0.001) and professional category (F=6.385; p=0.002). There was a positive, moderate and highly significant correlation between quality of life at work and work engagement (r=0.658; p<0.001). It is concluded that the evaluations and, consequently, the interventions in terms of quality of work life and the work engagement of the professionals of the National Network of Integrated Continuity Care, must take into account the sociodemographic and professional variability of these individuals and that actions in terms of quality of work Life and specifically in terms of professionals' updating needs, can have positive effects on work engagement levels.

Post-measurement compressed calibration for ICP-MS-based metal quantification in mine residues bioleaching.

Bioleaching is an actual economical alternative to treat residues, which allows, depending on the chosen strategy, two possible outcomes: (1) a leachate enriched with target metals, or (2) a residue enriched in target metals through the leaching of interfering components (IC). This work aimed to study the metals released by bioprocessing the Panasqueira mine tailings, as a strategy to increase critical metals' relative concentration in residues. Biostimulation of the local microbiota was compared to a bioaugmentation approach using the autochthonous Diaphorobacter polyhydroxybutyrativorans strain B2A2W2. Inductively Coupled Plasma Mass Spectrometry (ICP-MS) was selected to study the metals released in the leachate through multi-element external standards. A new data treatment method was developed to use a preliminary sweep of intensities to quantify the non-initial target metals concentration in the leachate, based on preliminary ICP-MS intensity measurements. The results demonstrated that biostimulation was an efficient bioleaching strategy for the IC silicon, aluminium, magnesium, selenium, manganese, zinc, iron, and copper, by decreasing concentration, resulting in a relative increase in the gallium and yttrium (10x) levels in the treated residue. The strategy followed to quantify a large number of elements with ICP-MS using a reduced number of data points for calibration proved valid and speeded up the analytical process.

Green and blue spaces and lung function in the Generation XXI cohort: a life-course approach.

BACKGROUND: Exposure to natural environments may affect respiratory health. This study examined the association of exposure to green and blue spaces with lung function in children, and assessed the mediation effect of air pollution and physical activity. METHODS: The study used data from the Generation XXI, a population-based birth cohort from the Porto Metropolitan Area (Portugal). Residential Normalised Difference Vegetation Index (NDVI) at different buffers (100, 250 and 500 m), the accessibility to urban green spaces (UGS) within 400 and 800 m and the minimum distance to the nearest UGS and to the nearest blue spaces were assessed at birth, 4, 7 and 10 years of age. Three life-course measures were calculated: averaged exposure, early-life exposure (birth) and exposure trend over time (change in exposure). Forced vital capacity (FVC), forced expiratory volume in 1 s (FEV1) and forced expiratory flow between 25% and 75% of FVC (FEF25-75%) at 10 years were used as outcomes. To assess associations, linear regression models and path analysis were used. RESULTS: This study included 3278 children. The adjusted models showed that increasing the NDVI exposure over time within 100 m of the child's residence was associated with higher values of FEV1 (L) and FEF25-75% (L·s-1) (ß 0.01, 95% CI 0.0002-0.03 and ß 0.02, 95% CI 0.001-0.05, respectively). No significant associations were observed for the remaining measures of exposure, and no mediation effect was found for pollution or physical activity. CONCLUSION: Increasing exposure to greenness at close proximity from residences was associated with improved lung function. While the mechanism remains unknown, this study brings evidence that city greening may improve children's respiratory health.

Risk Factors for Infection, Predictors of Severe Disease, and Antibody Response to COVID-19 in Patients With Inflammatory Rheumatic Diseases in Portugal-A Multicenter, Nationwide Study.

Objective: To identify risk factors for SARS-CoV-2 infection and for severe/critical COVID-19, and to assess the humoral response after COVID-19 in these patients. Methods: Nationwide study of adult patients with inflammatory RMDs prospectively followed in the Rheumatic Diseases Portuguese Register-Reuma.pt-during the first 6 months of the pandemic. We compared patients with COVID-19 with those who did not develop the disease and patients with mild/moderate disease with those exhibiting severe/critical COVID-19. IgG antibodies against SARS-CoV-2 were measured ≥3 months after infection and results were compared with matched controls. Results: 162 cases of COVID-19 were registered in a total of 6,363 appointments. Patients treated with TNF inhibitors (TNFi; OR = 0.160, 95% CI 0.099-0.260, P < 0.001) and tocilizumab (OR 0.147, 95% CI 0.053-0.408, P < 0.001) had reduced odds of infection. Further, TNFi tended to be protective of severe and critical disease. Older age, major comorbidities, and rituximab were associated with an increased risk of infection and worse prognosis. Most patients with inflammatory RMDs (86.2%) developed a robust antibody response. Seroconversion was associated with symptomatic disease (OR 13.46, 95% CI 2.21-81.85, P = 0.005) and tended to be blunted by TNFi (OR 0.17, 95% CI 0.03-1.05; P = 0.057). Conclusions: TNFi and tocilizumab reduced the risk of infection by SARS-CoV-2. Treatment with TNFi also tended to reduce rates of severe disease and seroconversion. Older age, general comorbidities and rituximab were associated with increased risk for infection and worse prognosis, in line with previous reports. Most patients with RMDs developed a proper antibody response after COVID-19, particularly if they had symptomatic disease.

Portuguese recommendations for the use of biological and targeted synthetic disease-modifying antirheumatic drugs in patients with rheumatoid arthritis - 2020 update.

OBJECTIVE: To update the recommendations for the treatment of rheumatoid arthritis (RA) with biological and targeted synthetic disease-modifying antirheumatic drugs (bDMARDs and tsDMARDs), endorsed by the Portuguese Society of Rheumatology (SPR). METHODS: These treatment recommendations were formulated by Portuguese rheumatologists taking into account previous recommendations, new literature evidence and consensus opinion. At a national meeting, in a virtual format, three of the ten previous recommendations were re-addressed and discussed after a more focused literature review. A first draft of the updated recommendations was elaborated by a team of SPR rheumatologists from the SPR rheumatoid arthritis study group, GEAR. The resulting document circulated among all SPR rheumatologists for discussion and input. The level of agreement with each of all the recommendations was anonymously voted online by all SPR rheumatologists. RESULTS: These recommendations cover general aspects such as shared decision, treatment objectives, systematic assessment of disease activity and burden and its registry in Reuma.pt. Consensus was also achieved regarding specific aspects such as initiation of bDMARDs and tsDMARDs, assessment of treatment response, switching and definition of persistent remission. CONCLUSION: These recommendations may be used for guidance of treatment with bDMARDs and tsDMARDs in patients with RA. As more evidence becomes available and more therapies are licensed, these recommendations will be updated.

Residential and school green and blue spaces and intelligence in children: The Generation XXI birth cohort.

Green and blue spaces have numerous health benefits. It has been hypothesized that contact with these spaces can have an important role in cognitive development, because it provides children with unique stimuli that can help develop curiosity, creativity, awareness, and control. This study aimed to estimate the association between exposure to green and blue spaces and the various measures of intelligence quotient (IQ) among children from Porto Metropolitan Area (Portugal). This investigation used data from the Generation XXI birth cohort. Exposure was assessed by Geographic Information Systems using vegetation indexes and measures of accessibility to urban green spaces and blue spaces in the surroundings of the residence and school, measured at 0, 4, 7 and 10 years. The outcome was the verbal, performance, and global IQ, measured using the Wechsler Intelligence Scale for Children (WISC-III), administered at age 10. Associations were estimated using crude and adjusted multilevel models. The mediation role of air pollution and physical activity was quantified. This study included 3827 children. The adjusted models showed that having green spaces up to 800 m of the residence was positively associated with performance IQ (1.30 95%CI [0.26; 2.35]) and global IQ (1.27 [0.18; 2.36]). No clear associations were observed regarding accessibility to blue spaces. Physical activity appeared to have a minor mediation role. Children with urban green spaces around their residences had higher performance and global IQ. Local actors in the sectors of public health and urban planning should promote the creation of green spaces close to residential areas.

Advances in Ultrasound Imaging in Rheumatology-What Do They Mean and What Challenges Do They Pose? The Example of Microvascular Imaging.

Musculoskeletal ultrasound is an important tool for the monitorization of inflammatory rheumatic diseases, with subclinical synovitis being frequently detected in ultrasound scans of patients in clinical remission. It has been shown, for example, that the presence of Power Doppler signal synovial membrane has prognostic value for patients with rheumatoid arthritis. Microvascular imaging technologies significantly improve the sensitivity for slow-flow vessels and may potentially detect subclinical inflammation when Power Doppler fails to do so. The authors briefly discuss the implications of the use of such techniques in rheumatology setting and review available evidence.