ObjectivesIdiopathic 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.MethodsMulticenter, 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.Results230 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.ConclusionAnti-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.

Background:Low bone mineral density (BMD) is common in ankylosing spondylitis (AS). The fracture risk (FR) is increased and its reduction with pharmacologic therapy is not clearly defined in this population. However, early screening and bisphosphonates as first-line treatment are recommended.Objectives:To investigate the influence of dual-energy X-ray absorptiometry (DXA) in the ten-year risk of fracture assessed by FR Assessment Tool (FRAX) and to determine possible demographic or clinical factors associated with an increased FR in a spondyloarthritis (SpA) population.Methods:Retrospective study including all the over 40 years-old SpA patients (ASAS classification criteria) followed at our Rheumatology Department and registered in the national database. Demographic, clinical and laboratorial data were collected at the time of the last follow-up visit. Data from the last DXA (until 3 years prior to the last visit) were collected. Indication for pharmacological treatment by FRAX was assessed according to the national recommendations.Results:A total of 231 SpA patients were included: 126 males (54.5%), 53 (22.9%) smokers; 171 (74%) had AS, 23 (10%) had Inflammatory Bowel Disease Associated SpA and 37 (16%) had Undifferentiated SpA. At the last follow-up visit, the mean age was 52.9 years (±9.6) and the median disease duration was 21.9 years [1.0-55.5]. The mean ASDAS-CRP was 2.5 (±0.9) and the majority of patients had moderate (25.5%) or high (48.5%) disease activity (according to ASDAS). One hundred and thirty patients (56.3%) were taking NSAIDs, 45 (19.5%) were taking glucocorticoids, 85 (36.8%) were under csDMARDs and 170 (73.6%) under bDMARDs [157 (68%) under TNFi, 11 (4.8%) under secukinumab and 2 (0.9%) under ustekinumab].Eleven patients (4.8%) had previous fragility fractures, 118 (51.1%) had DXA in the last 3 years and 167 (72.3%) were taking calcium and/or vitamin D supplements.Sixteen patients (6.9%) had indication for treatment by FRAX without DXA and 9 of these (56.3%) were already under treatment. Similarly, 16 (6.9%) had indication for treatment by FRAX with DXA and 13 of these (81.3%) were already under treatment. Ten patients (4.3%) were reclassified in FRAX with DXA: 7 (3%) had no indication for treatment by FRAX without DXA but obtained it by FRAX with DXA and 3 (1.3%) had indication for treatment by FRAX without DXA but they lost it by FRAX with DXA. We found a moderate level of agreement in the indication for treatment between FRAX with and without DXA (kappa=0.595; p<0.001). The use of DXA in FRAX estimated a significant higher median FR, both for major osteoporotic fracture (2.4% [0.8-31.0] vs 1.8% [0.6-20.0]; p<0.001) and for hip fracture (0.5% [0.0-23.0] vs 0.2% [0.0-14.0]; p<0.001).We found significant correlations between FR and some disease-related variables (table 1).Table 1.Correlations between the risk of fracture estimated by FRAX and disease-related variables.Disease durationBASDAIASDAS-CRPBASMIBASFIEstimated fracture risk by FRAX:without DXAmajor osteoporotic fracturer=0.352p<0.001r=0.204p=0.002r=0.214p=0.001r=0.301p<0.001r=0.317p<0.001hip fracturer=0.389p<0.001r=0.142p=0.034r=0.170p=0.011r=0.305p<0.001r=0.275p<0.001with DXAmajor osteoporotic fracturer=0.227p=0.014r=0.314p=0.001r=0.356p<0.001r=0.293p=0.002r=0.379p<0.001hip fracturen.s.r=0.197p=0.036r=0.269p=0.004r=0.271p=0.004r=0.258p=0.006Conclusion:Our results showed that a similar number of patients had indication for pharmacological treatment by FRAX both with and without DXA. Although the inclusion of DXA resulted in a higher estimated FR by FRAX, the observed moderate level of agreement between FRAX with and without DXA suggests that the FR estimation by FRAX, even without DXA, may be a reasonable approach in SpA patients. In line with literature, we found significant associations between the estimated risk fracture by FRAX and some disease activity and function measures.Disclosure of Interests:Bruno Miguel Fernandes: None declared, Salomé Garcia: None declared, Sara Ganhão: None declared, Maria Rato: None declared, Filipe Pinheiro: None declared, Miguel Bernardes Speakers bureau: Abbvie, Amgen, Biogen, Eli-Lilly, Glaxo-Smith-Kline, Pfizer, Janssen, Novartis, Lúcia Costa: None declared

(1) Background: The UCLA GIT 2.0 questionnaire has been recognized as a feasible and reliable instrument to assess gastrointestinal (GI) symptoms in systemic sclerosis (SSc) patients and their impact on quality of life. The aim of this study was to create and validate UCLA GIT 2.0 for Portuguese patients with SSc. (2) Methods: A multi-center study was conducted enrolling SSc patients. UCLA GIT 2.0 was validated in Portuguese using reliability (internal consistency, item­–total correlation, and reproducibility) and validity (content, construct, and criterion) tests. Criterion tests included EQ-5D and SF-36v2. Social–demographic and clinical data were collected. (3) Results: 102 SSc patients were included, 82.4% of them female, and with a mean sample age of 57.0 ± 12.5 years old. The limited form of SSc was present in 62% of the patients and 56.9% had fewer than five years of disease duration. Almost 60% presented with SSc-GI involvement with a negative impact on quality of life. The means for SF-36v2 were 39.3 ± 10.3 in the physical component summary and 47.5 ± 12.1 in the mental component summary. Total GI score, reported as mild in 57.8% of the patients, was highly reliable (ICC = 0.912) and the Cronbach’s alpha was 0.954. There was a high correlation between the total GI score and EQ-5D-5L and SF-36v2 scores. (4) Conclusion: The Portuguese version of UCLA GIT 2.0 showed good psychometric properties and can be used in research and clinical practice.

ObjectiveTo 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.MethodsNationwide 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.Results162 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 &lt; 0.001) and tocilizumab (OR 0.147, 95% CI 0.053–0.408, P &lt; 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).ConclusionsTNFi 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.

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Background:Enthesitis is a hallmark clinical feature of spondyloarthritis (SpA), but to date, few studies have investigated how the overall response to biological treatment relates to the evolution of enthesitis counts.Objectives:Assess whether the variation in enthesitis indices reflects the overall response to bDMARD therapy in SpA.Methods:This longitudinal, retrospective study included patients who met Assessment of Spondyloarthritis international Society (ASAS) criteria for SpA followed at the Rheumatology Department of a tertiary hospital, under bDMARD therapy. Demographic, laboratorial and clinical data were collected, including Bath Ankylosing Spondylitis Metrology Index (BASMI), Bath Ankylosing Spondylitis Functional Index (BASFI), Bath Ankylosing Spondylitis Activity Index (BASDAI), Ankylosing Spondylitis Disease Activity Score-C-reactive protein (ASDAS-CRP), Maastrich Ankylosing Spondylitis Enthesitis Score (MASES), Leeds Enthesitis Index (LEI) and Spondyloarthritis Research Consortium of Canada (SPARCC) scores. All were evaluated at baseline and at 6, 12, 18 and 24 months after starting the first biological therapy. The variation in each parameter compared with the baseline values was calculated at 6, 12, 18 and 24 months and represented in the form of delta. Correlations between variables were assessed using Spearman test and comparison between groups using Wilcoxon, Mann-Whitney U and Kruskal-Wallis tests.Results:We included 273 patients, 123 (45,1%) females, aged 42,0±12,3 years and with diagnosis of SpA for 15,4±11,2 years at the start of bDMARD therapy. Eighteen (6,6%) had depression. At baseline, mean BASDAI was 6,43 ±1,62, ASDAS-CRP was 4,01 ± 0,86, median MASES was 1 (0-4), LEI 0 (0-1,75) and SPARCC 1 (0-4). Seventy-two patients (26,4%) started golimumab, 71 (26,0%) adalimumab, 66 (24,2%) infliximab, 54 (19,8%) etanercept, 9 (3,3%) certolizumab and 1 (0,4%) secukinumab. Enthesitis indices were significantly higher at baseline in females [median MASES-females 2 (0-5) vs 0 (0-2), p<0,001; LEI-females 0 (0-2) vs 0 (0-1), p=0,03; and SPARCC-females 2 (0-5) vs 0 (0-2), p<0,001], and remained so at 24 months [median MASES-females 1 (0-3,5) vs 0 (0-0), p<0,001; LEI-females 0 (0-0,5) vs 0 (0-0), p<0,001; and SPARCC-females 1 (0-3) vs 0 (0-0), p<0,001]. MASES and SPARCC, but not LEI, at baseline were significantly higher in patients with depression [median MASES-depression 3,5 (2-6) vs 1 (0-4), p=0,01; SPARCC-depression 4 (0-8) vs 1 (0-3), p=0,03], but at 24 months no differences were observed. There was a significant difference between each of the 3 scores of enthesitis when assessed at 6, 12, 18 and 24 months, compared to baseline (p <0.004). No differences were observed regarding the choice of bDMARD. At baseline, MASES had a significant correlation with patient visual analogic scale (VAS) (r=0,18; p=0,01), BASDAI (r=0,36; p<0,001) and BASFI (r=0,21; p=0,003); LEI had a significant correlation with BASDAI (r=0,31; p<0,001) and BASFI (r=0,21; p=0,003); SPARCC had a significant correlation with patient VAS (r=0,19; p=0,01), BASDAI (r=0,37; p<0,001) and BASFI (r=0,26; p<0,001). ΔLEI at 6 months had a significant correlation with ΔBASDAI (r=0,25; p=0,005), ΔASDAS (r=0,190; p=0,03), Δpatient VAS (r=0,23; p=0,01) and Δphysician VAS (r=0,25; p=0,01), but not with ΔESR, ΔCRP and ΔBASMI; no correlation was found at 6 months for ΔMASES or ΔSPARCC. At 12 months, ΔMASES had a significant correlation with ΔBASDAI (r=0,18; p=0,03); ΔLEI with ΔBASDAI (r=0,23; p=0,01) and Δpatient VAS (r=0,19; p=0,03); for ΔSPARCC no significant correlations were found. At 18 months and 24 months, no correlations were found.Conclusion:The initiation of bDMARD led to improved enthesitis indices over a 24-month period. ΔLEI correlates better with SpA activity scores and measurements than the other indices, especially at the first 12 months of initiation of bDMARD therapy.Disclosure of Interests:Filipe Pinheiro: None declared, Maria Rato: None declared, Bruno Miguel Fernandes: None declared, Salomé Garcia: None declared, Sara Ganhão: None declared, Pedro Madureira: None declared, Miguel Bernardes Speakers bureau: Abbvie, Amgen, Biogen, Eli-Lilly, Glaxo-Smith-Kline, Pfizer, Janssen, Novartis, Lúcia Costa: None declared

Background:Patients with rheumatoid arthritis (RA) have a higher risk of osteoporosis not only due to chronic inflammation status, but also due to the treatment with glucocorticoids. FRAX is a computer-based algorithm developed by the World Health Organization for estimation of the 10-year risk of a hip or major osteoporotic fracture. Inclusion of femoral neck bone mineral density (BMD) in the estimation is optional.Objectives:The study aimed to identify the RA patients under treatment with biological disease-modifying antirheumatic drug (bDMARD), who have FRAX scores, calculated with and without BMD, classified as high fracture risk and evaluate if they are receiving treatment for osteoporosis. The authors also investigated the intra-individual agreement between FRAX fracture risk calculated with and without BMD.Methods:Demographic and clinical data and BMD results from RA patients followed in a tertiary university hospital and registered in the Rheumatic Diseases Portuguese Register were used for analysis. Patients under 40 years of age at the last visit were excluded. McNemar test was applied for the identification of discordance of risk categories. The Wilcoxon test was used to characterize the intraindividual differences between paired FRAX risks with and without BMD. Correlations between pairs of variables were evaluated by the Spearman test. For independent variables Mann-Whitney test was used.Results:A total of 303 patients were included, 244 were females (80.5%) and 49 current smokers (16.2%). Mean age was 59.5 ± 9.54 years and mean disease duration 18.5 ± 10.4 years. Two hundred and twenty patients (72.4%) and 243 (80.2%) were RF and ACPA positive, respectively, and 51.5% had erosive disease. Mean disease activity score (DAS28-4V-CRP) was 3.08 ± 1.18 and mean femoral neck BMD 0.84 ± 0.12 g/cm2. One hundred and seventy nine patients (58.9%) were concomitantly treated with conventional synthetic DMARDs and 215 (70.7%) with glucocorticoids. Among all the patients, 35 (11.6%) had previous fractures and 19 (6.3%) have family history of fracture. The median 10-year risk of a major fracture and a hip fracture, calculated without BMD, was 6.0 (1.2-50) and 1.5 (0.1-39), respectively; with BMD it was 6.9 (1.3-61) and 1.7 (0-49). When FRAX score is calculated without BMD (n=303), 76 (25.1%) patients were categorized as high fracture risk. Among them, only 41 (54%) were receiving osteoporosis treatment. FRAX assessment with BMD (n=231) identified 99 (32.7%) patients with high fracture risk, 51 (51,5%) in treatment for osteoporosis. Thirty patients (21%) previously classified as low fracture risk using FRAX without BMD were recategorized as high risk (p<0.001). Despite that, there was a strong correlation between fracture risks assessed with and without BMD for both major and hip fracture (r = 0.867, p < 0.0001 and r = 0.728, p < 0.0001, respectively). ACPA and RF positive patients did not have higher FRAX scores (including or not BMA). Patients with erosive disease had a higher 10-year probability of major fracture evaluated by FRAX when it includes BMD (p=0.041).Conclusion:It is very important to accurately assess the risk of osteoporotic fractures in RA patients to treat them properly. The authors highlight the high number of patients who are not receiving treatment according to FRAX categorization. In spite of the correlation between estimated fracture risk by FRAX with and without BMD, there is a discordance in fracture risk categorization, as one fifth of patients of low risk were reclassified as high risk. For the RA population treated with bDMARDS, our findings raise the need to request a DXA not only for patients classified as having an intermediate risk of fracture, but also for low-risk patients.Disclosure of Interests:Maria Rato: None declared, Filipe Pinheiro: None declared, Salomé Garcia: None declared, Bruno Miguel Fernandes: None declared, Sara Ganhão: None declared, Rita Gaio: None declared, Miguel Bernardes Speakers bureau: Abbvie, Amgen, Biogen, Eli-Lilly, Glaxo-Smith-Kline, Pfizer, Janssen, Novartis, Alexandra Bernardo: None declared, Lúcia Costa: None declared