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.

BackgroundVarious nationwide studies have been already published to better understand Mixed Connective Tissue Disease (MCTD) (1,2). However, Portuguese data is not available.ObjectivesTo characterize clinical and immunological features of a Portuguese cohort of patients with MCTD.MethodsRetrospective, multicenter study including adult-onset patients with clinical diagnosis of MCTD and fulfilling at least one of the following classification criteria: Sharp, Kasukawa, Alarcón-Segovia or the Kahn’s criteria. Positivity to other autoantibodies besides anti-U1-RNP were allowed. SPSS was used for statistical analysis and significance level was defined as 2-sided p<.05.ResultsA total of 98 patients were included, with a mean age at diagnosis and disease duration of 40.5±13.7 and 7.0±6.5 years, respectively. Most patients were female (87.8%) and Caucasian (70.4%). Raynaud’s phenomenon (96.9%), arthralgia/arthritis (94.9/74.5%) and puffy fingers (60.2%) were the most common and early manifestations. Gastroesophageal (GE), respiratory and muscular involvement were also prevalent, mostly during the follow up, affecting 30.6%, 34.7% and 43.9% of the patients, respectively. Clinical and immunological characteristics are described in Table 1. Males were older at symptom’s onset (65.0 VS 46.7, p=.035), having more respiratory involvement (OR=4.5, 95% CI 1.3-16.4), and positivity to anti-ACPA (OR=20.0, 95% CI: 3.1-129.4). GE involvement occurred more often in Caucasian patients (OR=3.8; 95% CI: 1.0-14.1), while anemia of chronic diseases (OR=2.7; 95% CI: 1.0-7.2), myositis (OR=3.6; 95% CI: 1.3-9.9) and constitutional symptoms (OR=3.2; 95% CI: 1.2-8.3) were more frequent in Afro-American patients, whose were also younger at disease (34.1 VS 50.6, p=.01). After a median follow-up time of 4 (IQR 8) years, 4 deaths occurred (4.1%), mostly (75%) due to infectious complications.Table 1.Clinical and immunological characteristicsClinical ManifestationsAt presentationFollow-upMucocutaneous systemRaynaud’s phenomenon, n (%)85 (86.7)95 (96.9)Puffy hands, n (%)48 (49.0)59 (60.2)SSc-like, n (%)43 (44.8)59 (60.8)SLE-like, n (%)28 (28.9)35 (35.7)Musculoskeletal systemArthralgia/Arthritis, n (%)/n (%)81 (82.7) / 56 (57.1)93 (94.9) / 73 (74.5)Myositis, n (%)26 (25.6)43 (43.9)Hematological system, n (%)46 (46.9)70 (71.4)Respiratory system, n (%)14 (14.3)34 (34.7)Cardiovascular system3 (3.1)4 (4.1)Pulmonary hypertension*2 (2.0)15 (15.3)Gastroesophageal involvement, n (%)11 (11.2)30 (30.6)Renal involvement, n (%)2 (2.0)10 (10.2)Neurological involvement, n (%)6 (6.3)14 (14.3)Constitutional symptoms, n (%)26 (26.5)30 (30.6)Immunological characteristicsAnti-dsDNA, n (%)21 (21.4)Anti-smith antibody, n (%)21 (21.4)Anti-Ro/SSA, n (%)31 (31.6)Anti-La/SSB, n (%)7 (7.1)Anti-centromere, n (%)3 (4.1)Rheumatoid Factor, n (%)39 (39.8)Anti- anti-citrullinated protein antibodies, n (%)6 (6.1)Antiphospholipid antibodies, n (%)7 (7.1)Myositis antibodies, n (%)9 (9.2)Complement activation, n (%)27 (27.6)Hypergammaglobulinemia, n (%)51 (52.0)Legend: Anti-dsDNA: anti-double stranded deoxyribonucleic acid antibody; SLE: systemic lupus erythematosus, SSc: systemic sclerosis. *No information regarding cardiac catheterism, then compatible alterations in the echocardiogram.ConclusionRaynaud’s phenomenon, puffy fingers and arthritis were the most common manifestations in Portuguese patients, with similar proportions found in literature (1,2). However, we reported some differences in mucocutaneous, renal and serosa involvement and higher prevalence of probable pulmonary hypertension (1,2), which may be explained by the heterogeneity of the inclusion criteria. Except for respiratory, myositis, GE and constitutional symptoms, there were no differences regarding gender and ethnicity.Here, we characterize the largest cohort of MCTD in Portugal.References[1]Cappelli S, et al. Semin Arthritis Rheum. 2012 Feb;41(4):589–98.[2]Alves MR et al. Clin Exp Med. 2020 May;20(2):159–66.Disclosure of InterestsNone 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.

Background:Systemic Sclerosis (SSc) is a chronic disease with multi-organ manifestations that may contribute to disability and low quality of life.1 Therefore, anxiety and depression are more frequent in SSc patients than in general population.2Objectives:To assess the prevalence of anxiety and depression in a SSc cohort and to evaluate its correlation with function, quality of life and assessment of gastrointestinal (GI) involvement scores.Methods:A cross-sectional study was conducted evaluating a cohort of SSc patients. All patients answered to the Hospital Anxiety and Depression Scale (HADS) questionnaire. A cut-off score < 8 was considered normal. Health Assessment Questionnaire (HAQ), Scleroderma HAQ (SHAQ), 36-Item Short Form Health Survey (SF-36), EuroQol-5D (EQ-5D) and University Of California, Los Angeles, Scleroderma Clinical Trials Consortium Gastrointestinal Scale (UCLA SCTC GIT) 2.0 questionnaires were also obtained. Clinical data was obtained and analyzed.Results:We included 20 patients, 17 females [n = 17 (85%)], median (min, max) age was 52.5 (28, 75) years-old. Regarding disease classification, 13 (65%) had limited SSc, 4 (20%) had diffuse SSc and 3 (15%) had early SSc. A score ≥ 8 was found in 14 (70%) patients on HADS-A [median (min, max) = 9 (2, 19)] and in 12 (60%) patients on HADS-D [median (min, max) = 8 (1, 15)]. Depressive patients had significantly worst scores on the measures of function, such as HAQ and lung and gastrointestinal involvements and patient global assessment of SHAQ, of quality of life, such as EQ-5D and physical functioning, role physical, bodily pain, vitality, social functioning and mental health domains of SF-36, and on the UCLA SCTC GIT 2.0 scale. Anxious patients had significantly worst scores on social functioning and mental health domains of SF-36 and on the UCLA SCTC GIT 2.0 scale (Table 1).Conclusion:The prevalence of depression and anxiety on SSc patients is high and should not be neglected. Overall disability and multiorgan manifestations, particularly GI involvement, may contribute to a low quality of life and consequently to depression and anxiety.References:[1]Firestein & Kelley’s Textbook of Rheumatology 2-Volume Set, 11th Edition[2]Brett D. Thombs et al. Depression in Patients With Systemic Sclerosis: A Systematic Review of the Evidence. Arthritis & Rheumatism (Arthritis Care & Research) Vol. 57, 2007, pp 1089–1097Table 1.Function, quality of life and gastrointestinal (GI) involvement assessment according to HADS score.Results, median [min, max]HADS-D ≥ 8 (n = 12)HADS-D < 8 (n = 8)P-valueHADS-A ≥ 8 (n = 14)HADS-A < 8 (n = 6)P-valueSHAQ- GI involvement26.5 [0, 90]2 [0, 40]0.00918.5 [0, 90]2.5 [0, 40]0.091- Lung involvement48.5 [5, 90]2.5 [0, 30]0.00118 [0, 90]3 [0, 65]0.126- Patient global assessment67.5 [30, 100]4 [0, 85]0.01153.5 [2, 100]41.5 [0, 85]0.509HAQ1.375 [0.5, 2]0.1875 [0, 1]0.0011.25 [0, 2]0.875 [0, 1.125]0.147EQ5D0.3667 [-0.0573, 0.6937]0.6752 [0.2870, 1]0.0060.4640 [-0.0573, 0.7667]0.6752 [0.287, 1]0.075SF36- Physical functioning25 [15, 75]75 [50, 100]0.00140 [15, 100]72.5 [25, 85]0.106- Role physical31.25 [0, 75]72.875 [31.25, 100]0.02537.5 [0, 100]65.625 [31.25, 100]0.214- Bodily pain41 [0, 74]68 [20, 88]0.01141 [0, 88]61.5 [20, 74]0.428- Vitality25 [0, 43.75]65.625 [25, 75]0.00137.5 [0, 75]65.625 [12.5, 75]0.135- Social functioning37.5 [12.5, 87.5]87.5 [50, 100]0.00250 [12.5, 100]87.5 [87.5, 100]0.003- Mental health45 [25, 80]65.7 [51.4, 85]0.01245 [25, 75]77.5 [51.4, 85]0.005UCLA SCTC GIT 2.0- Reflux0.38 [0, 1.25]0 [0, 1.25]0.0240.25 [0, 1.25]0 [0, 1]0.139- Distension1 [0.5, 2]0.25 [0, 1.5]0.0171 [0.25, 2]0.125 [0, 1]0.024- Total0.44[0.1, 0.99]0.04 [0, 0.97]0.0100.34 [0.04, 0.99]0.02 [0, 0.44]0.018Disclosure of Interests:None declared

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