ABSTRACT
BackgroundAnti-synthetase syndrome (ASS) is a rare auto-immune condition that combines autoantibodies and specifics clinical manifestations, including myositis, interstitial lung disease (ILD), polyarthritis, mechanic's hands, Raynaud's phenomenon, and unexplained fever. The hallmark of this syndrome is the presence of anti-aminoacyl-tRNA-synthetase (ARS) antibodies. Several anti-ARS antibodies have been described, anti-Jo1 being the most common, followed by anti-PL7, anti-PL12, anti-OJ, anti-EJ, anti-KS, anti-YRS, and anti-Zo. According to a recent epidemiological survey, the rising number of patients with autoimmune diseases, including idiopathic inflammatory myopathies (IIM) coincides with the COVID-19 pandemic.ObjectivesTo evaluate the clinical characteristics of ASS patients with different anti-ARS antibodies from a tertiary rheumatology center.MethodsWe conducted a retrospective, single-centered study on consecutive patients diagnosed with ASS from 1 January 2015 to 31 December 2022. Clinical and serologic data were obtained by medical records review from hospital database. Myositis-specific antibodies (MSA) and myositis-associated antibodies (MAA) were tested using commercial ELISA kits. We included all patients fulfilling Connor's criteria for ASS.ResultsSixty-one patients (44 females) with mean age 54.4 (13.8) years were included. The most frequently reported clinical manifestation was arthralgia (68.8%), followed by Raynaud's phenomenon (67.2%), ILD (65.6%), myositis (46%), mechanic's hands (44.3%), arthritis (39.3%), and fever (18.0%). The typical triad for ASS, including myositis, arthritis and ILD was present in 17 patients. Twenty-eight (45.9%) patients were PL7+, 21 (34.4%) were Jo1+, 3 (4.9%) were PL12+, and 2 (3.2%) were OJ+. Seven patients were positive for more than two anti-ARS antibodies. The most frequently found MAA was anti-Ro52 (n=23, 37.7%). Of the 61 patients included, 41 (67.2%) patients were diagnosed in the last 3 years (COVID-19 pandemic). The most frequently detected MSA in ASS patients diagnosed during COVID-19 pandemic was anti-PL7 (25/28), while anti-Jo1 was the most common MSA in ASS patients diagnosed before 2020 (p<0.05) (Fig 1).The anti-Jo1+ patients were younger, have significantly more frequent muscle involvement and significantly higher levels of CK than anti-PL7+ patients (p<0.05). The co-occurance of anti-Ro52 antibodies was more frequently observed in anti-Jo1+ patients (n=11, 52.4%) than in anti-PL7+ patients (n=6, 21.4%) (p<0.05). We did not find statistically significant differences between ASS groups regarding sex, disease duration, clinical manifestations including dermatologic lesions, Raynaud's phenomenon, arthralgia/arthritis, ILD, fever, and cancers (all p>0.05).ConclusionASS patients have heterogenous manifestations, and different types of anti-ARS antibodies are associated to distinct clinical and immunological features. The COVID-19 pandemic led to increase prevalence of ASS cases and to a remarkable shift in the anti-ARS antibodies profile, with increased frequency of anti-PL7 antibodies. Further studies are needed to investigate the link between SARS-CoV-2 infections and myositis.References[1]Witt LJ, et al. The Diagnosis and Treatment of Antisynthetase Syndrome. Clin Pulm Med. 2016 Sep;23(5):218-226.[2]Gracia-Ramos AE, et al. New Onset of Autoimmune Diseases Following COVID-19 Diagnosis. Cells. 2021 Dec 20;10(12):3592.[3]Connors GR, et al. Interstitial lung disease associated with the idiopathic inflammatory myopathies: what progress has been made in the past 35 years? Chest. 2010 Dec;138(6):1464-74.[4]García-Bravo Let al. Association of anti-SARS-COV-2 vaccine with increased incidence of myositis-related anti-RNA-synthetases auto-antibodies. J Transl Autoimmun. 2022 Jun 30;5:100160.Figure 1.ASS patients with positive anti-ARS antibodies per year (from 2015 to 2022). The green line shows the PL7+ patients;and the orange line shows the Jo1+ cases.[Figure omitted. See PDF]AcknowledgementsI have no acknowledgements to declare.Disclosure of Inter stsNone Declared.
ABSTRACT
Understanding COVID-19 vaccination attitudes is of paramount importance in order to estimate and influence the willingness to vaccinate. A cross-sectional study was performed in the spring of 2021 in Romania by sending a web-based questionnaire to patients with rheumatic autoimmune diseases in order to assess their willingness to vaccinate against SARS CoV-2 and to identify factors that might influence their decision. Variables derived from the questionnaire were analyzed in univariable and multivariable analysis. 71 patients, of which 60 (84.5%) women, were included in the study, with a 53.5% (38 subjects) willingness to vaccinate. A positive association was observed between the willingness to vaccinate and the patients' trust in authorities, trust in the healthcare system and the choice of scientific sources of information in their decision making. A positive correlation was observed between the intention to vaccinate and the global VAX score, as well as the subscale VAX scores (p<0.05). Global VAX score emerged as a predictive factor for the intention to vaccinate on logistic regression (p<0.05). The present study underlines the importance of reliable scientific sources of information for pa-tients with autoimmune diseases and how the general attitude about vaccination becomes a pre-dictive factor for accepting COVID-19 vaccine.
ABSTRACT
In order to address the main challenges related to the rare diseases (RDs) the European Commission launched the European Reference Networks (ERNs), virtual networks involving healthcare providers (HCPs) across Europe. The mission of the ERNs is to tackle low prevalence and RDs that require highly specialised treatment and a concentration of knowledge and resources. In fact, ERNs offer the potential to give patients and healthcare professionals across the EU access to the best expertise and timely exchange of lifesaving knowledge, trying to make the knowledge travelling more than patients. For this reason, ERNs were established as concrete European infrastructures, and this is particularly crucial in the framework of rare and complex diseases in which no country alone has the whole knowledge and capacity to treat all types of patients. It has been five years since their kick-off launch in Vilnius in 2017. The 24 ERNs have been intensively working on different transversal areas, including patient management, education, clinical practice guidelines, patients' care pathways and many other fundamental topics. The present work is therefore aimed not only at reporting a summary of the main activities and milestones reached so far, but also at celebrating the first 5 years of the ERN on Rare and Complex Connective Tissue and Musculo-skeletal Diseases (ReCONNET), in which the members of the network built together one of the 24 infrastructures that are hopefully going to change the scenario of rare diseases across the EU.
ABSTRACT
Background: In the context of the coronavirus (SARS-CoV-2) pandemic, several studies looked at the relation between rheumatic disease and SARS-CoV-2. It remains unclear whether these patients are at increased risk of developing more severe cases of coronavirus disease (COVID-19) or not. Objectives: The objective of this descriptive study is to report the characteristics and outcomes of rheumatic patients that had a history of confirmed SARS-CoV-2 infection. Findings have also been compared to some of the existing publications on COVID-19 in these patients. Methods: Between November 17, 2020 and January 18, 2021, a single-centre observational study was conducted in the rheumatology department of the Emergency Clinical County Hospital and the University of Medicine and Pharmacy Iuliu Hatieganu in Cluj-Napoca, Romania. The sample consisted of 62 rheumatic patients with a positive polymerase chain reaction test from nasopharyngeal/oral swab. Data on both systemic autoimmune disease and COVID-19 was collected using a survey, by means of telephone or in the outpatient clinic setting. Data on the patient rheumatologic condition was also collected from the electronic health records available within our department Results: 62 patients were included, with 85.48% females and 14.51% males, and a median age of 52 years (SD +/-14). The most frequent comorbidities were high blood pressure (HBP) 46.77%, dyslipidaemia 19.35%, liver disease 17.74%, and interstitial lung disease (ILD) 12.90%. Recurrent COVID-19 symptoms included: cough (62%), fever (46,77%), anosmia (46.77%), ageusia (30.64%), headache (29.03%), gastrointestinal symptoms (27.41%) and myalgia (25.80%). Out of the entire 62 sample, 41 patients had an inflammatory arthritis (IA) diagnosis, with the most frequent being rheumatoid arthritis (RA) -68.29%, followed by ankylosing spondylitis -21.95%, psoriatic arthritis -7.31% and 2.43% with Still disease. Only 10 patients suffered from connective tissue disease (CTD): 3 systemic lupus erythematosus, 2 poli/dermatomyositis, 2 Sjogren syndrome (SjS), 2 mixed connective tissue disease, 1 systemic sclerosis (SSc). Another 10 patients had overlapping syndromes with the most frequent (40%) overlap between RA and SSc. One patient had osteoarthritis. 49 patients followed a treatment with conventional synthetic disease-modifying anti-rheumatic drugs with 51.2% of them being treated with Methotrexate. 14 of our patients received glucocorticoids (GC), but no relation between the GC dose and COVID-19 severity could be observed. Only 3 patients with doses greater than 10mg/day were present in the cohort and 2 developed mild while 1 developed an asymptomatic COVID-19 course. 22 patients had received biological treatment. Anti TNF alpha medication was administered to 13 of these, and mostly consisted of Adalimumab, Etanercept and Golimumab. The anti TNF alpha patients were asymptomatic or had mild forms of COVID-19 (93.30%). 8 cases had ILD: 3 RA patients, 3 overlapping syndromes, 1 SSc and 1 SjS. The median age was 59,5 years (SD +/-10). 25% exhibited severe, 37,5% moderate, 25% mild and 12.5% asymptomatic COVID-19. The COVID-19 severity in our sample was as follows: 12.90% of the patients were asymptomatic, 59.67% exhibited a mild form, 19.35% a moderate one, and out of the 8.06% with a severe case of COVID-19, 1 patient died. The median age in the severe cases of COVID-19 was 66 years (SD +/-12) and HBP was the most common comorbidity. Conclusion: Most patients in this sample were either asymptomatic or had a mild COVID-19 evolution. Although the research design has multiple limitations, rheumatic pathology does not seem to be a higher risk factor for severe COVID-19 than other associated comorbidities. With that in mind, ILD patients should be closely monitored as even in on our limited sample size a worse evolution of COVID-19 has been observed. Biological treatments, especially anti TNF alpha might help in reducing the severity of COVID-19, but this outcome could have been associated in our sample with other factors like lower media age and less comorbidities.