Incidence and Clinical Outcomes of Newonset Autoimmune Connective Tissue Diseases after Covid-19 Infection- a Systematic Literature Review

Background/Aims Cases of new autoimmune and autoinflammatory conditions have been reported among COVID-19 survivors. A literature review on newonset autoimmune connective tissue diseases (ACTDs) following infection with COVID-19 is lacking.This systematic literature review aimed to evaluate the potential association between COVID-19 infection and the development of new-onset ACTDs in adults. Methods Articles published until September 2022, investigating the association between COVID-19 infection and new-onset ACTDs were included. The ''population'' searched was patients with disease terms for autoimmune connective tissue diseases, including (but not limited to) systemic lupus erythematosus (SLE), Sjogren's syndrome, systemic sclerosis (SSc), any idiopathic inflammatory myositis (IIM), antisynthetase syndrome, mixed CTD and undifferentiated CTD (and related MeSH terms), with ''intervention'' as COVID-19 and related terms. For terms for COVID-19, a dedicated search strategy developed by the National Institute for Clinical Excellence was used.Medline, Embase, and Cochrane databases were searched, restricted to English-language articles only. Eligible articles were: case reports and series (of any sample size), observational studies, qualitative studies and randomised controlled trials. Patients developing ACTDs without prior COVID-19 or reporting flares of existing ACTDs were excluded. Information was extracted on patient demographics, new ACTDs' onset time, clinical characteristics, COVID-19 and ACTD treatment, and COVID-19 and ACTDs outcomes. The protocol was registered in PROSPERO (CRD42022358750). Results After deduplication, 2239 articles were identified. After screening title and , 2196 papers were excluded, with 43 proceeding to fulltext screening. Ultimately, 28 articles (all single case reports) were included. Of the 28 included patients, 64.3% were female. The mean age was 51.1 years (range 20-89 years). The USA reported the most cases (9/28). ACTD diagnoses comprised: 11 (39.3%) IIM (including 4 cases of dermatomyositis);7 (25%) SLE;4 (14.3%) anti-synthetase syndrome;4 (14.3%) SSc;2 (7.1%) other ACTD (one diagnosed with lupus/MCTD overlap). Of eight, four (14.3%) patients (including that with lupus/MCTD) were diagnosed with lupus nephritis. The average onset time from COVID-19 infection to ACTD diagnosis was 23.7days. A third of the patients were admitted to critical care, one for ACTD treatment for SLE with haemophagocytic lymphohistiocytosis (14 sessions of plasmapheresis, rituximab and intravenous corticosteroids) and nine due to COVID-19. The majority (80%) of patients went into remission of ACTD following treatment, while two (10%) patients died- one due to macrophage activation syndrome associated with anti-synthetase syndrome and two from unreported causes. Conclusion Our results suggest a potential association between COVID-19 infection and new-onset ACTDs, predominantly in young females, reflective of wider CTD epidemiology. The aetiology and mechanisms by which ACTDs arise following COVID-19 infection remain unknown and require more robust epidemiological data.

A Systematic Review of New Rheumatic Immune-Mediated Inflammatory Diseases (R-Imid) Following Sars-Cov-2 Vaccinations

Background/Aims There are sporadic reports about the development of new rheumatic immune-mediated inflammatory diseases (R-IMIDs) in adults after receiving SARS-CoV-2 vaccines. This systematic review (SR) aimed to critically review and summarize the clinical profile, patient demographics, treatment, and prognosis of new-onset R-IMIDs following SARS-CoV-2 vaccination. Methods We retrieved English-language articles (Case reports and series and observational studies) on new-onset R-IMIDs following SARS-CoV-2 vaccination, published until June 2022, from standard databases (MEDLINE, Embase, Cochrane). The search strings used during the literature search incorporated 'SARS-CoV-2 vaccination' (along with related MeSH terms) and various key terms for R-IMIDs [which included (but was not limited to) inflammatory arthritis, connective tissue disease (CTD), vasculitis, systemic lupus erythematosus, Sjogren's syndrome, sarcoidosis, systemic sclerosis, idiopathic inflammatory myositis, anti-synthetase syndrome, Adult-onset Stills disease (AOSD), giant cell arteritis (GCA), and polymyalgia rheumatica (PMR)]. The protocol was registered in PROSPERO (CRD42022318561). Results Of the total 2179 articles retrieved, 1986 articles were excluded following the title- screening, and 107 articles that did not meet inclusion criteria. We included the remaining 86 articles (130 cases) upon full-text screening. Furthermore, we added four articles (six cases) based on a manual search, comprising 90 articles (136 cases) for final analysis. These 136 new R-IMID cases were reported from 27 different countries. Of these, more than one-third of the cases were reported from three countries (viz., Italy, Japan, and the USA). The patients had a mean age of 57 (range:17-90) years, and the majority were females (63.0%). Most patients developed R-IMIDs after receiving Pfizer-BioNTech vaccine (76;55%), followed by Oxford AstraZeneca vaccine (35;25%). The mean duration between SARSCoV- 2 vaccination and R-IMIDs development was 9.2 (range:1-90) days. The second dose of the vaccine resulted in more R-IMIDs (74;54%) than the first (53;39%). CTDs (34;25%) and small vessel vasculitis (33;24%) were the commonest R-IMID manifestations, followed by inflammatory arthritis and AOSD, each in 13 (9.5%) cases. Nearly half of the patients with CTDs had Idiopathic Inflammatory Myositis. PMR and GCA accounted for 16 (11.7%) and 5 (3.6%) cases, respectively. However, no cases of axial spondylarthritis were reported. Most (118;86%) R-IMID patients were treated with corticosteroids, with a small number receiving steroid-sparing drugs, such as methotrexate, rituximab and cyclophosphamide. Most (125;91%) went into either disease remission or improvement following the treatment. Only three patients were admitted to the intensive care unit (ICU) to manage their disease;One of them died due to fatal myositis and rhabdomyolysis;two surviving ICU patients had ANCA-associated vasculitis with lung involvement. Conclusion Although rare, this SR highlights the emergence of de novo R-IMIDs following SARS-CoV-2 vaccination. We cannot confirm the causality between the vaccination and the onset of R-IMID. However, further research is warranted in this area.

Hypogammaglobulinemia after Rituximab Treatment in an Italian Real-Life Cohort of Iim

Background. Rituximab (RTX) is a chimeric monoclonal antibody that binds the CD20 molecule on the surface of B cells and leads to B cell depletion. RTX is recommended by the European League Against Rheumatism (EULAR) as off-label in patients affected by idiopathic inflammatory myopathies (IIM). The real-world experience has shown that hypogammaglobulinemia occurring early after anti-CD20 treatment can be multifactorial (active disease, effect of other drugs) and usually transient, with a minimal increase in the risk of infections. The present study aimed to analyse the differences in the rate of RTX-associated hypogammaglobulinemia in a cohort of IIM patients in clinical practice, as well as the onset of major infections and its correlation with hypogammaglobulinemia. Methods. Patients followed at Rheumatology Unit of Siena University Hospital from January 2020 to September 2021 were retrospectively enrolled. Inclusion criteria were as follows: fulfilment of disease-specific classification criteria 2017 EULAR criteria and /or Peter and Bohan criteria for dermatomyositis (DM) and polymyositis (PM), positivity of anti-synthetase antibody and typical clinical features for anti-synthetase syndrome (ASS) and the measurement of serum Ig levels at the time of RTX administration (maximum 2 weeks before) (T0) and 6 (T1) to 12 (T2) months later, consistently with previous studies. Ig serum levels, measured by standard nephelometry (normal ranges: IgG 700-1600 mg/dL, IgM 40-240 mg/dL, IgA 70-400 mg/dL) were assessed as part of routine clinical care. Hypogammaglobulinemia was defined as moderate (serum IgG <600 mg/dL) and severe (IgG <400 mg/dL), as previously reported. Results. Seven patients (mean+/-SD, 57.3+/-19.7 years;7 female) were enrolled. Three of them had diagnosis of DM, three ASS and one PM. Two patients showed MDA5-positivity, two JO1-positivity, one TIF1-gamma-positivity, one PL7-positivity and the other one PM/Scl-positivity. All patients had at least two organs involved, and 4 out of 7 (57%) suffered from interstitial lung disease. Before starting RTX treatment, three and four patients underwent at least one and two synthetic immunosuppressants. All patients underwent low dosage of corticosteroids, and four patients underwent concomitant synthetic immunosuppressants (2 hydroxychloroquine and 2 MTX). IgG concentrations were statically lower at T2 compared to those at baseline (p=0.0391). None of them showed severe hypogammaglobulinemia. Similarly, IgM concentration significantly decreased at T2 compared to those at baseline (p=0.0078). Two patients showed major infections and two patients had paucisymptomatic COVID-19 (one of them had twice). Corticosteroids dosages were inversely correlated with IgG T2 concentrations (p=0.040, r=-0.919). Conclusion. Hypogammaglobulinemia following RTX is uncommon in IIM and is more likely in patients with high glucocorticoids, immunosuppressants and CYC exposure. IgG monitoring at least 6 months after RTX treatment may be useful in stratifying patients to identify those who require closer monitoring. These results shine a spotlight for increased awareness of the role of immunoglobulin measurement before maintenance doses of RTX.

Impaired Promis Physical Function in Idiopathic Inflammatory Myopathy Patients: Results from the Multicenter Covad Patient-Reported E-Survey

Background. Evaluation of physical function is fundamental in the management of idiopathic inflammatory myopathies (IIMs). Patient-Reported Outcome Measurement Information System (PROMIS) is a National Institute of Health initiative established in 2004 to develop patient-reported outcome measures with improved validity and efficacy. The present study aims to investigate the physical function status of IIM patients compared to those with non-IIM autoimmune diseases (AIDs) and healthy controls (HCs) utilizing PROMIS Physical Function (PF) data obtained in the coronavirus disease-2019 (COVID-19) Vaccination in Autoimmune Diseases (COVAD) study, a large-scale, international self-reported e-survey assessing the safety of COVID-19 vaccines in AID patients. Methods. The survey data regarding demographics, IIM and AID diagnosis, disease activity, fatigue and pain VAS, and PROMIS PF short form-10a were extracted from the COVAD study database. The disease activity (active vs inactive) of each patient was assessed in 3 different ways: (1) physician's assessment (active if there was increased immunosuppression), (2) patient's assessment (active vs inactive as per patient), and (3) current steroid use. These 3 definitions of disease activity were applied independently to each patient. PROMIS PF-10a scores were compared between each disease category (IIMs vs non-IIM AIDs vs HCs), stratified by disease activity based on the 3 definitions stated above, employing negative binomial regression model, and the predicted PROMIS PF-10a score adjusted for age, gender, and ethnicity was calculated. Factors affecting PROMIS PF-10a scores other than disease activity were identified by multivariable regression analysis in the patients with inactive disease (IIMs or non-IIM AIDs). The association between fatigue or pain VAS and PROMIS PF-10a scores was also assessed. Results. 1057 IIM patients, 3635 non-IIM AID patients, and 3981 HCs responded to the COVAD survey until August 2021. The median age of the respondents was 43 [IQR 30-56] years old, and 74.8% were female. Among IIM patients, dermatomyositis was the most prevalent diagnosis (34.8%), followed by inclusion body myositis (IBM) (23.6%), polymyositis (PM) (16.2%), anti-synthetase syndrome (11.8%), overlap myositis (7.9%), and immune-mediated necrotizing myopathy (IMNM) (4.6%). The predicted mean of PROMIS PF-10a scores was significantly lower in IIMs compared to non-IIM AIDs or HCs (36.3 [95% (CI) 35.5-37.1] vs 41.3 [95% CI 40.2-42.5] vs 46.2 [95% CI 45.8-46.6], p<0.001), irrespective of disease activity (Figure 1). The results were consistent across analyses using different disease activity definitions (physician's assessment, patient's assessment, and steroid use), while the largest difference between active IIMs and non-IIM AIDs was observed when the disease activity was defined by patient's assessment (35.0 [95% CI 34.1-35.9] vs 40.1 [95% CI 38.7-41.5]). Considering the subgroups of IIMs, the scores were significantly lower in IBM in comparison with non-IBM IIMs (p<0.001). The independent factors associated with low PROMIS PF-10a scores in the patients with inactive disease were older age, female gender, and the disease category being IBM, PM, or IMNM. Higher fatigue or pain VAS was associated with lower PROMIS PF-10a scores in each disease category (p<0.001), while the scores were still lower in IIMs compared to non-IIM AIDs even after being adjusted for fatigue and pain. Conclusion. Physical function is significantly impaired in IIMs compared to non-IIM AIDs or HCs, even in patients with inactive disease. The elderly, women, and IBM groups are the worst affected, suggesting that developing targeted strategies to minimize functional disability in certain groups may improve patient-reported physical function and disease outcomes.

Rituximab in the Treatment of Idiopathic Inflammatory Myopathies with Interstitial Lung Disease, Long-Term Follow-Up

Background. Interstitial lung disease (ILD) is the common internal organ manifestation of idiopathic inflammatory myopathies (IIM) that can severely affect the course and prognosis of the disease. Rituximab (RTX) has been used to treat IIM, including variants with ILD. Objectives. To describe the course of disease in IIM patients with ILD, treated with RTX in long-term follow-up. Methods. Our prospective study included 35 pts with IIM fulfilling Bohan and Peter criteria and having ILD. The mean age was 51.8+/-11.9 years, female-26 pts (74%);24 (68.5%) with antisynthetase syndrome, 5 (14.3%) dermatomyositis (DM), 5 (14.3%) with a-Pm/Scl overlap myositis and 1 (2,9%) with a-SRP necrotizing myopathy were included. 25 (71,4% ) patients had nonspecific interstitial pneumonia, 9 (25,7%) organizing pneumonia (OP) and 1 (2,9%) OP, transformed to diffuse alveolar damage. All pts had the standard examination including manual muscle testing (MMT), creatinkinase (CK) anti-Jo-1 antibodies (anti-Jo-1) assay;forced vital capacity (FVC) and carbon monoxide diffusion capacity (DLCO) evaluation as well as high-resolution computed tomography (HRCT) scanning of the chest were performed at baseline, and 36 and more months. The median disease duration was 3.2 [0.16-18] years, 21 (60%) of pts were positive for a-Jo-1 antibody. All pts received prednisolone at a mean dose of 24.3+/-13 mg/day, immunosupressants at inclusion received 25 (71%) pts: cyclophosphamide 18 , mycophenolate mofetil 6 and comdination 1;Rituximab (RTX) was administered in case of severe course of disease and intolerance or inadequate response to GC and other immunosuppressive drugs. Results. The mean follow-up period after the first infusion of RTX was 47.2+/-11.9 months. Pts received 1-11 courses of RTX . The cumulative mean dose of RTX was 4.6 +/-2.5g. MMT 8 increased from 135.8+/-13.5 to 148.75+/-3.5 (p=0.000001). CK level decreased DELTACK - 762 u/l(median 340;25th% 9;75th% 821). anti-Jo-1 decreased from 173.4+/-37 to 96.5+/-79 u/ml (p=0.00002), FVC increased from 82+/-22.6 to 96,9+/-22% (p=0.00011). DLCO increased from 51.4+/-15.2 to 60+/-77.8% (p=0.0001). The mean prednisone dose was reduced from 24.3+/-13 to 5.7+/-2.4 mg/day. 3 pts died: ILD progression was the cause of death in 1 case, 1 bacterial pneumonia and COVID19 pneumonia. Conclusions. The results of this study confirm the positive effect of RTX in IIM patients with ILD (increase of muscle strength and improve lung function, decrease in anti-Jo-1 levels) and also its good steroid-sparing effect. RTX could be considered as an effective drug for the complex therapy of IIM patients with ILD when standard therapy is ineffective or impossible.

Working Group for Myositis within the Swedish Rheumatism Association

The Swedish Rheumatism Association, our umbrella Organization: In Sweden, there are approximately one million people with different rheumatic diseases, and about 1400 of them have a myositis diagnosis. In addition to several local associations, there are 3 nationwide diagnostic groups for systemic inflammatory diseases: Working group for systemic lupus erythematosus (SLE), Working group for Systemic Sclerosis and Working group for Myositis. Goals and vision: We form opinion and influence politicians and decision-makers at all levels in issues that are important to us, such as access to rapid care and opportunities for rehabilitation. Knowledge and Education: We educate: * Representatives who can share knowledge based on their own experience and to provide support and help for people living with rheumatic disease. * Volunteers for patient schools. * Patient Research Partners since 2008. Research and fundings: : * We are the single largest private funder of Swedish rheumatology research. * Patient Research Partners should become obvious members in research projects. Working group for Myositis was established in 2020 and most of our activities have been on-line. The number of members is growing as we spread out the information. We will continue with our on-line events and together with our experts arrange our first patient conference in 2022. We are a member of the Swedish Rare Disease Association and European Network ERN ReCONNET. We have now three Patient Research Partners with myositis and we will continue to participate in international research projects, such as IMACS, Rehabilitation & exercise SIG. Our mission is to give support to myositis patients and their families, share knowledge of their disease, facilitate meeting with others with the same diagnosis for an exchange of experiences or just for fun. Our goals are to: * Inform through newsletters, patient meetings, website and webcasts. * Arrange lectures by myositis experts. * Arrange annual patient conference. * Raise awareness for the disease in society and inform healthcare professionals within primary care units. * Contribute to that all patients receives equally good care all over the country. * Inform about research results, ongoing studies and update information on new treatments and drugs. * Contribute to that all newly diagnosed patients have access to patient education and written information material about myositis. * Contribute for opportunities for rehabilitation, such as training in warm water pools and access to rehabilitation facilities in warm climate. * Collaborate with the Youth organization of the Swedish Rheumatism Association for Juvenile Dermatomyositis and provide support for parents, children and adolescents. * Collaborate with the myositis organizations in other countries. Our Webinars: The experts who have shared their knowledge on our webinars are: Ingrid Lundberg, Professor;Maryam Dastmalchi, MD, Rheumatologist;Helene Alexanderson, PhD, Associate professor, PT;Malin Regardt, PhD, OT;Balsam Hanna, Specialist Rheumatology;Dag Leonard, MD, Rheumatologist;Antonella Notarnicola, MD, Rheumatologist;Fabricio Espinosa, Rheumatologist, PhD candidate;Kristofer Andreasson, PT, PhD candidate;Jonatan Sjogren, OT;Lars Nordelv, CBT Therapist, also a patient;Helena Andersson, MD, Rheumatologist;Hanna Brauner, PhD, Dermatologist. Among the topics our webinars have covered so far are: Diagnostic criteria of myositis, new research findings, existing treatments and ongoing studies, Physical activity and its effects on depression, safety of high-intensity interval training, Occupational therapy, Patient Reported Outcomes, Myositis Associated Antibodies and how to deal with anxiety, cardiac involvement and osteoporosis in myositis, clinical findings and treatments for Antisynthetase syndrome skin involvement in Dermatomyositis, Covid-19 and vaccination.

Dutch Myositis Working Group (Dmwg)

Introduction. The poster of the Dutch Myositis Working Group (DMWG) aims to inform people about her goals, activities and ambitions. The group is run by seven patients, representing all types of myositis, supported by Spierziekten Nederland, the umbrella patient organization for neuromuscular disorders in The Netherlands and 4 myositis specialists as medical advisors. Chair: Ingrid de Groot. Contact email: myositis@spierziekten.nl Goals and ambitions of the Dutch myositis working group: * I n collaboration with medical advisors to provide information about IIM (idiopathic inflammatory myopathies) or myositis to newly diagnosed patients and their families: IIM types, symptoms, diagnosis, (new) treatment options, prognosis, inform them about the myositis expertise centres etc. * To connect and support people with all types of IIM: dermatomyositis (DM), polymyositis (PM), Anti Synthetase Syndrome (ASyS), immune mediated necrotizing myopathy (IMNM), juvenile dermatomyositis (JDM), overlap myositis. * To raise awareness of myositis among the public, health care professionals and researchers, pharmaceutical companies? * To collaborate with clinicians, researchers and funds on a national and international level with the aim to improve (clinical) care and research. * To stimulate and participate in the development and conducting of clinical trials. * To collaborate with myositis working groups and patient organisations abroad. * To represent the patient perspective within in the Myositis Network Netherlands and (inter)national myositis study groups. * Patient advocacy. Activities and services: * In person or online meetings aiming to offer moral support and an opportunity to share experiences, concerns etc. or just to socialize. Three times a year we organize separate meetings for people with IBM, for people with other IIM and for caregivers. * Website updates on treatment, guidelines, (inter)national research, activities and actualities (e.g. Covid situation). * Supply patients with brochures for GP/ family doctor, physiotherapist etc. * Online (secured) platform for members. * Annual patient conference with diagnosis specific scientific programs. * Monthly newsletters: these are personalized which means they contain mainly news on the receivers type of IIM (e.g. IBM or ASyS) and information on general topics concerning all people with IIM or neuromuscular disorder. * In person meetings and / or online webinars on general topics e.g. living with a chronic condition, work, pain, fatigue. * Annual meetings with medical advisors: the working group pays a visit to all medical advisors in their respective hospitals. * Representation at (inter)national conferences. * Representation in projects such as guidelines development. * Collaboration in (inter)national studies leading to enrolling Dutch patients, researchers and clinicians in multi-centre studies, (co-) authorships in publications and to presentations during conferences (Treat NMD, IMACS, MNN). * To advise and recommend on research proposals from patient perspective. * To advise decision makers on continuation of expert centres from patient perspective. Collaborations: * Myositis Network Netherlands: patient representation on the board. * OMERACT (Outcome Measures in Rheumatology): Patient Research Partner of the Myositis Working Group. * IMACS (International Myositis Assessment and Clinical Studies Group): steering committee member of Exercise & Rehabilitation Group, led by Helene Alexanderson, ass.prof PhD, RPT). * ENMC (European Neuromuscular Centre): patient representation in myositis workshops. * EULAR (European League against Rheumatism): member of PARE and Patient Research Partner. * GCOM. * ERN - NMD (European Reference Network for Neuromuscular Diseases): member of NMD working group led by em. prof. dr. Marianne de Visser. * Patient organizations for people living with myositis . We are in this together Since myositis is a (very) rare disease, the 'myositis community' is a small one although we're happy to say that it is expanding quite rapidly. Through our inte sive involvement in several national and international studies and research projects we now have close contacts with many myositis experts across the globe, which makes it easier to keep up with actualities and developments concerning research, treatment etc. and to disseminate this knowledge to our members. This helps us to inform, support and advocate for the Dutch people living with myositis and their families and at the same time it offers opportunities to give something back: by sharing with the research community and clinicians our experiential knowledge of the consequences of myositis on everyday life. That way we can contribute to more meaningful research. We can only go forward if we do this together! That is why we are very ambitious in our efforts to contribute to myositis research. Here we list our collaborative efforts: * In 2019 the Myositis Network Netherlands of clinicians and researchers with expertise in IIM was established in which the DMWG is representing the patient perspective by a member on the board. * In OMERACT Myositis Working Group a member of the DMWG is one of the two Patient Research Partners and as such an equal partner of this study aiming to define a set of core patient reported domains with regard to the quality of life and respective instruments for use in IIM. The involvement of the DMWG has led to the opportunity for Dutch patients to participate in Delphi surveys and to an opportunity for Dutch myositis clinics to collaborate in the longitudinal study that emerged from this. * The IMACS network is an important part of our international network. One of our DMWG members is member of the Executive Committee of the Exercise & Rehabilitation Group and as such can facilitate for Dutch patients to become involved in the current study with the ultimate objective to develop recommendations for exercise in all types of IIM. * Members of the DMWG participated in several ENMC workshops on IIM as patient representatives and will continue to do so in the future. * Through a PARE membership in EULAR and membership of the study group of 'collaborative research' the DMWG hopes to raise awareness of myositis within the influential EULAR community and to speak up on behalf of the patients in Europe living with IIM. * One of our members is member of the GCOM committee responsible for the patient program of GCOM and shares the ambitions of this GCOM committee to increase the involvement of patients in this very important IIM conference. * One DMWG member joined the ERN- Neuromuscular Disease group and as such represents the people with IIM living throughout Europe. * DMWG has ambitions to empower people living with IIM and to connect with them, crossing borders by doing so. We have close and amicable relationships with patient organisations in Australia, Czech Republic, Germany, Sweden, UK and USA. * Empowering patients is one of our goals and we accomplished this for instance in Sweden. On invitation by prof. dr. Ingrid Lundberg our chair visited the Karolinska Institute, spent a week with their myositis team and in return was one of the speakers on the annual patient meeting and helped the Swedish patients establish their own myositis working group.

Rheumatic diseases and Covid-19 infection -A case series

Purpose: To describe the outcome of Covid-19 infected patients with underlying rheumatic diseases in a rheumatology center in Malaysia. Introduction: Several risk factors for Covid-19 infection have been recognized since the onset of pandemic. Whether patients with rheumatic diseases are more susceptible to severe Covid-19 infection remain unclear. Method(s): This was a retrospective study. The electronic medical records of all Covid-19 infected patients with underlying rheumatic diseases who follow up in rheumatology clinic Hospital Sultan Ismail from March 2020 to December2021 were reviewed and identified. Result(s): There were total of 40 patients with 95% of them were female (38/40). Majority of them were Malay (31/40) followed by Chinese (6/40), Indian (2/40) and others (1/40). The mean age group was 46 (range from 21 to 75). 55% of them were diagnosed to have Systemic Lupus Erythematosus (SLE), followed by Rheumatoid Arthritis (RA, 27.5%), scleroderma (Ssc,5%) and 2.5 % each for Sjogren syndrome (Sjog), psoriatic arthritis (PsA), gout, antisynthetase syndrome and dermatomyositis (DM) overlap RA. 72.5% of them were unvaccinated and only 7.5% of them were completed 2 doses of covid-19 vaccine whereas the rest of them only had single dose. The results showed 17.5% of them succumbed to covid-19 infection and 5 of them succumbed for Covid-19 pneumonia stage 5. 70% of the patients who succumbed were unvaccinated during covid-19 infection. Conclusion(s): There were 5 patients from chronic inflammatory arthritis and 2 from systemic autoimmune conditions succumbed due to Covid-19 infection. Whether patients with chronic inflammatory arthritis more prone to infection required more data. Majority of patients who succumbed were unvaccinated. (Table Presented).

A Case of Antisynthetase Syndrome Initially Presented With Interstitial Lung Disease Mimicking COVID-19.

In this case report, we present a case of antisynthetase syndrome which is a rare disease that can be easily missed, if not specifically looked for in adults, whose initial presentation is combination of myopathic and respiratory symptoms. In clinical practice, patients presenting with coronavirus disease 2019 (COVID-19) symptoms, whose computed tomography (CT) imaging is consistent with COVID-19, were accordingly isolated and treated as COVID-19 awaiting reverse transcription polymerase chain reaction (RT-PCR) results. However, there are many COVID-19 mimics on chest CT, which can make the CT-based diagnosis of COVID-19 unsafe.

A case of antisynthetase syndrome presenting solely with life-threatening interstitial lung disease.

A previously fit and well 38-year-old man presented during the COVID-19 pandemic with dyspnoea, cough and palpitations. C-reactive protein was elevated and chest X-ray demonstrated bilateral lower zone consolidation. SARS CoV-2 swab was negative. He was diagnosed with community-acquired pneumonia and treated with oral antibiotics. He developed severe type 1 respiratory failure and was admitted to the high-dependency unit for non-invasive ventilation. CTPA was negative for pulmonary embolism, instead demonstrating bilateral organising pneumonia. Empirical treatment for swab-negative COVID-19 pneumonitis was started; however, further deterioration ensued and prompted intubation and ventilation. Microbiological testing did not yield any positive results, thereby raising suspicion for the presence of an autoimmune disease. Pulsed intravenous methylprednisolone was administered with good effect. ENA screen was positive for anti-Jo1 and myositis-specific autoantibodies were positive for Ro-52, Ku and PL-12. The patient was extubated and did not exhibit any muscle weakness on clinical examination. Creatine kinase was only mildly elevated. He was diagnosed with amyopathic antisynthetase syndrome - frequently considered as a form of idiopathic inflammatory myopathy (IIM) - and treated with further intravenous methylprednisolone and cyclophosphamide. Oxygen therapy was gradually weaned and the patient discharged on mycophenolate mofetil and a weaning course of oral steroids.

ANTISYNTHETASE SYNDROME PRESENTING AS INTERSTITIAL LUNG DISEASE: A CASE OF ANCHORING BIAS DURING THE COVID-19 PANDEMIC

SESSION TITLE: Dyspne Mysteries SESSION TYPE: Rapid Fire Case Reports PRESENTED ON: 10/18/2022 01:35 pm - 02:35 pm INTRODUCTION: Anti-synthetase (AS) syndrome is characterized by interstitial lung disease (ILD), arthritis, myositis, fever, or Raynaud's phenomenon in the presence of an AS autoantibody (1). At least 70% of patients with AS syndrome develop ILD (2), and it represents the major cause of mortality in these patients with a 10 year survival rate of 73%. In a small cohort study, the anti-PL-12 antibody subtype was found to be strongly associated with ILD (3). CASE PRESENTATION: A 35 year old female with a history of tobacco use disorder presented to the hospital with three months of recurrent subjective fevers, non-productive cough, and dyspnea on exertion. She denied arthralgias, muscle weakness and hemoptysis. She initially presented to her primary care physician with these symptoms and was prescribed amoxicillin for streptococcal pharyngitis. The patient continued to be symptomatic and was treated empirically for COVID-19 pneumonia twice despite two negative COVID-19 tests and without any significant clinical improvement in her respiratory status. On admission, she was febrile, tachycardic, and had a new oxygen requirement with bilateral coarse breath sounds on exam. She had no leukocytosis and her COVID-19 test was negative. CT angiography of the chest showed extensive mixed reticular and airspace opacities with peribronchial predilection and peripheral sparing (figure 1). A bronchial alveolar lavage was notable only for neutrophilia (19%) and eosinophilia (4%). Rheumatological workup revealed elevated rheumatoid factor, positive antinuclear antibody (1:40), weakly positive anti–Sjögren's-syndrome-related antigen A antibody (50 AU/ml), undetectable anti-Jo-1 antibody and positive anti-PL-12 antibody. Pulmonary function testing revealed a TLC of 40% and DLCO of 28%, consistent with a restrictive pattern. Considering the patient's organizing pneumonia, positive antibodies, and findings of "mechanic's hands,” the patient was diagnosed with anti-synthetase syndrome with ILD. She was started on oral prednisone and mycophenolate mofetil. On follow-up, she was noted to have symptomatic improvement and stable hypoxia without clinical signs of disease progression. DISCUSSION: During the coronavirus pandemic, the resemblance of COVID-19 pneumonia to other diseases, in the absence of conscious suspicion for other etiologies, can lead to anchoring and availability bias thereby delaying diagnosis and appropriate treatment. Additionally, anti-synthetase syndrome should be considered in the differential diagnosis of ILD even in the absence of arthritis and myositis, as respiratory symptoms are often the first presenting signs. CONCLUSIONS: Increased responsibility is required of diagnosticians to exercise due diligence and active recognition of COVID availability and anchor bias to avoid missing crucial diagnoses. Reference #1: Cojocaru, Manole, Inimioara Mihaela Cojocaru, and Bogdan Chicos. "New insights into antisynthetase syndrome.” Maedica 11.2 (2016): 130. Reference #2: Marco, Joanna L., and Bridget F. Collins. "Clinical manifestations and treatment of antisynthetase syndrome.” Best Practice & Research Clinical Rheumatology 34.4 (2020): 101503. Reference #3: Kalluri, Meena, et al. "Clinical profile of anti-PL-12 autoantibody: cohort study and review of the literature.” Chest 135.6 (2009): 1550-1556. DISCLOSURES: No relevant relationships by Mario Flores No relevant relationships by David Jackson No relevant relationships by Lisa Saa No relevant relationships by Abu Baker Sheikh

IDIOPATHIC INFLAMMATORY MYOPATHIES WITH INTERSTITIAL LUNG DISEASE TREATED WITH RITUXIMAB AND LONG-TERM FOLLOW-UP

SESSION TITLE: Treatment Debates in Critical Care SESSION TYPE: Rapid Fire Original Inv PRESENTED ON: 10/17/2022 12:15 pm - 1:15 pm PURPOSE: Interstitial lung disease (ILD) is the common internal organ manifestation of idiopathic inflammatory myopathies (IIM) that can severely affect the course and prognosis of the disease. Rituximab (RTX) has been used to treat IIM, including variants with ILD. OBJECTIVES:To describe the course of disease in IIM patients with ILD, treated with RTX in long-term follow-up. METHODS: Our prospective study included 35 pts with IIM fulfilling Bohan and Peter criteria and having ILD. The mean age was 51.8±11.9 years, female-26 pts (74%);24 (68.5%) with antisynthetase syndrome, 5 (14.3%) dermatomyositis (DM), 5 (14.3%) with a-Pm/Scl overlap myositis and 1 (2,9%) with a-SRP necrotizing myopathy were included. 25 (71,4% ) patients had nonspecific interstitial pneumonia, 9 (25,7%) organizing pneumonia (OP) and 1 (2,9%) OP, transformed to diffuse alveolar damage. All pts had the standard examination including manual muscle testing (MMT), creatinkinase (CK) anti-Jo-1 antibodies (anti-Jo-1) assay;forced vital capacity (FVC) and carbon monoxide diffusion capacity (DLCO) evaluation as well as high-resolution computed tomography (HRCT) scanning of the chest were performed at baseline, and 36 and more months. The median disease duration was 3.2 [0.16-18] years, 21 (60%) of pts were positive for a-Jо-1 antibody. All pts received prednisolone at a mean dose of 24.3±13 mg/day, immunosupressants at inclusion received 25 (71%) pts: cyclophosphamide 18, mycophenolate mofetil 6 and comdination 1;Rituximab (RTX) was administered in case of severe course of disease and intolerance or inadequate response to GC and other immunosuppressive drugs. RESULTS: The mean follow-up period after the first infusion of RTX was 47.2±11.9 months. Pts received 1-11 courses of RTX. The cumulative mean dose of RTX was 4.6 ±2.5g. MMT 8 increased from 135.8±13.5 to 148.75±3.5 (p=0.000001). CK level decreased ΔCK – 762 u/l(median 340;25th% 9;75th% 821). anti-Jo-1 decreased from 173.4±37 to 96.5±79 u/ml (p=0.00002), FVC increased from 82±22.6 to 96,9±22% (p=0.00011). DLCO increased from 51.4±15.2 to 60±77.8% (p=0.0001). The mean prednisone dose was reduced from 24.3±13 to 5.7±2.4 mg/day. 3 pts died: ILD progression was the cause of death in 1 case, 1 bacterial pneumonia and COVID19 pneumonia. CONCLUSIONS: The results of this study confirm the positive effect of RTX in IIM patients with ILD (increase of muscle strength and improve lung function, decrease in anti-Jo-1 levels) and also its good steroid-sparing effect. CLINICAL IMPLICATIONS: RTX could be considered as an effective drug for the complex therapy of IIM patients with ILD when standard therapy is ineffective or impossible DISCLOSURES: No relevant relationships by Lidia Ananyeva No relevant relationships by Maria Aristova No relevant relationships by Oxana Desinova No relevant relationships by Liudmila Garzanova No relevant relationships by Anna Khelkovskaya-Sergeeva No relevant relationships by Olga Koneva No relevant relationships by Dmitry Kulikovsky

A CASE OF ANTISYNTHETASE SYNDROME: THE IMPORTANCE OF HISTORY IN DISTINGUISHING ETIOLOGY OF INTERSTITIAL LUNG DISEASE IN A COVID-19 WORLD

SESSION TITLE: Systemic Diseases Causing Pulmonary Havoc SESSION TYPE: Rapid Fire Case Reports PRESENTED ON: 10/18/2022 10:15 am - 11:10 am INTRODUCTION: In the coronavirus disease 2019 (COVID-19) era, the etiology of interstitial lung disease (ILD) should remain broad to ensure accurate diagnosis and the proper treatment of patients. Vital to the art of medicine is taking a comprehensive history, and anchoring on a common diagnosis such as COVID-19 can result in early dismissal of alternate etiologies that physicians have an obligation to explore. CASE PRESENTATION: A 58-year-old male with a history of diabetes, hypothyroidism, and hypertension presented to the emergency department (ED) with dyspnea and fever. Initial CT chest imaging was significant for reticular and fibrotic changes with peripheral ground-glass and solid nodular opacities, some with areas of central clearing. Despite negative PCR testing, he was diagnosed with COVID-19 and discharged on oxygen with pulmonary follow-up. He continued to have arthralgias, proximal muscle weakness, low-grade fevers, and weight loss. He re-presented to the ED and was admitted for hypovolemia and further exploration into a potential autoimmune etiology of his symptoms. Labs were significant for a creatine kinase of 3,381 U/L, positive autoimmune antibodies [ANA (1:320), Jo-1 (>8.0 U), and SS-A/Ro (1.4 U)], and elevated ESR and CRP (30 mm/hr and 81 mg/L). Repeat CT revealed persistent parenchymal changes. Bronchoscopy was performed without anatomical abnormalities, and bronchoalveolar lavage (BAL) fluid was normal in appearance and negative for infectious etiologies. Though the patient was a farmer and possessed risk factors for hypersensitivity pneumonitis, lack of lymphocytic predominance on BAL, negative hypersensitivity panel, and uncharacteristic CT findings helped exclude this diagnosis. The patient was diagnosed with antisynthetase syndrome and treated with pulse dose intravenous solumedrol before transitioning to prednisone with resolution of muscle weakness and radiographic improvement in lung infiltrates. Muscle biopsy was deferred given the rapid clinical response and serum markers consistent with the diagnosis. DISCUSSION: Antisynthetase syndrome is a rare cause of ILD and often presents with myositis, arthritis, skin changes, Raynaud's phenomenon, and fever [1]. These symptoms, combined with the aminoacyl-tRNA synthetase antibody—most commonly the Jo-1 antibody—help confirm the diagnosis [2]. Due to a lack of established diagnostic criteria, muscle biopsy is often used to exclude other causes of myositis [3]. The ILD associated with antisynthetase syndrome is a significant cause of morbidity and mortality, and delay in diagnosis can lead to progression of lung injury. CONCLUSIONS: Chest imaging findings in COVID-19 are nonspecific, and post-COVID lung disease often presents similarly to other ILDs [1]. Because of this, history and physical exam remain crucial tools to reflect on alternate diagnoses for ILD and will continue to be necessary as we evolve through this COVID-19 era. Reference #1: Devi HG, Pasha MM, Padmaja MS, Halappa S. Antisynthetase Syndrome: A Rare Cause for ILD. Journal of Clinical and Diagnostic Research : JCDR. 2016;10(3):OD08. doi:10.7860/JCDR/2016/16872.7361 Reference #2: Cavagna L, Trallero-Araguás E, Meloni F, et al. Influence of Antisynthetase Antibodies Specificities on Antisynthetase Syndrome Clinical Spectrum Time Course. Journal of Clinical Medicine. 2019;8(11). doi:10.3390/jcm8112013 Reference #3: Schmidt J. Current Classification and Management of Inflammatory Myopathies. J Neuromuscul Dis. 2018;5(2):109-129. doi: 10.3233/JND-180308. PMID: 29865091;PMCID: PMC6004913. DISCLOSURES: No relevant relationships by Dustin Norton No relevant relationships by Alyssa Simon No relevant relationships by Kang Rui Xiang

UNUSUAL CASE OF ANTI-SYNTHETASE SYNDROME CORRELATED WITH EXPOSURE TO COVID-19 INFECTION OR VACCINE

SESSION TITLE: Systemic Disease with Diffuse Lung Symptoms Case Posters SESSION TYPE: Case Report Posters PRESENTED ON: 10/17/2022 12:15 pm - 01:15 pm INTRODUCTION: Since its first detection at Wuhan, China, SARS-CoV-2 (novel coronavirus 2019) has engulfed the world with more than 100 million cases and manifestations of COVID19 have been evolving over time. Various post COVID19 syndromes are being recognized. Reactive arthritis, connective tissue disorders such as myositis and pulmonary complications have been correlated with exposure to COVID infection. We describe the case of antisynthetase syndrome in a patient correlated with exposure to COVID infection or vaccine. CASE PRESENTATION: A 68 year old female with history of hypertension and exposure to COVID infection in the family member, presented with 2-3 months worsening generalized body ache/pain started 2 weeks after receiving second dose of mRNA vaccine. Patient also reported dyspnea and leg swelling for 1 month. Upon presentation, she was placed on 4 liter oxygen via nasal cannula. Chest x-ray concerning for infiltrates, possibly COVID. CT chest no pulmonary embolism but evidence of pneumonia superimposed on chronic appearing bronchiectasis. Flu and Covid testing were negative. Patient was started on IV antibiotics for community acquired pneumonia. Labs showed elevated ESR, CRP and CK level. No fever, weakness, mechanics hands, rash or Raynaud's phenomenon. Infectious work up remained negative. No lymphadenopathy on CT chest to suggest sarcoid. ACE level normal. ANA and anti aminoacyl-tRNA synthetase antibody positive but other ENA were negative. HMG-COA ab negative. MPO/PR3 neg. Echocardiogram was unremarkable. Work up was suggestive of Anti synthetase syndrome with interstitial lung disease(ILD), a form of dermatomyositis. Patient was started on intravenous steroid with good improvement in symptoms and later transitioned to oral prednisone. Patient was discharged on minimal home oxygen with plan to start immunosuppressive medications. DISCUSSION: We are unsure if our patient had COVID19 infection since COVID testings were negative (antigen, antibody and nucleic acid detection ). The likelihood of autoimmune and rheumatic diseases in COVID19 survivors is a big issue. COVID19 infection may unmask previously undiagnosed rheumatic conditions and precipitate de novo disease, both of which may persist after resolution of the initial infection. Corticosteroids remain the cornerstone of early treatment with initial doses at 1mg/kg of the ideal body weight. In an effort to reduce steroid related side effects, other immunosuppressive agents should be considered at the outset of therapy, particularly when treating anti-synthetase syndrome with manifestations of ILD. CONCLUSIONS: Patients with anti-synthetase syndrome with ILD could have correlation with exposure to COVID infection or vaccination, and are steroid responsive. It is likely that clinical improvement may result from prompt suppression of inflammatory systemic response by corticosteroid. Reference #1: 1. Ahmed S, Zimba O, Gasparyan AY. COVID-19 and the clinical course of rheumatic manifestations. Clin Rheumatol. 2021;40(7):2611-2619. doi:10.1007/s10067-021-05691-x Reference #2: 2. Witt LJ, Curran JJ, Strek ME. The Diagnosis and Treatment of Antisynthetase Syndrome. Clin Pulm Med. 2016;23(5):218-226. doi:10.1097/CPM.0000000000000171 DISCLOSURES: No relevant relationships by ELINA MOMIN No relevant relationships by Mohammedumer Nagori

IS BIOLOGIC DISEASE-MODIFYING ANTIRHEUMATIC DRUG USE IN PATIENTS WITH AUTOIMMUNE DISEASES A RISK FACTOR FOR SEVERE SARS-COV-2 INFECTION?

SESSION TITLE: COVID-19 Case Report Posters 3 SESSION TYPE: Case Report Posters PRESENTED ON: 10/19/2022 12:45 pm - 01:45 pm INTRODUCTION: Biological disease modifying anti-rheumatic drugs (bDMARDs) are commonly used to treat interstitial lung disease (ILD) in patients with connective tissue disease (CTD). These patients often develop concurrent COVID-19 disease and existing data is scarce to guide treatment. We describe a case with a rare anti-Pl-7 Anti-Synthetase Syndrome (ASS) receiving Rituximab treatment for ILD who developed acute respiratory distress syndrome (ARDS) secondary to COVID-19 disease. CASE PRESENTATION: A 58-year-old female presented with worsening shortness of breath, loss of taste and smell, cough and headaches for 1 week. She had pre-existing severe chronic ILD secondary to ASS on Rituximab therapy. She tested positive on SARS-CoV-2 PCR testing, CT chest showed bilateral lung honeycombing, reticulations, traction bronchiectasis along with ground glass opacities consistent with active inflammatory interstitial process superimposed on ILD. She was diagnosed with COVID-19 pneumonia. She was initially started on high-dose Dexamethasone, Remdesivir and supplemental oxygen via high flow nasal cannula and supportive care for ARDS, however level of care was escalated due to worsening respiratory distress. Rituximab was discontinued due to active COVID-19 infection, the decision was made to start Baricitinib at 4 mg daily. She received treatment for 14 days, that led to a significant improvement in her respiratory status. DISCUSSION: ASS is a rare autoimmune condition involving multiple organs, with ILD being the major cause of morbidity. bDMARDS, especially Rituximab, have shown promising results in management of severe and refractory ILD in ASS. However, the role of bDMARDs as protective or risk factor for developing severe COVID-19 disease in these patients is unclear. ARDS in COVID-19 disease involves a vigorous inflammatory response and cytokine production leading to diffuse alveolar damage. Literature supports that use of corticosteroids, IL-1 and IL-6 receptor blockers and Janus Kinase (JAK) inhibitors for severe COVID-19 pneumonia is associated with decreased morbidity. Baricitinib is a JAK1 and JAK2 with anti-cytokine and anti-viral properties and has been associated with reduction in morbidity and mortality in patients with COVID-19 as demonstrated in our case. Generally, use of bDMARDs does not contribute to worse outcomes in COVID-19 disease in patients receiving these agents for rheumatological conditions. However, use of Rituximab and high dose glucocorticoids have been associated with worse outcomes, while Baricitinib may have a protective effect. Therefore, holding Rituximab in those with active COVID-19 infection is recommended. CONCLUSIONS: Management of COVID-19 in patients with CTD is a challenge due to the novel nature of the disease and scarcity of available data. The association of use of bDMARDs in rheumatological disease with outcomes in SARS-CoV-2 infection is yet to be elucidated. Reference #1: Barbosa AN, Silvinato A, Bacha H, Floriano I, Tanni S, Bernardo W. Use of disease-modifying drugs (tocilizumab, tofacitinib, and baricitinib-a biological or synthetic target specific) in patients hospitalized with COVID-19. Rev Assoc Med Bras (1992). 2022;68(1):3-8. Reference #2: Santos CS, Férnandez XC, Moriano Morales C, Álvarez ED, Álvarez Castro C, López Robles A, Pérez Sandoval T. Biological agents for rheumatic diseases in the outbreak of COVID-19: friend or foe? RMD Open. 2021 Jan;7(1):e001439. doi: 10.1136/rmdopen-2020-001439. PMID: 33455920;PMCID: PMC7813407. Reference #3: Galarza-Delgado DÁ, Serna-Peña G, Compeán-Villegas JE, Cardenas-de la Garza JA, Pineda-Sic RA, Colunga-Pedraza IJ, Vega-Morales D, Pérez-Barbosa L, Skinner-Taylor CM, Flores-Alvarado DE. Characteristics and evolution of 38 patients with rheumatic diseases and COVID-19 under DMARD therapy. Clin Rheumatol. 2021 Mar;40(3):1197-1199. doi: 10.1007/s10067-020-05510-9. Epub 2020 Nov 24. PMID: 33231774;PM

Myositis and Myocarditis With Anti- Jo-1 Antibodies Following SARS-CoV-2 mRNA Vaccination Or COVID-19 Infection

Background: Myocarditis without myositis has been described following mRNA SARS-CoV-2 vaccination. The literature on post-vaccine antibody mediated myositis is limited and to date no case series have been reported with a distinct clinical syndrome and a single myositis specific antibody, related to SARS-CoV-2 mRNA vaccination or COVID-19. Over a 6-month period in 2021, 54 patients were referred to our tertiary referral centre for suspected myositis. Out of 25 patients with a diagnosis of myositis, we identified three patients with a distinct clinical syndrome with myositis and myocarditis with anti-Jo-1 antibodies, following SARS-CoV-2 mRNA vaccination (BNT162-Pfizer-BioNtech;n=2) or following a mild COVID-19 infection (n=1). Results: Three patients (one woman, two men;49, 50 and 58 years old) developed progressive muscle weakness and muscle pain following either vaccination (patient 1 and 2) or mild COVID-19 infection (patient 3). Patients 2 and 3 had a history of anti- CCP positive rheumatoid arthritis (RA), which had been untreated for three years in patient 2. Both post-vaccine cases had severe pitting edema of the legs, patient 2 also had arthritis. None of the patients had mechanic's hands, Raynaud's phenomenon, or interstitial lung disease (ILD). The time interval between the SARS-CoV-2 trigger and the onset of progressive muscle weakness was between 10 and 14 days (patient 1 and 3) and was estimated cybetween three and seven days in patient 2. Laboratory tests showed highly elevated CK levels (17-32 times upper limit of normal (ULN)) and troponine T levels (14-34 times ULN). In patient 2, in addition, troponin I was tested (42 times ULN), which is more specific for myocardial involvement. In patient 1 supraventricular tachycardia, unspecific ST- and Twave abnormalities and elevated NTproBNP were found. In all patients, testing for myositis specific antibodies (MSAs;EUROline myositis 16 Ag. lineblot assay) showed anti-Jo-1 antibodies (semi-quantitatively in the highest possible range). Muscle MRI showed widespread muscle edema in all patients and extensive fascial and subcutaneous edema in the legs in the post-vaccine cases (figure 1). Muscle biopsies showed inflammatory myopathy. Cardiac MRI showed abnormalities in all patients: Pericardial effusion and/or late contrast enhancement of the epicardial myocardium (figure 1). All patients showed major improvement in response to immunosuppressive therapy and could discontinue highdosed steroids after three and six months. Discussion: In conclusion, we report three patients with a distinct clinical picture of anti-Jo-1 myositis and myocarditis without ILD, following SARS-CoV-2 mRNA vaccination or COVID-19. Although it is difficult to determine a causal relationship between SARS-CoV-2 and anti-Jo-1 myositis based on these small numbers, we suspect a SARSCoV- 2 trigger of anti-synthetase syndromes given the typical combination of symptoms and previously demonstrated association with antecedent viral infections. In addition, we have collected nationwide data on myositis specific antibodies (MSAs) in 2019 (pre- COVID-19) and 2021 (during COVID-19) from six medical centers in the Netherlands. We are currently analysing these data to examine whether the proportion of positive MSAs in 2021 is higher as compared to 2019. The results will be presented at the ICNMD.

To drain, or not to drain: Diagnostic and therapeutic challenges associated with cardiac tamponade in Right heart sided heart failure and pulmonary hypertension

Background: In clinical practice, cardiac tamponade is not an all-or-none phenomenon, but rather a continuum of hemodynamic impairment. Diagnosis depends on an overall assessment of clinical and echocardiographic findings, hemodynamic measurements, and other corresponding patient-level variables must be considered to make a diagnosis to initiate timely intervention.1 The identification of cardiac tamponade in the presence of severe pulmonary hypertension and right ventricular failure can be even more challenging, because the classic findings are often not observed. Our patient clearly had hemodynamic compromise (orthostatic collapses and then persistent hypotension from a large pericardial effusion but did not exhibit the common features of tamponade on ECHO. This can be explained by the preexisting, markedly elevated right-sided pressures, which prevented typical findings of pulsus paradoxus, right atrial and ventricular diastolic collapse, and equalization of diastolic pressures.1 Case presentation: 44 Years old lady background of Interstitial lung disease, pulmonary artery hypertension, Right heart failure and anti-synthetase syndrome. Recently required Intensive care admission for COVID pneumonitis and was discharged on home oxygen. Now admitted to hospital after she boarded the flight without oxygen and became unwell. She was treated on lines of exacerbation of interstitial lung disease, Right heart failure and moderate pericardial effusion without signs of tamponade in ECHO and was given adequate diuresis and responded very well to it. Her oxygen requirement came down and she clinically improved. Few days after, she developed diarrhea and prerenal Acute kidney injury while in ward and became borderline hypotensive which improved after her diuretic doses were reduced and then held. She then after few days started to develop orthostatic collapses with hypotension and then became persistently hypotensive. Her CT Pulmonary angiogram showed unchanged moderate circumferential pericardial effusion, and no Pulmonary embolism. She was reviewed by Critical care outreach team and an urgent bedside Echocardiogram was performed to rule out features of tamponade which showed moderate pericardial effusion, severely dilated Right heart with massive Right ventricular pressures compressing her Left ventricle. She was urgently reviewed by cardiology and was taken to Intensive care unit for invasive hemodynamic monitoring, where she was given inotropes and inhaled pulmonary artery vasodilators. The decision was taken to cautiously diurese and not to drain the pericardial effusion due to risk of developing further instability by increasing right ventricular expansion causing further collapse and pressure on Left ventricle. Discussion and conclusion: Our case stresses on the importance of Bedside Echocardiogram in timely identifying the atypical features of cardiac tamponade and to understand the different hemodynamics and mechanism of obstructive shock in patients with pre-existing right sided heart failure. After the establishing that patient was in obstructive cardiogenic shock with atypical findings of tamponade, the next most important step was to decide whether to drain the pericardial effusion or not. In our literature search, we found that the drainage of a large pericardial effusion in patients with pulmonary hypertension has been accompanied by catastrophic, sudden hemodynamic collapse and it has been postulated that the presence of pericardial fluid limits right ventricular distension in response to pressure and volume overload. When the pericardial fluid is removed, rapid enlargement of the right ventricle causes: (1) reduced right ventricular systolic function due to muscle fiber distension;and (2) compression of the left ventricle, which leads to impaired diastolic filling and left ventricular outflow track obstruction.1.

RELAPSES of IDIOPATHIC INFLAMMATORY MYOPATHIES after VACCINATION AGAINST COVID19: A REAL-LIFE ITALIAN STUDY

Background: Severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) vaccination plays a crucial role as pivotal strategies to curb the coronavirus dis-ease-19 (COVID-19) pandemic. Despite the mass-scale vaccination, literature data about the incidence of disease fares in IIM patients are still not reported as well as the immunological condition. Objectives: The present study aimed to describe the clinical status of patients affected by IIM after vaccination against COVID19 in order to assess the number of relapses or immune-mediated reactions in a cohort of Italian patients with such disease. Methods: We included all patients affected by IIM and followed by Myositis Clinic, Rheumatology and Respiratory Diseases Units, Siena University Hospital, Bari University Hospital, Policlinico Umberto I, Sapienza University, Rome, and Policlinico Paolo Giaccone, Palermo. Inclusion criteria were a recent (<3 months) clinical and serological assessment before the survey and a defnite diagnosis of dermatomyosi-tis, polymyositis and anti-synthetase syndrome. All patients underwent a telephone survey in order to establish their clinical status and potential relapses after vaccination. Results: A total of 119 IIM patients (median, IQR 58 (47-66) years;32 males) were consecutively enrolled. Fifty had a diagnosis of DM, 39 had PM and 30 had ASS. The median months of disease duration was 79.62±83.98. According to number of organs involvement, forty-two had only one, 45 had two organs involvement, 20 had three, 11 had four and one had five. The majority of them received two doses of COVID-19 vaccine, except four patients who refused the vaccination: 94 (78.9%) Cominarty, 16 (13.4%) Moderna, 5 (0.04%) AZ. Seven (0.06%) patients had fare after vaccination, the majority of them were mild except one major with three organs involved and one life-threatening with systemic involvement. In order to understand or predict the effect of demographic and clinical features on the fare development after vaccination, a logistic regression analysis was performed. The goodness-of-ft statistics showed a Chi2 associated with the Log ratio (L.R.) of 0.045. From the probability associated with the Chi-square tests, the Type II analysis showed the variable that most influences the development of fare was the number of organs involved (p=0.047). Sixty-eight patients received the third dose of COVID-19 vaccination: 51 (75%) Cominarty and 17 (25%) Moderna. Only one (0.01%) patient (the same who had life-threatening fare with systemic involvement after two doses) had fare after third dose and eventually died. Conclusion: Vaccines against SARS-CoV2 have provided, both in registratory studies and in preliminary real-life evidence, an overall good efficacy and safety. Nevertheless, only scanty data are available for rheumatic patients in general and the ones affected by IIM in particular. To the best of our knowledge, ours represent the largest cohort of IIM patients in which immunogenicity of anti-SARS-CoV2 vaccine was assessed. In line with real-life data from other diseases, we found a non-statistically signifcant risk of relapse in our patients, which occurred seldom, usually mild and in patients with a more severe and aggressive course of disease.

COVID-19 in PATIENTS with INFLAMMATORY MYOPATHY

Background: Older age, male sex, multimorbidity and glucocorticoids emerged in rheumatic patients as risk factors for severe COVID-19. Objectives: We aimed to evaluate the frequency and severity of COVID-19 in a well-defned cohort of patients with idiopathic infammatory myopathy (IIM). Methods: We analyzed medical records of IIM patients diagnosed and followed at our secondary/tertiary center between January 2005 and December 2021. Results: During the 204-month period IIM was newly diagnosed in 191 patients, of whom 52 died before COVID-19 pandemic. Of the remaining 139 patients (69.8% females;9 polymyositis, 47 dermatomyositis;38 antisynthetase syndrome, 26 overlap syndrome;17 immune mediated necrotizing myopathy;2 inclusion body myositis), SARS-CoV-2 infection was proven in 13 (9.4%) patients (61.5% females, mean (SD) age at infection 62.9 (±16.8 years)). Seven/13 COVID-19 patients (53.8%) had a diagnosis of antisynthetase syndrome. At the time of infection IIM was in a remission in 12/13 patients and relapsed 5 weeks earlier in one patient. Seven patients were without immunomodulatory therapy, 1 patient was on steroids alone, 2 on DMARD alone, 3 on steroids and DMARD;a mean daily prednisolone equivalent dose was 5 mg). Eleven/13 (84.6%) patients had mild COVID-19 (one had an asymptomatic infection) and were treated sympto-matically, while 2 patients were hospitalized due to severe infection (respiratory insufficiency). Table 1 shows clinical characteristics and duration of COVID-19 symptoms. During pandemic overall 9/139 (6.5%) patients with IIM died, including one patient due to COVID-19. Conclusion: In our IIM cohort, antisynthetase syndrome represented a higher relative risk for COVID-19 compared to other IIIM subtypes.

NOT EVERYTHING IS COVID

CASE: 45-year-old African American female with history of hypertension, hypothyroidism and prior tobacco abuse was admitted to hospital with shortness of breath and hypoxia. She was diagnosed with COVID-19 pneumonia due to her respiratory symptoms, CT scan findings of bilateral pulmonary infiltrate and positive COVID IgG antibodies although a PCR test was negative. The patient was discharged and later seen in pulmonary clinic where on further questioning she complained of fatigue, bilateral wrist and knee pain, and exertional dyspnea. On auscultation, bilateral rales were noted in the lower lung fields. She was noted to desaturate upon ambulation. PFTs (pulmonary function tests) revealed severe restrictive spirometry and severe gas transfer defect. A HRCT revealed bilateral infiltrates suggestive of organizing pneumonia. CPK was elevated at 449. Serologies were positive for ANA and antijo1 and negative for other connective tissue diseases. The patient was diagnosed with anti-synthetase syndrome (ASS). She was treated with oxygen, steroids and tacrolimus with reported improvement in her symptoms. IMPACT/DISCUSSION: ASS is a rare chronic systemic autoimmune disorder that predominantly affects females with a median age of 50. It is characterized by autoantibodies against aminoacyl-tRNA synthetase enzyme. The role of these autoantibodies in the development of ASS is not fully understood. Several autoantibodies have been identified including anti-Jo1, anti-EJ, antiOJ, anti-PL7, anti-PL12, anti-SC, anti-KS, anti-JS, anti-HA, anti-YRS. Among them, anti-Jo1 is the most common. The ASS is characterized by myositis, interstitial lung disease( ILD), arthritis, fever, Raynaud's phenomenon, mechanic's hand plus positive serologic testing of the Anti- aminoacyl-tRNA synthetase enzyme. The majority of the patients with Anti- Jo-1 antibodies develop ILD. An organizing pneumonia pattern can be seen in the settings of connective tissue disease and is commonly found in those with the ASS. ILD may be the first manifestation of the disease. CONCLUSION: We present a case of a 45 year old female mistakenly diagnosed with COVID pneumonia who on further evaluation was found to have ILD secondary to Antisynthetase Syndrome, a form of inflammatory myositis. An organizing pneumonia pattern on HRCT can be found in many settings other than COVID pneumonia. Careful attention to the history, physical examination, lab findings and COVID test results remain important in identifying etiologies other than COVID 19 for a patient's respiratory symptoms during the pandemic. Delays in diagnosis can be quite harmful to patients.