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Juvenile Dermatomyositis (JDM) is an autoimmune disease that involves skin, muscle and internal organ disorders. Its mechanisms still not well established, but the triggering role of viral infections has been described. In this context, the effect of the COVID-19 on the onset of autoimmune disorders such as JDM remains a matter of study and research. We report a severe JDM, following a confirmed COVID-19 infection in a previously healthy 8 year-old boy who presented with various skin lesions and a cholestatic liver involvement. Laboratory findings were consistent with an inflammatory myositis and an autoimmune liver disease. Skin and muscle biopsies confirmed the diagnosis of JDM. The therapy choice was difficult. Finally, he received a second line therapy of the JDM with a favorable outcome. The liver fragment analysis showed a steatosis. This case supports the hypothesis of COVID-19 triggering role in the genesis of JDM and autoimmune diseases.Copyright © 2023 Scientific Publishers of India. All rights reserved.
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Background. Prior research has shown that viruses may trigger JDM, although the degree to which COVID-19 may serve as a trigger for JDM remains unknown. We present two case reports of JDM occurring after COVID-19 infection. We also provide case numbers of new JDM diagnoses pre-and post-COVID-19 as well as an analysis of JDM population characteristics pre-and post-COVID-19. A 5year-old female developed upper respiratory infection (URI) symptoms and was diagnosed with COVID-19 in December of 2020. She developed Gottron's sign, heliotrope rash, and weakness resulting in admission in February of 2021. She had elevated CK, AST, ALT, LDH, and aldolase. Her CMAS (childhood myositis assessment scale) was 24. An MRI showed diffuse myositis. Myositis specific antibody (MSA) testing revealed a positive MJ antibody. She was diagnosed with JDM and started on steroids, methotrexate, hydroxychloroquine, and IVIG with improvement. The second patient was a 4year-old female who was diagnosed with COVID-19 in October 2020. In January 2021, she developed heliotrope rash and Gottron's papules. She developed decreased exercise tolerance in May 2021 found to have elevated Aldolase and LDH. Her CMAS was 34. An MRI showed diffuse myositis. MSA testing was significant for a positive P155/140 antibody. She was started on hydroxychloroquine, steroids, IVIG and methotrexate with improvement. Due to the aforementioned cases a retrospective analysis was performed assessing the characteristics of JDM pre-and post-COVID-19 at Lurie Children's Hospital. Methods. The Cure JM biorepository houses clinical data, laboratory data, and patient samples obtained at the onset of JDM. The following information was obtained from newly diagnosed JDM patients: MSA, DAS (disease activity score), flow cytometry results, vWF antigen, neopterin, CMAS, capillary end row loop(ERL), LDH, Aldolase, ESR, CRP, IgG, complements, ANA, and age at diagnosis. We identified 10 patients with a diagnosis of JDM from January 1st 2020 -July 1st 2021 who were designated as the post-COVID-19 group. This population was compared to a total of 51 patients diagnosed with JDM between Jan 1st 2010 and December 31st 2019 who were designated as the pre-COVID-19 group. Data analysis was performed using Welch T-testing. Research enrollment was impacted due to the COVID-19 pandemic. To better assess JDM rates, chart review and EMR reports were obtained to determine the total number of JDM diagnoses. Results. T-testing showed no significant change in DAS, ERL count, T or B cell flow cytometry, vWF antigen, CK, CMAS, CRP, Aldolase, LDH, IgG, complements or ANA titer between the pre-and post-COVID-19 JDM groups. The analysis showed a significant change in NK cell population with a decrease in the absolute NK cell number (pre 163, post 90.75. P value 0.03), and NK cell percentage (pre 6.6%, post 3.625%, P value 0.008). Both of the patients presented in this case report showed a low NK cell number (1% and 3% respectively). The total number of new JDM cases rose from an average of 6.3 cases per year to an average 9 cases per year from January 1st 2020 to December 31st 2021. Conclusion. This study provides two case reports of COVID-19 likely triggering JDM. This study also shows a modest increase in the number of new JDM cases since the onset of the pandemic. Interestingly, the NK cell population in the post-COVID-19 JDM patients were significantly decreased. NK cells have multiple roles in not only immune regulation, but also the immune response to viruses. This study suggests that NK cells play a role in the development of in virally mediated JDM, specifically in cases triggered by COVID-19. Future studies will be important to further delineate the function of NK cells in these patients. Markers of JDM disease severity, including DAS, Neopterin, CK, and CMAS, did not significantly change in our institution's JDM population after the onset of the COVID-19 pandemic.
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Background. Juvenile Dermatomyositis (JDM) is a chronic systemic vasculopathy of unknown etiology characterized by symmetrical proximal muscle weakness, raised serum concentration of muscle enzymes and pathognomonic skin rashes. Although JDM is the most common pediatric idiopathic inflammatory myopathy, it is still quite rare with an annual incidence of 3.2 cases per million children in the US. Youth with chronic disease are reported to have a higher prevalence of mental health disorders compared to healthy peers, with some research reporting up to a fourfold increase in risk. The COVID-19 pandemic has raised psychological distress among youth;data from the first year of the pandemic suggests that 1 in 4 youth globally are experiencing clinically elevated depression symptoms. The primary aim of this study is to describe the prevalence of depression in a cohort of patients with JDM to help providers better understand the mental health issues that arise in this population. This is critically important as early intervention for depression in youth with JDM has the potential to improve both medical and psychosocial outcomes. Methods. This was a cohort study measuring depression in youth with JDM at Children's Healthcare of Atlanta (CHOA). Subjects were recruited during routine outpatient visits to CHOA rheumatology clinics from August to December 2020. Subjects had a diagnosis of JDM for at least 3 months, were between 5-20 years old, and had no cognitive deficit precluding questionnaire completion. Parent completed a proxy questionnaire if the child was 5-7 years old. Depression was assessed using the Patient Questionnaire-9 (PHQ-9). Of 15 eligible subjects, all consented to the study. Informed consent/assent was obtained. CHOA Institutional Review Board approved the study. Upon identification of depression, an educational handout was offered, which also included mental health care providers. Identified suicide risk was addressed with immediate direct questioning of suicidal intent, plan or attempt within the prior week;endorsement of any of these prompted enactment of a safety plan and urgent psychiatric evaluation. Statistical comparisons were performed using SAS. Medians and interquartile ranges (IQR), mean and standard deviation and frequencies were calculated for demographic and disease related variables. The presence of depression symptoms were analyzed as binary covariates for positive screens on the PHQ-9. Results. Demographics of the 15 participants included 53% female, median age of 12 years (IQR 10.0, 19.0;range 5-20) with a range of 5-20 years. The sample was heterogeneous with respect to race/ethnicity, with 8 (53.3%) Black, 6 (40%) White and 1 (6.7%) Asian participant. Median disease duration was 4.1 years (IQR 2.2, 6.9). Calcinosis was present in 10 (67%) of patients. Five (33%) participants had active disease at the time of completing PHQ-9, all of whom had mild disease with median Physician Global Score of 0.6 (IQR 0, 0.9). Depression was identified in 6 subjects (40%): 5 subjects (33%) were classified as having mild depression and 1 subject (7%) was classified as having moderate depression. No subjects had severe depression nor endorsed suicidal ideation. There was no significant difference in depression prevalence in patients with active disease versus inactive disease. The prevalence of depression in this small cohort is similar to previously reported rates of depression in patients with JIA and SLE;notably, it is higher than rates of depression in healthy children in the US. Conclusions. This pilot study adds to our understanding of the relationship between JDM diagnosis and psychosocial functioning in children and youth. The COVID-19 pandemic has been associated with a rise in depression in all children. Our findings suggest that regardless of disease status, there is a higher prevalence of depression in JDM patients compared to their healthy peers. Given the small sample size, further studies are needed to assess depression in paediatric rheumatology clinics.
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Background. Several studies and cohorts with adult populations with rheumatic diseases (RD) were performed since pandemic outbreak. RD patients were more susceptible to infections and may develop severe forms of COVID-19, since they present immunosuppressive mechanisms inherent to the disease itself and to its treatment. Healthy children and adolescents seem to be less infected and present milder diseases. However, juvenile dermatomyositis patiets and immunosuppressed children have not been extensively studied. The objectives of the study are to evaluate asymptomatic SARS-CoV-2 infection in pediatric RD patients, to identify the risk factors related to contagion and to describe demographics and the profile of COVID-19 in juvenile dermatomyositis (JDM) patients followed. Methods. A cross-sectional study was conducted in March 2021, including 77 pediatric RD patients followed at a Brazilian tertiary hospital and 45 healthy controls. Data was obtained through a questionnaire applied to outpatients during the month of March 2021, before the vaccine, and contained demographic data, symptoms compatible with COVID-19 over the past year, and contact with people with confirmed COVID-19. Patients' medical records were reviewed to access data regarding disease and current medications. A qualitative immunochromatographic SARS-CoV-2 test was performed in all participants. All patients who were using rituximab or intravenous human immunoglobulin, or had symptoms of COVID-19, were excluded. Results. Patients' group included 11 (14.3%) JDM patients, 31 (40.2%) JIA, 25 (32.4%) JSLE, six patients with vasculitis, two with SS, one MCTD and one with autoinflammatory syndrome. Patients and controls were similar in terms of female gender (70.1% vs. 57.8%, p=0.173), median age (14 vs. 13 years, p=0.269) and SARS-CoV-2 serology positivity (22% vs. 15.5%, p=0.481). 80.5% of rheumatic patients were in use of immunosuppressive drugs, 27.3% of them using corticosteroids, 33.3% in high doses, and 7.8% on immunobiologicals. No statistical differences were found between positive (n=17) and negative serology (n=60) patients regarding demographic/socioeconomic data, contact with people with confirmed COVID-19, use and number of immunosuppressive drugs, use and dose of corticosteroids, use of hydroxychloroquine and immunobiological drugs (p>0.05). Regarding the profile of JDM patients, 6/11 (54%) were female, the median age was 13 years (range 9-17) and 3/11 (27%) presented COVID-19 serology positivity. 2/11 were in immunosuppressive treatment, however none of them were in use of glucocorticoids and biologic agents. Conclusions. Pediatric JDM and other rheumatic diseases patients were infected at the same rate as healthy ones. Neither the underlying pathology nor its treatment seemed to interfere with the contagion risk.
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Background. Viruses are thought to play a role in triggering juvenile idiopathic inflammatory myopathies (JIIM), which include juvenile dermatomyositis (JDM), juvenile polymyositis (JPM), and overlap myositis. There is growing evidence that infection with severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) can trigger autoimmune diseases in genetically susceptible individuals, including idiopathic inflammatory myopathies (IIM). Studies have shown similarities between SARS-CoV-2 infection and anti-melanoma differentiation-associated gene 5 (MDA5) antibody-related dermatomyositis, suggesting possible shared underlying autoimmune and/or inflammatory mechanisms. To date, there are few studies describing individual cases of JIIM following SARS-CoV-2 infection, and, to our knowledge, none have explored the effects of SARS-CoV-2 on the clinical presentation of JIIM. In this study, we aim to investigate the impact of SARS-CoV-2 on JIIM by comparing the onset of new JIIM cases, as well as clinical and laboratory characteristics at disease onset, in patients diagnosed before and after onset of the Coronavirus Disease 2019 pandemic (COVID 19). Methods. Patients diagnosed with JIIM prior to age 19 at The Children's Hospital at Montefiore were eligible for study inclusion. Demographic, clinical, and laboratory data, as well as evidence of exposure to SARS-CoV-2, were collected retrospectively by manual chart review. Patients were grouped into pre-COVID 19 (defined as prior to January 1, 2020) and post-COVID 19 (defined as January 1, 2020, or later). Descriptive statistics were used to summarize each variable. Given the small sample size, non-parametric testing was performed using Fischer's exact test and Wilcoxon rank sum test. Results. Forty-four patients were included in the analysis (Table I). Thirty-four patients (77.3%) were diagnosed pre-COVID 19 and ten patients (22.7%) were diagnosed post-COVID 19. Of the ten patients diagnosed post-COVID 19, five (50%) had known exposure to or infection with SARS-CoV-2. Patients diagnosed with JIIM post-COVID 19 were more likely to be of non-Hispanic Black or Asian descent (p=0.041), develop disease at an older age (p=0.009), and present with non-classic cutaneous manifestations (as opposed to classic findings of Gottron's papules/sign or Heliotrope rash) (p=0.031), despite similar frequencies of JDM versus overlap myositis. While presence of muscle weakness did not differ between the groups, patients diagnosed post-COVID 19 tended to have more severe weakness, though results were not statistically significant. Interestingly, despite delays to diagnosis reported during the pandemic, there was no difference between time from symptom onset to diagnosis. Conclusion. This is the first study to explore the effects of SARS-CoV-2 on the clinical presentation of JIIM. In our center, we found that patients diagnosed with JIIM after COVID-19 were more likely to be racial minorities, older at onset, and present with non-classic cutaneous manifestations. While there were no significant differences in myositis specific or associated antibodies, patients diagnosed post-COVID 19 did not have complete autoantibody investigation performed at the time of this study. Clinicians should consider JIIM even in the absence of classic cutaneous manifestations, particularly in the post-COVID 19 era. Patients should be followed longitudinally to explore long-term impacts of SARS-CoV-2 on JIIM. Further investigation is warranted to identify the mechanisms by which SARS-CoV-2 impacts JIIM and how these differ from the effects of other viruses.
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Background. Autoimmune rheumatic diseases (ARD) include various chronic conditions with high morbidity and mortality rates, and an increased risk of infections, including the new COVID-19. It is possible that adolescents with ARD have higher levels of psychological distress which may affect their mental health and life conditions. The objectives were to assess mental health and life conditions in adolescents with autoimmune rheumatic diseases (ARD) and healthy controls in social isolation, emphasizing some demographic aspects and daily routine of adolescents with juvenile dermatomyositis (JDM) during the COVID-19 quarantine. Methods. A cross-sectional study, performed from July 2020 to October 2020, included 155 ARD adolescents and 105 healthy controls. Online survey composed by self-reported strengths and difficulties questionnaire (SDQ) and a semi-structured questionnaire was filled in regarding demographic data, daily home and school routine, physical activities and COVID-19 information during the pandemic. Results. The patients included in the study presented the following underlying diseases: 15% JDM, 29% juvenile systemic lupus erythematosus (JSLE) and 56% juvenile idiopathic arthritis (JIA). Among adolescents with JDM, 71% were female, 54% Caucasian and the median age was 14 years (range 10-18). Regarding school data, 92% JDM participants attended school before pandemic, 75% studied in public schools and up to 17% did not present home schooling during the quarantine. All JDM patients agreed with stay-home policy after pandemic outbreak, and they reported change in life routine (96%), sleep problems (29%), sleep after midnight (75%) and increased screen time (87%). Worsening of family financial situation (37%) and increased family violence (8%) were also observed. Concerning mental health assessment, it was verified that one third of JDM subjects presented abnormal total difficulties and emotional scores of SDQ. No differences were found regarding sex, ethnicity and current age between ARD patients and controls (p>0.05). The frequencies of abnormal SDQ total (32% vs. 32%, p=0.901) and emotional (38% vs. 35%, p=0.653) were similar in both groups. Logistic regression analyses in ARD patients demonstrated that female (OR=2.4;95%CI 1.0-6.0;p=0.044) was associated with severe emotional SDQ dysfunction, whereas poor sleep quality was considered risk factor for both worse total SDQ (OR 2.6;95% CI 1.2-5.5;p=0.009) and emotional SDQ scores (OR=4.6;95%CI 2.2-9.7;p<0.001). Comparisons between ARD patients with and without current prednisone use showed higher median scores of peer problems in the first group [3(0-10) vs. 2(0-7), p=0.049]. The median and frequencies of SDQ scores and domains were similar between JDM, JSLE and JIA (p>0.05). Conclusions. Approximately one third of JDM, JSLE and JIA patients presented abnormal total difficulties and emotional scores of SDQ. Female sex and poor sleep quality were the main factor associated with emotional impact in these ARD adolescents.
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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.
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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.
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INTRODUCTION: Juvenile dermatomyositis (JDM), a rare multisystemic autoimmune disease of unknown cause, leads to chronic inflammation of both striated and smooth muscles. SARS -Co V2 virus infection in children generally remain asymptomatic. However, in some children it leads to a detailed immunological response named as multisystem inflammatory syndrome in children (MIS-C). Post recovery, occasionally, children are susceptible to other autoimmune disorders. CASE PRESENTATION: Our case post MIS-C developed JDM. 8-year-old malnourished child developed proximal myopathy of both upper and lower limbs post recovery from COVID 19. His disease severity increased within a short span of time and he went on to develop contractures and deformity of both upper and lower limbs. He developed an uncommon complication of JDM in form of high-grade non-Hodgkin's lymphoma. CONCLUSION: This case highlights the importance of long-term complications of COVID-19 in children which would gradually evolve in the next few years.
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RNA viruses have been posited as triggers for Juvenile Dermatomyositis (JDM). The COVID-19 pandemic proved a unique opportunity to observe the effect of a novel RNA virus on JDM incidence and phenotype. We found the incidence of JDM increased from average of 6.9 cases per year from 2012-2019 to 9 cases per year from 2020-2021. We compared markers of disease activity in the patients diagnosed with JDM prior to and during the pandemic and found that patients diagnosed with JDM during the pandemic had significantly lower average NK cell counts 90.75(± 76) vs 163(±120) (P= 0.038) and NK cell percentage 3.63% (±2.3) vs. 6.6% (±4.1), (P= 0.008). Other markers of JDM did not significantly change. This study suggests that COVID-19 may be a viral trigger for JDM in selected cases and that NK cell dysregulation may be of particular interest in future research of virally triggered JDM.
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The proceedings contain 539 papers. The topics discussed include: advances in the understanding and management of atherosclerosis in inflammatory arthritis;long-term safety and efficacy of voclosporin in Asian patients with lupus nephritis;clinical profile of four children with juvenile dermatomyositis and anti-SAE antibody positivity: a single center experience from north India;the MMP degraded and citrullinated vimentin (VICM) is a diagnostic and treatment response biomarker;incidence and outcome of covid-19 in AIRD patients on concomitant treatment with tofacitinib- results from KRA covid cohort (KRACC) subset;are we treating-to-target in spondyloarthritis (SPA)? a cross-sectional analysis from the Asia Pacific league of associations for rheumatology (APLAR) SPA registry;utilities of low-dose computed tomography (LDCT) on identifying patient with axial psoriatic arthritis (AXPSA) a cross-sectional study;age-related genes USP2 and ARG2 are involved in the reduction of immune cell infiltration in elderly patients with rheumatoid arthritis;and MICRORNA-27a-3p inhibits lung and skin fibrosis of systemic sclerosis by negatively regulating SPP1.
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Background: Dermatomyositis is a type of systemic inflammatory autoimmune disorder characterised by muscle inflammation and skin rashes. We present a rare adult onset refractory Nxp2 dermatomyositis following COVID 19 infection Methods: 36-year- old male came with the complaints of: Redness of right eye, Easy fatgiuability ,dysphagia of 3 months duration * Patient had uncomplicated COVID-19 1 month prior to onset of present complaints * On examination he had anasarca proximal muscle weakness and muscle tenderness and had neck and pharyngeal muscle weakness dysphagia and nasal regurgitation.He also had malar rash and periribital rash and swelling (Figure 1) * Investigations revealed biochemical radiological and Electrophysiological evidence of myositis (Table 1) * He was managed with pulse sterids ivig rituximab and tacrolimus with gradual but definite resolution Conclusion(s): Auto-antibodies against NXP2 are detected in 15% to 25% cases of Juvenile dermatomyositis and in only 1% of adult cases. This form of DM is characterized by accompanying calcinosis and severe and chronic disease course and is often carcinoma-associated (breast, uterine or pancreatic carcinoma). Post COVID NXP2 DM has not yet been reported. (Figure Presented).
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Purpose of Study: Report a rare case of onset of seronegative, juvenile dermatomyositis likely potentiated by Covid-19 infection Methods Used: Case analysis and literature research Summary of Results: A 7 year-old previously healthy male presented with 3 weeks of progressive, bilateral upper and lower extremity weakness, difficulty swallowing, voice changes, periorbital edema, and rash. Recent history was notable for diagnoses of COVID-19 one month prior to presentation and streptococcal pharyngitis 2 months prior to presentation. Notably, there is a family history of systemic lupus erythematosus. On examination, the patient demonstrated bilateral periorbital swelling with purple discoloration of the upper eyelids, a violaceous, pruritic, macular rash on his upper extremities and on his abdomen. Musculoskeletal exam was significant for severe axial (strength 2/5) and proximal (strength 3/5) muscleweakness with notable inability to sit unsupported or maintain head control. His neurologic exam was nonfocal;however, diffuse hyporeflexia in both upper and lower extremities were elicited. Initial screening labs were notable for mild transaminitis;positive ANA (1:80 in speckled pattern), negative ANCA, negative dsDNA/Anti- Sm, elevated aldolase of 10.3, CK 464, and LDH 665;normal thyroid studies and normal inflammatory markers. MRI with and without contrast of the spine indicated diffuse myositis of all muscle groups. Due to concern for autoimmune mediated myositis, Rheumatology was involved early in the patient's course. Empiric treatment was initiated early in the patient's presentation with IVIG, steroids, methotrexate, and plaquenil leading to gradual improvement in symptoms. Subsequent muscle biopsy was consistent with juvenile dermatomyositis (JDM). Conclusion(s): JDM is rare, occurring in 1 to 15 per million children. It classically presents with proximal myopathy and dermatologic findings of Gattron's papules, a heliotrope and malar rash. Its pathophysiology is not yet well defined but is thought to be a humoral mediated autoimmune disease. Muscle biopsies characteristically show perifascicular and perivascular infiltration. Early diagnosis and treatment with steroids, immune modulators, and physical therapy is critical to limit muscle atrophy. Viral infections are known triggers of rheumatologic diseases broadly;however, the more pronounced type 1 interferon response associated with COVID-19, which is known to be a driving pathway of JDM, may be a risk factor for severe, recalcitrant disease. Future research is needed to better identify involved pathophysiology and target future treatment efforts. Additionally, more education and case reports could focus on dermatologic presentations of individuals with pigmented skin. Copyright © 2023 Southern Society for Clinical Investigation.
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Background: According to newspaper Bernama, 87.6% of adolescents in Malaysia aged between 12 and 17 have completed their vaccination and 97.7% of the adult population have completed theirs as of 2nd January 2022.The acceptance of patients with rheumatic diseases on Covid-19 vaccination are crucial in the successful long term protection against Covid-19 infection. We conducted a phone interview to determine the acceptance of Covid-19 vaccination amongst adolescents with underlying rheumatic diseases. Objective(s): To determine the acceptance of Covid-19 vaccination amongst adolescents with underlying rheumatic diseases. Method(s): This was a phone survey. The electronic medical records of all rheumatology patients follow up in rheumatology clinic Hospital Sultan Ismail, Malaysia from 1st January 2012 to 31th December 2021 were reviewed and patients with age group from 12 to 21 were identified. Demographic and diagnosis of the patients collected. Result(s): Phone survey was done after data extracted from medical records. For those under the age of 18, guardian of the patients was interviewed. A total of 50 patients were identified. 36 of them were having systemic lupus erythematosus (SLE), 5 of them were having juvenile idiopathic arthritis (JIA),2 of them were having psoriatic arthritis (PSA) and another 2 of them were having Rheumatoid arthritis (RA), followed by rheumatoid arthritis (RA) overlapped SLE, juvenile dermatomyositis, Henoch-Schonlein purpura, SLE overlapped with JIA and mixed connective tissue disease, 1 each respectively. Most of the patients were female (46/50) and majority of them were Malay (33/50). This was followed by Chinese (10/50), Indian (4/50) and others (3/50). The mean age group was 18 (range from 13 to 21). Majority of them patients are keen or already completed Covid-19 vaccination with the acceptance rate as high as 92% (46/50). Only 8% of them not keen for vaccination with the reason of worrying the risk of myocarditis post vaccination. Conclusion(s): The overall acceptance rate of Covid-19 vaccination amongst adolescents with rheumatic diseases are very encouraging with the percentage of >90% despite of lacking knowledge about vaccine Covid-19. This result can assist our Ministry of Health plan for future battle to improve vaccine uptake that hopefully can lead to herd immunity against COVID-19 infection.
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We present a case of severe juvenile dermatomyositis with limited response to steroids in an adolescent who developed symptoms within hours after receiving Pfizer BNT162b2 coronavirus disease 2019 vaccine. The patient presented with severe weakness of proximal muscles, dyspnoea, and tachycardia. His muscle enzymes were raised, and he was diagnosed with severe juvenile dermatomyositis following magnetic resonance imaging and muscle biopsy. His management was challenging, requiring multidisciplinary input, and difficult decisions with regard to the appropriate immunomodulatory treatments. The patient had to undergo escalating immunosuppressive treatments before he began to recover clinically and biochemically. To our knowledge, this is the first case in an adolescent although a few cases of similar presentations following coronavirus disease 2019 vaccination have been reported in adults. Elucidating the potential relationship of the vaccine with this severe myopathy in an adolescent is important for global vaccination policies, but avoiding the conflation of association with causation is also crucial in the context of the pandemic.
Subject(s)
COVID-19 , Dermatomyositis , Muscular Diseases , Male , Adult , Humans , Adolescent , Dermatomyositis/complications , BNT162 Vaccine , COVID-19 Vaccines , COVID-19/complicationsABSTRACT
Juvenile dermatomyositis (JDM) is a heterogeneous autoimmune inflammatory myositis with symmetrical proximal muscle weakness and a characteristic rash. Juvenile dermatomyositis is characterized by variable presentation and phenotypes. Detection of myositis autoantibodies is useful in improving JDM diagnosis and predicting the prognosis. In this literature review based on case series we analyze clinical and autoantibody phenotypes of JDM in four patients who were hospitalized in one regional center in Ukraine during the last 3 years and three of them presented in the time of the COVID-19 pandemic. The reviewed literature showed the last updates for the JDM diagnosis and the role of myositis autoantibodies in the prediction of disease course, systemic involvement, and malignancy risk. The presence of anti-synthetase syndrome in all presented patients, mainly due to anti-PL-7 autoantibodies, encourages further study with more patients and with detection of other myositis-specific autoantibodies to identify or refute certain regional features.
ABSTRACT
Anti-MDA5 antibodies characterise a distinct phenotype of dermatomyositis in adults as well as children, with ethnic disparity in clinical presentation and severity. They often present as a diagnostic conundrum with rash, ulceration, and polyarthritis, but minimal muscle disease. Mechanic's hands are typically associated with anti-synthetase syndrome, but their presence in anti-MDA5 antibody positive patients, although reported, is not well known. We present the case of a boy in whom mechanic's hand heralded a relapse of juvenile dermatomyositis which was suspected based on remotely assessed patient-reported outcome measures on teleconsultation. This report suggests that mechanic's hands should also prompt testing for myositis antibodies including anti-MDA5 in Indian children with JDM. Diligent awareness of the condition, and timely use of patient reported outcome measures of muscle power and skin assessment may guide management while delivering remote care in challenging situations such as a global pandemic.
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Objectives: To describe the clinical and laboratory characteristics of pediatric patients diagnosed with dermatomyosis during the COVID pandemic. Method: Description of the clinical and laboratory findings in patients under 15 years of age, who were admitted at our hospital with signs and symptoms suggestive of dermatomyositis, from March 2020 until November 2021. Results: Five patients, three boys and two girls, aged between 8 and 13 years, were diagnosed with juvenile dermatomyositis (JDM). The most frequent symptom was asthenia. Heliotrope erythema, malar rash, periungual erythema, and papules on elbows and knees were present in all cases. Three of our patients had perniosis on their toes. Four patients presented lesions in the oral mucosa, such as geographic tongue or gingivitis. The time between the onset of symptoms and the first visit with the pediatrician ranged from 1 to 6 months. In all cases, GOT/GPT enzymes, and aldolase were elevated at diagnosis, and the SARS-CoV-2 PCR was negative. Two patients had anti-TIF1 antibodies, and two had anti-MDA5 antibodies. In one girl, no specific autoantibodies for JDM were detected. Magnetic resonance imaging showed muscle oedema in all patients. All cases are in remission after systemic treatment with steroids, methotrexate or immunoglobulins. Discussion: JDM is a severe disease of childhood. Our cases did not present symptoms suggestive of COVID and the PCR for SARS-CoV-2 was negative on admission in all of them, but the presence of perniosis on the toes of three patients could correspond to “COVID toes,” and be a late manifestation of an asymptomatic or oligosymptomatic infection of SARS-CoV-2. It has been suggested that SARS-CoV-2 infection could trigger the development of JDM, possibly through the induction of IFNα. Long-term follow-up is necessary to establish a relationship between the prognosis of specific autoantibodies, the involvement of acral and mucosal areas, and the possible relation with COVID.
ABSTRACT
Background/Aims According to newspaper Bernama, 6.5% of adolescents in Malaysia aged between 12 and 17 have completed their vaccination and 89.7% of the adult population have completed theirs as of 9th October 2021. The acceptance of patients with rheumatic diseases on Covid-19 vaccination are crucial in the successful long-term protection against COVID-19 infection. We conducted a phone survey to determine the acceptance of COVID-19 vaccination amongst adolescents with underlying rheumatic disease. Methods This was a phone survey. The electronic medical records of all rheumatology patients follow up in rheumatology clinic Hospital Sultan Ismail, Malaysia from 2019 to 2021 were reviewed and patients with the age group between 12 to 21 were identified. Demographic data, diagnosis of the disease and outcome of the survey were collected and analysed. Results Phone survey was done after data extracted from medical records. For those under the age of 18, guardian of the patients was interviewed. A total of 50 patients were identified. Most of the patients were female (46/50) and majority of them were Malay (33/50). This was followed by Chinese (10/50), Indian (4/50) and others (3/50). The mean age group was 18 (range from 13 to 21). 36 of them were having systemic lupus erythematosus (SLE), 5 of them were having juvenile idiopathic arthritis (JIA) ,2 of them were having psoriatic arthritis (PSA) and another 2 of them were having rheumatoid arthritis (RA), followed by RA overlapped SLE, juvenile dermatomyositis, Henoch-Schönlein purpura, SLE overlapped with JIA and mixed connective tissue disease about 1 each respectively. Majority of them patients are keen or already completed COVID-19 vaccination with the acceptance rate of as high as 92% (46/50). Only 8% of them are not keen for vaccination with the only reason of worrying the risk of myocarditis post vaccination (1 SLE patient and 1 PSA patient). Conclusion The overall acceptance rate of COVID-19 vaccination amongst adolescents with rheumatic diseases are very encouraging with the percentage of>90% despite lacking knowledge about vaccination for COVID-19. This result can assist our Ministry of Health in planning for future battles to improve vaccine uptake that hopefully can lead to herd immunity against COVID-19 infection.
ABSTRACT
Introduction: A working hypothesis is that juvenile dermatomyositis (JDM) is a type 1 interferon driven inflammatory response, triggered by one or more environmental stimuli, such as infection. Objectives: We aimed to test the hypothesis that SARS-CoV-2 infection could promote JDM onset or relapse. Methods: We studied SARS-CoV-2 infection history in all JDM patients seen in our center for disease onset (n=6) or relapse (n=4) since the start of the pandemic. IgG and IgM directed against whole spike protein, spike Receptor Binding Domain (RBD), spike S2 subunit, nucleocapsid protein (NP) and Membrane glycoprotein (ME) were measure in the plasma by multiplex bead-based assay at diagnosis. IFNα2 level in the plasma was measure by digital ELISA. Results: Out of the 10 patients we identified concomitant infection by SARS-CoV-2 with disease onset in one patient, and concomitant infection by SARS-CoV-2 with disease relapse after 8 years out of treatment in one other. IFNa2 dosages in the plasma of these two patients revealed abnormally elevated concentrations (73476 fg/ml and 4612fg/ml respectively, median active JDM: 491fg/ml). Conclusion: Our results strongly suggest that SARS-CoV-2 infection could trigger the development of JDM, possibly through induction of IFNα.