scholarly journals Challenges in Diagnosing COVID-19 Related Disease in Pediatric Patients With Rheumatic Disease

2021 ◽  
Author(s):  
Seher Şener ◽  
Özge Başaran ◽  
Sibel Laçinel Gürlevik ◽  
Ümmüşen Kaya Akça ◽  
Erdal Atalay ◽  
...  
Keyword(s):  
Rheumatic Disease ◽  
Rheumatic Diseases ◽  
Pediatric Patients ◽  
Juvenile Dermatomyositis ◽  
Systemic Juvenile Idiopathic Arthritis ◽  
Severe Condition ◽  
Delay In Diagnosis ◽  
Systemic Jia ◽  
Mediterranean Fever ◽  
Inflammatory Syndrome

Abstract Objectives Multisystem inflammatory syndrome in children (MIS-C) is a rare but severe condition associated with coronavirus disease 2019 (COVID-19). Here we aimed to raise awareness for the symptoms of MIS-C in patients with rheumatic diseases, emphasizing the challenges of the differential features. Methods We retrospectively evaluated the demographic and clinical characteristics, laboratory and imaging findings, treatments, and outcomes of six MIS-C patients with previous rheumatic disease. Results Three of the patients had familial Mediterranean fever (FMF), one had juvenile dermatomyositis (JDM), one had systemic juvenile idiopathic arthritis (JIA), and another patient had oligoarticular JIA. All FMF patients presented with fever and abdominal pain, two also had chest pain. The patient with systemic JIA presented with fever, rash, and myalgia. All patients had elevated inflammatory markers and high d-dimer levels. Chest imaging of two FMF patients showed infiltrations compatible with pneumonia. One FMF patient had mildly decreased systolic functions with a shortening fraction of 48% in his echocardiography. Intravenous immunoglobulin and methylprednisolone were administered to all patients. Anakinra was given to four patients. Conclusions Clinical and laboratory signs of MIS-C may overlap with the findings of various rheumatic diseases, and this may cause a delay in diagnosis.

scholarly journals AB0978 EFFICACY OF ANAKINRA TREATMENT IN PEDIATRIC RHEUMATIC DISEASES; A SINGLE-CENTER EXPERIENCE

2020 ◽  
Vol 79 (Suppl 1) ◽  
pp. 1783.1-1784
Author(s):  
F. Demir ◽  
E. Gürler ◽  
B. Çolak ◽  
B. Sözeri
Keyword(s):  
Familial Mediterranean Fever ◽  
Injection Site ◽  
Rheumatic Diseases ◽  
Systemic Juvenile Idiopathic Arthritis ◽  
Inactive Disease ◽  
Daily Injection ◽  
Recurrent Pericarditis ◽  
Injection Site Reactions ◽  
Mediterranean Fever ◽  
Anakinra Treatment

Background:Anakinra, a recombinant IL-1 receptorantagonist, is a treatment option that acts byblocking the biological activity of IL-1 in autoinflammatory conditions. The diseases that the IL-1 was over expressed are the potential conditions for this treatment. Such as familial Mediterranean fever (FMF), cryopyrin-associated periodic syndrome (CAPS), and hyperimmunglobulin D syndrome (HIDS) with monogenic inheritance, and systemic juvenile idiopathic arthritis (SoJIA) or idiopathic recurrent pericarditis as non-Mendelian polygenic diseases, can be listed as examples of these diseases.Objectives:The aim of this study was to review the efficacy of anakinra treatment in children with rheumatic disease followed in our center.Methods:The study group consisted of children with pediatric rheumatic diseases followed up in the Pediatric Rheumatology Department of University of Health Sciences and treated with anakinra (anti-IL 1) for at least one month, between 1 July 2016 and 1 January 2020. The data of these patients were collected retrospectively. The disease activity of the patients at 3rd month and 12th month after the treatment were assessed. We aim to report our experiences of pediatric rheumatic diseases treated with anakinra.Results:There were 28 patients treated with anakinra for the different pediatric rheumatic diseases. The diagnoses of these patients were as follows; eight were macrophage activation syndrome (MAS) complicating SoJIA, six were HIDS, four were CAPS, four were FMF, four were idiopathic recurrent pericarditis, one was deficiency of interleukin-36 receptor antagonist (DITRA), and one was undefined systemic autoinflammatory disease. 46.4% of the patients were male and 53.6% were female. The median age of diagnosis of the patients was 6.5 ((interquartile range (IQR): 4-12.7) years. The median follow-up duration of the patients was 14 (IQR: 3.7-28) months. The patients median anakinra treatment duration was 3 (IQR: 1-4) months. Fever reduced and C-reactive protein normalized within median 2 (IQR: 1-3) and 5 (IQR: 5-7) days, respectively. In the 3rd month after treatment; It was observed that 53.6% of patients achieved a complete remission (no attack was seen or MAS was improved). The frequency of attacks were decreased more than 50% in 35.7% of patients and less than 50% in 7.1%. 3.6% of patients were unresponsive to treatment. In the 12th month assessment after the initiation of treatment, it was observed that 28.6% of patients were still under anakinra treatment and in remission, 10.7% of them were in remission without anakinra treatment. In 60.7% of patients, anakinra was switch to other biological treatments for different reasons (35.7% partial response or unresponsiveness, 17.8% injection site reactions and 7.1% daily-injection difficulty). Biologic drug switch to canakinumab and tocilizumab was observed in 88.2% and 11.8% of patients, respectively. One patient developed recurrent MAS episodes when the anakinra dose was tapered, and one another patient was unresponsive to the anakinra and dead due to secondary to MAS.Conclusion:Anakinra seems to be a successful treatment to achieve inactive disease in a significant portion of patients in the early period. The recurrence of disease attacks while drug tapering and injection site reactions were appears the main causes of treatment switch or discontinuation.References:[1]Ben-Zvi I, Kukuy O, Giat E, Pras E, Feld O, Kivity Set al. Anakinra for Colchicine-Resistant Familial Mediterranean Fever: A Randomized, Double-Blind, Placebo-Controlled Trial. Arthritis Rheumatol. 2017;69:854-862.Acknowledgments:We thank our patients and their familiesDisclosure of Interests:None declared

scholarly journals Outcomes of COVID-19 in a cohort of pediatric patients with rheumatic diseases

2021 ◽  
Author(s):  
Diana Sofia Villacis-Nunez ◽  
Christina A. Rostad ◽  
Kelly Rouster-Stevens ◽  
Arezou Khosroshahi ◽  
Shanmuganathan Chandrakasan ◽  
...  
Keyword(s):  
Rheumatic Disease ◽  
Rheumatic Diseases ◽  
Pediatric Patients ◽  
Univariate Analysis ◽  
Case Series ◽  
Hospitalized Patients ◽  
High Dose ◽  
High Dose Corticosteroid ◽  
Severe Immunosuppression ◽  
Dose Corticosteroid

Abstract Background There are few reports of COVID-19 in pediatric patients with rheumatic diseases. This study describes the clinical presentation and outcomes of COVID-19 in this population. Methods We analyzed a single-center case series of pediatric patients with rheumatic diseases and laboratory-confirmed COVID-19. Demographic, baseline and COVID-19 associated clinical features were compared between ambulatory and hospitalized patients using univariate analysis. Results Forty cases were identified: 32 (80%) in the ambulatory group and 8 (20%) hospitalized. Older age (median age 18 years vs 16 years; p = 0.01) and African American race (OR 8.42; 95% CI [1.20-101.69]; p = 0.01) predominated in hospitalized patients. Systemic lupus erythematosus (OR 6.77; 95% CI [1.01–56.71]; p = 0.02), medium/high-dose corticosteroid use (OR 10.62; 95% CI [1.46–99.57]; p = 0.008), mycophenolate use (OR 11.91; 95% CI [1.64-149.35]; p = 0.005), and severe immunosuppression (OR 16.83; 95% CI [1.74-861.43]; p = 0.004) were associated with increased odds of hospitalization. Patients with fever (OR 11.91; 95% CI [1.64-149.35]; p = 0.004), dyspnea (OR 16.51; CI [1.10-998.37]; p = 0.02), and myalgias (OR 13.40; 95% CI [1.43-194.56)]; p = 0.009) were more commonly encountered in the hospitalized group. Rheumatic disease flares were almost exclusive to hospitalized patients (OR 42.13; 95% CI [3.40-2463.87]; p < 0.001).. All patients recovered. Conclusions Medium/high-dose corticosteroid use, mycophenolate use, and severe immunosuppression were risk factors for hospitalization. Fever, dyspnea and myalgias were high-risk symptoms. The type of rheumatic disease, as well as disease flare could be contributing factors to the need for hospitalization.

scholarly journals Outcomes of COVID-19 in a cohort of pediatric patients with rheumatic diseases

2021 ◽  
Vol 19 (1) ◽  
Author(s):  
D. Sofia Villacis-Nunez ◽  
Christina A. Rostad ◽  
Kelly Rouster-Stevens ◽  
Arezou Khosroshahi ◽  
Shanmuganathan Chandrakasan ◽  
...  
Keyword(s):  
Chest Pain ◽  
Rheumatic Disease ◽  
Rheumatic Diseases ◽  
Pediatric Patients ◽  
Univariate Analysis ◽  
Hospitalized Patients ◽  
High Dose ◽  
High Dose Corticosteroid ◽  
Severe Immunosuppression ◽  
Dose Corticosteroid

Abstract Background There are few reports of COVID-19 in pediatric patients with rheumatic diseases. This study describes the clinical presentation and outcomes of COVID-19 in this population. Methods We analyzed a single-center case series of pediatric patients with rheumatic diseases and laboratory-confirmed COVID-19. Demographic, baseline and COVID-19 associated clinical features were compared between ambulatory and hospitalized patients using univariate analysis. Results Fifty-five cases were identified: 45 (81.8%) in the ambulatory group and 10 (18.2%) hospitalized. African American race (OR 7.78; 95% CI [1.46–55.38]; p = 0.006) and cardiovascular disease (OR 19.40; 95% CI 2.45–254.14; p = 0.001) predominated in hospitalized patients. Active rheumatic disease (OR 11.83; 95% CI 1.43–558.37; p = 0.01), medium/high-dose corticosteroid use (OR 14.12; 95% CI [2.31–106.04]; p = 0.001), mycophenolate use (OR 8.84; 95% CI [1.64–63.88]; p = 0.004), rituximab use (OR 19.40; 95% CI [2.45–254.14]; p = 0.001) and severe immunosuppression (OR 34.80; 95% CI [3.94–1704.26]; p = < 0.001) were associated with increased odds of hospitalization. Fever (OR 7.78; 95% CI [1.46–55.38]; p = 0.006), dyspnea (OR 26.28; 95% CI [2.17–1459.25]; p = 0.003), chest pain (OR 13.20; 95% CI [1.53–175.79]; p = 0.007), and rash (OR 26.28; 95% CI [2.17–1459.25]; p = 0.003) were more commonly observed in hospitalized patients. Rheumatic disease flares were almost exclusive to hospitalized patients (OR 55.95; 95% CI [5.16–3023.74]; p < 0.001).. One patient did not survive. Conclusions Medium/high-dose corticosteroid, mycophenolate and rituximab use, and severe immunosuppression were risk factors for hospitalization. Fever, dyspnea, chest pain, and rash were high-risk symptoms for hospitalization. Rheumatic disease activity and flare could contribute to the need for hospitalization.

scholarly journals Experience of Vaccination of a Patient with Systemic Juvenile Idiopathic Arthritis (sJIA) with a 13-Valent Pneumococcal Conjugate Vaccine, prior to the Appointment of Therapy with Tocilizumab, an Anti-IL-6-Receptor Monoclonal Antibody

2018 ◽  
Vol 17 (4) ◽  
pp. 322-327 ◽  
Author(s):  
Daria D. Vankova ◽  
Ekaterina I. Alekseeva ◽  
Margarita А. Soloshenko ◽  
Tatiana М. Dvoriakovskaia ◽  
Ksenia B. Isaeva ◽  
...  
Keyword(s):  
Juvenile Idiopathic Arthritis ◽  
Conjugate Vaccine ◽  
Rheumatic Diseases ◽  
Pneumococcal Conjugate Vaccine ◽  
Respiratory Infections ◽  
Systemic Juvenile Idiopathic Arthritis ◽  
Genetically Engineered ◽  
Antibacterial Drugs ◽  
Systemic Jia ◽  
First Choice

Background. Infections are the main cause of death for patients with autoimmune rheumatic diseases. In adult patients with rheumatoid arthritis (RA), mortality caused by respiratory infections is 2–5 times higher than in the population. One of the frequent infectious complications in the course of treatment with tocilizumab, the first-choice drug for treating systemic juvenile idiopathic arthritis (sJIA), is pneumonia characterized by a poor clinical picture, normal values of laboratory indices of the disease activity (ESR, C-reactive protein) with pronounced changes in the lungs revealed by computed tomography. In case of acute respiratory infection in children with systemic JIA, immunosuppressants and genetically engineered biological preparations (GEBP) are discontinued. This often leads to an exacerbation of the underlying disease and the progression of a pathological process. At present, vaccination against pneumococcal infection in Russia is not included in the standard for managing patients with rheumatic diseases. Studies of the safety and efficacy of vaccination with 13-valent pneumococcal conjugate vaccine (PCV) in patients with sJIA receiving genetically engineered biological preparations were not conducted. Clinical Case Description. The article shares the experience of vaccination of a girl aged 9 years with a 13-valent PCV that was conducted in the course of a scientific investigation, which studied the efficacy and safety of vaccination of children with systemic JIA prior to prescription of GEBP tocilizumab. Vaccination did not cause a deterioration in the course of the main disease (1 month), led to a reduction in the incidence of acute respiratory infections (from 4 to 1 time within 6 months before and after vaccination), and discontinuation of antibacterial drugs within 6 months after vaccination. Conclusion. The safety of a 13-valent PCV in a child with sJIA and a decrease of the incidence of respiratory diseases after vaccination, their complications, and the use of antibacterial drugs have been shown.

scholarly journals COVID-19 Could Trigger Systemic Juvenile Idiopathic Arthritis: First Case Report

2020 ◽  
Author(s):  
Vadood Javadi Parvaneh ◽  
Khosro Rahmani
Keyword(s):  
Juvenile Idiopathic Arthritis ◽  
Case Report ◽  
Acute Abdomen ◽  
Systemic Juvenile Idiopathic Arthritis ◽  
Signs And Symptoms ◽  
The Body ◽  
Case Presentation ◽  
Systemic Jia ◽  
First Case ◽  
Inflammatory Syndrome

Abstract Background: COVID-19 has been reported to cause a variety of signs and symptoms during its three known phases.Case presentation: We report a 7 years-old boy with COVID-19 first presented with an acute abdomen. Then he showed pictures of Kawasaki-like syndrome, a multiorgan inflammatory syndrome in children (MIS-C), and finally systemic juvenile idiopathic arthritis.Conclusion: MIS-C is a result of the hyperinflammatory response of the body to SARS-CoV-2. Although there are increasing reports of this state in children, we reported the first case presenting systemic JIA triggered by COVID-19.

scholarly journals Pain, functional disability, and their Association in Juvenile Fibromyalgia Compared to other pediatric rheumatic diseases

2019 ◽  
Vol 17 (1) ◽  
Author(s):  
Mark Connelly ◽  
◽  
Jennifer E. Weiss
Keyword(s):  
Rheumatic Disease ◽  
Rheumatic Diseases ◽  
Pediatric Rheumatology ◽  
Functional Disability ◽  
Juvenile Dermatomyositis ◽  
Functional Limitations ◽  
Well Being ◽  
Effect Sizes ◽  
Juvenile Fibromyalgia ◽  
Disease Effect

Abstract Background Severe pain and impairments in functioning are commonly reported for youth with juvenile fibromyalgia. The prevalence and impact of pain in other diseases commonly managed in pediatric rheumatology comparatively have been rarely systematically studied. The objective of the current study was to determine the extent to which high levels of pain and functional limitations, and the strength of their association, are unique to youth with juvenile primary fibromyalgia syndrome/JPFS) relative to other pediatric rheumatic diseases. Methods Using data from 7753 patients enrolled in the multinational Childhood Arthritis and Rheumatology Research Alliance (CARRA) Legacy Registry, we compared the levels and association of pain and functional limitations between youth with JPFS and those with other rheumatic diseases. Results Pain levels were rated highest among youth with JPFS (M = 6.4/10, SD = 2.4) and lowest for juvenile dermatomyositis (M = 1.7/10, SD = 2.2), with pain significantly higher in the JPFS group than any other pediatric rheumatic disease (effect sizes = .22 to 1.05). Ratings on measures of functioning and well-being also were significantly worse for patients with JPFS than patients with any other rheumatic disease (effect sizes = .62 to 1.06). The magnitude of association between pain intensity and functional disability, however, generally was higher in other rheumatic diseases than in JPFS. Pain was most strongly associated with functional limitations in juvenile dermatomyositis, juvenile idiopathic arthritis, and mixed connective tissue disease. Conclusions JPFS is unique among conditions seen in pediatric rheumatology with regard to ratings of pain and disability. However, pain appears to be comparably or more highly associated with level of functional impairment in other pediatric rheumatic diseases. Pain in childhood rheumatic disease thus would benefit from increased prioritization for research and treatment.

scholarly journals OP0147 RHEUMATIC? - A DIGITAL DIAGNOSTIC DECISION SUPPORT TOOL FOR INDIVIDUALS SUSPECTING RHEUMATIC DISEASES: A MULTICENTER VALIDATION STUDY

2021 ◽  
Vol 80 (Suppl 1) ◽  
pp. 87.1-88
Author(s):  
R. Knevel ◽  
J. Knitza ◽  
A. Hensvold ◽  
A. Circiumaru ◽  
T. Bruce ◽  
...  
Keyword(s):  
Health Care ◽  
Decision Support ◽  
Rheumatic Disease ◽  
Rheumatic Diseases ◽  
Receiver Operating Curve ◽  
Musculoskeletal Complaints ◽  
Immune Mediated ◽  
Diagnostic Decision ◽  
Ocean Observations ◽  
Receiver Operating

Background:Digital diagnostic decision support tools promise to accelerate diagnosis and increase health care efficiency in rheumatology. Rheumatic? is an online tool developed by specialists in rheumatology and general medicine together with patients and patient organizations for individuals suspecting a rheumatic disease.1,2 The tool can be used by people suspicious for rheumatic diseases resulting in individual advise on eventually seeking further health care.Objectives:We tested Rheumatic? for its ability to differentiate symptoms from immune-mediated diseases from other rheumatic and musculoskeletal complaints and disorders in patients visiting rheumatology clinics.Methods:The performance of Rheumatic? was tested using data from 175 patients from three university rheumatology centers covering two different settings:A.Risk-RA phase setting. Here, we tested whether Rheumatic? could predict the development of arthritis in 50 at risk-individuals with musculoskeletal complaints and anti-citrullinated protein antibody positivity from the KI (Karolinska Institutet)B.Early arthritis setting. Here, we tested whether Rheumatic? could predict the development of an immune-mediated rheumatic disease in i) EUMC (Erlangen) n=52 patients and ii) LUMC (Leiden) n=73 patients.In each setting, we examined the discriminative power of the total score with the Wilcoxon rank test and the area-under-the-receiver-operating-characteristic curve (AUC-ROC).Results:In setting A, the total test score clearly differentiated between individuals developing arthritis or not, median 245 versus 163, P < 0.0001, AUC-ROC = 75.3 (Figure 1). Also within patients with arthritis the Rheumatic? total score was significantly higher in patients developing an immune-mediated arthritic disease versus those who did not: median score EUMC 191 versus 107, P < 0.0001, AUC-ROC = 79.0, and LUMC 262 versus 212, P < 0.0001, AUC-ROC = 53.6.Figure 1.(Area under) the receiver operating curve for the total Rheumatic? scoreConclusion:Rheumatic? is a web-based patient-centered multilingual diagnostic tool capable of differentiating immune-mediated rheumatic conditions from other musculoskeletal problems. A following subject of research is how the tool performs in a population-wide setting.References:[1]Knitza J. et al. Mobile Health in Rheumatology: A Patient Survey Study Exploring Usage, Preferences, Barriers and eHealth Literacy. JMIR mHealth and uHealth. 2020.[2]https://rheumatic.elsa.science/en/Acknowledgements:This project has received funding from EIT Health. EIT Health is supported by the European Institute of Innovation and Technology (EIT), a body of the European Union that receives support from the European Union’s Horizon 2020 Research and Innovation program.This project has received funding from the Innovative Medicines Initiative 2 Joint Undertaking under grant agreement No 777357, RTCure.Disclosure of Interests:Rachel Knevel: None declared, Johannes Knitza: None declared, Aase Hensvold: None declared, Alexandra Circiumaru: None declared, Tor Bruce Employee of: Ocean Observations, Sebastian Evans Employee of: Elsa Science, Tjardo Maarseveen: None declared, Marc Maurits: None declared, Liesbeth Beaart- van de Voorde: None declared, David Simon: None declared, Arnd Kleyer: None declared, Martina Johannesson: None declared, Georg Schett: None declared, Thomas Huizinga: None declared, Sofia Svanteson Employee of: Elsa Science, Alexandra Lindfors Employee of: Ocean Observations, Lars Klareskog: None declared, Anca Catrina: None declared

scholarly journals Macrophage Activation Syndrome in Adults: A Retrospective Case Series

2021 ◽  
Vol 9 ◽  
pp. 232470962110264
Author(s):  
Taylor Warmoth ◽  
Malvika Ramesh ◽  
Kenneth Iwuji ◽  
John S. Pixley
Keyword(s):  
Macrophage Activation Syndrome ◽  
Macrophage Activation ◽  
Pediatric Patients ◽  
Inflammatory Diseases ◽  
Systemic Juvenile Idiopathic Arthritis ◽  
Case Series ◽  
Adult Patients ◽  
Retrospective Case ◽  
Life Threatening ◽  
Activation Syndrome

Macrophage activation syndrome (MAS) is a form of hemophagocytic lymphohistocytosis that occurs in patients with a variety of inflammatory rheumatologic conditions. Traditionally, it is noted in pediatric patients with systemic juvenile idiopathic arthritis and systemic lupus erythematous. It is a rapidly progressive and life-threatening syndrome of excess immune activation with an estimated mortality rate of 40% in children. It has become clear recently that MAS occurs in adult patients with underlying rheumatic inflammatory diseases. In this article, we describe 6 adult patients with likely underlying MAS. This case series will outline factors related to diagnosis, pathophysiology, and review present therapeutic strategies.

scholarly journals New onset diabetes with diabetic ketoacidosis in a child with multisystem inflammatory syndrome due to COVID-19

2020 ◽  
Vol 0 (0) ◽  
Author(s):  
Monica N. Naguib ◽  
Jennifer K. Raymond ◽  
Alaina P. Vidmar
Keyword(s):  
Metabolic Acidosis ◽  
Diabetic Ketoacidosis ◽  
Pediatric Patients ◽  
Systolic Function ◽  
Hemodynamic Instability ◽  
Case Presentation ◽  
Onset Diabetes ◽  
Potential Trigger ◽  
Inflammatory Syndrome ◽  
New Onset

AbstractIntroductionMultisystem inflammatory syndrome in children (MIS-C) is a unique clinical complication of SARS-CoV-2 infection observed in pediatric patients. COVID-19 is emerging as a potential trigger for the development of diabetes in children. Here, we report a patient presenting with MIS-C and new onset diabetes, and discuss the implication and clinical management of these concomitant conditions.Case presentationAn eight-year-old female presented with hyperglycemia, ketosis and metabolic acidosis consistent with diabetic ketoacidosis (DKA) in the setting of fever, rash, respiratory distress, hemodynamic instability, reduced systolic function with dilation of the left anterior descending artery, and positive SARS-CoV-2 antibodies suggestive of MIS-C.

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