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Background: Patients with rheumatic disease may mount a suboptimal serologic response to COVID-19 vaccination. We evaluated predictors of low antibody response in a clinic-based cohort. Methods: We conducted a cross-sectional study using electronic health record (EHR) data at Brigham and Women's Hospital, Boston, MA. Patients with systemic rheumatic disease that had SARS-CoV-2 spike antibody (Ab) tested using the Roche Elecsys immunoassay, February–August 2021, after 2 doses of mRNA vaccine or 1 dose of adenovirus vector vaccine were identified. Demographics, systemic rheumatic disease, vaccination dates, and disease-modifying antirheumatic drugs (DMARDs) were extracted. The primary outcome was low spike Ab (≤ 200 U/mL). Logistic regression models estimated predictors of low spike Ab. Results: Among 382 patients, the mean age was 57 years, 77% were female, and 37% had low spike Ab. Older age (OR 1.03, 95% CI [1.02, 1.05]), SLE (OR 4.81 [2.08, 8.43], reference: inflammatory arthritis), prednisone (OR 1.67 [1.03, 2.74]), and rituximab (OR 22.91 [9.85, 53.29]) were significantly associated with higher odds of low spike Ab. Use of csDMARD monotherapy (OR 0.12 [0.04, 0.33]) and JAK inhibitors (OR 0.41 [0.18, 0.92]) were associated with significantly lower odds for low spike Ab. After adjusting for systemic rheumatic disease and DMARDs, SLE and rituximab remained significantly associated with low spike Ab. Conclusions: Over a third of patients with systemic rheumatic disease with spike Ab tested in routine care had low spike Ab after 2 doses of mRNA or 1 dose of adenovirus vector COVID-19 vaccine. SLE and rituximab were significant risk factors for low spike Ab. Key Points: • More than one-third of patients with systemic rheumatic disease that had spike Ab tested in routine care had low spike Ab after 2 doses of mRNA or 1 dose of adenovirus vector COVID-19 vaccine. • Diagnosis of SLE, use of prednisone, and use of rituximab were significantly associated with greater odds of low spike antibodies. • These data underscore the importance of additional doses of COVID-19 vaccine and prophylactic Evusheld in immunosuppressed patients with systemic rheumatic disease as recommended by the US Centers for Disease Control. [ABSTRACT FROM AUTHOR]

Introduction: Restless leg syndrome (RLS) is a neurological disorder characterized by an uncontrollable desire to move legs along with abnormal sensations, particularly at night, which can lead to sleep disturbance. RLS may mimic rheumatic diseases or can be associated with them, hence their identification and treatment are important to improve sleep quality and overall quality of life in rheumatic diseases. Methods: We conducted a search of the PubMed, SCOPUS, and EMBASE databases to identify studies reporting a prevalence of RLS in patients with rheumatic disease. Two authors independently screened, selected, and extracted the data. Heterogeneity was assessed using I2 statistics and random effect method of the meta‐analysis was used to synthesize the results. Results: Out of 273 unique records, 17 eligible studies including 2406 rheumatic patients were identified. RLS prevalence (95% CI) among patients of rheumatoid arthritis, systemic lupus erythematosus, osteoarthritis, fibromyalgia and ankylosing spondylitis are found to be 26.6% (18.6 34.6); 32.5% (23.1–41.9), 4.4% (2.0–6.8), 38.1% (31.3–45.0) and 30.8% (23.48–39.16) respectively. RLS prevalence was similar for males and females. Conclusion: Our study indicates a high prevalence of RLS in patients with rheumatic diseases. Early detection and treatment of RLS in patients with rheumatic conditions could be beneficial in improving their overall health and quality of life. [ABSTRACT FROM AUTHOR]

Diagnostic and prognostic markers are necessary to help in patient diagnosis and the prediction of future clinical events or disease progression. As promising biomarkers of selected diseases, the free light chains (FLCs) κ and λ were considered. Measurements of FLCs are currently used in routine diagnostics of, for example, multiple myeloma, and the usefulness of FLCs as biomarkers of monoclonal gammopathies is well understood. Therefore, this review focuses on the studies concerning FLCs as new potential biomarkers of other disorders in which an inflammatory background has been observed. We performed a bibliometric review of studies indexed in MEDLINE to assess the clinical significance of FLCs. Altered levels of FLCs were observed both in diseases strongly connected with inflammation such as viral infections, tick-borne diseases or rheumatic disorders, and disorders that are moderately associated with immune system reactions, e.g., multiple sclerosis, diabetes, cardiovascular disorders and cancers. Increased concentrations of FLCs appear to be a useful prognostic marker in patients with multiple sclerosis or tick-borne encephalitis. Intensive synthesis of FLCs may also reflect the production of specific antibodies against pathogens such as SARS-CoV-2. Moreover, abnormal FLC concentrations might predict the development of diabetic kidney disease in patients with type 2 diabetes. Markedly elevated levels are also associated with increased risk of hospitalization and death in patients with cardiovascular disorders. Additionally, FLCs have been found to be increased in rheumatic diseases and have been related to disease activity. Furthermore, it has been suggested that inhibition of FLCs would reduce the progression of tumorigenesis in breast cancer or colitis-associated colon carcinogenesis. In conclusion, abnormal levels of κ and λ FLCs, as well as the ratio of κ:λ, are usually the result of disturbances in the synthesis of immunoglobulins as an effect of overactive inflammatory reactions. Therefore, it seems that κ and λ FLCs may be significant diagnostic and prognostic biomarkers of selected diseases. Moreover, the inhibition of FLCs appears to be a promising therapeutical target for the treatment of various disorders where inflammation plays an important role in the development or progression of the disease. [ABSTRACT FROM AUTHOR]

Systemic Lupus Erythematosus (SLE) is a systemic autoimmune disease that attacks various organ systems with a variety of clinical implications, ranging from mild skin and mucosal manifestations to severe central nervous system manifestations and death. Cases of SLE have been documented nearly two centuries ago when scholars used the terms 'erythema centrifugum' and 'seborrhea congestiva' to describe the discoid skin lesions and the butterfly or malar rash in SLE. Since then, knowledge about this disease has developed rapidly, especially knowledge related to the underlying pathogenesis of SLE. To date, it is known that immune system dysregulation, supported by genetic and environmental predisposition, can trigger the occurrence of SLE in a group of susceptible individuals. Various inflammatory mediators, cytokines and chemokines, as well as intra- and intercellular signaling pathways, are involved in the pathogenesis of SLE. In this review, we will discuss the molecular and cellular aspects of SLE pathogenesis, with a focus on how the immune system, genetics and the environment interact and trigger the various clinical manifestations of SLE. [ABSTRACT FROM AUTHOR]

Introduction: Pain is a prominent contributor to negative personal and social outcomes, including increased disability and mortality, in many rheumatic diseases. In the Biopsychosocial model of chronic pain, psychological and social factors share roles with the biology of the injury in determining each patient's pain and suffering. The current study explored factors associated with clinical pain intensity and interference among patients with chronic secondary musculoskeletal pain in rheumatic diseases. Methods: In total, 220 patients experiencing chronic secondary musculoskeletal pain participated. Biological factors (age, biological sex, pain condition, pain duration, pain sensitivity, and comorbidity), socio-economic factors, psychological factors (pain catastrophizing and depressive symptoms), and pain intensity and interference were measured. Descriptive, multivariable linear regression and partial correlation analyses were conducted. Subgroup analysis by sex was conducted to examine differences in how different factors affect the pain experience. Results: The mean age of the participants was 52.3 years (SD = 12.07) and ranged from 22 to 78. Average pain intensity was 3.01 (0-10 scale) and average total pain interference score was 21.07 (0-70 scale). Partial correlation found positive correlations between pain intensity and interference with depression (intensity: R = 0.224; p = 0.0011; interference: R = 0.351; p < 0.001) and pain catastrophizing (intensity: R = 0.520; p < 0.001; interference: R = 0.464; p < 0.001). In males, pain condition (β = -0.249, p = 0.032) and pain catastrophizing (R = 0.480, p < 0.001) were associated with pain intensity. In males, the simple correlation between pain intensity and depression (R = 0.519; p < 0.001) was driven by pain catastrophizing. In females, pain catastrophizing (R = 0.536, p < 0.001) and depressive symptoms (R = 0.228, p = 0.0077) were independently associated with pain intensity. Age (β = -0.251, p = 0.042) and pain catastrophizing (R = 0.609, p < 0.001) were associated with pain interference in males, while depressive symptoms (R = 0.439, p < 0.001) and pain catastrophizing (R = 0.403, p < 0.001) were associated with pain interference in females. Again, in males, the simple correlation between pain interference and depression (R = 0.455; p < 0.001) was driven by pain catastrophizing. Discussion: In this study, females were more directly affected by depressive symptoms than males, regarding pain intensity and interference. Pain catastrophizing was a significant factor influencing chronic pain for both males and females. Based on these findings, a sex-specific approach to the Biopsychosocial model should be considered in understanding and managing pain among Asians with chronic secondary musculoskeletal pain. [ABSTRACT FROM AUTHOR]

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We described the profile and outcome of Filipino patients with inflammatory rheumatic diseases (IRDs) who developed COVID-19 (IRD-C19) during the onset of the pandemic, prior to vaccinations and variants. We obtained de-identified data of Filipino patients with IRD-C19 from the Global Rheumatology Alliance registry from March 2020 to August 2021. Descriptive statistics and multivariate analyses were applied. Registered were 164 patients (mean age 44 years; 70% female). The most common IRDs were systemic lupus erythematosus (SLE, 41.4%), rheumatoid arthritis (RA, 15.2%), and gout (14.6%). Majority were receiving conventional DMARDs (59.1%) and/or glucocorticoid therapy (GC, 51.2%). Half (58.5%) were hospitalized, with risk higher in active IRD (OR 3.7), heart disease (8.52), and hypertension (8.73); and lower in SLE patients (0.15). Among hospitalized patients, 76% needed supplemental oxygen. Heart disease (6.28), hypertension (7.6), and moderate-to-high IRD activity (3.37) were associated with higher odds of requiring oxygen supplementation. Hypertension was associated with mechanical ventilation (8.23). Twenty-four (15%) patients died, with odds lower if on prednisone ≥ 10 mg/day (0.17) and with other autoimmune IRDs aside from SLE and RA (0.05). Among patients with IRD-C19 prior to vaccinations and variants, higher disease activity, hypertension, and heart disease were associated with poorer outcomes. Prednisone ≥ 10 mg/day was associated with lower odds of death. This study provides valuable historical information, emphasizing the need for continued data collection to clarify COVID-19's impact. Key Points • Filipinos with rheumatic diseases who developed COVID-19 at the pandemic outset had manifestations similar to other ethnicities and geographic locations. • Hospitalization and need for oxygen supplementation were higher among patients with active inflammatory rheumatic disease, heart disease, and hypertension. • Use of csDMARDs did not show negative effects on COVID-19 outcomes. • A reduced odds of dying was found among patients on prednisone > 10 mg/day. [ABSTRACT FROM AUTHOR]

The article presents guidelines on prescribing comorbidity medications used in rheumatology practice to pregnant and breastfeeding women from the British Society for Rheumatology. The objective is to update previous guidelines on prescribing for drug categories like pain management, anticoagulants and anti-hypertensives. It excludes the management of infertility or acute pain relief during labour.

The article presents guidelines on prescribing immunomodulatory anti-rheumatic drugs and corticosteroids to pregnant and breastfeeding women from the British Society for Rheumatology. The objective is to update the previous guidelines and highlight medications that have to be stopped and avoided in the reproductive age group. It excludes the management of infertility or the indications for the drugs in rheumatic diseases in pregnancy.

The article presents guidelines on prescribing comorbidity medications used in rheumatology practice to pregnant and breastfeeding women from the British Society for Rheumatology. The objective is to update previous guidelines on prescribing for drug categories like pain management, anticoagulants and pulmonary vasodilators. It excludes the management of infertility or acute pain relief during labour.

The article presents guidelines on prescribing immunomodulatory anti-rheumatic drugs and corticosteroids to pregnant and breastfeeding women from the British Society for Rheumatology. Uncertainty over the use of the drugs in pregnancy and breastfeeding has led to the expansion of existing and novel disease-modifying antirheumatic drugs. The guideline excludes the management of infertility or indications in specific rheumatic diseases in pregnancy.

Objectives To determine COVID-19 vaccine hesitancy rates in inflammatory arthritis patients and identify factors associated with changing vaccine hesitancy over time. Methods This investigation was a prospective cohort study of inflammatory arthritis patients from community and public hospital outpatient rheumatology clinics enrolled in the Australian Rheumatology Association Database (ARAD). Two surveys were conducted, one immediately prior to (pre-pandemic) and another approximately 1 year after the start of the pandemic (follow-up). Coronavirus disease 2019 (COVID-19) vaccine hesitancy was measured at follow-up, and general vaccine hesitancy was inferred pre-pandemic; these were used to identify factors associated with fixed and changing vaccine beliefs, including sources of information and broader beliefs about medication. Results Of the 594 participants who completed both surveys, 74 (12%) were COVID-19 vaccine hesitant. This was associated with pre-pandemic beliefs about medications being harmful (P  < 0.001) and overused (P  = 0.002), with stronger beliefs resulting in vaccine hesitancy persistent over two time points (P  = 0.008, P  = 0.005). For those not vaccine hesitant pre-pandemic, the development of COVID-19 vaccine hesitancy was associated with a lower likelihood of seeking out vaccine information from health-care professionals (P  < 0.001). COVID-19 vaccine hesitancy was not associated with new influenza vaccine hesitancy (P  = 0.138). Conclusion In this study of vaccine beliefs before and during the COVID-19 pandemic, factors associated with COVID-19 vaccine hesitancy in inflammatory arthritis patients varied, depending on vaccine attitudes immediately prior to the start of the pandemic. Fixed beliefs reflected broader views about medications, while fluid beliefs were highly influenced by whether they sought out information from health-care professionals, including rheumatologists. [ABSTRACT FROM AUTHOR]

Background: Coronavirus disease‐2019 (COVID‐19) vaccine hesitancy is a significant threat to the success of COVID‐19 vaccination programs. Objectives: We aimed to assess attitudes and factors affecting the decision‐making vis‐à‐vis COVID‐19 vaccination among patients with autoimmune rheumatic diseases (ARDs). Method: A cross‐sectional survey of adults with ARDs was conducted between January 2022 and April 2022. All enrolled ARDs patients were asked to answer a questionnaire about their attitudes regarding COVID‐19 vaccination. Results: Three hundred patients were included with a female‐to‐male ratio of 2.5:1. The mean age of the patients was 49.2 ± 15.6 years. Around 37% of patients who hesitated to get the COVID‐19 vaccination were apprehensive regarding potential adverse events from the vaccine. About 25% (76 cases) were hesitant about vaccination, of which 15% were uncertain about the vaccine's efficacy, and 15% thought the vaccine was unnecessary because they lived in rural areas where they practiced social distancing. "Family role as a non‐working member" was the only factor strongly associated with hesitancy for vaccination (odds ratio of 2.42; 95% CI 1.06–5.57). The attitudes to vaccination showed that the patients were concerned about disease flaring and believed all medicine should be stopped before vaccination. Conclusion: Around one‐quarter of ARDs sufferers hesitated to get COVID‐19 vaccination. In addition, some patients were disinclined to get vaccinated because they were worried about its efficacy and/or associated adverse events. The findings help healthcare providers plan to counter negative attitudes toward vaccination in ARDs patients to protect them during the COVID‐19 era. [ABSTRACT FROM AUTHOR]

Objective. Contemporary studies reporting outcomes of critical care in patients with inflammatory and autoimmune rheumatological diseases are scarce. This study describes 15 years of experience from 2005-2019 in a Colombian referral hospital. Methods. This observational, descriptive, consecutive case series study was performed on adult patients with inflammatory and autoimmune rheumatic diseases who were admitted to the intensive care unit (ICU) of the San Ignacio University Hospital in Bogotá (Colombia), from January 1, 2005, to December 21, 2019. We describe the sociodemographic characteristics, admission causes and criteria, lengths of stay, immunosuppressive treatment, systemic support, and mortality. Results. The study included 300 patients with a median age of 48 years [interquartile range (IQR) 31-62 years], predominantly female (76%). Disease exacerbations (30%), infections (17.6%), and cardiovascular diseases (15%) were the main causes of admission. Respiratory failure (23%) most commonly caused by septic shock (24%) was the principal indication for intensive care admission. The most frequent infections were community-acquired pneumonia (11.6%) and soft-tissue infections (9%). In 40.3% of patients, inotropic and vasopressor support was required. The median length of stay was 4 days (IQR 2-8), and global mortality was 21.6%. Conclusions. Rheumatic diseases in the ICU are still associated with high morbidity and mortality. Patients with inflammatory and autoimmune rheumatic diseases require a meticulous clinical approach, strict clinical monitoring, frequent assessment of complications, evaluation of systemic support needs, and specific management.

Summary: Background: Due to geographic isolation and border controls Aotearoa New Zealand (AoNZ) attained high levels of population coronavirus disease-19 (COVID-19) vaccination before widespread transmission of COVID-19. We describe outcomes of SARS-CoV-2 infection (Omicron variant) in people with inflammatory rheumatic diseases in this unique setting. Methods: This observational study included people with inflammatory rheumatic disease and SARS-CoV-2 infection in AoNZ between 1 February and 30 April 2022. Data were collected via the Global Rheumatology Alliance Registry including demographic and rheumatic disease characteristics, and COVID-19 vaccination status and outcomes. Multivariable logistic regression was used to explore associations of demographic and clinical factors with COVID-19 hospitalisation and death. Findings: Of the 1599 cases included, 96% were from three hospitals that systematically identified people with inflammatory rheumatic disease and COVID-19. At time of COVID-19, 1513 cases (94.6%) had received at least two COVID-19 vaccinations. Hospitalisation occurred for 104 (6.5%) cases and 10 (0.6%) patients died. Lower frequency of hospitalisation was seen in cases who had received at least two vaccinations (5.9%), compared to the unvaccinated (20.6%) or those with a single vaccine dose (10.7%). In multivariable adjusted models, people with gout or connective tissue diseases (CTD) had increased risk of the combined outcome of hospitalisation/death, compared to people with inflammatory arthritis. Glucocorticoid and rituximab use were associated with increased rates of hospitalisation/death. All patients who died had three or more co-morbidities or were over 60 years old. Interpretation: In this cohort with inflammatory rheumatic diseases and high vaccination rates, severe outcomes from SARS-CoV-2 Omicron variant were relatively infrequent. The outcome of Omicron variant infection among vaccinated but SARS-CoV-2 infection-naive people with inflammatory rheumatic disease without other known risk factors were favourable. Funding: Financial support from the American College of Rheumatology (ACR) and European Alliance of Associations for Rheumatology (EULAR) included management of COVID-19 Global Rheumatology Alliance funds.

The goal of this study is to develop a rapid diagnostic test for rheumatic disease and systemic lupus erythematosus (SLE) screening. A novel rapid vertical flow assay (VFA) was engineered and used to assay anti-nuclear (ANA) and anti-dsDNA (αDNA) autoantibodies from systemic lupus erythematosus (SLE) patients and healthy controls (HCs). Observer scores and absolute signal intensities from the VFA were validated via ELISA. The rapid point-of-care VFA test that was engineered demonstrated a limit of detection of 0.5 IU/mL for ANA and αDNA autoantibodies in human plasma with an inter-operator CV of 19% for ANA and 12% for αDNA. Storage stability was verified over a three-month period. When testing anti-dsDNA and ANA levels in SLE and HC serum samples, the duplex VFA revealed 95% sensitivity, 72% specificity and an 84% ROC AUC value in discriminating disease groups, comparable to the gold standard, ELISA. The rapid αDNA/ANA duplex VFA can potentially be used in primary care clinics for evaluating patients or at-risk subjects for rheumatic diseases and for planning follow-up testing. Given its low cost, ease, and rapid turnaround, it can also be used to assess SLE prevalence estimates.

Abstract Background Non-criteria antiphospholipid antibodies (NC-aPL) are a relatively undefined subgroup of antiphospholipid antibodies (aPL). Knowledge about NC-aPL in adults is limited and even less is known in pediatric patients. Routine tests for antiphospholipid syndrome (APS)—a clinical state marked by the presence of aPL in association with vascular thrombosis—usually include lupus anticoagulant (LAC), anti-cardiolipin (aCL) and -beta-2 glycoprotein I (aβ2GPI). LAC is a functional screen for prothrombotic aPL, while the latter tests identify specific autoantibodies. Specific targets of NC-aPL include, but are not limited to, phosphatidylethanolamine, phosphatidylserine, and prothrombin. Presentation of cases We present single-center data from eight pediatric patients with NC-aPL identified during a three-year period. All patients had presenting features raising suspicion for APS. Most patients were female with a primary rheumatic disease. One patient had a stroke. Another patient had alveolar hemorrhage and pulmonary hypertension. Raynaud’s phenomenon, rashes involving distal extremities, and headaches were common. Most patients had a positive LAC, yet their routine aPL tests were negative, prompting testing for NC-aPL. Conclusions Our findings suggest NC-aPL are associated with typical signs and symptoms of APS in pediatric patients. Pediatricians and pediatric subspecialists should consider NC-aPL when clinical suspicion is high and routine aPL tests are negative, particularly when LAC is positive. While guidelines for NC-aPL do not yet exist for children or adults, these autoantibodies have pathogenic potential. Actionable items could include evaluation for the presence of other (primary) rheumatic diseases, and consultation with hematologists and/or obstetricians regarding anticoagulation/platelet inhibition and thrombosis education. Future guidelines regarding NC-aPL will only be generated by gathering more data, ideally prospectively.

Abstract Background Pulmonary complications of rheumatic diseases may cause functional impairment and increase mortality. However, reports regarding detection of lung involvement in children with treatment-naive, newly diagnosed rheumatic diseases are scarce. Herein, we aimed to describe the characteristics of such patients and explore the association between lung involvement and rheumatic disease. Methods From January 2019 to June 2021, 48 pediatric patients with treatment-naive, newly diagnosed rheumatic diseases at Department of Rheumatology and Immunology, Shanghai Children's Medical Center, School of Medicine, Shanghai Jiao Tong University were included with pulmonary function tests (PFTs) and high-resolution computed tomography (HRCT) findings, and 51 age-matched healthy controls were examined based on PFTs. Univariate and multivariable logistic regression analyses were used to investigate the clinical characters and laboratory parameters associated with lung involvement in these patients. Results Asymptomatic patients had a faster respiratory rate and a higher ratio of forced expiratory volume in 1 s/forced vital capacity than the controls (P

Feng Wang Rheumatology and Immunology Department, Heping Hospital Affiliated to Changzhi Medical College, Changzhi, 046000, People’s Republic of ChinaCorrespondence: Feng Wang, Department of Rheumatology and Immunology, Heping Hospital Affiliated to Changzhi Medical College, No. 110, Yan ‘an Nan Lu, Changzhi City, Shanxi Province, 046000, People’s Republic of China, Tel +0086-355-3128612, Email wangfengwf10@163.comIntroduction: The COVID-19 pandemic is an ongoing global pandemic. Patients with rheumatic disease are more likely to be infected with COVID-19 than the general population, and they should be vaccinated against COVID-19 for protection. This study aimed to understand the willingness to receive the COVID-19 vaccine among these patients.Methodology: Patients who came to the Rheumatology and Immunology Department of our hospital from July 3– 20, 2021, were randomly selected for a self-designed survey via an online questionnaire platform. Four hundred seventeen questionnaires were validated.Results: Males were more likely to underestimate the risk of COVID-19 infection than females, and medical workers had a higher perception of COVID-19 infection risk than nonmedical workers (both P < 0.05). Among the included patients, 149 (35.73%) patients were hesitant to be vaccinated, while 268 (64.27%) patients are willing to accept vaccination. Logistic regression analysis showed that men were more willing to be vaccinated than women, and nonmedical workers were more willing to be vaccinated than medical workers (both P < 0.05). A total of 55.40% of the patients did not receive the COVID-19 vaccine. The incidence of adverse reactions for the 167 patients who received the COVID-19 vaccine was 8.98%.Conclusion: The vaccination rate of the patients with rheumatic disease was relatively low, as was their vaccination willingness. Patient sex, whether the patient was a medical worker, and the patient’s level of knowledge about the risk of COVID-19 infection and the impact of vaccination on the disease were key factors.Keywords: COVID-19 vaccine, COVID-19, rheumatic disease, vaccination willingness

Abstract Background Vitamin D deficiency has been associated with autoimmune diseases and oral health. Knowledge about the association between vitamin D status and oral conditions in JIA is limited. We aimed to investigate vitamin D status in a cohort of Norwegian children and adolescents with JIA and possible associations between serum vitamin D levels, clinical indicators of oral health, and JIA disease characteristics. Methods This multi-center, cross-sectional study, included individuals with JIA aged 4–16 years from three geographically spread regions in Norway. Demographic data, age at disease onset, disease duration, JIA category, disease status, medication, and vitamin D intake were registered. One blood sample per individual was analyzed for 25(OH) vitamin D, and the level of insufficiency was defined as

Summary: Background: Rheumatic disease (RD) represents a broad spectrum of systemic conditions characterized by inflammation and pain in muscles or joints with a significant burden on quality of life. Increasing evidence suggests that diet could play a modulatory role in RD by influencing cardiovascular diseases (CVD) risk factors frequently present in these patients as well as inflammation and antioxidant defence. Objectives: This review aims to summarize the available evidence on the effect of nutrients, foods and dietary patterns on the most common autoimmune inflammatory RD including rheumatoid arthritis, Sjogren's syndrome, systemic lupus erythematosus and systemic sclerosis. Results: We documented that MUFAs and PUFAs seem to have positive effects in modulating the inflammatory process. Regarding the dietary interventions, low-calorie diets, Mediterranean diet and fasting appear to be effective in reducing the symptoms of the most common RD. Positive results were also obtained in some cases with gluten-free, low-fat, vegan, elimination or anti-inflammatory diets. Conclusion: Although further and specific studies are needed, the fact that people obtained an improvement in clinical outcomes after almost all these dietary patterns suggests that a healthy diet could play a pivotal role in the RD management.

Purpose: We analyzed data collected for ascertaining severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) vaccine acceptance, perceptions, and post-vaccination side effects among Kuwaiti inflammatory rheumatic disease (IRD) patients. Methods: The current cross-sectional study was conducted on patients attending governmental rheumatology clinics across seven hospitals in Kuwait between July and September 2021. We included adults of both sexes who are national/residents of the state of Kuwait and who had a confirmed diagnosis of any IRD disease. Data on patients' demographics, history of IRD, SARS-CoV-2 infection, vaccination status, as well as post-vaccination side effects and disease flare were collected from the included participants through a self-administered questionnaire. Stata MP/17 for macOS was used for statistical analyses. Results: We included 501 IRD patients, a group with a mean age of 43.38 years and a mean disease duration of 10.46 years. The majority of the included patients were female (79.8%), and the most common primary rheumatology diagnosis was rheumatoid arthritis (42.5%), followed by spondyloarthritis (19.4%) and systemic lupus erythematosus (19.0%). One hundred and five patients (21.0%) had SARS-CoV-2 infection confirmed by PCR-positive swab; of them, 17 patients were hospitalized. None of the included patients were using steroids alone. cDMARDs, bDMARDs, and sDMARDs were reported in 37.3%, 18.0%, and 3.8% of the patients, respectively. Three hundred and fifty-one patients (70.1%) were vaccinated; 40.9% received Pfizer/BioNTech, whereas 28.7% received AstraZeneca/Oxford vaccines. Fear that the vaccination will aggravate the condition or interfere with the present therapy and concerns about its effectiveness as well as its side effects were the most prevalent causes for refusing to accept the SARS-CoV-2 vaccine. Other patients were worried about the paucity of the data because individuals with IRD had been omitted from earlier research, resulting in a dearth of information. The commonly reported post-vaccination side effects were body ache/pain, fatigue, and pain at the injection site (32.1%, 30.3%, and 29.7%, respectively). IRD flare post-SARS-CoV-2 vaccination was self-reported in only 9 patients, and 342 did not report a flare. Conclusions: This study's findings highlight that SARS-CoV-2 vaccines have an acceptable safety profile, with the majority of their side effects being temporary and mild. The occurrence of flares was low after immunization. Reassurance and trust in the safety of the SARS-CoV-2 vaccination in IRD patients should be reassuring to rheumatologists and vaccine recipients. [ABSTRACT FROM AUTHOR]

There is an increased risk of malignancies in patients with many systemic rheumatic diseases, which negatively impact on their quality of life. The risk and types of malignancies can differ by the type of rheumatic diseases. Possible mechanisms linking them are dynamic and complicated, including chronic inflammation and damage in rheumatic disease, inability to clear oncogenic infections, shared etiology and some anti-rheumatic therapies. Although certain disease-modifying anti-rheumatic drugs (DMARDs) have been proved to be potentially carcinogenic, the majority of them were not associated with increased risk of most malignancies in patients with systemic rheumatic diseases. [ABSTRACT FROM AUTHOR]

Interleukin-32 (IL-32) is an important cytokine involved in the innate and adaptive immune responses. The role of IL-32 has been studied in the context of various diseases. A growing body of research has investigated the role of IL-32 in rheumatic diseases including inflammatory arthritides (rheumatoid arthritis, ankylosing spondylitis, and psoriatic arthritis) and connective tissue diseases (systemic lupus erythematosus, systemic sclerosis, granulomatosis and polyangiitis, and giant cell arteritis). IL-32 has been shown to play different roles according to the type of rheumatic diseases. Hence, the putative role of IL-32 as a biomarker is also different in each rheumatic disease: IL-32 could serve as a biomarker for disease activity in some diseases, whereas in other diseases it could be a biomarker for certain disease manifestations. In this narrative review, we summarize the associations between IL-32 and various rheumatic diseases and discuss the putative role of IL-32 as a biomarker in each disease. [ABSTRACT FROM AUTHOR]

Medical nutrition therapy (MNT) is an indisputable component of the multidisciplinary therapeutic approach in rheumatoid arthritis (RA). Previous research has suggested that in chronic disease where nutrition is an important effector of prognosis, healthy dietary choices might take an unhealthy turn, with patients developing disordered eating in the form of orthorexia nervosa (ON). ON is characterized by a pathological preoccupation with "healthy", "pure" eating, associated with restrictive dietary patterns, nutrient deficiencies and worsening disease outcomes. The aim of the present cross-sectional study was to evaluate ON tendencies in a sample of adult patients with RA. A total of 133 patients with RA were recruited, and completed the ORTO-15 questionnaire for the assessment of ON tendencies. Most of the patients were overweight/obese (53.4%). The results revealed ON tendencies in the sample, with the median ORTO-15 score reaching 36 (IQR: 33–39). Greater ON tendencies were associated with the female gender, and lowered ON tendencies with increasing age and body mass index. The present findings highlight the need for health professional awareness regarding the problem of ON in patients with RA and the importance of screening patients. [ABSTRACT FROM AUTHOR]

In rheumatoid arthritis (RA), diet quality and nutritional status have been shown to impact the disease activity and adherence to the Mediterranean diet (MD) has been suggested as an anti-inflammatory regime to improve disease status and reduce cardiovascular risk. The Mediterranean DiEt In Rheumatoid Arthritis (MADEIRA) was a single-blind (statistician), two-arm randomized clinical trial, investigating the effects of a 12-week lifestyle intervention, including a personalized isocaloric MD plan with the promotion of physical activity (PA), supported through a clinical decision support systems (CDSS) platform, versus usual care in women with RA. Forty adult women with RA on remission were randomly allocated (1:1 ratio) to either the intervention or the control arm. The intervention group received personalized MD plans and lifestyle consultation on improving PA levels, whereas the controls were given generic dietary and PA advice, based on the National Dietary Guidelines. The primary outcome was that the difference in the MD adherence and secondary outcomes included change in disease activity (DAS28), anthropometric indices (BodPod), dietary intake, PA, vitamin D concentrations, and blood lipid profiles after 12 weeks from the initiation of the trial. At 3 months post-baseline, participants in the MD arm exhibited greater adherence to the MD compared with the controls (p < 0.001), lower DAS28 (p < 0.001), favorable improvements in dietary intake (p = 0.001), PA (p = 0.002), body weight and body composition (p < 0.001), blood glucose (p = 0.005), and serum 1,25(OH)2D concentrations (p < 0.001). The delivery of the MD and PA promotion through CDSS nudges in women with RA in an intensive manner improves the MD adherence and is associated with beneficial results regarding disease activity and cardiometabolic-related outcomes, compared with the usual care. [ABSTRACT FROM AUTHOR]

The purpose of the research was to verify the effects of a supervised telehealth stretching exercise program on pain, sleep, depression, and functionality of women with fibromyalgia during the COVID-19 pandemic. This randomized clinical trial comprised 28 participants allocated into 2 groups. The experimental group (EG; n = 13; 49.9 ± 8.0 years) received a stretching exercise program with remote supervision for 12 weeks; the active control group (CG; n = 15; 47.0 ± 6.5 years) received counseling for stretching via an exercise guide and videos to be performed without supervision for 12 weeks. The following outcomes were assessed online: pain index, sleep quality, symptom severity, the impact of fibromyalgia on daily activities, depressive symptoms, pain intensity at rest and after movement, and flexibility. Data analysis was performed by intention to treat (ITT) and per protocol (PP) using generalized estimating equations (α = 0.05). Significant improvements were observed post-intervention for all outcomes in both groups, except for the flexibility by ITT (p = 0.136) and depressive symptoms by PP analysis (p = 0.065). In addition, the impact of fibromyalgia on daily activities presented better responses for EG than CG (ITT: p = 0.027). It was concluded that the telehealth stretching program, supervised or not, was effective in promoting the improvement in the main symptoms in women with fibromyalgia. [ABSTRACT FROM AUTHOR]

Objectives COVID-19 vaccines have been proven to be safe in the healthy population. However, gaps remain in the evidence of their safety in patients with systemic autoimmune and inflammatory disorders (SAIDs). COVID-19 vaccination-related adverse events (AEs) in patients with SAIDs and healthy controls (HC) seven days post-vaccination were assessed in the COVAD study, a patient self-reported cross-sectional survey. Methods The survey was circulated in early 2021 by >110 collaborators (94 countries) to collect SAID details, COVID-19 vaccination details and 7-day vaccine AEs, irrespective of respondent vaccination status. Analysis was performed based on data distribution and variable type. Results Ten thousand nine hundred respondents [median (interquartile range) age 42 (30–55) years, 74% females and 45% Caucasians] were analysed; 5867 patients (54%) with SAIDs were compared with 5033 HCs. Seventy-nine percent had minor and only 3% had major vaccine AEs requiring urgent medical attention (but not hospital admission) overall. Headache [SAIDs = 26%, HCs = 24%; odds ratio (OR) = 1.1 (95% CI: 1.03, 1.3); P  = 0.014], abdominal pain [SAIDs = 2.6%, HCs = 1.4%; OR = 1.5 (95% CI: 1.1, 2.3); P  = 0.011], and dizziness [SAIDs = 6%, HCs = 4%; OR = 1.3 (95% CI: 1.07, 1.6); P  = 0.011], were slightly more frequent in SAIDs. Overall, major AEs [SAIDs = 4%, HCs = 2%; OR = 1.9 (95% CI: 1.6, 2.2); P  < 0.001] and, specifically, throat closure [SAIDs = 0.5%, HCs = 0.3%; OR = 5.7 (95% CI: 2.9, 11); P  = 0.010] were more frequent in SAIDs though absolute risk was small (0–4%). Major AEs and hospitalizations (<2%) were comparable across vaccine types in SAIDs. Conclusion Vaccination against COVID-19 is safe in SAID patients. SAIDs were at a higher risk of major AEs than HCs, though absolute risk was small. There are small differences in minor AEs between vaccine types in SAID patients. [ABSTRACT FROM AUTHOR]

Objective: Children with suspicious complaints of rheumatic diseases are generally referred to a pediatric rheumatologist. We aimed to evaluate the profile of patients referred to the pediatric rheumatology unit and were not diagnosed with a rheumatic disease and to assess the impact of the coronavirus disease-2019 pandemic on referral complaints. Materials and Methods: All new outpatients who applied to the pediatric rheumatology department between March 2019 and February 2021 and were not diagnosed with rheumatic disease were included. We also compared the frequency of admission symptoms during the pre-pandemic (March 2019-February 2020) and pandemic periods (March 2020-February 2021). Results: A total of 1089 patients without a rheumatic disease diagnosis (568 female, 52.2%; median age 10.0 years) were included in this study. The most common complaint for referral was prolonged or recurrent fevers (13.4%) followed by anti-nuclear antibody positivity (13.1%), arthralgia (13.0%), skin findings (7.5%), and the presence of heterozygous mutations in the Mediterranean fever gene (6.9%). During the pandemic year, the number of patients referred for back pain increased significantly (P = .028). A total of 682 of 1089 patients were consulted from other departments in our center (62.6%). Of these, the most frequent consultation request was from general pediatrics (43.6%). The rheumatic disease was excluded in 11.3% of the patients. Conclusion: Prolonged or recurrent fever and anti-nuclear antibody positivity were the most frequent complaints of referrals to a pediatric rheumatology unit in patients who did not have a rheumatic disease. The rate of back pain was more common in children during the pandemic period. [ABSTRACT FROM AUTHOR]

Background: Social media data may augment understanding of the disease and treatment experiences and quality of life of youth with chronic medical conditions. Little is known about the willingness to share social media data for health research among youth with chronic medical conditions and the differences in health status between sharing and nonsharing youth with chronic medical conditions. Objective: We aimed to evaluate the associations between patient-reported measures of disease symptoms and functioning and the willingness to share social media data. Methods: Between February 2018 and August 2019, during routine clinic visits, survey data about social media use and the willingness to share social media data (dependent variable) were collected from adolescents in a national rheumatic disease registry. Survey data were analyzed with patient-reported measures of disease symptoms and functioning and a clinical measure of disease activity, which were collected through a parent study. We used descriptive statistics and multivariate logistic regression to compare patient-reported outcomes between youth with chronic medical conditions who opted to share social media data and those who did not opt to share such data. for research. Sharers reported worse symptoms and functioning compared to those of nonsharers. Social media may offer a potent information source and engagement pathway for youth with rheumatic diseases, but differences between sharing and nonsharing youth merit consideration when designing studies and evaluating social media-derived findings. [ABSTRACT FROM AUTHOR]

Objective There is dearth of data regarding the outcomes of coronavirus disease 2019 (COVID-19) among rheumatic and musculoskeletal disease (RMD) patients from Southeast Asia. We report the clinicodemographic profile and identify predictors of COVID-19 outcomes in a large cohort of Indian RMD patients. Methods This prospective cohort study, carried out at the Postgraduate Institute of Medical Education and Research, Chandigarh (a tertiary care centre in India), included RMD patients affected with COVID-19 between April 2020 and October 2021. Demographic and clinical and laboratory details of COVID-19 and underlying RMD were noted. Predictors of mortality, hospitalization and severe COVID-19 were identified using stepwise multivariable logistic regression. Results A total of 64 severe acute respiratory syndrome coronavirus-2-infected RMD patients [age 41.5 (19–85) years; 46 (72%) females] were included. Eighteen (28%) patients had severe COVID-19. Twenty-three (36%) required respiratory support [11 (17%) required mechanical ventilation]. Thirty-six (56%) patients required hospitalization [median duration of stay 10 (1–42) days]; 17 (27%) required intensive care unit admission. Presence of co-morbidities [odds ratio (OR) = 4.5 (95% CI: 1.4, 14.7)] was found to be an independent predictor of COVID-19 severity. Co-morbidities [OR = 10.7 (95% CI: 2.5, 45.4)] and underlying lupus [OR = 7.0 (95% CI: 1.2, 40.8)] were independently associated with COVID-19 hospitalization. Ongoing rheumatic disease activity [OR = 6.8 (95% CI: 1.3, 35.4)] and underlying diagnosis of lupus [OR = 7.1 (95% CI: 1.2, 42.4)] and SSc [OR = 9.5 (95% CI: 1.5, 61.8)] were found to be strong independent predictors of mortality. Age, sex, underlying RMD-associated interstitial lung disease and choice of immunosuppressive therapy were not associated with COVID-19 severity or adverse outcomes. Conclusion The presence of co-morbidities was independently associated with COVID-19 severity and hospitalization. Ongoing rheumatic disease activity and the presence of lupus or SSc independently predicted mortality. Age, sex, type of immunosuppressive therapy and presence of RMD-associated interstitial lung disease did not affect COVID-19 severity or outcomes in Indian RMD patients. [ABSTRACT FROM AUTHOR]

Objective The aim was to evaluate the impact of the coronavirus disease 2019 (COVID-19) pandemic and stringent social isolation measures on patients with rheumatic disease (RD) from the beginning of the pandemic (April 2020). Methods In this UK-based single-centre, prospective, observational cohort study, all RD follow-up patients at our centre were invited by SMS text message in April 2020 to participate in the study. Participants completed questionnaires at four time points between April 2020 and December 2021. We collected demographics, clinically extremely vulnerable (CEV) status, short form 12 mental (MCS) and physical health component scores (PCS) for health-related quality of life, vaccination status, COVID-19 infection rates and incidence of long COVID. Results We enrolled 1605 patients (female, 69.0%; CEV, 46.5%); 906 of 1605 (56.4%) completed linked responses to our final questionnaire. MCS improved (+0.6, P  < 0.05), whereas PCS scores deteriorated (−1.4, P  < 0.001) between April 2020 and December 2021. CEV patients had worse mental and physical health scores than non-CEV patients at entry (PCS, 36.7 and 39.3, respectively, P  < 0.001; MCS, 40.9 and 43.0, respectively, P  < 0.001) and at each time point throughout the study; both mental and physical health outcomes were worse in CEV compared with non-CEV patients (P  < 0.001 and P  = 0.004, respectively). At study close, 148 of 906 (16.3%) reported COVID infection, with no difference in infection, vaccination or long COVID rates between CEV and non-CEV patients. Conclusions Mental and physical health in RD patients has changed throughout the pandemic; outcomes for both metrics of health were worse in CEV patients, although there were no differences in infection rates between the groups. These data might assist the understanding and planning of future health-care policy and social restrictions in RD patients. Trial registration ClinicalTrials.gov, www.clinicaltrials.gov , NCT04542031. [ABSTRACT FROM AUTHOR]

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A comprehensive, up-to-date systematic review (SR) of the new-onset rheumatic immune-mediated inflammatory diseases (R-IMIDs) following COVID-19 vaccinations is lacking. Therefore, we investigated the demographics, management, and prognosis of new R-IMIDs in adults following SARS-CoV-2 vaccinations. A systematic literature search of Medline, Embase, Google Scholar, LitCovid, and Cochrane was conducted. We included any English-language study that reported new-onset R-IMID in adults following the post-COVID-19 vaccination. A total of 271 cases were reported from 39 countries between January 2021 and May 2023. The mean age of patients was 56 (range 18–90), and most were females (170, 62.5%). Most (153, 56.5%) received the Pfizer BioNTech COVID-19 vaccine. Nearly 50% of patients developed R-IMID after the second dose of the vaccine. Vasculitis was the most prevalent clinical presentation (86, 31.7%), followed by connective tissue disease (66, 24.3%). The mean duration between the vaccine’s ‘trigger’ dose and R-IMID was 11 days. Most (220, 81.2%) received corticosteroids; however, 42% (115) received DMARDs such as methotrexate, cyclophosphamide, tocilizumab, anakinra, IV immunoglobulins, plasma exchange, or rituximab. Complete remission was achieved in 75 patients (27.7%), and 137 (50.6%) improved following the treatment. Two patients died due to myositis. This SR highlights that SARS-CoV-2 vaccines may trigger R-IMID; however, further epidemiology studies are required.

Inflammasome is a molecular platform that is formed in the cytosolic compartment to mediate host immune responses to infection and cellular damage. Inflammasome can activate caspase-1, leading to the maturation of two inflammatory cytokines interleukin 1β (IL-1β) and IL-18 and initiation of a proinflammatory form of cell death called pyroptosis. Among various inflammasome complexes, the NLRP3 inflammasome is by far the most studied inflammasome. NLRP3 inflammasome is a key factor in regulating host immune defense against infectious microbes and cellular damage. However, the dysregulated NLRP3 inflammasome activation also participates in the pathogenesis of many human disorders. NLRP3 inflammasome plays an important role in the pathogenesis of rheumatic disease such as rheumatoid arthritis (RA), systemic lupus erythematosus (SLE), ankylosing spondylitis (AS), Sjögren's syndrome (SS), dermatomyositis/polymyositis (DM/PM), gout, and systemic sclerosis (SSc). For example, NLRP3 inflammasome has been found highly activated in synovial tissues and peripheral blood mononuclear cells from RA patients. In this paper, we will discuss the role of NLRP3 inflammasome in the pathogenesis of rheumatic disease.

The impact of immunosuppressant therapy in COVID-19 patients with autoimmune rheumatic disease remains unclear based on previous studies. Here, we reviewed the clinical evidence to evaluate COVID-19 patients with rheumatic disease outcomes, which previously used immunosuppressant therapy to control the disease. We used PubMed and Science Direct database to search literature up to April 2021 for publications with confirmed COVID-19 infection with rheumatic disease. The outcomes of this review were the infection rate of COVID-19 and the rate of hospitalization, ICU admission, and mortality. A total of 16 articles were included in this review. The overall rates of COVID-19 infection in patients with autoimmune rheumatic disease did not differ from the general population. Rheumatic disease patients who previously used hydroxychloroquine showed a similar infection risk of COVID-19 with those who did not use hydroxychloroquine. Furthermore, immunosuppressant therapies were associated with poor clinical outcomes, increase risk of hospitalization, ICU admission, and mortality, particularly in patients with comorbidities. The use of bDMARD, such as TNF-? inhibitor, showed a protective effect to reduce the risk of hospitalization and mortality. The administration of immunosuppressant therapy must be closely monitored in rheumatic disease patients due to unfavorable outcomes. More studies are urgently required to map risk factors of clinical outcomes with the specific immunosuppressant therapy and specific rheumatic disease.

Patients with pediatric rheumatic diseases (PRDs) have higher morbidity and mortality associated with infectious diseases. Vaccination is an effective way to prevent infection. This study aimed to understand the vaccination status, vaccination-related attitudes, and adverse reactions in patients with PRDs in one of the largest Pediatric Rheumatic and Immune centers in China. A cross-sectional study using an online questionnaire was conducted among the caregivers of patients with PRDs admitted to the Children’s Hospital of Chongqing Medical University. 189 valid questionnaires were collected. The most two common PRDs in this study were juvenile idiopathic arthritis (29.6%) and systemic lupus erythematosus (19.6%). Univariate analysis and multivariate logistic regression were used to identify potential factors associated with vaccination completion among these patients. Univariate analysis suggested that the age of onset, course of the disease, treatment duration, disease duration

Over the last decades, Inborn Errors of Immunity (IEI) characterized by an immune dysregulatory picture, isolated or combined with infections, have been increasingly identified and referred as Primary Immune Regulatory Disorders (PIRD). PIRD diagnosis may be difficult due to heterogeneity of time onset, sequence of clinical manifestations and laboratory abnormalities. Moreover, the dissection of a PIRD vs. a secondary immunodeficiency (SID) might be a real challenge since the same indications for immunosuppressant treatments might represent per se a PIRD clinical expression. Here we report a female patient with a history of recurrent respiratory and urinary tract infections since early infancy and a diagnosis of Rheumatoid Arthritis in adulthood. After poor response to several biologicals she was treated with Rituximab and sent to immunology referral for a severe hypogammaglobulinemia. Clinical and immunological features matched a diagnosis of common variable immunodeficiency and when IgG replacement therapy and antibiotic prophylaxis were added a good infectious control was obtained. Next generation sequencing analysis has revealed a novel heterozygous VUS in the IKBKB gene (c.1465A > G; p.Ser489Gly). Functional analysis has shown a reduced capacity of B lymphocytes and CD4 positive T cells in inducing IκBα degradation, with negative impact on NF-kB pathway. Due to recurrent infections attributed to a common condition in childhood and to an exclusive autoimmunity-centered approach in adulthood, both diagnosis and suitable treatment strategies have suffered a significant delay. To reduce the diagnostic delay, pediatricians, general practitioners and specialists should be aware of IEI and the challenges to differentiate them from SID. Furthermore, genetic characterization and functional analysis may contribute to a personalized approach, in a perspective of targeted or semi-targeted therapy.

BackgroundCollection of patient-reported outcomes measures (PROs) may augment clinical data and inform health research, improving care, yet approaches to sustaining interest among patient cohorts in research participation are needed. One approach may involve returning aggregate research results (ARRs), which may help patients contextualize personal experiences, prompt conversations with providers or family, and encourage information seeking. This model has been demonstrated for Web-based patient-centered registries. Studies with clinical cohorts may further elucidate the model, its impacts on interest in research participation and planned actions, and potential for participants to experience this as helpful or harmful-gap areas.ObjectiveWe sought to investigate the impacts of returning ARRs comprising summaries of PROs and clinical metrics to parents of children with rheumatic disease, assessing interest in future research participation among parents who viewed ARRs and plans for acting on returned information. Further, we sought to investigate reactions to viewing ARRs and how these reactions impacted planned actions.MethodsClinical and PRO data were obtained about children in a national clinical disease registry, summarized, and processed into annotated infographics, comprising ARRs for children's parents. Parents who viewed ARRs (n=111) were surveyed about the information's perceived value and their reactions. Reaction patterns were summarized using principal components analysis (PCA), and associations among reaction patterns and interest in research participation and planned actions were estimated using multivariate logistic regression.ResultsParental endorsement of the value of ARRs for understanding their child's condition and making care decisions was high (across 10 topics for which ARRs were shared, 42.2%-77.3% of the parents reported information was "very valuable"). Most (58/111, 52.3%) parents reported being more interested in participating in research after viewing ARRs, with the remainder reporting that their interest levels were unchanged. Reactions to viewing ARRs reflected experiencing validation/affirmation and information burden based on PCA. Reactions were not associated with child demographic or clinical characteristics and PROs, except that parents from households with less education reported greater information burden than those from more educated households (P=.007). In adjusted models, parents with higher validation/affirmation scores had increased odds of reporting heightened interest in research participation (adjusted odds ratio [AOR] 1.97, 95% CI 1.18-3.30), while higher information burden scores were associated with decreased odds of planned discussions with their child (AOR 0.59, 95% CI 0.36-0.95) and increased odds of planned discussions with providers (AOR 1.75, 95% CI 1.02-3.00).ConclusionsReturning ARRs may foster a "virtuous cycle" of research engagement, especially where ARRs are experienced favorably and affect plans to share and discuss ARRs in support of a child's chronic disease care and treatment. Reactions to ARRs vary with education level, underscoring the need for attention to equity for this model.

Background: Pneumocystis jirovecii pneumonia (PJP) is a life-threatening disease with increasing prevalence in patients with rheumatic disease. Trimethoprim/sulfamethoxazole (TMP/SMX) is an effective treatment for patients with rheumatic disease hospitalized for PJP. This study aimed to describe the 90-day mortality of patients with rheumatic disease complicated by PJP and investigate whether the administration of TMP/SMX after 7 days from initial symptoms correlates with 90-day mortality. Methods: We enrolled consecutive patients with rheumatic disease complicated with PJP in our center from August 2018 to August 2021. The participants were classified into two groups according to when TMP/SMX was initiated: early (within the first 7 days) and late (after 7 days). The primary outcome was 90-day PJP-related mortality. Multivariate cox regression and Kaplan–Meier survival analyses were conducted to identify the risk factors for mortality and examine differences in survival between early and late use of TMP/SMX. Results: Thirty-seven patients with rheumatic disease (median age 50.1 years, 24.3% male) complicated by PJP were enrolled in our study, and 15 (40.5%) patients died at or before 90 days of follow-up. The most common comorbidity was systemic lupus erythematosus (14, 37.8%), followed by inflammatory myopathy (11, 27.9%). Patients in the early group were less likely to require mechanical ventilation (8/27, 29.6% vs. 9/10, 90.0%, P = 0.002), lower doses glucocorticoids (43.2 mg/d vs. 72.2 mg/d, P = 0.039) and had lower mortality (7/27, 25.9% vs. 8/10, 80.0%, P = 0.006) than those in the late group. In the Kaplan–Meier analysis, the survivor probability of the early group was notably higher than that of the late group (P = 0.007). Multivariate cox regression analysis showed that initiation of TMP/SMX after 7 days from admission (hazard ratio [HR]: 5.9, 95% confidence interval [CI]: 1.1–30.4; P = 0.034) and a higher level of lactate dehydrogenase (LDH; HR: 6.0, 95% CI: 1.1–31.8; P = 0.035) were associated with 90-day mortality in patients with rheumatic disease complicated by PJP. Conclusion: Patients with rheumatic disease complicated by PJP had poor prognoses, with mortality rates as high as 40.5%. TMP/SMX initiation after 7 days from initial symptoms and a higher level of serum LDH were significantly associated with increased 90-day mortality. [ABSTRACT FROM AUTHOR]

Background: Infective endocarditis is a vascular-associated infectious disease that affects the vascular endothelium outer layer of the heart, intravascular as well as a prostheses control valve. Infective endocarditis (IE) is a widespread infectious disease that necessitates the collaboration of an interdisciplinary approach to cure fatal infections. IE is a rapidly evolving disease with a significant death and morbidity rate. The incidence rate of IE in advanced nations ranges from 1.7 to 6.2 for every 100,000 people. Aims and Objectives: This study intends to evaluate the demographics, clinical features, microbiological status, echocardiographic results and outcomes of the various treatment among the patients of Infective Endocarditis who were admitted to the tertiary care hospital in Tamil Nadu. Methods: A retrospective study was conducted on patients with IE was performed by collecting various required data demographic, clinical features, echocardiographic, treatment and outcomes data. The data was evaluated and studied accordingly based on the types of endocarditis (native valve endocarditis or prosthetic valve endocarditis), cultures (positive or negative), etc. Results: The study found that all the PVE cases were community-acquired. The study has shown that 89% of the patients but absent in 11% of them. Before hospital admission, fevers lasted between 30 and 90 days in 31%, over 90 days in 47% of patients, and over 90 days in 11% of patients. 34% of patients reported weight loss and 19% of patients with neurological problems (focal deficits and headache). In 12% of patients, who presented with IE, there was evidence of a distant abscess, past bacteremia, or septic foci, and 4% of patients had undergone a previous dental operation. Diabetes mellitus (14%), chronic renal disease (11%), decompensated chronic liver disease (4%), and exposure to steroids or other cytotoxic drugs (6%), among other co-morbidities, were all significant (p<0.05). NVE shows high culture- positive cases (88.3%) than PVE (11.7%). Dead cases are 13.3% in culture-positive and 15% in culture-negative. Conclusion: The study concluded that rheumatic heart disease was the main risk factor. Hospital-acquired IE is also on the rise and trans-oesophagal echo (TEE) is a crucial diagnostic technique with exceptional sensitivity for finding vegetation. [ABSTRACT FROM AUTHOR]

Background: Systemic sclerosis (SSc) is an incurable autoimmune disease characterized by progressive skin fibrosis and organ failure. Tenosynovitis is a common musculoskeletal manifestation, but tendon rupture has seldom reported in SSc.Case Presentation: We present a rare case of a 49-year-old female with SSc who has suffered from bilateral tendon rupture of the fourth and fifth digits with positive antinuclear antibody (ANA) and anti-centromere B antibody, but negative rheumatoid factor in serum. In the extensor tendons of the patient's hands, inflammation, edema, hypertrophy and tendon interruption were detected with ultrasound and magnetic resonance imaging(MRI). Tendon transfer repair surgery was performed and 10 mg/week methotrexate was then used in this patient. Her hand function was improved well with methotrexate and rehabilitation treatment postoperatively.Conclusions: Early detection of tenosynovitis is necessary to prevent tendon rupture in SSc patients. Ultrasound and Magnetic Resonance Imaging appear to be useful examinations for evaluating tendon pathology for early detection. [ABSTRACT FROM AUTHOR]

Objectives: Multisystem inflammatory syndrome in children (MIS-C) is a rare but severe condition associated with coronavirus disease 2019. 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, 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. [ABSTRACT FROM AUTHOR]

Purpose: The COVID-19 pandemic might add to the stressors experienced by people living with rheumatic diseases. This study aimed to examine rheumatic patients' functional and psychosocial states during the pandemic and assess its impact on their quality of life.Methods: Our time-series study included a patient-centered electronic survey, sampling adult rheumatic patients living in Saudi Arabia at different time points from March to August 2020. Patient-reported outcomes included physical function, anxiety, depression, fatigue, sleep disturbance, ability to participate in social roles, and pain interference domains were measured using the Patient-Reported Outcomes Measurement Information System (PROMIS-29 Profile v2.1).Results: A total of 1278 respondents were enrolled. Results showed significant variation in patients' experiences. Our analyses revealed that the physical well-being of rheumatic patients was significantly impacted, and such effect was persistent over time irrespective of public health measures to control the COVID-19 outbreak.Conclusion: Our findings consistently demonstrated the need for psychological and social consideration to improve rheumatic patients' quality of life. Nevertheless, there is still a lot to be learned about the extent of COVID-19 impact on rheumatic patients and the implications it has on long-term disease outcomes. [ABSTRACT FROM AUTHOR]