Κυριακή 4 Απριλίου 2021

Lung cancer mimicking systemic lupus erythematosus: case-based review

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Abstract

The aim of this study was to analyze the clinical features of lupus-like lung adenocarcinoma, thus improving both the recognition of lupus mimickers and diagnosis accuracy. We collected three cases of lung adenocarcinoma in which the clinical characteristics and laboratory profiles imitated systemic lupus erythematosus (SLE) in our hospital, and also we had a literature review using search engine. There are few reports of lung adenocarcinoma for which the clinical and laboratory profiles meet the criteria for SLE diagnosis. Follow-up and pathological biopsy are beneficial for the differential diagnosis. Few lung adenocarcinoma cases resemble SLE. Gene pleiotropy and immune dysregulation might be contributing factors. Lung adenocarcinoma should be considered in the differential diagnosis of SLE. Follow-up and pathological biopsy should be improved to enable early detection of lung adenocarcinoma-associated lupus-like conditions.

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Increased influenza vaccination rates in patients with autoimmune rheumatic diseases during the Covid-19 pandemic: a cross-sectional study

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Abstract

To assess non-compliance and potential changes in seasonal flu vaccination coverage before and during the Covid-19 pandemic in patients with autoimmune rheumatic diseases (ARDs). Consecutive patients with ARDs followed-up in 2 tertiary hospitals were telephone-interviewed (December 12–30, 2020) regarding seasonal flu vaccination during the 2019/20 and 2020/21 time periods. Self-reported disease flares that occurred after flu vaccination, as well as reasons for non-vaccination were recorded. One thousand fifteen patients were included. The rate of flu vaccination increased from 76% before to 83% during the COVID-19 pandemic (p = 0.0001). The rate of self-reported disease flares was < 1% among vaccinated patients. Reasons for not vaccination in both periods, respectively, included: 'was not recommended by their rheumatologists' (35.0vs.12.2%, p < 0.0001), 'did not feel that they would have any benefit' (36.9 vs. 32.6%), felt unsafe to do so (27.5 vs. 30.2%), or other reasons (18.9 vs. 23.8%). By multivariate analysis, age [OR = 1.03 (95% CI 1.02–1.04)] vs. [1.04 (95% CI 1.02–1.05)] and treatment with biologics [OR = 1.66 (95% CI 1.22–2.24) vs. [1.68 (95% CI 1.19–2.38)] were independent factors associated with vaccination in both periods. These findings, although are temporally encouraging, emphasize the need for continuous campaigns aiming at increasing patients' and physicians' awareness about the bene fits of vaccination.

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Knowledge gap about immune checkpoint inhibitors among rheumatologists and medical students: a survey

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Abstract

Previous studies found that physicians working in developed countries in Europe and in the USA declared insufficient knowledge concerning immune-related adverse events (irAE) following use of immune checkpoint inhibitors (ICI) in cancer treatment. We determined this knowledge gap among rheumatologists and medical students (MS) in Brazil. A web-based structured survey or a direct interview was applied to 1428 board-certified Brazilian rheumatologists and an adapted questionnaire was sent to 840 undergraduate MS attending the last 2 years of Medical Schools in Fortaleza-CE, Brazil, in September 2019. 228 (15.9%) rheumatologists and 145 (17.2%) MS answered the survey; 136 (60%) rheumatologists worked at Institutions with Oncology service. Rheumatologists had 22.6 ± 12.6 years of medical practice, most [116 (50.9%)] worked in private practice and 9 (3.9%) were on training. Fifty-three (23.4%) declared being familiar [40 (17.6%)] or very familiar [13 (5.8% )] with irAE. Almost two-thirds declared having never managed irAE and about a third (38.6%) felt confident in managing such patients. Knowledge among rheumatologists was similar regardless of having more or less than 10 years of practice (P = 0.758). Less than 5% MS declared being familiar with ICI and most have never heard of irAE. There is a large gap concerning knowledge about ICI and irAE among rheumatologists and MS in Brazil. Continuing medical education strategies are needed to improve this knowledge.

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The global prevalence of rheumatoid arthritis: a meta-analysis based on a systematic review

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Abstract

The objective is to determine the global population prevalence of rheumatoid arthritis (RA) based on population-based studies and assess factors that influence RA prevalence estimates. Four electronic databases were searched (ProQuest Central, MEDLINE, Web of Science, and EMBASE) for peer-reviewed English publications that report prevalence estimates of RA from 1980 and 2019. We included case–control studies, cross-sectional studies, and prospective or retrospective cohort studies in our search strategy. A random-effect meta-analysis model was used to produce the pooled prevalence estimates. The potential between-study heterogeneity was identified using sensitivity analysis, sub-group and meta-regression analyses. A total of 67 studies were included in the meta-analysis, containing 742,246 RA patients and 211,592,925 healthy controls in the study period. The global RA prevalence estimate was 0.46% (95% confidence interval [CI] 0.39–0.54; I2 = 99.9%) with a 95% prediction interval (0.06–1.27). The RA point-prevalence was 0.45% (95% CI 0.38–0.53%) between 1986 and 2014, while the pooled period-prevalence was 0.46% (95% CI 0.36% and 0.57%) from 1955 to 2015. The highest RA pooled prevalence (0.69%; 95% CI 0.47–0.95) was derived from linked data source studies. Based on meta-regression, the factors that explain the studies' heterogeneity of RA prevalence, including geographical location, the risk bias assessment of studies and sample size. The global prevalence of RA between 1980 and 2019 was 460 per 100,000 population, with variations due to geographical location and study methodology. Linked data are the preferred method to estimate RA population prevalence as they provide the best case ascertainment.

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A retrospective comparison of respiratory events with JAK inhibitors or rituximab for rheumatoid arthritis in patients with pulmonary disease

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Janus kinase inhibitors (JAKi) are an exciting option for the treatment of rheumatoid arthritis (RA) but little is known about their safety and tolerability in patients with existing respiratory disorders. The objective was to compare pulmonary safety of JAKi versus rituximab in patients with concurrent interstitial lung disease (ILD) or bronchiectasis. We performed a retrospective electronic patient record review of patients with known ILD or bronchiectasis commencing JAKi or rituximab for the treatment of RA. Patients initiating treatment from January 2016 to February 2020 were included. Respiratory events (hospitalization or death from a respiratory cause) were compared using Kaplan–Meier survival analysis. We analysed patients who received JAKi (n = 28) and rituximab (n = 19) for a mean (SD) of 1.1 (0.62) and 2.14 (1) years respectively. Patients were predominantly female (68%), anti-CCP antibody positive (94%) and non-smoking (8 9%) with a median (IQR) percentage predicted FVC at baseline of 100% (82–115%) and percentage predicted TLCO of 62% (54.5–68%). Respiratory events occurred in five patients treated with JAKi (18%; 5 hospitalizations, 2 deaths) and in four patients treated with rituximab (21%; 3 hospitalizations, 1 death). Respiratory event rates did not differ between groups (Cox-regression proportional hazard ratio = 1.38, 95% CI 0.36–5.28; p = 0.64). In this retrospective study, JAKi for the treatment of RA with existing ILD or bronchiectasis did not increase the rate of hospitalization or death due to respiratory causes compared to those treated with rituximab. JAK inhibition may provide a relatively safe option for RA in such patients.

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Plasmacytoid dendritic cells and type I interferons in flares of systemic lupus erythematosus triggered by COVID-19

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Increased risk of mental health disorders in patients with RA during the COVID-19 pandemic: a possible surge and solutions

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Depression is a common co-morbidity among rheumatoid arthritis (RA) patients, which may translate into difficulty performing activities of daily living. COVID-19 is an unprecedented disaster that has disrupted lives worldwide and led to a rise in the incidence of mental health disorders. Given the widespread economic devastation due to COVID-19, many RA patients, already susceptible to mental illness, maybe at an increased risk of inaccessibility to medical care, accentuated stress, and consequent worsening of existent mental health disorders, or the onset of new mental health disorders such as anxiety, post-traumatic stress disorder, or depression. The objective of this review is to assess if there is an increased risk of mental health disorders in patients with RA during the COVID-19 pandemic. Telemedicine has bridged the transition to remote chronic care in the pandemic period, though certain accessibility and technological challenges are to be addressed. De creased access to care amid lockdowns and a proposed triggering of disease activity in patients with autoimmune disorders may potentially herald a massive spike in incidence or flares of patients diagnosed with RA in the coming months. Such a deluge of cases may be potentially devastating to an overburdened healthcare system. Rheumatologists may need to prepare for this eventuality and explore techniques to provide adequate care during these challenging times. The authors found that there is a significant association between the adverse impact on the mental health of RA patients and the COVID-19 pandemic. However, more research is needed to highlight individual risk factors.

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Primary central nervous system lymphoma in neuropsychiatric systemic lupus erythematosus: case-based review

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Primary central nervous system lymphoma (PCNSL) sometimes occurs in immune-compromised hosts or patients with autoimmune diseases. Some cohort studies have previously reported an increased risk of non-Hodgkin's lymphoma in systemic lupus erythematosus (SLE), while some cases of PCNSL in patients with SLE were reported. We present the case of PCNSL which developed in a patient with the active phase of neuropsychiatric SLE (NPSLE). Furthermore, we reviewed published English articles to confirm the characteristics of PCNSL related to SLE. To our knowledge, this is the first report of PCNSL occurring in NPSLE. Histology demonstrated B-cell lymphoma with a positive Epstein–Barr virus-encoded RNA. This patient recovered following surgical resection of the lymphoma, whole brain radiation therapy, intravenous infusion of rituximab (RTX), and administration of belimumab after RTX. Given the series of reviews, our report suggests that the persistence of damage in the central nervous system (CNS) and long-term exposure to immunosuppressants may impact oncogenic immune responses within the CNS, leading to PCNSL development.

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Cross-cultural adaptation and psychometric testing of the Dutch and German versions of the Evaluation of Daily Activity Questionnaire in people with rheumatoid arthritis

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The Evaluation of Daily Activity Questionnaire (EDAQ) is a detailed patient-reported outcome measure of activity ability. The objective of this research was to assess the linguistic and cross-cultural validity and psychometric properties of the EDAQ in rheumatoid arthritis for Dutch and German speakers. The EDAQ was translated into Dutch and German using standard methods. A total of 415 participants (Dutch n = 252; German n = 163) completed two questionnaires about four weeks apart. The first included the EDAQ, Health Assessment Questionnaire (HAQ) and 36-item Short-Form v2 (SF-36v2) and the second, the EDAQ only. We examined construct validity using Rasch analysis for the two components (Self-Care and Mobility) of the Dutch and German EDAQ. Language invariance was also tested from the English version. We examined internal consistency, concurrent and discriminant validity and test–retest reliability in the 14 EDAQ domains. The Self-C are and Mobility components satisfied Rasch model requirements for fit, unidimensionality and invariance by language. Internal consistency for all 14 domains was mostly good to excellent (Cronbach's alpha ≥ 0.80). Concurrent validity was mostly strong: HAQ rs = 0.65–0.87; SF36v2 rs = − 0.61 to − 0.87. Test–retest reliability was excellent [ICC (2,1) = 0.77–0.97]. The EDAQ has good reliability and validity in both languages. The Dutch and German versions of the EDAQ can be used as a measure of daily activity in practice and research in the Netherlands and German- speaking countries.

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Patient perspectives on how to improve education on medication side effects: cross-sectional observational study at a rheumatology clinic in The Netherlands

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Rheumatoid arthritis (RA) patients often report lacking information on medication side effects. The aims of this study were to observe how rheumatology healthcare providers deliver medication information and to determine in which specific domains information is missing. First, 12 single-blinded structured observations were performed during regular RA patient consultations. The observers noted whether and how medication and medication side effects were discussed. Second, 100 RA patients were asked to fill out an adaptation of the Satisfaction with Information about Medicines Scale (SIMS). Medication was discussed during all observed consultations. With new medication, its purpose and mode of action were explained in all cases, but possible side effects in only 33%. Overall, medication side effects were discussed in 58% of consultations. Most information delivery was verbal (92%). Response rate to the questionnaire was 61%. Overall satisfaction with medication ed ucation was mean 7.3 (± 1.9) (NRS 0–10) with a comparable high SIMS total satisfaction sum score of mean 12.3 (± 4.4). At subscale score levels, 89% were satisfied with the amount of information on the action and usage of medication, but only 47% with the information on the potential problems of medication. RA patients express overall high satisfaction with their medication education but there is still an unmet need for information on potential risks and side effects. Using the SIMS questionnaire in daily clinical practice may help focus medication education to the needs of the individual patient.

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Use of conventional synthetic and biologic disease-modifying anti-rheumatic drugs in patients with rheumatic diseases contracting COVID-19: a single-center experience

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To examine whether patients with inflammatory arthritis (IA) treated with conventional synthetic (cs) disease-modifying anti-rheumatic drugs (DMARDs) and/or biologic (b) DMARDs, could be affected from SARS-CoV-2 infection and to explore the COVID-19 disease course and outcome in this population. This is a prospective observational study. During the period February–December 2020, 443 patients with IA who were followed-up in the outpatient arthritis clinic were investigated. All patients were receiving cs and/or bDMARDs. During follow-up, the clinical, laboratory findings, comorbidities and drug side effects were all recorded and the treatment was adjusted or changed according to clinical manifestations and patient's needs. There were 251 patients with rheumatoid arthritis (RA), 101 with psoriatic arthritis (PsA) and 91 with ankylosing spondylitis (AS). We identified 32 patients who contracted COVID-19 (17 RA, 8 PsA, 7 AS). All were in remission and all drugs were discontinued. They presented mild COVID-19 symptoms, expressed mainly with systemic manifestations and sore throat, while six presented olfactory dysfunction and gastrointestinal disturbances, and all of them had a favorable disease course. However, three patients were admitted to the hospital, two of them with respiratory symptoms and pneumonia and were treated appropriately with excellent clinical response and outcome. Patients with IA treated with cs and/or bDMARDs have almost the same disease course with the general population when contract COVID-19.

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Antibody subtypes and titers predict clinical outcomes in ANCA-associated vasculitis

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The objective of this study is to evaluate the association between antineutrophil cytoplasmic autoantibody (ANCA) subtype and ANCA titers on clinical outcomes and disease activity among a cohort of patients from Central Appalachia diagnosed with ANCA-associated vasculitis (AAV) over a 3-decade period. This is a retrospective chart review of all patients diagnosed with AAV. ANCA subtypes (myeloperoxidase (MPO) and proteinase 3 (PR3)) and titers at the time of diagnosis and at the time of relapse or last follow-up were evaluated along with patient outcomes. Outcomes of interest included relapse, development of end-stage renal disease (ESRD) and mortality. Sensitivity analysis and multivariable analysis were performed. Of the 202 patients, 111 patients were MPO-ANCA positive and 91 patients were PR3-ANCA positive. Relapse was more frequent among patients with PR3-ANCA compared to MPO-ANCA (35% vs 12%, p < 0.001). In both ANCA subgroups, the stronge st predictor of relapse was an increase in titers prior to relapse, HR 8.1 (95% CI 1.6–40), p 0.009. Patients who achieved serological remission had a lower risk of ESRD [sub-HR 0.31 (95% CI 0.11–0.89)] and mortality [HR (95% CI) 0.24 (0.07–0.7)]. PR3-ANCA was associated with higher risk of ESRD [sub-HR 3.1 (95% CI 1.1–8.5)]. There was no difference in mortality between patients with MPO-ANCA and PR3-ANCA. Our study supports the use of both ANCA subtypes and titer levels for predicting clinical outcomes in patients receiving treatment for AAV. Monitoring of ANCA antibody titers may be useful since both serological remission and increase in titers provide prognostic information.

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