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Yao syndrome, a rare autoinflammatory disorder linked to mutations in the nucleotide-binding oligomerization domain-containing protein-2 (NOD2) gene, manifests through periodic fever, polyarthritis, dermatitis, gastrointestinal disturbances, and sicca-like symptoms. The therapeutic landscape is limited, primarily encompassing glucocorticoids, interleukin-1 (IL-1), and IL-6 inhibitors. This report details the case of a teenager with periodic fevers, arthritis, livedo reticularis, and NOD2 gene mutations R702W and IVS8+158C consistent with Yao syndrome. The individual demonstrated significant improvement with canakinumab therapy. This case report aims to enhance recognition and understanding of Yao syndrome's clinical spectrum and management options.
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OBJECTIVE: To describe people with gout who were diagnosed with coronavirus disease 2019 (COVID-19) and hospitalized and to characterize their outcomes. METHODS: Data on patients with gout hospitalized for COVID-19 between March 12, 2020, and October 25, 2021, were extracted from the COVID-19 Global Rheumatology Alliance registry. Descriptive statistics were used to describe the demographics, comorbidities, medication exposures, and COVID-19 outcomes including oxygenation or ventilation support and death. RESULTS: One hundred sixty-three patients with gout who developed COVID-19 and were hospitalized were included. The mean age was 63 years, and 85% were male. The majority of the group lived in the Western Pacific Region (35%) and North America (18%). Nearly half (46%) had two or more comorbidities, with hypertension (56%), cardiovascular disease (28%), diabetes mellitus (26%), chronic kidney disease (25%), and obesity (23%) being the most common. Glucocorticoids and colchicine were used pre-COVID-19 in 11% and 12% of the cohort, respectively. Over two thirds (68%) of the cohort required supplemental oxygen or ventilatory support during hospitalization. COVID-19-related death was reported in 16% of the overall cohort, with 73% of deaths documented in people with two or more comorbidities. CONCLUSION: This cohort of people with gout and COVID-19 who were hospitalized had high frequencies of ventilatory support and death. This suggests that patients with gout who were hospitalized for COVID-19 may be at risk of poor outcomes, perhaps related to known risk factors for poor outcomes, such as age and presence of comorbidity.
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Objective: The aim was to assess rheumatology clinicians' perceptions of telemedicine and their experiences before and during the coronavirus disease 2019 (COVID-19) pandemic. Methods: We conducted a cross-sectional online survey and collected responses from rheumatology clinicians worldwide, between November 2020 and February 2021, regarding use and perceptions of telemedicine in rheumatology. We summarized data with descriptive statistics and qualitative analysis for free-text responses. Results: The survey was completed by 349 rheumatology clinicians from 49 countries; 59% were female and about two-thirds were in the 30-50 years age group. Academic affiliations were held by 55% of participants, and 44% were from North America. Before the pandemic, 24% of participants had experience with telemedicine, whereas about three-quarters used telemedicine for the first time during the pandemic. Overall, 56% thought they provided less adequate care with telemedicine. More than half of clinicians felt that telemedicine was adequate for evaluating crystalline arthritis, inflammatory arthritis and lupus flares. Telemedicine was felt to be inadequate for flares of myositis, vasculitis and scleroderma. Technical problems were reported in 29% of telemedicine encounters and were most commonly related to patient-encountered difficulties. Conclusion: Most rheumatology clinicians used telemedicine for the first time during the pandemic. The quality of care provided was thought to be inferior to that provided in person for specific clinical situations. Additional efforts are needed to address barriers to effective telemedicine, such as patient-related technology issues, challenges with building rapport and performing a physical examination, and to define the appropriate scope of clinical scenarios conducive to telemedicine.
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Objective: The aim was to evaluate the impact of the coronavirus disease 2019 (COVID-19) pandemic on the clinical experiences, research opportunities and well-being of rheumatology trainees. Methods: A voluntary, anonymous, Web-based survey was administered in English, Spanish or French from 19 August 2020 to 5 October 2020. Adult and paediatric rheumatology trainees were invited to participate via social media and email. Using multiple-choice questions and Likert scales, the perceptions of trainees regarding the impact of the COVID-19 pandemic on patient care and redeployment, learning and supervision, research and well-being were assessed. Results: There were 302 respondents from 33 countries, with 83% in adult rheumatology training. An increase in non-rheumatology clinical work was reported by 45%, with 68% of these having been redeployed to COVID-19. Overall, trainees reported a negative impact on their learning opportunities during rheumatology training, including outpatient clinics (79%), inpatient consultations (59%), didactic teaching (55%), procedures (53%), teaching opportunities (52%) and ultrasonography (36%). Impacts on research experiences were reported by 46% of respondents, with 39% of these reporting that COVID-19 negatively affected their ability to continue their pre-pandemic research. Burnout and increases in stress were reported by 50% and 68%, respectively. Physical health was negatively impacted by training programme changes in 25% of respondents. Conclusion: The COVID-19 pandemic has had a substantial impact on rheumatology training and trainee well-being. Our study highlights the extent of this impact on research opportunities and clinical care, which are highly relevant to future curriculum planning and the clinical learning environment.
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OBJECTIVE: To evaluate the impact of telemedicine use during the coronavirus disease 2019 (COVID-19) pandemic on rheumatology trainees. METHODS: A voluntary, anonymous, web-based survey was administered in English, Spanish, or French from August 19 to October 5, 2020. Adult and pediatric rheumatology trainees were invited to participate via social media and email. Using multiple-choice questions and Likert scales, the survey assessed prior and current telemedicine use, impact on training, and supervision after COVID-19 prompted rapid telemedicine implementation. RESULTS: Surveys were received from 302 trainees from 33 countries, with 83% in adult rheumatology training programs. Reported telemedicine use increased from 13% before the pandemic to 82% during the pandemic. United States trainees predominantly used video visits, whereas outside the United States telemedicine was predominantly audio only. Most (65%) evaluated new patients using telemedicine. More respondents were comfortable using telemedicine for follow-up patients (69%) than for new patients (25%). Only 39% of respondents reported receiving telemedicine-focused training, including instruction on software, clinical skills, and billing, whereas more than half of United States trainees (59%) had training. Postconsultation verbal discussion was the most frequent form of supervision; 24% reported no supervision. Trainees found that telemedicine negatively impacted supervision (50%) and the quality of clinical teaching received (70%), with only 9% reporting a positive impact. CONCLUSIONS: Despite widespread uptake of telemedicine, a low proportion of trainees received telemedicine training, and many lacked comfort in evaluating patients, particularly new patients. Inadequate supervision and clinical teaching were areas of concern. If telemedicine remains in widespread use, ensuring appropriate trainee supervision and teaching should be prioritized.
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OBJECTIVE: The relative risk of SARS-CoV-2 infection and COVID-19 disease severity among people with rheumatic and musculoskeletal diseases (RMDs) compared to those without RMDs is unclear. This study was undertaken to quantify the risk of SARS-CoV-2 infection in those with RMDs and describe clinical outcomes of COVID-19 in these patients. METHODS: We conducted a systematic literature review using 14 databases from January 1, 2019 to February 13, 2021. We included observational studies and experimental trials in RMD patients that described comparative rates of SARS-CoV-2 infection, hospitalization, oxygen supplementation/intensive care unit (ICU) admission/mechanical ventilation, or death attributed to COVID-19. Methodologic quality was evaluated using the Joanna Briggs Institute critical appraisal tools or the Newcastle-Ottawa scale. Risk ratios (RRs) and odds ratios (ORs) with 95% confidence intervals (95% CIs) were calculated, as applicable for each outcome, using the Mantel-Haenszel formula with random effects models. RESULTS: Of the 5,799 abstracts screened, 100 studies met the criteria for inclusion in the systematic review, and 54 of 100 had a low risk of bias. Among the studies included in the meta-analyses, we identified an increased prevalence of SARS-CoV-2 infection in patients with an RMD (RR 1.53 [95% CI 1.16-2.01]) compared to the general population. The odds of hospitalization, ICU admission, and mechanical ventilation were similar in patients with and those without an RMD, whereas the mortality rate was increased in patients with RMDs (OR 1.74 [95% CI 1.08-2.80]). In a smaller number of studies, the adjusted risk of outcomes related to COVID-19 was assessed, and the results varied; some studies demonstrated an increased risk while other studies showed no difference in risk in patients with an RMD compared to those without an RMD. CONCLUSION: Patients with RMDs have higher rates of SARS-CoV-2 infection and an increased mortality rate.
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COVID-19 , Enfermedades Reumáticas , Hospitalización , Humanos , Enfermedades Musculares , Respiración Artificial , Enfermedades Reumáticas/epidemiología , SARS-CoV-2RESUMEN
BACKGROUND: We describe the early experiences of adults with systemic rheumatic disease who received the COVID-19 vaccine. METHODS: From 2 April to 30 April 2021, we conducted an online, international survey of adults with systemic rheumatic disease who received COVID-19 vaccination. We collected patient-reported data on clinician communication, beliefs and intent about discontinuing disease-modifying antirheumatic drugs (DMARDs) around the time of vaccination, and patient-reported adverse events after vaccination. RESULTS: We analysed 2860 adults with systemic rheumatic diseases who received COVID-19 vaccination (mean age 55.3 years, 86.7% female, 86.3% white). Types of COVID-19 vaccines were Pfizer-BioNTech (53.2%), Oxford/AstraZeneca (22.6%), Moderna (21.3%), Janssen/Johnson & Johnson (1.7%) and others (1.2%). The most common rheumatic disease was rheumatoid arthritis (42.3%), and 81.2% of respondents were on a DMARD. The majority (81.9%) reported communicating with clinicians about vaccination. Most (66.9%) were willing to temporarily discontinue DMARDs to improve vaccine efficacy, although many (44.3%) were concerned about rheumatic disease flares. After vaccination, the most reported patient-reported adverse events were fatigue/somnolence (33.4%), headache (27.7%), muscle/joint pains (22.8%) and fever/chills (19.9%). Rheumatic disease flares that required medication changes occurred in 4.6%. CONCLUSION: Among adults with systemic rheumatic disease who received COVID-19 vaccination, patient-reported adverse events were typical of those reported in the general population. Most patients were willing to temporarily discontinue DMARDs to improve vaccine efficacy. The relatively low frequency of rheumatic disease flare requiring medications was reassuring.
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COVID-19 , Enfermedades Reumáticas , Reumatología , Adulto , Vacunas contra la COVID-19 , Femenino , Humanos , Masculino , Persona de Mediana Edad , Enfermedades Reumáticas/tratamiento farmacológico , SARS-CoV-2 , Encuestas y Cuestionarios , VacunaciónRESUMEN
OBJECTIVE: Antirheumatic disease therapies have been used to treat coronavirus disease 2019 (COVID-19) and its complications. We conducted a systematic review and meta-analysis to describe the current evidence. METHODS: A search of published and preprint databases in all languages was performed. Included studies described ≥1 relevant clinical outcome for ≥5 patients who were infected with severe acute respiratory syndrome coronavirus 2 and were treated with antirheumatic disease therapy between January 1, 2019 and May 29, 2020. Pairs of reviewers screened articles, extracted data, and assessed risk of bias. A meta-analysis of effect sizes using random-effects models was performed when possible. RESULTS: The search identified 3,935 articles, of which 45 were included (4 randomized controlled trials, 29 cohort studies, and 12 case series). All studies evaluated hospitalized patients, and 29 of the 45 studies had been published in a peer-reviewed journal. In a meta-analysis of 3 cohort studies with a low risk of bias, hydroxychloroquine use was not significantly associated with mortality (pooled hazard ratio [HR] 1.41 [95% confidence interval (95% CI) 0.83, 2.42]). In a meta-analysis of 2 cohort studies with some concerns/higher risk of bias, anakinra use was associated with lower mortality (pooled HR 0.25 [95% CI 0.12, 0.52]). Evidence was inconclusive with regard to other antirheumatic disease therapies, and the majority of other studies had a high risk of bias. CONCLUSION: In this systematic review and meta-analysis, hydroxychloroquine use was not associated with benefit or harm regarding COVID-19 mortality. The evidence supporting the effect of other antirheumatic disease therapies in COVID-19 is currently inconclusive.
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Antirreumáticos/uso terapéutico , Tratamiento Farmacológico de COVID-19 , Anticuerpos Monoclonales/uso terapéutico , Anticuerpos Monoclonales Humanizados/uso terapéutico , Azetidinas/uso terapéutico , Sesgo , COVID-19/mortalidad , COVID-19/fisiopatología , Cloroquina/uso terapéutico , Progresión de la Enfermedad , Glucocorticoides/uso terapéutico , Humanos , Hidroxicloroquina/uso terapéutico , Inmunoglobulinas Intravenosas/uso terapéutico , Factores Inmunológicos/uso terapéutico , Proteína Antagonista del Receptor de Interleucina 1/uso terapéutico , Inhibidores de las Cinasas Janus/uso terapéutico , Modelos de Riesgos Proporcionales , Purinas/uso terapéutico , Pirazoles/uso terapéutico , SARS-CoV-2 , Sulfonamidas/uso terapéuticoRESUMEN
The novel coronavirus disease (COVID-19) pandemic has significantly impacted the field of rheumatology, in both the delivery of clinical care and didactic education for our trainees. These changes have generated significant strain for program directors and clinical educators who have had to leverage technology and develop new systems to ensure continued trainee education and assessment. We aim to outline the impacts on formal education programs presented by these unprecedented disruptions, describe the development and deployment of online teaching, reflect on the challenges and opportunities for technology-enabled learning and use of social media for education, and give some international perspectives on impacts on postgraduate rheumatology training outside the USA. With the rapid dissolution of barriers in place during the pre-COVID-19 era, we have the opportunity to assess the efficacy of new methods of care and further integrate technology into teaching and assessment. We propose that a hybrid in-person and technology-enabled learning approach, so-called blended learning, is likely to remain the most desirable future model for supporting trainee learning.
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Infecciones por Coronavirus/epidemiología , Educación a Distancia/métodos , Educación de Postgrado en Medicina/métodos , Neumonía Viral/epidemiología , Reumatología/educación , COVID-19 , Curriculum , Humanos , Difusión de la Información , Pandemias , Medios de Comunicación SocialesRESUMEN
INTRODUCTION: COVID-19 is an acute respiratory viral infection that threatens people worldwide, including people with rheumatic disease, although it remains unclear to what extent various antirheumatic disease therapies increase susceptibility to complications of viral respiratory infections. OBJECTIVE: The present study undertakes a scoping review of available evidence regarding the frequency and severity of acute respiratory viral adverse events related to antirheumatic disease therapies. METHODS: Online databases were used to identify, since database inception, studies reporting primary data on acute respiratory viral infections in patients utilizing antirheumatic disease therapies. Independent reviewer pairs charted data from eligible studies using a standardized data abstraction tool. RESULTS: A total of 180 studies were eligible for qualitative analysis. While acknowledging that the extant literature has a lack of specificity in reporting of acute viral infections or complications thereof, the data suggest that use of glucocorticoids, JAK inhibitors (especially high-dose), TNF inhibitors, and anti-IL-17 agents may be associated with an increased frequency of respiratory viral events. Available data suggest no increased frequency or risk of respiratory viral events with NSAIDs, hydroxychloroquine, sulfasalazine, methotrexate, azathioprine, mycophenolate mofetil, cyclophosphamide, or apremilast. One large cohort study demonstrated an association with leflunomide use and increased risk of acute viral respiratory events compared to non-use. CONCLUSION: This scoping review identified that some medication classes may confer increased risk of acute respiratory viral infections. However, definitive data are lacking and future studies should address this knowledge gap.
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Antirreumáticos/farmacología , Infecciones por Coronavirus , Pandemias , Neumonía Viral , Enfermedades Reumáticas , Betacoronavirus , COVID-19 , Comorbilidad , Infecciones por Coronavirus/diagnóstico , Infecciones por Coronavirus/epidemiología , Humanos , Huésped Inmunocomprometido , Neumonía Viral/diagnóstico , Neumonía Viral/epidemiología , Enfermedades Reumáticas/tratamiento farmacológico , Enfermedades Reumáticas/epidemiología , Medición de Riesgo , SARS-CoV-2 , Índice de Severidad de la EnfermedadRESUMEN
The Institute of Medicine states that most diagnostic errors are caused by flaws in clinician diagnostic thinking. Accurately inferring the correct diagnosis from the patient history is the best way to improve diagnostic accuracy and efficiency. Such an improvement is contingent upon training early phase medical learners how to organize data from a patient history to arrive at the most likely diagnosis of the patient's chief health concern (CC). We describe how organizing the traditional history of present illness into what our trainees have come to call the "All-Inclusive History of Present Illness" (AIHPI) by applying the Bayesian statistical concepts of chronologically sequencing, as suggested by Skeff, both relevant historical risks and known medical events generate a series of pre-event probabilities of the most likely disease causing a patient's CC. Our trainees have enthusiastically recognized that the AIHPI organization process helps them improve both their ability to deliver well-organized, succinct verbal case presentations and the efficiency of generating and communicating what they think is the most likely disease causing a patient's CC.
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The United States is facing a rheumatology provider shortage over the next decade, which will negatively affect care for patients with rheumatic disease across the nation if this deficit is not thoughtfully addressed. The increasing numbers of retiring rheumatology specialists, women entering the workforce, and rheumatology graduates seeking part-time employment were identified as the most significant factors driving the projected decline in supply of providers. The major factors driving the projected increase in demand include an aging and growing population and improved treatment options, both of which increase disease prevalence and the challenge of managing chronic rheumatologic diseases.
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Fuerza Laboral en Salud/tendencias , Reumatólogos/provisión & distribución , Reumatología/tendencias , Accesibilidad a los Servicios de Salud , Necesidades y Demandas de Servicios de Salud , Humanos , Admisión y Programación de Personal , Médicos Mujeres , Jubilación , Enfermedades Reumáticas/epidemiología , Enfermedades Reumáticas/terapiaRESUMEN
BACKGROUND: The Centers for Medicare and Medicaid Services Innovation Center introduced the Bundled Payments for Care Improvement (BPCI) initiative in 2011 as 1 strategy to encourage healthcare organizations and clinicians to improve healthcare delivery for patients, both when they are in the hospital and after they are discharged. Mercy Health Saint Mary's, a large urban academic medical center, engaged in BPCI primarily with a group of medical diagnosis-related groups (DRGs). OBJECTIVES: In this article, we describe our experience creating a system of response for the diverse people and diagnoses that fall into the medical DRG bundles and specifically identify organizational factors for enabling successful implementation of bundled payments. RESULTS: Our experience suggests that interprofessional collaboration enabled program success. CONCLUSIONS: Although still in its early phases, observations from our program's strategies and tactics may provide potential insights for organizations considering engagement in the BPCI initiative.
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Ahorro de Costo/economía , Atención a la Salud/economía , Medicaid/economía , Medicare/economía , Paquetes de Atención al Paciente/economía , Mejoramiento de la Calidad/economía , Centros Médicos Académicos/economía , Grupos Diagnósticos Relacionados , Hospitales Urbanos/economía , Humanos , Estados UnidosRESUMEN
INTRODUCTION: High-need, high-cost (HNHC) patients can over-use acute care services, a pattern of behavior associated with many poor outcomes that disproportionately contributes to increased U.S. healthcare cost. Our objective was to reduce healthcare cost and improve outcomes by optimizing the system of care. We targeted HNHC patients and identified root causes of frequent healthcare utilization. We developed a cross-continuum intervention process and a succinct tool called a Complex Care Map (CCM)© that addresses fragmentation in the system and links providers to a comprehensive individualized analysis of the patient story and causes for frequent access to health services. METHODS: Using a pre-/post-test design in which each subject served as his/her own historical control, this quality improvement project focused on determining if the interdisciplinary intervention called CCM© had an impact on healthcare utilization and costs for HNHC patients. We conducted the analysis between November 2012 and December 2015 at Mercy Health Saint Mary's, a Midwestern urban hospital with greater than 80,000 annual emergency department (ED) visits. All referred patients with three or more hospital visits (ED or inpatient [IP]) in the 12 months prior to initiation of a CCM© (n=339) were included in the study. Individualized CCMs© were created and made available in the electronic medical record (EMR) to all healthcare providers. We compared utilization, cost, social, and healthcare access variables from the EMR and cost-accounting system for 12 months before and after CCMs© implementation. We used both descriptive and limited inferential statistics. RESULTS: ED mean visits decreased 43% (p<0.001), inpatient mean admissions decreased 44% (p<0.001), outpatient mean visits decreased 17% (p<0.001), computed tomography mean scans decreased 62% (p<0.001), and OBS/IP length of stay mean days decreased 41% (p<0.001). Gross charges decreased 45% (p<0.001), direct expenses decreased 47% (p<0.001), contribution margin improved by 11% (p=0.002), and operating margin improved by 73% (p<0.001). Patients with housing increased 14% (p<0.001), those with primary care increased 15% (p<0.001), and those with insurance increased 16% (p<0.001). CONCLUSION: Individualized CCMs© for a select group of patients are associated with decreased healthcare system overutilization and cost of care.