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Objectives To assess people's level of recognition of rheumatic disease symptoms and their awareness that the rheumatologist is the main effector when it comes to these disorders. Materials and methods Survey performed in 8 towns in the Buenos Aires Province. Every town had at least one rheumatologist. Three clinical cases were presented: (1) inflammatory low back pain, (2) systemic disease, and (3) chronic polyarthritis. The population was asked whether (a) a physician should be immediately consulted, or (b) they could wait. They were asked whether they would advocate any initial treatment. They were also asked which physician should be consulted. Results Out of 150 surveys, 68% were female, the average age was 51.7 years old. Most people asserted that treatment with anti-inflammatory drugs should come first: 83% in inflammatory low back pain, 70% in systemic disease, and 70% in chronic polyarthritis (p = 0.02). The number of men that suggested waiting was higher (47% vs. 28% of women; p = 0.04). A rheumatologist was recommended by 51% for chronic polyarthritis, 15% for systemic disease, and 8% for inflammatory low back pain (p Conclusions Chronic polyarthritis was the disease people identified best as within the rheumatologist's field of expertise. Men tended to delay consultation more than women. Consultation is less likely when the level of education is lower.

Objectives We aimed to identify, appraise, synthesize, and contextualize rapidly emerging reports on medication taking (‘adherence’) among patients with rheumatic diseases during the COVID‐19 pandemic. Methods We searched MEDLINE, EMBASE, and CINAHL for peer‐reviewed communications, letters, and manuscripts published during the COVID‐19 pandemic evaluating medication taking among individuals with rheumatic diseases. We appraised assessment and reporting of medication adherence according to established definitions of three distinct problems of medication taking (i.e., non‐initiation, poor implementation, and discontinuation) and pooled findings using random effects models. Results We included 31 peer‐reviewed studies in our synthesis from various jurisdictions, of which 25 described medication taking among rheumatology patients and 6 described medication prescribing among rheumatology providers. The pooled prevalence of overall medication non‐adherence was 14.8% (confidence interval [CI]: 12.3%, 17.2%) and that of medication discontinuation (i.e., stopping of prescriptions) and poor implementation (i.e., not taking medication at the dose/frequency prescribed) as 9.5% (CI: 5.1%, 14.0%) and 9.6% (CI: 6.2%, 13.0%), respectively. Non‐initiation (i.e., not starting/not filling new prescriptions) was not addressed. Conclusions Medication taking among individuals with rheumatic diseases during the COVID‐19 pandemic varies globally. Unclear reporting and extensive variation in research methods between studies creates barriers to research replication, comparison, and generalization to specific patient populations. Future research in this area should use consistent and transparent approaches to defining and measuring medication taking problems in order to ensure findings appropriately describe the epidemiology of medication adherence and have the potential to identify modifiable targets for improving patient care.

Introduction/objective To assess the positioning that patients with systemic lupus erythematosus (SLE), rheumatoid arthritis (RA), ankylosing spondylitis (AS) and their proxies give to their diseases. Methods Subjects completed a self-administered questionnaire to rank 11 diseases from “worst” to “least bad”. Then they defined the “worst” disease and ranked 10 diseases from highest to lowest importance from a list including “my rheumatic disease/my relative's disease”. The lists of the included diseases represented the mindshare from a sample of healthy adults. Results There were 570 respondents (104 SLE, 99 RA, 82 AS, and 285 proxies). Rheumatoid arthritis was considered the third-worst disease (recoded ranking first by 41% of patients and 43% proxies, second by 49% and 44%, and third by 10% and 13%). A disease that kills was the preferred definition for the worst disease. “My disease/my relative's disease” was ranked fourth in importance (first by 41% of patients, second by 38%, and third by 21%). Rankings were not associated with age, schooling, disease duration, or setting. Discussion and conclusions Most respondents ranked their own disease considerably lower than other non-rheumatic conditions.

The causes of mortality among rheumatic diseases vary widely between geographic areas and cannot be generalized, however, they are frequently associated with the aggressiveness of the clinical presentation and the secondary effects of the therapy used. The main purpose of this study was to characterize the causes of death in a group of patients with autoimmune rheumatic disease. For this purpose, a retrospective study was carried out over a period of 10 years (2009–2019), deceased patients were included from a referral centre in Guatemala City, the records were reviewed in search of demographic and clinical variables. In total, 185 deceased patients were identified from 898 admissions during that period, 85.9% were women and the main disease reported was systemic lupus erythematosus in 49.7% of the cases. Among the causes of mortality, infections were reported in 53% and those attributed to disease activity in 15% of cases. The main conclusion is that that most of the deaths were of infectious origin.

INTRODUCTION The antinuclear antibody (ANA) test is a screening test for systemic autoimmune rheumatic disease (SARD). We hypothesised that the presence of anti-DFS70 in ANA-positive samples was associated with a false-positive ANA test and negatively associated with SARD. METHODS A retrospective analysis of patient samples received for ANA testing from 1 January 2016 to 30 June 2016 was performed. Patient samples underwent ANA testing via indirect immunofluorescence method and anti-DFS70 testing using enzyme-linked immunosorbent assay. RESULTS Among a total of 645 ANA-positive samples, the majority (41.7%) were positive at a titre of 1:80. The commonest nuclear staining pattern (65.5%) was speckled. Only 9.5% of ANA-positive patients were diagnosed with SARD. Anti-DFS70 was found to be present in 10.0% of ANA-positive patients. The majority (51/59, 86.4%) of patients did not have SARD. Seven patients had positive ANA titre > 1:640, the presence of anti-double stranded DNA and/or anti-Ro60. The presence of anti-DFS70 in ANA-positive patients was not associated with the absence of SARD (Fisher’s exact test, p = 0.245). CONCLUSION The presence of anti-DFS70 was associated with a false-positive ANA test in 8.6% of our patients. Anti-DFS70 was not associated with the absence of SARD.

Pediatric rheumatology subspecialists treat chronic autoimmune diseases with onset in childhood. Prompt diagnosis and ongoing management of these conditions are imperative to prevent damage from ongoing inflammation. Here, we aim to describe the current landscape of pediatric rheumatic disease in lower to middle-income countries (LMICs) and explore current barriers to understanding global disease burden. We then examine innovative strategies to promote a more equitable future for children and young people living with rheumatic diseases worldwide.

Both pediatric and adult rheumatology care teams play a central role in health care transition, the shift from child- and family-centered to adult-oriented health care. Components of transition preparation include readiness assessment, setting self-management goals, and spending time in clinical visits without a parent present. Pediatric providers and families should work together to create a transfer plan, identifying a new adult rheumatology care provider, providing a medical summary before transfer, and anticipating changes in health insurance. For high-risk transfers, direct communication between providers is recommended. Finally, adult rheumatologists need to build rapport with young adults to support future engagement in care.

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.

Muscle echogenicity; Musculoskeletal ultrasound; Rheumatic diseases Ecogenicidad muscular; Ecografía musculoesquelética; Enfermedades reumáticas Ecogenicitat muscular; Ecografia musculoesquelètica; Malalties reumàtiques Objectives: To investigate the inter/intra-reliability of ultrasound (US) muscle echogenicity in patients with rheumatic diseases. Methods: Forty-two rheumatologists and 2 radiologists from 13 countries were asked to assess US muscle echogenicity of quadriceps muscle in 80 static images and 20 clips from 64 patients with different rheumatic diseases and 8 healthy subjects. Two visual scales were evaluated, a visual semi-quantitative scale (0–3) and a continuous quantitative measurement (“VAS echogenicity,” 0–100). The same assessment was repeated to calculate intra-observer reliability. US muscle echogenicity was also calculated by an independent research assistant using a software for the analysis of scientific images (ImageJ). Inter and intra reliabilities were assessed by means of prevalence-adjusted bias-adjusted Kappa (PABAK), intraclass correlation coefficient (ICC) and correlations through Kendall’s Tau and Pearson’s Rho coefficients. Results: The semi-quantitative scale showed a moderate inter-reliability [PABAK = 0.58 (0.57–0.59)] and a substantial intra-reliability [PABAK = 0.71 (0.68–0.73)]. The lowest inter and intra-reliability results were obtained for the intermediate grades (i.e., grade 1 and 2) of the semi-quantitative scale. “VAS echogenicity” showed a high reliability both in the inter-observer [ICC = 0.80 (0.75–0.85)] and intra-observer [ICC = 0.88 (0.88–0.89)] evaluations. A substantial association was found between the participants assessment of the semi-quantitative scale and “VAS echogenicity” [ICC = 0.52 (0.50–0.54)]. The correlation between these two visual scales and ImageJ analysis was high (tau = 0.76 and rho = 0.89, respectively). Conclusion: The results of this large, multicenter study highlighted the overall good inter and intra-reliability of the US assessment of muscle echogenicity in patients with different rheumatic diseases. RH and JH were supported by Ministry of Health, Czech Republic – conceptual development of research organization, Motol University Hospital, Prague, Czech Republic (00064203).

The impact of the COVID-19 pandemic, and implemented restrictions on the frequency of pediatric rheumatic diseases remain unknown, while they have probably prevented common infections in children. We present the effects of the COVID-19 on our pediatric rheumatology practice in a main referral center. We retrospectively reviewed the medical records of patients presenting to pediatric rheumatology department in 4 years before March 2020 and compared it to the pandemic year (March 2020–March 2021). Since there was an overall decrease in patient numbers, we calculated the percentage according to the total number of that year. A total of 32,333 patients were evaluated. The mean annual number of patients decreased by 42% during the COVID-19 pandemic. When follow-up visits (25,156) were excluded, there were 2818 new diagnoses of rheumatic diseases. In the pre-pandemic period, familial Mediterranean fever (FMF) (n = 695, 28.1%) was the most frequent, whereas in the pandemic period multisystem inflammatory syndrome in children (MIS-C) (n = 68, 19.2%) was the most common diagnosis. There were no significant differences in the percentages of juvenile idiopathic arthritis, autoimmune diseases, rare autoinflammatory diseases, and other vasculitides. However, there was a significant decrease in patients diagnosed with FMF, IgA vasculitis (IgAV), acute rheumatic fever (ARF), classic Kawasaki disease (KD), and macrophage activation syndrome (MAS) (all p

Reliable and responsive outcome measures that accurately detect changes in disease state, activity, and damage are crucial to conducting observational and interventional trials that can directly transform care for children with rheumatic disease. A combination of consensus-based and direct measurement approaches has led to the development of several validated, composite outcome measures in juvenile idiopathic arthritis, juvenile dermatomyositis, childhood-onset systemic lupus erythematosus, and pediatric vasculitis. This review outlines clinician-reported, disease-specific outcome measures developed for these conditions.

The Childhood Arthritis & Rheumatology Research Alliance (CARRA) launched in 2000 as a small network of pediatric rheumatologists and investigators dedicated to promoting collaborative research to improve the care and outcomes of childhood-onset rheumatic diseases. Over the past 2 decades, CARRA has grown to become a major driver of advances in evidence-based medicine and career development in pediatric rheumatology. Its research approach has transformed pediatric rheumatology. CARRA is a vibrant organization that will continue to facilitate impactful research in the care of children, adolescents, and young adults with rheumatic disease in the years to come.

Systemic rheumatoid diseases form a large group of entities with variable clinical picture and different involvement and degree of organ impairment. Respiratory tract represents major site of damage, with lung interstitium, pleura, pulmonary vasculature and airways possibly affected. In systemic sclerodermia and rheumatoid arthritis, lung disease is the most significant cause of morbidity and mortality. Breathing difficulties may either present as first symptoms of underlying rheumatoid disease or may appear at any time during the course of the disease. Rheumatologists should routinely screen their patients for possible lung impairment. Similarly, extrapulmonal signs should be assessed by pulmonologists in patients referred for dyspnea. Currently, novel antifibrotic therapy is available not only for patients with idiopathic pulmonary fibrosis (IPF), but also for selected group of patients with non-IPF progressive fenotype associated interstitial lung disease having solid evidence-based background. Interdisciplinary approach in terms of collaboration between pulmonologist and rheumatologist is of key importance as proper identification of possible candidates and early onset of therapy is crucial.

Objectives Immunosuppressed paediatric patients with rheumatic disease (RD) may be at risk for severe or critical disease related to severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) infection. Data remain scarce on coronavirus disease 2019 (COVID-19) outcomes in paediatric RD patients. The aim of this study was to determine the seroprevalence of SARS-CoV-2 IgG and to describe COVID-19 outcomes in immunosuppressed paediatric RD patients. Methods Patients diagnosed with RD before age 18 years and treated with at least one immunosuppressive medication for at least 3 months were enrolled from a tertiary paediatric rheumatology practice in New York and also underwent routine SARS-CoV-2 IgG testing from May to November 2020. A total of 571 patients were screened and 262 were enrolled. SARS-CoV-2 IgG-positive subjects were assessed for symptoms of COVID-19 infection. SARS-CoV-2 PCR results were recorded where available. Demographic, diagnostic, medication and outcome data were collected. Results Of 262 subjects (186 female), 35 (13%) were SARS-CoV-2 IgG positive; 17 (49%) had symptoms suggestive of COVID-19. Of the 17 patients who had SARS-CoV-2 PCR testing, 11 (65%) were PCR positive, 7 of whom were IgG positive. Most SARS-CoV-2 IgG-positive subjects were not PCR tested. The most common symptoms in IgG- and/or PCR-positive subjects were fever, fatigue and cough. No SARS-CoV-2 IgG- or PCR-positive subject developed severe or critical COVID-19 or required hospitalization. Conclusions This is the first report of clinical outcomes of SARS-CoV-2 infection and seroprevalence of SARS-CoV-2 IgG in a large cohort of paediatric RD patients. Most SARS-CoV-2 IgG-positive subjects had no symptoms of COVID-19 infection. Symptomatic subjects all had mild COVID-19 symptoms, suggesting that the risk of severe or critical COVID-19 in immunosuppressed paediatric RD patients is minimal.

Graphical abstract, Highlights • Tricuspid valve stenosis following pacemaker implantation is rare. • Advanced echocardiography can identify the cause. • Invasive hemodynamic evaluation can quantify the effects. • A multidisciplinary approach ensures an optimal treatment strategy.

Introducción: la osteoartritis es una enfermedad articular crónica degenerativa con componente inflamatorio caracterizado por degeneración y pérdida progresiva del cartílago hialino y hueso subcondral y daño del tejido sinovial. Objetivo: describir los aspectos clínicos y epidemiológicos de los pacientes con diagnóstico de osteoartritis en el Hospital Provincial de Zaire en la República de Angola, en el período comprendido entre septiembre de 2021 a septiembre de 2022. Método: se realizó un estudio descriptivo, de cohorte longitudinal, con datos del Departamento de Ortopedia y Traumatología de dicha institución. El universo estuvo constituido por 489 pacientes, la muestra del estudio quedó conformada por n꓿167. Se realizaron pruebas de correlación bivariable con el análisis del coeficiente de correlación de Pearson. Resultados: el grupo etario predominante fue de 61 a 70 años (28,7 %), con predominio del sexo femenino (58,1 %), con un tiempo de evolución mayor a 5 años (35,9 %), la articulación mayormente afectada fue la rodilla con 62 pacientes. Se demostró que existe una correlación positiva considerable entre el tiempo de evolución de la enfermedad y el grado de discapacidad, mediante valor del coeficiente de Pearson (0,407), resultado que muestra significación estadística. Conclusiones: la osteoartritis es una enfermedad degenerativa de etiología variada que se presenta en edades tempranas, con mayor frecuencia en el sexo femenino asociada a la articulación de la rodilla, existiendo correlación entre el tiempo de evolución con el grado de discapacidad que produce.

The effects of biological disease-modifying antirheumatic drugs (bDMARDs) in the clinical course of COVID-19 on children with underlying rheumatologic diseases have not been fully demonstrated. To evaluate the course of COVID-19 infection in patients with rheumatic disease receiving bDMARD treatment. This was a retrospective, multicenter study conducted in pediatric patients infected by SARS-CoV-2 and under bDMARDs therapy. The study population consisted of 113 patients (72 female/41 male). The mean age of the patients was 12.87 ± 4.69 years. The primary diagnosis of the cohort was as follows: 63 juvenile idiopathic arthritis, 35 systemic autoinflammatory diseases, 10 vasculitides, and five cases of connective tissue diseases. The mean duration of the primary disease was 4.62 ± 3.65 years. A total of 19 patients had additional comorbid diseases. Thirty-five patients were treated with canakinumab, 25 with adalimumab, 18 with etanercept, 10 with infliximab, nine with tocilizumab, six with rituximab, four with anakinra, three with tofacitinib, and one with abatacept. The median exposure time of the biological drug was 13.5 months. Seventy-one patients had symptomatic COVID-19, while 42 were asymptomatic. Twenty-four patients required hospitalization. Five patients presented with MIS-C. The hospitalized patients were younger and had a shorter duration of rheumatic disease compared to ambulatory patients, although the difference was not statistically significant. Steroid usage, presence of fever, and dyspnea were more common among the hospitalized patients. A worsening in the course of both COVID-19 and current disease was not noticed under bDMARDs, however, to end with a strong conclusion multicentric international studies are required. © 2021, The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.

Glukokortikoide sind von erheblicher therapeutischer Bedeutung bei der Behandlung entzundlicher Erkrankungen, jedoch insbesondere bei langfristiger Anwendung mit Knochenmineraldichteverlust, Osteoporose und Frakturen assoziiert. Es sollten Empfehlungen zum Management der Glukokortikoid-induzierten Osteoporose (GIOP) bei erwachsenen Patienten unter Langzeit-Glukokortikoid(GC)-Therapie entwickelt werden. Es wurde eine systematische Literatursuche (SLR) durchgefuhrt, um die Evidenz fur Praventions- und Behandlungsoptionen der GIOP zusammenzufassen. Mithilfe zuvor definierter Fragestellungen wurden, basierend auf SLR und dem Evidenzgrad, Empfehlungen in einem strukturierten Gruppenkonsensprozess erarbeitet. Die Empfehlungen umfassen zunachst die Supplementation mit Kalzium und Vitamin D bei Erwachsenen unter Langzeit-GC-Therapie. Wenn eine spezifische osteologische Therapie angezeigt ist, empfehlen wir als Erstlinientherapie Bisphosphonate oder Denosumab. Bei hohem Frakturrisiko empfehlen wir Teriparatid als primare spezifische osteologische Therapie. Bei hochgradiger Niereninsuffizienz soll Denosumab (nach Ausschluss renaler Osteopathie) eingesetzt werden und bei Schwangerschaft keine spezifische osteologische Therapie erfolgen. Fur Patienten, die das Therapieziel nicht erreicht haben, sollte die Umstellung auf eine andere Klasse spezifischer osteologischer Medikamente erfolgen. Wir empfehlen eine Reevaluation nach einer Therapiedauer von 3 bis 5 Jahren oder nach Beendigung der Langzeit-GC-Therapie. Diese Arbeit soll evidenz- und konsensbasierte Empfehlungen fur ein bestmogliches Management der GIOP in Deutschland geben und bei Behandlungsentscheidungen unterstutzen.

Autoimmune disorders are typically categorized into systematic and local diseases that affect a single organ or tissue. Organs and tissues affected by autoimmune disorders include components of the endocrine system, such as thyroid, pancreas, and adrenal glands; blood, such as red blood cells; and the connective tissues, skin, muscles, and joints. Rhupus is a complex musculoskeletal autoimmune disease (AD) in which features of both rheumatoid arthritis (RA) and systemic lupus erythematosus (SLE) are simultaneously present. The cause and trigger of rhupus is still unknown, suggesting the role of genetic, immunological, hormonal, and environmental factors in the initiation and progression of the disease. The role of immune system has been established by the presence of specific autoantibodies as markers for diagnosis of rhupus and the formation of immune complex in serum. The complications of rhupus include the presence of rheumatoid factor (RF) and nodules and the involvement of nervous and renal systems that complicates its diagnosis and prognosis. The rhupus arthritis resembles RA-like pathophysiology and leads to inflammation, deformation, and disability. Due to the heterogeneity and rarity of the disease, the prevalence, pathophysiology, and natural history as well as radiological and immunological profiles of rhupus are still not properly understood.

Published by Elsevier Ltd., Background: Differences in the distribution of individual-level clinical risk factors across regions do not fully explain the observed global disparities in COVID-19 outcomes. We aimed to investigate the associations between environmental and societal factors and country-level variations in mortality attributed to COVID-19 among people with rheumatic disease globally. Methods: In this observational study, we derived individual-level data on adults (aged 18-99 years) with rheumatic disease and a confirmed status of their highest COVID-19 severity level from the COVID-19 Global Rheumatology Alliance (GRA) registry, collected between March 12, 2020, and Aug 27, 2021. Environmental and societal factors were obtained from publicly available sources. The primary endpoint was mortality attributed to COVID-19. We used a multivariable logistic regression to evaluate independent associations between environmental and societal factors and death, after controlling for individual-level risk factors. We used a series of nested mixed-effects models to establish whether environmental and societal factors sufficiently explained country-level variations in death. Findings: 14 044 patients from 23 countries were included in the analyses. 10 178 (72·5%) individuals were female and 3866 (27·5%) were male, with a mean age of 54·4 years (SD 15·6). Air pollution (odds ratio 1·10 per 10 μg/m3 [95% CI 1·01-1·17]; p=0·0105), proportion of the population aged 65 years or older (1·19 per 1% increase [1·10-1·30]; p, MAG is supported by the National Institute of Arthritis and Musculoskeletal and Skin Diseases (grant numbers K01 AR070585 and K24 AR074534 [JY]). KDW is supported by the Department of Veterans Affairs and the Rheumatology Research Foundation Scientist Development award. JAS is supported by the National Institute of Arthritis and Musculoskeletal and Skin Diseases (grant numbers K23 AR069688, R03 AR075886, L30 AR066953, P30 AR070253, and P30 AR072577), the Rheumatology Research Foundation (K Supplement Award and R Bridge Award), the Brigham Research Institute, and the R. Bruce and Joan M. Mickey Research Scholar Fund. NJP is supported by the National Institute of Arthritis and Musculoskeletal and Skin Diseases (T32-AR-007258). AD-G is supported by grants from the Centers for Disease Control and Prevention and the Rheumatology Research Foundation. RH was supported by the Justus-Liebig University Giessen Clinician Scientist Program in Biomedical Research to work on this registry. JY is supported by grants from the National Institutes of Health (K24 AR074534 and P30 AR070155).

Study Aims To synthesize the impact of HIIT on disease activity, physical functioning, and quality of life in people diagnosed with SARDs To identify gaps in the appraised literature and present a need for further research into the potential of HIIT as a management strategy for SARDs.

An observational study based on a national quality register for pregnancy and rheumatic diseases

BACKGROUND Detection of early capillaroscopic alterations in the preclinical phase may prove useful in patients with non-scleroderma connective tissue disease (CTD). We aimed to verify whether certain capillaroscopic alterations, alone or in combination, might be predictive of CTD. METHODS We retrospectively collected data on patients with Raynaud's phenomenon who underwent capillaroscopy conducted by highly expert examiners with a degree in vascular medicine at our institutions. Included subjects were divided in two groups: those developing rheumatic disease during follow-up, and those without subsequent diagnosis of CTD. Notably, we excluded subjects who presented with an evident scleroderma pattern or rheumatic disease during their initial examination. RESULTS We included a total of 76 patients, 60 who developed CTD during follow-up, which spanned in this group 23±7 months, and 16 who did not develop CTD during follow-up, which spanned 23±9 months. The following features were significantly associated with Raynaud's phenomenon: 1) angiotectonic disorder (p

One of the primary tasks for a doctor when meeting a patient for the first time is to gain his affection and trust. It is not always possible during the first visit to make an accurate diagnosis and prescribe pathogenetic therapy, because this often requires a deep additional examination. However, it is imperative to demonstrate knowledge and confidence, provide psychological support and alleviate the suffering of the patient, especially in the case of rheumatic disease accompanied by severe pain. Such a patient should be immediately prescribed adequate analgesic therapy. The main tool for controlling pain in diseases of the joints and spine are non-steroidal anti-inflammatory drugs. Moreover, their prescription should be deliberate and balanced, taking into account the clinical picture and comorbid pathology.The article presents two clinical observations, illustrating the formation of a diagnostic and therapeutic concept at an outpatient appointment.

Aim People with rheumatic diseases (PRD) remain vulnerable in the era of the COVID‐19 pandemic. We formulated recommendations to meet the urgent need for a consensus for vaccination against SARS‐CoV‐2 in PRD. Methods Systematic literature reviews were performed to evaluate: (a) outcomes in PRD with COVID‐19; (b) efficacy, immunogenicity and safety of COVID‐19 vaccination; and (c) published guidelines/recommendations for non‐live, non‐COVID‐19 vaccinations in PRD. Recommendations were formulated based on the evidence and expert opinion according to the Grading of Recommendations Assessment, Development and Evaluation methodology. Results The consensus comprises 2 overarching principles and 7 recommendations. Vaccination against SARS‐CoV‐2 in PRD should be aligned with prevailing national policy and should be individualized through shared decision between the healthcare provider and patient. We strongly recommend that eligible PRD and household contacts be vaccinated against SARS‐CoV‐2. We conditionally recommended that the COVID‐19 vaccine be administered during quiescent disease if possible. Immunomodulatory drugs, other than rituximab, can be continued alongside vaccination. We conditionally recommend that the COVID‐19 vaccine be administered prior to commencing rituximab if possible. For patients on rituximab, the vaccine should be administered a minimum of 6 months after the last dose and/or 4 weeks prior to the next dose of rituximab. Post‐vaccination antibody titers against SARS‐CoV‐2 need not be measured. Any of the approved COVID‐19 vaccines may be used, with no particular preference. Conclusion These recommendations provide guidance for COVID‐19 vaccination in PRD. Most recommendations in this consensus are conditional, reflecting a lack of evidence or low‐level evidence.

Aim To update previous guidance of the Asia Pacific League of Associations for Rheumatology (APLAR) on the management of patients with rheumatic and musculoskeletal diseases (RMD) during the coronavirus disease 2019 (COVID‐19) pandemic. Methods Research questions were formulated focusing on diagnosis and treatment of adult patients with RMD within the context of the pandemic, including the management of RMD in patients who developed COVID‐19. MEDLINE was searched for eligible studies to address the questions, and the APLAR COVID‐19 task force convened 2 meetings through video conferencing to discuss its findings and integrate best available evidence with expert opinion. Consensus statements were finalized using the modified Delphi process. Results Agreement was obtained around key aspects of screening for or diagnosis of COVID‐19; management of patients with RMD without confirmed COVID‐19; and management of patients with RMD with confirmed COVID‐19. The task force achieved consensus on 25 statements covering the potential risk of acquiring COVID‐19 in RMD patients, advice on RMD medication adjustment and continuation, the roles of telemedicine and vaccination, and the impact of the pandemic on quality of life and on treatment adherence. Conclusions Available evidence primarily from descriptive research supported new recommendations for aspects of RMD care not covered in the previous document, particularly with regard to risk factors for complicated COVID‐19 in RMD patients, modifications to RMD treatment regimens in the context of the pandemic, and COVID‐19 vaccination in patients with RMD.

Infections, including opportunistic infections, are a major and frequent cause of morbidity and mortality in patients with systemic autoimmune and rheumatic diseases. Pneumocystis jirovecii pneumonia, classically considered to be typical of HIV patients, transplanted patients or patients treated with oncological chemotherapy, is appearing increasingly frequently in these patients. Therefore, rheumatologists should know its mechanism of production, clinical manifestations, treatment and prophylaxis, all of which are addressed in this review.

Background: Reports of leishmaniasis in immunosuppressed patients after visiting the Mediterranean Basin are becoming increasingly common. Still, awareness of the risk of infection and its clinical manifestations may be insufficient among healthcare professionals in the travellers' home countries. Methods: This observational, longitudinal study included 47 patients from Sweden with rheumatic disease and ongoing immunomodulatory treatment, who visited a rehabilitation centre in southern Spain where leishmaniasis is endemic. Patients were evaluated for clinical signs of leishmaniasis at baseline and after three years. Patients with leishmaniasis were followed for 4-5 years. The treatment outcome was assessed by clinical evaluation and determination of the cell-mediated immunological response to Leishmania by a whole blood cytokine release assay. Results: Seven patients (15%) were diagnosed with leishmaniasis. The median time from exposure to the onset of symptoms was 3 [1-17] months. The median delay between the onset of symptoms and treatment start was 9 [1-12] months. All patients with leishmaniasis responded well to treatment. Only one patient had a relapse, which occurred within the first year. Conclusion: Healthcare professionals need to be aware of the increased risk of leishmaniasis for travellers who are immunosuppressed. Knowledge of the symptoms is crucial for a timely diagnosis and early treatment. Sí

Biological disease-modifying drugs (DMARDs), and more recently targeted synthetic DMARDs, have revolutionized the management of autoimmune rheumatic disease. However, these medications should be used with caution, taking into account individual patient co-morbidities and evaluating the possibility of risks associated with their use. Patients given these therapies require regular blood test monitoring, close follow-up and access to prompt rheumatology advice. Patients should be aware of the possible risks, especially infection. Clinicians must be vigilant when suspecting infections in these patients, as they do not always develop the typical clinical or biochemical picture of sepsis.

Patients with rheumatic diseases are susceptible to infections due to their underlying disease states as well as from immunosuppressive medications, highlighting the importance of vaccination, these same factors also pose challenges to vaccine efficacy, safety, and uptake. This article reviews the impact of immunosuppressive therapies and rheumatic disease on vaccine efficacy in this vulnerable patient population as well as discusses best practices.

Patients with rheumatic disease, including those with systemic lupus erythematous, rheumatoid arthritis, and spondyloarthritis, use total hip and knee arthroplasties at high rates. They represent a particularly vulnerable population in the perioperative setting because of their diseases and the immunosuppressant therapies used to treat them. Careful planning among internists, medical specialists, and the surgical team must therefore occur preoperatively to minimize risks in the postoperative period, particularly infection. Management of immunosuppressant medications, such as conventional synthetic disease-modifying antirheumatic drugs and targeted therapies including biologics, is one avenue by which this infectious risk can be mitigated.

Rheumatic disease is a disease affecting the joints and surrounding structures, consisting of more than 100 types. Rheumatoid Arthritis (RA) is a progressive autoimmune disease with chronic inflammation that attacks the musculoskeletal system but can involve the organs and systems of the body as a whole, characterized by swelling, joint pain and destruction of synovial tissue accompanied by movement disorders followed by premature death. This type of research is analytical, with a cross sectional design, where in this study, the dependent and independent variables will be observed at the same time, this method is expected to be known "Factors Associated with Rheumatic Incidence in Rheumatism Patients in Village VI Ethnic Work Area. Puskesmas Tanah Garam Kota Solok ". Based on the results of research conducted on 40 respondents, it can be concluded that there is a relationship between genetics and the incidence of rheumatism, there is a relationship between age and the incidence of rheumatism, there is a relationship between sex and the incidence of rheumatism, there is a relationship between obesity and the incidence of rheumatism and a relationship between lifestyle and incidence rheumatism in Kelurahan VI Tribe, Tanah Garam Public Health Center, Solok City.

Objectives We sought to gain insight into the prevalence of COVID-19 and the impact stringent social distancing (shielding) has had on a large cohort of rheumatology (RD) follow-up patients from a single large UK centre. Methods We linked COVID-19-related deaths, screening and infection rates to our RD population (1.2.20–1.5.20) and audited active rheumatology follow-up patients through survey data communicated via a linked mobile phone SMS message. We assessed epidemiology, effect of stringent social distancing (shielding) and quality of life (HRQoL) by Short Form 12 (SF12). Results There were 10,387 active follow-up patients, 7911 had linked mobile numbers. 12/10,387 RD patients died from COVID-19 (0.12%); local population 4131/7,415,149 (0.12%). For patients with mobile phones, 1693/7911 (21%) responded and of these, 1605 completed the SF12. Inflammatory arthritis predominated 1174/1693 (69%); 792/1693 (47%) were shielding. Advice on shielding/distancing was followed by 1372/1693(81%). 61/1693 (4%) reported COVID-19 (24/61 shielding); medication distribution was similar in COVID and non-COVID patients. Mental SF12 (MCS) but not physical (PCS) component scores were lower in COVID (60) vs. non-COVID (1545), mean differences: MCS, − 3.3; 95% CI − 5.2 to − 1.4, P

Telerheumatology is the application of telehealth to rheumatic disease. Although generally acceptable to both providers and patients, little is known about the safety of telerheumatology or about when, how, and for whom it is best used. Telerheumatology's impact on the rheumatology workforce as well as access to care and health disparities in rheumatic disease is not known. These outcomes likely will depend on the specific telemedicine modalities employed.

Children with rheumatic disease have rare pulmonary manifestations that may cause significant morbidity and mortality. These children are often clinically asymptomatic until disease has significantly progressed, so they should be screened for pulmonary involvement. There has been recent recognition of a high mortality-related lung disease in systemic-onset juvenile idiopathic arthritis; risk factors include onset of juvenile idiopathic arthritis less than 2 years of age, history of macrophage activation syndrome, presence of trisomy 21, and history of anaphylactic reaction to biologic therapy. Early recognition and treatment of lung disease in children with rheumatic diseases may improve outcomes.

Rheumatic diseases reach across continents with some similarities as well as unique challenges. The intersection between genetic factors, environmental exposures and socioeconomic factors, as well as challenges, with delays in access to subspecialty care and medications, manifest in different ways. By understanding both the challenges and successes in different countries, while also recognizing the significant diversity both within and across continents, unified strategies to improve rheumatic disease outcomes and decrease disparities among the most vulnerable groups can be developed and disseminated.

Summary Sjogren's syndrome was considered for many years a disease of adulthood, characterised by immune infiltration of exocrine glands, leading to dryness (eg, dry mouth and eyes), which is a cardinal symptom. As of the last 20 years, it became apparent that although the disease is very rare in children, its clinical presentation differs from that of adults, posing substantial challenges to the recognition, diagnosis, and classification of patients with childhood-onset Sjogren's syndrome. This Viewpoint explores comparative classification criteria for children (not validated) and adults with Sjogren's syndrome, as well as differences in the clinical presentation of childhood-onset versus adult-onset Sjogren's syndrome, offering ideas about how we can improve the diagnosis of Sjogren's syndrome in children. A review of the role of medical history and clinical assessment, serology, glandular function assessment, and imaging, as well as salivary and lachrymal gland biopsy in the diagnosis of children with Sjogren's syndrome is included. Additionally, we provide suggestions about further research and registry data collection that is required to address the unmet needs of these patients.

Systemic lupus erythematosus (SLE) is an autoimmune rheumatic disease characterized by multiple pathologies in which sustained inflammatory activity leads to progressive tissue destruction and organ damage. One of the main proinflammatory cytokines playing a key role in autoimmune diseases such as rheumatoid arthritis (RA) or SLE, is tumor necrosis factor (TNF) alpha.The introduction of TNF-alpha inhibitors revolutionized the treatment of RA and other conditions including psoriatic arthritis and ankylosing spodylitis. We review here the efficacy and safety of TNF-alpha blockers in SLE focussing on why it has not been more widely used since TNF-alpha was reported to be increased in SLE patients and to correlate with disease activity.We summarize the reported SLE cases that have received TNF-alpha blockers and the main results to date. We reflect on whether there is a case to reconsider the use of TNF-alpha blockade in SLE.

Background/purpose Autoimmune diseases are diseases in which the body produces an abnormal immune response to self-antigens and damages its own tissues. Programmed death-1 (PD-1) and its ligands (PD-Ls) have been discovered to be important negative regulators of the immune system, playing crucial roles in autoimmunity. Methods We analyzed the existing scientific literature dealing with this issue. In this review, the PD-1/PD-L pathway in the genetic susceptibility to and pathogenesis of rheumatic diseases is discussed. The PD-1/PD-L pathway might be helpful for diagnosing, evaluating the disease activity of and treating rheumatic diseases. Results PD-1/PD-L gene polymorphisms are associated with a genetic predisposition to rheumatic disorders, which can provide reference information for diagnosis and disease activity. The conclusion of the crucial role of the PD-1/PD-L pathway in the pathogenesis of rheumatic diseases is consistent, but the details remain controversial. In some animal models, manipulating the PD-1/PD-L pathway could decrease disease severity. PD-1/PD-Ls may enable us to develop new therapeutics for patients with rheumatic diseases in the future. Conclusion The PD-1/PD-L pathway plays crucial roles in rheumatic disease. More work is needed to provide a better mechanistic understanding of the PD-1/PD-L pathway and to facilitate the precise therapeutic manipulation of this pathway.

The article describes the main pathophysiological mechanisms underlying the potential use of antirheumatic therapy in the new COVID-19 in patients with rheumatic diseases. Also, it summarizes current data on the risk and outcome of COVID-19 in patients with systemic autoimmune diseases. To date, there are no large randomized studies on the use of antirheumatic drugs in patients with rheumatic diseases in the setting of COVID-19. Besides, there is no convincing evidence that any disease-modifying antirheumatic drug (conventional synthetic, biological, or targeted synthetic) can prevent the development of a severe COVID-19 course. At the same time, the importance of concomitant pathology (hypertension, obesity, cardiovascular diseases, diabetes mellitus) and risk factors (smoking) in the development of a severe COVID-19 course in patients with rheumatic diseases is shown. The article presents possible options for initiating and continuing treatment with antirheumatic drugs in patients with rheumatic diseases, depending on the stage of the infectious disease process. KEYWORDS: COVID-19, rheumatic diseases, disease-modifying antirheumatic drug, interleukin, tumor necrosis factor, glucocorticosteroids. FOR CITATION: Vakhlevskiy V.V., Tyrenko V.V., Svintsitskaya I.S., Kryukov E.V. Patterns of the rheumatic disease course in the setting of a new coronavirus infection. Russian Medical Inquiry. 2021;5(2):84–88. DOI: 10.32364/2587-6821-2021-5-2-84-88.

Objective We sought to evaluate and improve knowledge of lactation compatibility of medications commonly used to treat rheumatic diseases among rheumatology, obstetric, and newborn providers practicing at an academic safety net hospital. Methods Baseline knowledge of rheumatic disease medication compatibility with lactation among 49 providers was obtained via a multiple‐choice questionnaire. Following initial evaluation, providers were given a rheumatic diseases and lactation information card. The questionnaire was readministered at the time of card distribution and 5 months later. Results At baseline, more rheumatology providers correctly identified a higher number of lactation‐compatible and noncompatible medications than nonrheumatology providers (78% and 65% vs 31% and 46%, respectively; P < 0.0001). After the intervention, rheumatology providers correctly identified lactation‐compatible and noncompatible medications 98% and 100% of the time, compared with 78% and 65% of the time before the intervention (P < 0.0001 and P < 0.0001). This improvement was durable because rheumatology providers correctly identified lactation‐compatible and noncompatible medications 96% and 98% of the time 5 months following the initial intervention (P = 0.0021 and P < 0.0001). Nonrheumatology providers correctly identified lactation‐compatible and noncompatible medications 31% and 46% of the time before the intervention and 95% and 100% of the time after the intervention (P < 0.0001 and P < 0.0001). Conclusion Rheumatology providers had better baseline knowledge than obstetric and newborn providers of the breastfeeding compatibility of medications used to treat rheumatic diseases. However, all providers had knowledge gaps. After a simple educational intervention, the knowledge gap was significantly narrowed in all provider groups. This improvement was durable because repeat testing of the rheumatology provider subset 5 months post intervention continued to show significant improvement.

Background: We investigated how the non-medical switching (NMS) between Etanercept (ETN)/originator and SB4/biosimilar affects treatment efficacy in a rheumatic disease (RD) cohort, evaluating some laboratory parameters as loss of efficacy predictors after NMS. Methods: We enrolled 124 patients with RD (rheumatoid arthritis, ankylosing spondylitis, psoriatic arthritis): 79 switchers from ETN/originator to SB4 and 45 naïve patients receiving SB4 (first biological treatment). At baseline, 6 (T1), and 12 months (T2), clinical and laboratory parameters were evaluated. Results: In naïve patients, TNF-α significantly increased at T1 in responders (NR) and non-responders (NNR). TNF-α was lower in NNR than in NR at T1 and T2. In NR and NNR, drug levels (DL) increased between T1 and T2. However, DLs were lower in NNR than in NR at T1 and T2. TNF-α was higher in switcher responders (SR) than in non-responders (SNR) at T1 and T2. In SNR, DLs were higher at baseline than in SR, but they decreased significantly at T1 and T2. Conclusions: We observed a decrease in DL and TNF-α levels after NMS in SNR. Moreover, in naïve patients, DL and TNF-α levels were higher in NR than in NNR. Monitoring DL and TNF-α levels may represent a future precision medicine approach to predict loss of efficacy after NMS.

Objective: TURKBIO registry, established in 2011, is the first nationwide biological database in Turkey. This study aimed to provide an overview of TURKBIO data collected by June 2018. Methods: The registry included adult patients with rheumatoid arthritis (RA), ankylosing spondylitis (AS), nonradiographic axial spondyloarthritis (nr-AxSpA), and psoriatic arthritis (PsA). Demographic and clinical features, disease activity markers, and other follow-up parameters, current and previous treatments, and adverse events were registered electronically at each visit using open-source software. The registration of patient-reported outcome measures was carried out electronically by the patients using touch screens. Results: TURKBIO registry included a total of 41,145 treatment series with biologicals. There were 2,588 patients with axSpA (2,459 AS and 129 nr-axSpA), 2,036 with RA, and 428 with PsA. The total number of patients, including those with other diagnoses, was 5,718. In the follow-up period, the number of patients and also visits steadily increased by years. The yearly mean number of visits per patient was found to be 2.3. Significant improvements in disease activity and health assessment parameters were observed following the biological treatments. Biologics were often given in combination with a conventional synthetic disease-modifying antirheumatic drug in patients with RA. Infections were the most commonly seen adverse events, followed by allergic reactions. Tuberculosis was observed in 12 patients, malignancy in 18, and treatment-related mortality in 31. Conclusion: TURKBIO provided a valuable real-life experience with the use of biologics in rheumatic diseases in Turkey

Lymphadenopathy is a common manifestation of active rheumatic disease, although other causalities, such as infections and malignancies, always need to be taken into account. For rheumatic diseases,...

Objective. To evaluate sexual function in a cohort of men with rheumatic disease compared to healthy controls. Patients and methods. The study included 60 patients (ankylosing spondylitis, rheumatoid arthritis, systemic sclerosis, gout, psoriatic arthritis) and 60 healthy age-matched controls. Sexual function of patients and controls was evaluated by the Sexual Health Inventory for Men and a detailed questionnaire. Results. The mean age of study group was 45,26 (7.8) years. In 90% of the cases sexual life changed after diagnosis: only 1.67% described their sexual life as being very satisfying, while 21.67% as being satisfying, 68.33% as not satisfying and 8.33% as absent. 40% of the patients had diminished libido, significantly lower compared to the study group (p = 0.043). Most patients presented with mild erectile dysfunction (60%), while most of the systemic sclerosis patients presented with mild to moderate erectile dysfunction. Erectile dysfunction scores were significantly lower compared to controls (0.009). 55% of the patients reported no influence of the treatment on their sexual life, 38% of the patients reported that specific treatment improved their sexual life.There was no statistical difference between SHIM scores of patients using biological therapies and other drugs. Conclusions. This work suggests that men with rheumatic conditions have a worse sexual function in comparison with healthy control. However, specific disease parameters or treatments were not linked to sexual dysfunction.

Amaç: İnflamatuar romatizmal hastalıklar her yaşta görülebilen remisyon ve alevlenmelerle seyreden kronik hastalıklardır. Bu anket çalışması, hastaların tedavilerini kesmelerine etki eden faktörleri araştırmak ve hangi tedavi yöntemlerini seçtiklerini belirlemek amacıyla düzenlenmiştir.Gereç ve Yöntem: Bu çalışma Ocak 2019-Aralık 2019 arasında Romatoloji Bilim Dalında yapıldı. Çalışmaya 18 yaş üzeri hayatının bir döneminde tedavisini kesen 150 hasta alındı. Çalışmaya romatoid artrit, ankilozan spondilit, bağ doku hastalığı tanısı olan hastalar dahil edildi. Bulgular: Kliniğimizde değerlendirilen 150 hastanın 101 tanesi kadın, 49 tanesi erkekti. Hastaların yaş ortalaması 46 olarak bulundu. Hastaların tanılarına göre değerlendirildiğinde romatoid artrit %50, ankilozan spondilit %19,3, bağ doku hastalığı %14, diğerleri %16 bulundu. Hastaların tedaviyi kesme nedeni en sık ilaç ilaç yan etkisi %32,7 olup diğerleri sosyal güvenlik sorunları %23,3, hastalığın iyileştiğini düşünme %16, tedaviye inanmama %12,7, hamilelik %6, görsel medya %3, tamamlayıcı ve alternatif tıb (TAT) %6, diğerleri %2 olarak bulundu. Hastaların eğitim düzeyi, yaşadığı yer, tanı farklılıkları ile medikal tedaviyi kesme ilişkisi bulunmadı. Tedaviyi kesen hastaların %80,6’sı hiçbir ilaç almadığı %19,4’ü TAT yöntemlerine başvurduğu bulundu. En sık kullanılan TAT yöntemi %68 bitkisel yöntemdi.Medikal tedaviyi kesen hastalarda alternatif tedavi seçimi ile eğitim, yaşadığı yer arasında ilişki bulunmadı. Medikal tedaviyi kesen romatoid artrit hastalarında %72, ankilozan spondilit hastalarında %65,5, bağ doku hastalığında %57,1 oranında ilk 6 ay içinde hastalık alevlenmesi gözlendi. Tartışma-Sonuç: İnflamatuar romatizmal hastalarında hayatının bir döneminde tıbbi tedaviyi bırakarak takip dışı kaldığı gözlenmiştir. Bu nedenle hastalar medikal tedavinin sürekli olması gerektiği ve ilaçların hastalık üzerinde etkileri konusunda bilgilendirilmelidir. Medikal tedaviyi kesen hastaların ilk 6 ayda hastalık alevlenmesi nedeniyle medikal tedaviye başladığı gözlenmiştir.

Objective Hashimoto’s thyroiditis is known to cluster with other systemic autoimmune disorders. Rheumatic manifestations, such as a seronegative non-erosive polyarthritis have been described. The aim of this study was to evaluate the characteristics and the prevalence of rheumatic features in thyroiditis patients, and to ascertain whether the association with systemic autoimmune disorders improved the arthritis manifestations. Methods In total, 180 thyroiditis patients were enrolled. Major clinical and demographic characteristics have been recorded. Patients underwent a rheumatological clinical assessment and extra-articular manifestations allowing for a differential diagnosis with systemic autoimmune diseases and spondyloarthropathy. Presence of systemic autoimmune diseases was recorded. Results A total of 8.33% of thyroiditis patients shown a peripheral inflammatory arthritis (P = 0.002). Female gender (P = 0.042) and thyroid peroxidase (TPOAbs) positivity (P = 0.001) were more frequent. In total, 37 patients had systemic autoimmune diseases (P = 0.0003). A significant high prevalence of coeliac disease and Addison disease was found (P = 0.034 and P = 0.049, respectively). In patients with coeliac disease, the articular manifestations were more frequent (21.21%) (P = 0.001) and the risk to develop joint involvement was 2.96. Conclusion Although we found an articular involvement in about one-third of thyroiditis patients, the prevalence of inflammatory arthropathy was only 8.33%. The prevalence of other coexisting autoimmune disorders was 34.26% with a significant prevalence of coeliac disease (7.41%). Thyroiditis patients with coeliac disease have an articular involvement more frequently than those without. In these patients, we have found a high risk of developing arthritis than patients with only thyroiditis, suggesting cumulative autoimmune effects in the developing articular involvement.