Rheumatology Practice Research Articles

Monitoring and Treatment of Juvenile Idiopathic Arthritis-associated Uveitis: Brazilian Evidence-based Practice Guidelines

ABSTRACT Purpose To present a national guideline for ophthalmologic care and surveillance of juvenile idiopathic arthritis-associated uveitis (JIA-uveitis). Methods Review article based on medical literature and the experience of an Expert Committee composed of members of the Brazilian Society of Pediatric Ophthalmology/Brazilian Council of Ophthalmology and the Brazilian Society of Pediatrics/Brazilian Society of Rheumatology. Studies with a high level of evidence were selected by searching the PubMed/Medline database. The final document was approved by the experts. Results The main recommendations are that children/adolescents with JIA should undergo screening according to their risk factors. Ophthalmological checkups should also consider ocular inflammation and therapy. Topical glucocorticoids should be the first line of therapy, with systemic glucocorticoids acting as bridge treatments in severe uveitis. Methotrexate should be the first-line systemic therapy and anti-tumor necrosis factor (anti-TNF alpha) the second for uncontrolled uveitis. Conclusions This evidence-based guideline for JIA-uveitis will be useful for both ophthalmology and rheumatology practice.

Central sensitisation in chronic pain conditions: latest discoveries and their potential for precision medicine

Summary Chronic pain is a leading cause of disability globally and associated with enormous health-care costs. The discrepancy between the extent of tissue damage and the magnitude of pain, disability, and associated symptoms represents a diagnostic challenge for rheumatology specialists. Central sensitisation, defined as an amplification of neural signalling within the CNS that elicits pain hypersensitivity, has been investigated as a reason for this discrepancy. Features of central sensitisation have been documented in various pain conditions common in rheumatology practice, including fibromyalgia, osteoarthritis, rheumatoid arthritis, Ehlers-Danlos syndrome, upper extremity tendinopathies, headache, and spinal pain. Within individual pain conditions, there is substantial variation among patients in terms of presence and magnitude of central sensitisation, stressing the importance of individual assessment. Central sensitisation predicts poor treatment outcomes in multiple patient populations. The available evidence supports various pharmacological and non-pharmacological strategies to reduce central sensitisation and to improve patient outcomes in several conditions commonly seen in rheumatology practice. These data open up new treatment perspectives, with the possibility for precision pain medicine treatment according to pain phenotyping as a logical next step. With this view, studies suggest the possibility of matching non-pharmacological approaches, or medications, or both to the central sensitisation pain phenotypes.

The role of antirheumatics in patients with COVID-19.

The COVID-19 pandemic has resulted in more than 2 million deaths globally. Two interconnected stages of disease are generally recognised; an initial viral stage and a subsequent immune response phase with the clinical characteristics of hyperinflammation associated with acute respiratory distress syndrome. Therefore, many immune modulators and immunosuppressive drugs, which are widely used in rheumatological practice, have been proposed as treatments for patients with moderate or severe COVID-19. In this Review, we provide an overview of what is currently known about the efficacy and safety of antirheumatic therapies for the treatment of patients with COVID-19. Dexamethasone has been shown to reduce COVID-19 related mortality, interleukin-6 inhibitors to reduce risk of cardiovascular or respiratory organ support, and baricitinib to reduce time to recovery in hospitalised patients requiring oxygen support. Further studies are needed to identify whether there is any role for glucocorticoids in patients with less severe COVID-19. Although evidence on the use of other antirheumatic drugs has suggested some benefits, results from adequately powered clinical trials are urgently needed. The heterogeneity in dosing and the absence of uniform inclusion criteria and defined stage of disease studied in many clinical trials have affected the conclusions and comparability of trial results. However, after the success of dexamethasone in proving the anti-inflammatory hypothesis, the next 12 months will undoubtedly bring further clarity about the clinical utility and optimal dose and timing of other anti-rheumatic drugs in the management of COVID-19.

Клинический случай развития ретроперитонеального фиброза (болезни Ормонда) в практике ревматолога

Забрюшинный (ретроперитонеальный) фиброз (РПФ) — редкое заболевание, характеризующееся разрастанием воспалительной и фиброзной ткани в забрюшинном пространстве. Обычно эти разрастания локализуются вокруг инфраренальной части брюшной аорты и подвздошных артерий, часто покрывая мочеточники или другие органы брюшной полости. Идиопатические заболевания составляют 70% случаев. РПФ может быть ассоциирован с иммуноглобулином G4 (IgG4), что является причиной двух третей случаев идиопатического РПФ. Вторичный РПФ может развиться вследствие инфекций, злокачественных новообразований, приема лекарственных препаратов, забрюшинного кровотечения или других заболеваний. Описан клинический случай идиопатического РПФ, вероятно, ассоциированного с IgG4, у пациента, прошедшего симптоматическое оперативное лечение в урологической клинике и продолжившего лечение у ревматолога патогенетической терапией (метилпреднизолон и мофетила микофенолат) со значительным улучшением. Для ревматологов данная патология представляет безусловный клинический интерес, поскольку, с одной стороны, в основе данных изменений лежат процессы, аналогичные таковым при системных заболеваниях соединительной ткани, а с другой стороны — это редкая патология, встречаемость РПФ составляет от 0,1 до 1,3 случая на 100000 человек в год. Однако чаще такие пациенты сразу попадают на прием к врачам смежных специальностей (урологам, нефрологам, хирургам, сосудистым хирургам), что подчеркивает важность разбора такого клинического случая.

Telerheumatology: before, during, and after a global pandemic.

In early 2020, the COVID-19 global pandemic shifted most healthcare to remote delivery methods to protect patients, clinicians, and hospital staff. Such remote care delivery methods include the use of telehealth technologies including clinical video telehealth or telephone visits. Prior to this, research on the acceptability, feasibility, and efficacy of telehealth applied to rheumatology, or telerheumatology, has been limited. Telerheumatology visits were found to be noninferior to in-person visits and are often more time and cost effective for patients. Clinicians and patients both noted the lack of a physical exam in telehealth visits and patients missed the opportunity to have lab work done or other diagnostic tests they are afforded with in-person visits. Overall, patients and clinicians had positive attitudes toward the use of telerheumatology and agreed on its usefulness, even beyond the pandemic. Although telerheumatology has the potential to expand the reach of rheumatology practice, some of the most vulnerable patients still lack the most basic resources required for a telehealth visit. As the literature on telerheumatology continues to expand, attention should be paid to health equity, the digital divide, as well as patient preferences in order to foster true shared decision-making over telehealth.

Exploring Pediatric Tele-Rheumatology Practices During COVID-19: A Survey of the PRCOIN Network.

Healthcare providers were rapidly forced to modify the way they practiced medicine during the coronavirus disease 2019 (COVID-19) pandemic. Many providers transitioned from seeing their patients in person to virtually using telemedicine platforms with limited training and experience using this medium. In pediatric rheumatology, this was further complicated as musculoskeletal exams typically require hands-on assessment of patients. The objective of this study was to examine the adoption of telemedicine into pediatric rheumatology practices, to assess its benefits and challenges, and to gather opinions on its continued use. A survey was sent to the lead representatives of each Pediatric Rheumatology Care and Outcomes Improvement Network (PR-COIN) site to collect data about their center's experience with telemedicine during the COVID-19 pandemic. Quantitative data were analyzed using descriptive statistics, and qualitative data were thematically analyzed. Responses were received from the majority [19/21 (90%)] of PR-COIN sites. All respondents reported transitioning from in-person to primarily virtual patient visits during the COVID-19 pandemic. All centers reported seeing both new consultations and follow-up patients over telemedicine. Most centers reported using both audio and video conferencing systems to conduct their telemedicine visits. The majority of respondents [13/19 (68%)] indicated that at least 50% of their site's providers consistently used pediatric Gait Arms Legs and Spine (pGALS) to perform active joint count assessments over telemedicine. Over half of the centers [11/19 (58%)] reported collecting patient-reported outcomes (PROs), but the rate of reliably documenting clinical components varied. A few sites [7/19 (37%)] reported performing research-related activity during telemedicine visits. All centers thought that telemedicine visits were able to meet providers' needs and support their continued use when the pandemic ends. Benefits reported with telemedicine visits included convenience and continuity of care for families. Conversely, challenges included limited ability to perform physical exams and varying access to technology. Pediatric rheumatology providers were able to transition to conducting virtual visits during the COVID-19 pandemic. Healthcare providers recognize how telemedicine can enhance their practice, but challenges need to be overcome in order to ensure equitable, sustainable delivery of quality and patient-centered care.

Is pain associated with premature mortality in patients with psoriatic arthritis? A nested case-control study using the DANBIO Register.

It has been hypothesized that the presence of chronic pain causes excess mortality. Since chronic pain is prevalent among patients with PsA this potential association should be explored. We aimed to investigate whether higher cumulative pain intensity is associated with an excess mortality risk in patients with PsA. A nested case-control study using data from the nationwide DANBIO Register (Danish Database for Biological Therapies in Rheumatology) Register and Danish healthcare registers. Cases were patients who died and corresponding to the date of death, matched on sex, year of birth and calendar period at the time of death with up to five controls. Exposure of interest was mean pain intensity reported during the time followed in routine rheumatology practice. Pain intensity was measured using a visual analogue scale from 0 to 100 and conditional logistic regression was used to calculate odds of mortality per 5 unit increase in pain while adjusting for confounders. The cohort consisted of 8019 patients. A total of 276 cases were identified and matched with 1187 controls. Higher mean pain intensity was associated with increased odds of mortality [odds ratio 1.06 (95% CI 1.02, 1.10)] in the crude model, but there was no association [odds ratio 0.99 (95% CI 0.95, 1.03)] when adjusting for additional confounders. Factors shown to increase the odds of mortality were recent glucocorticoid use, concomitant chronic obstructive pulmonary disease, diabetes mellitus, cancer and cardiovascular disease. These results indicate that experienced pain in itself is not associated with premature mortality in patients with PsA. However, recent glucocorticoid use and concurrent comorbidities were.

The Examination of a TPMT Gene Before Administration of Azathioprine in Rheumatology Practice and Identification of a Novel Variant p.W29R.

Two-hundred patients were assessed for the presence of genetic allelic variants using PCR amplification and direct sequencing. In 19 patients, we detected genetic allelic variants affecting TPMT activity; in 1 case, it was an unpublished heterozygous variant c.85T>C (p.W29R); of those, 15 patients were switched from AZA to a different medication, and 1 patient was prescribed a reduced dose of AZA. Our findings show the importance of testing for variants of the TPMT gene before the administration of AZA in clinical rheumatology practice. Patients with documented episodes of leukopenia or elevated liver biochemical tests while on AZA should undergo TPMT genotype testing and/or TPMT enzyme activity testing.

Hydroxychloroquine in systemic and autoimmune diseases: Where are we now?

Hydroxychloroquine (HCQ), one of the oldest drugs used in rheumatology, came recently into attention as one of the potential therapies tested for the severe acute respiratory syndrome coronavirus-2 disease treatment. Used initially as an antimalarial, then translated to rheumatic diseases, HCQ has been used in a wide range of pathologies, including infectious diseases, immune disorders, diabetes, dyslipidemia, or neoplasia. Regarding systemic diseases, HCQ is the mainstay treatment for systemic lupus erythematosus (SLE), where, according to last European guidelines, it is proposed to all SLE patients unless contraindicated or with side effects. HCQ proved positive impact in SLE on robust outcomes, such as accrual damage, disease activity and survival, but also pleiomorphic effects, including decrease in the need for glucocorticoids, reduction in the risk of neonatal lupus, lower fasting glucose and protection against diabetes, thrombotic risk, dyslipidemia, infections, etc. Moreover, HCQ can be used during pregnancy and breast-feeding. Besides SLE, the role for HCQ in the anti-phospholipid syndrome and Sjögren's disease is still under debate. On the contrary, recent advances showed only limited interest for rheumatoid arthritis, especially due the lack of structural damage prevention. There are still no strong data to sustain the HCQ use in other systemic diseases. In this review, we summarised the utility and efficacy of HCQ in different clinical conditions relevant for rheumatology practice.

Impact of COVID-19 on rheumatology practice in the UK-a pan-regional rheumatology survey.

The COVID-19 pandemic has disrupted healthcare services and rheumatology staff were redeployed to the frontline. The purpose of this survey was to evaluate the impact of the COVID-19 pandemic on the provision of rheumatology services as viewed by rheumatologists in the UK. Survey monkey questionnaire weblink was sent to 804 clinicians including consultant rheumatologists, speciality trainees, nurse specialists, and allied health professionals in 4 regions of the UK to evaluate personal effects of COVID-19 and redeployment, impact on current out-patient clinic activity, immunosuppressive drug use, and future rheumatology care. Response rate was 21%. One-fifth of the responders reported that their rheumatology departments were functioning less than 50% capacity during the pandemic. Two-third of responders felt anxious about the ill-effects of COVID-19 on their health and well-being, and one-third of them were redeployed. During the peak of the pandemic, 75% of clinicians stopped intravenous biologics. Although access to video consultation was available for up to three-fourths of the clinicians, the majority (90%) used this modality in less than 1 in 4 consultations. This survey highlights rheumatologists’ perception in the delivery of future care and anxiety they faced. As demonstrated by this survey, the National Institute for Health and Care Excellence (NICE) guidance did not influence clinician decision making in some aspects of patient care. Underutilization of tele-rheumatology in this survey should be considered whilst planning the restoration of rheumatology services in the post-COVID era.Key points• COVID-19 has generated significant concerns among rheumatology community about their mental well-being.• In over 50% of cases, rheumatologists stopped IV biologic drugs as per patients’ wishes during the first wave of the pandemic.• Tele-rheumatology has been used more widely during the pandemic, but the extent of its use in the post-COVID era is less clear. Evolving evidence will determine its future wider use.Supplementary InformationThe online version contains supplementary material available at 10.1007/s10067-021-05601-1.

Physicians' Biological Drug Preference in Patients With Rheumatoid Arthritis and Spondyloarthritis With a History of Malignancy: Perspectives From the Treasure Database.

Because of concerns about malignancy risks, using biological disease-modifying antirheumatic drugs (bDMARDs) in patients with a history of malignancy remains a challenging issue in rheumatology practice. This study aimed to investigate bDMARD preferences of physicians when treating of rheumatoid arthritis (RA) and spondyloarthritis (SpA) patients with a history of malignancy. The data for this cross-sectional study were gathered from the TReasure database using a date range of December 2017 and January 2020. Biological disease-modifying antirheumatic drug preferences were analyzed for 40 RA patients and 25 SpA patients with a history of malignancy. The most frequently prescribed bDMARD was rituximab, which was given to 28 RA patients (70%). For 25 patients (62.5%), the time between the diagnosis of malignancy and starting on a bDMARD regimen was less than 60 months, with a median interval of 43.5 months. Among SpA patients, the preferred bDMARDs were secukinumab and etanercept, which were each administered to 7 patients (28%). For 13 SpA patients (52%), the time between the diagnosis of malignancy and starting on bDMARDs was less than 60 months, with a median interval of 97 months. The observed bDMARD preferences may be related to the therapeutic effects of rituximab on lymphoproliferative malignancies, the protective effects of secukinumab on tumor progression, and the short half-life of etanercept. Biological disease-modifying antirheumatic drugs should be used in RA and SpA patients with malignancy in case of high inflammatory activity.

Orthopaedic rheumatology strategic recommendations and practice principles in SARS-CoV-2 Era

Orthopaedic rheumatology strategic recommendations and practice principles in SARS-CoV-2 Era - IJOR- Print ISSN No: - 2581-8112 Online ISSN No:- 2581-9151 Article DOI No:- 10.18231/j.ijor.2020.010, IP International Journal of Orthopaedic Rheumatology-IP Int J Orthop Rheumatol

Beyond the guidelines management of juvenile idiopathic arthritis: a case report of a girl with polyarticular disease refractory to multiple treatment options and Leri Weill syndrome

BackgroundThe last two decades brought new treatment options and high quality guidelines into the paediatric rheumatologic practice. Nevertheless, a number of patients still present a diagnostic and therapeutic challenge due to combination of vague symptoms and unresponsiveness to available treatment modalities.Case presentationWe report a case of sixteen years old girl suffering from polyarticular type of juvenile idiopathic arthritis refractory to multiple treatment options. She first presented at the age of 4 with swelling and contractures of both knees. Her symptoms were initially unresponsive to nonsteroidal anti-inflammatory drugs and progressed despite treatment with intraarticular and systemic glucocorticoids and methotrexate. Throughout the years, she received several biologics together with continuous administration of nonsteroidal anti-inflammatory drugs and disease modifying anti-rheumatic drugs as well as intraarticular and systemic glucocorticoids in disease flares. However, none of this options provided a permanent remission, so various other modalities, as well as other possible diagnoses were constantly being considered. Eventually she became dependent on a daily dose of systemic glucocorticoids. In 2018, the treatment with Janus kinase inhibitor tofacitinib was initiated, which led to gradual amelioration of musculoskeletal symptoms, improvement of inflammatory markers and overall well-being, as well as to the weaning of systemic glucocorticoids. As the swelling of the wrists subsided for the first time in many years, Madelung’s deformity was noticed, first clinically, and later radiographically as well. Genetic analysis revealed short-stature homeobox gene deficiency and confirmed the diagnosis of Leri Weill syndrome.ConclusionsThis case report emphasizes the need for reporting refractory, complicated cases from everyday clinical practice in order to build-up the overall knowledge and share experience which is complementary to available guidelines. Individual reports of difficult to treat cases, especially when additional diagnoses are involved, can be helpful for physicians treating patients with common rheumatological diseases such as juvenile idiopathic arthritis.

COVID-19: What Do Rheumatologists Need to Know?

Purpose of ReviewSevere acute respiratory syndrome-related coronavirus 2 (SARS-CoV-2) emerged in December 2019, rapidly reaching global pandemic proportions. Coronavirus disease 2019 (COVID-19) has presented unique challenges to the rheumatology community. It is known that many individuals with rheumatic disease are at increased risk of severe disease from other infections, sparking a similar fear for COVID-19. In addition, medications routinely used in rheumatology practice are being trialled as treatments, with the potential for drug shortages for rheumatology patients.Recent FindingsUnderlying comorbidities and active disease are associated with worse COVID-19 outcomes in patients with rheumatic disease. Tocilizumab and hydroxychloroquine have not proven to be effective treatments in the management of COVID-19. Telehealth has become an essential tool for the rheumatology community to monitor patients during the pandemic.SummaryIn this article, we summarise the available COVID-19 evidence that is of relevance to the rheumatology community. We discuss the risk of contracting COVID-19 in individuals with rheumatic disease, along with presenting features and clinical outcomes. We provide an overview of the treatments for COVID-19 which have significance for rheumatology. We highlight published recommendations which can guide our management of rheumatic disease populations during this pandemic. Finally, we discuss the challenges in delivering effective care virtually and present methods and tools which could be adapted for use.

Clinical utility of 18fluorodeoxyglucose positron emission tomography-computed tomography in rheumatology

Background: Several studies with 18fluorodeoxyglucose positron emission tomography with computed tomography (18FDG PET-CT) have indicated that 18fluorodeoxyglucose uptake in affected tissues reflects the disease activity. In addition, the usage of PET-CT for early detection, extent and monitoring of the treatment response has been reported.Methods: In this retrospective study, all 18FDG PET-CTs requested by rheumatologists were reviewed retrospectively. The clinical findings and scan findings were noted. Considering the final diagnosis made by the clinician as “gold standard”, he sensitivity, specificity and positive were calculated.Results: Out of 48, 18FDG PET-CT requests, two were excluded (39 females, mean age – 39.22 ± 15.349). The indications included establishing diagnosis (n = 31 [67.4%]) and disease activity/extent (n = 15 [32.6%]). It contributed to the diagnosis in 9 (31%), when 18F FDG PETCT is used for establishing the diagnosis. It identified abnormalities in 14/15 when used for disease activity and active disease was identified in 10. Seventeen patients had a final diagnosis of fibromyalgia. Overall, 18FDG PET-CT had 100 sensitivity and NPV. The diagnostic accuracy was 56.52%.Conclusions: The 18FDG PET-CT has high diagnostic sensitivity and poor specificity in rheumatology practice with respect to establishing the diagnosis as well as to detect the extent and activity of disease.

The role of musculoskeletal ultrasonography in rheumatoid arthritis and spondyloarthritis

The use of musculoskeletal ultrasonography in rheumatological practice has increased rapidly over the past years, as a result of both technological developments and a need to diagnose and treat inflammatory lesions early. Musculoskeletal ultrasonography completes the clinical examination in a rapid and accurate manner and allows the very early identification of a wide range of pathological findings involving different anatomical structures in the musculoskeletal system. Physical assessment and conventional radiography, currently the most widely used first-line approaches, showed low accuracy as lesion diagnostic methods. Moreover, the radiographic changes take long time to develop, which leads to much delay in diagnosis.

Pregnancy counseling in rheumatic diseases: Where science meets the steps

Most rheumatic diseases (RDs) have a predilection for women in the reproductive age group. Common drugs used in rheumatology practice have identified risks to the fetus; thus, adequate pregnancy counseling is of utmost importance. Contraception and adequate preparation for the same can enhance the experience of motherhood and decrease intra as well as peripartum complications. The knowledge, as well as practices of contraception in autoimmune diseases, are low and varied in various populations. The challenges faced in different RDs are unique and keen understanding can be fruitful toward better patient care. A multi-disciplinary team effort between the patient, obstetricians, and the rheumatologist is the key to better maternal and fetal outcomes.

Підвищення ефективності викладання проблемних питань ревматології на етапі післядипломної освіти шляхом інтеграції імунологічних знань

Clinical immunology is becoming an increasingly important area, in terms of its widespread use, in the teaching of rheumatology in cycles of thematic improvement. Purpose: development of a new diagnostic and therapeutic strategy for teaching rheumatological aspects based on the use of immunological knowledge. Rheumatologists are well aware of the simultaneous identification of antibodies to nuclear and cytoplasmic antigens, which contributes to the differential diagnosis of autoimmune pathology. The three versions of the quests we compiled had ambiguous answers from specialists who were well acquainted with clinical rheumatological pathology. 90% of physicians gave the correct, complete answer to the proposed first option (according to the WHO recommendations for the determination of molecular markers). The second variant of the task included activated forms of lymphocytes with phenotype CD69+, CD3+ HLA-DR+, CD19+ CD38+, CD8+ CD38+. Only 50% of physicians answered that such a test reflects the functional state of activated T- and B-lymphocytes and is recommended for monitoring the course of the disease. The next version of the quest was created taking into account the direct functions of receptor structures. Almost all physicians consider it unnecessary to assess minor subpopulations in patients with autoimmune diseases. When interpreting the results of the immunogram, a dissociation syndrome is established — a discrepancy between the direction of changes in the interrelated immunological parameters. The value of dissociation syndrome is that the latter may indicate latent immune defects. With the rapid expansion of new classes of drugs, clinical practice has changed dramatically, this is what drew the attention of rheumatologists to the fundamental knowledge of immunology and mechanisms of action of biological drugs. It should be noted that in many European countries, immunology courses have been developed specifically for rheumatology practice. Thus, the introduction of rheumatological aspects based on the use of immunological knowledge, especially at the stage of postgraduate education, which will contribute to professional development and the development of a full-fledged fully developed personality of the doctor.

Patient Experiences With Telemedicine in a National Health Service Rheumatology Outpatient Department During Coronavirus Disease-19.

The coronavirus disease-19 pandemic changed rheumatology practice with remote consultations being increasingly utilized where appropriate. We evaluated patient experiences with telephone consultations and report on patient attitudes toward current health care delivery and perspectives of telemedicine in a UK National Health Service rheumatology outpatient department. We analyzed 297 questionnaires from a postal survey conducted during the summer of 2020 after a telephone follow-up consultation. The mean age of respondents was 67 years and 68% were female. The 161 respondents (54%) reported it was their first telephone consultation and overall, 239 (84%) were satisfied with their health assessment. 60% would be happy to have future routine follow-up telephone consultations. Patients advised to shield shared similar satisfaction to the whole sample. However, with increasing age we identified a higher proportion were dissatisfied with telephone consultations and unlikely to have accessibility to video consultation or preferentially opt for this modality.

Greater awareness of biosimilars and shared decision-making among patients attending rheumatology practices in Colorado, United States: Real-world data

Greater awareness of biosimilars and shared decision-making among patients attending rheumatology practices in Colorado, United States: Real-world data