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1.
Trials ; 25(1): 683, 2024 Oct 15.
Article in English | MEDLINE | ID: mdl-39407290

ABSTRACT

BACKGROUND: Management of rheumatoid arthritis (RA) relies on symptoms reported by patients during infrequent outpatient clinic visits. These reports are often incomplete and inaccurate due to poor recall, leading to suboptimal treatment decisions and outcomes. Asking people to track symptoms in-between visits and integrating the data into clinical pathways may improve this. However, knowledge on how to implement this into practice and its impact on services and outcomes remains scarce in RA. Therefore, we evaluate the comparative effectiveness and cost-effectiveness of integrated symptom tracking in people with RA over and above usual care, while generating insights on factors for successful implementation. METHODS: In this superiority stepped wedge cluster-randomized controlled trial with continuous recruitment short exposure design, 16 rheumatology outpatient departments (clusters) recruit a total of 732 people with active RA. They initially offer clinic visits according to standard of care before switching in pairs to visits with integrated symptom tracking. Clusters switch in randomized order every 3 weeks. Integrated symptom tracking consists of (1) a mobile app for patients to track their symptoms daily and other RA aspects weekly/monthly, and (2) an interactive dashboard visualizing the app data, which healthcare professionals access from their electronic health record system. Clinic visits happen according to usual practice, with tracked symptom data only reviewed during visits. Our primary outcome is a difference in marginal mean disease activity score at 12 ± 3 months between standard of care and integrated symptom tracking, after accounting for baseline values, cluster, and other covariates. Secondary outcomes include patient-reported disease activity, quality of life and quality-adjusted life-years, medication/resource use, consultation and decision-making experience, self-management, and illness perception. We also conduct interviews and observations as part of a parallel process evaluation to gather information on implementation. DISCUSSION: Our trial will generate high-quality evidence of comparative and cost-effectiveness of integrated symptom tracking compared to standard of care in people with RA, with our process evaluation delivering knowledge on successful implementation. This optimizes the chances of integrated symptom tracking being adopted more widely if we find it is (cost-) effective. TRIAL REGISTRATION: Registered 4-Jun-2024 on https://www.isrctn.com/ , ISRCTN51539448. TRIAL OPEN SCIENCE FRAMEWORK REPOSITORY: https://osf.io/sj9ha/ .


Subject(s)
Arthritis, Rheumatoid , Cost-Benefit Analysis , Mobile Applications , Humans , Arthritis, Rheumatoid/diagnosis , Arthritis, Rheumatoid/therapy , Telemedicine , Randomized Controlled Trials as Topic , Time Factors , Multicenter Studies as Topic , Quality of Life , Treatment Outcome , Symptom Assessment/methods , Equivalence Trials as Topic , Comparative Effectiveness Research
2.
JCI Insight ; 2024 Oct 17.
Article in English | MEDLINE | ID: mdl-39418106

ABSTRACT

Rheumatoid Arthritis (RA) is an immune-mediated, chronic inflammatory condition. With modern therapeutics and evidence-based management strategies, achieving sustained remission is increasingly common. To prevent complications associated with prolonged use of immunosuppressants, drug tapering or withdrawal is recommended. However, due to the lack of tools that define immunological remission, disease flares are frequent, highlighting the need for a more precision medicine-based approach. Utilising high dimensional phenotyping platforms, we set out to define peripheral blood immunological signatures of sustained remission in RA. We identified that CD8+CD57+KIR2DL1+ NK cells are associated with sustained remission. Functional studies uncovered an NK cell subset characterized by normal degranulation responses and reduced pro-inflammatory cytokine expression, which was elevated in sustained remission. Furthermore, flow cytometric analysis of NK cells from synovial fluid combined with interrogation of a publicly available single cell RNA-seq dataset of synovial tissue from active RA identified a deficiency of the phenotypic characteristics associated with this NK cell remission signature. In summary, we have uncovered a novel RA remission signature associated with compositional changes in NK cell phenotype and function that has implications for understanding the impact of sustained remission on host immunity and distinct features which may define operational tolerance in RA.

3.
JCI Insight ; 2024 Oct 17.
Article in English | MEDLINE | ID: mdl-39418109

ABSTRACT

Systemic sclerosis (SSc) is characterized by immune system failure, vascular insult, autoimmunity, and tissue fibrosis. Transforming growth factor-beta (TGF-ß) is a crucial mediator of persistent myofibroblast activation and aberrant extracellular matrix production in SSc. The factors responsible for this are unknown. By amplifying pattern recognition receptor signaling, Triggering Receptor Expressed on Myeloid Cells 1 (TREM-1) is implicated in multiple inflammatory conditions. In this study, we used novel ligand-independent TREM-1 inhibitors in order to investigate the pathogenic role of TREM-1 in SSc, using preclinical models of fibrosis, and explanted SSc skin fibroblasts. Selective pharmacological TREM-1 blockade prevented and reversed skin fibrosis induced by bleomycin in mice and mitigated constitutive collagen synthesis and myofibroblast features in SSc fibroblasts in vitro. Our results implicate aberrantly activated TREM-1 signaling in SSc pathogenesis, identify a unique approach to TREM-1 blockade, and suggest a potential therapeutic benefit for TREM-1 inhibition.

4.
Pediatr Dermatol ; 2024 Oct 18.
Article in English | MEDLINE | ID: mdl-39420750

ABSTRACT

BACKGROUND/OBJECTIVES: Integrated care models, like combined rheumatology/dermatology clinics (RDCs), facilitate efficient coordination between specialists and provide comprehensive care. Given the limited literature on pediatric RDC logistics, outcomes, benefits and, challenges, we comprehensively characterized our patient cohort at the UCSF Benioff Children's Hospital RDC and surveyed pediatric dermatologists participating in RDCs. METHODS: We retrospectively reviewed 71 patients new to the UCSF Pediatric RDC between September 2017 and September 2023. A survey was distributed in 2024 to 17 dermatologists in North America, each representing a unique pediatric RDC. RESULTS: 69% of patients (49/71) were female. Seventeen (24%) presented without a known diagnosis; the first RDC visit established a diagnosis for 7 of them (41%). Of patients with a previously established diagnosis, initial RDC evaluation confirmed it in 52 (96%) and revised it for 2 (4%). The most encountered diagnoses were linear morphea (33%), lupus (23%), and psoriasis (13%). New systemic therapy was prescribed for 23% of patients, and additional work-up was recommended via skin biopsy (8%) and imaging (28%). Survey results revealed all pediatric RDCs include trainees, but only 59% (10/17) receive administrative support. All agreed that RDCs are valuable for patient care and most (15/17, 88%) felt that the RDC was a valuable use of their time. CONCLUSIONS: Pediatric RDCs are valuable for consensus diagnosis, streamlined evaluation, and management of complex patients. Though clinical and administrative support for RDCs is generally poor, RDCs are valuable to patients, a good use of time for clinicians, and offer educational opportunities for team members.

5.
Rheumatol Adv Pract ; 8(4): rkae091, 2024.
Article in English | MEDLINE | ID: mdl-39411286

ABSTRACT

Patient Initiated Follow-Up (PIFU) is gaining momentum in the NHS, aiming to optimize outpatient care amidst rising service demands. PIFU is valuable in rheumatology, where the increasing demand for ongoing management exacerbates the patient backlog. Importantly, PIFU has demonstrated comparable safety and outcomes to traditional care in numerous studies. PIFU empowers patients, drives personalized care, increases efficiency, and has the potential to reduce waiting lists by allowing services to focus on new and acute cases. Effective PIFU implementation includes careful selection of patients, educating patients and healthcare staff, well defined operational guidelines, and robust remote monitoring. Digital solutions can enhance PIFU through patient education, active remote monitoring and streamlined escalation. Electronic Patient Reported Outcome Measures (ePROMs) provide a suitable and safe metric to monitor patients remotely. Given the potential benefits, outpatient departments should consider investing in PIFU as a solution to current healthcare delivery challenges and as a means for future proofing clinical systems against increasing service demands.

6.
Rheumatol Int ; 2024 Oct 16.
Article in English | MEDLINE | ID: mdl-39412574

ABSTRACT

Virtual patients (VPs) are increasingly used in medical education to train clinical reasoning (CR) skills. However, optimal VP design for enhancing interactivity and authenticity remains unclear. Novel interactive modalities, such as large language model (LLM)-enhanced social robotic VPs might increase interactivity and authenticity in CR skill practice. To evaluate medical students' perceptions of CR training using an LLM-enhanced social robotic VP platform compared with a conventional computer-based VP platform. A qualitative study involved 23 third-year medical students from Karolinska Institutet, who completed VP cases on an LLM-enhanced social robotic platform and a computer-based semi-linear platform. In-depth interviews assessed students' self-perceived acquirement of CR skills using the two platforms. Thematic analysis was employed to identify themes and sub-themes. Three main themes were identified: authenticity, VP application, and strengths and limitations. Students found the social robotic platform more authentic and engaging. It enabled highly interactive communication and expressed emotions, collectively offering a realistic experience. It facilitated active learning, hypothesis generation, and adaptive thinking. Limitations included lack of physical examination options and, occasionally, mechanical dialogue. The LLM-enhanced social robotic VP platform offers a more authentic and interactive learning experience compared to the conventional computer-based platform. Despite some limitations, it shows promise in training CR skills, communication, and adaptive thinking. Social robotic VPs may prove useful and safe learning environments for exposing medical students to diverse, highly interactive patient simulations.

7.
BMJ Paediatr Open ; 8(1)2024 Oct 15.
Article in English | MEDLINE | ID: mdl-39414342

ABSTRACT

OBJECTIVE: Although Kawasaki disease (KD) is commonly regarded as a single disease entity, clinical subgroups have recently been described. We aimed to validate previous research on clinical subgroups and establish a KD subgroup differentiation model specific to China. METHODS: We analysed clinical data of 1682 patients diagnosed with KD at the Kunming Children's Hospital from December 2014 to December 2022. We performed principal component analysis and hierarchical clustering on 13 continuous variables. Then, we grouped the patients based on the optimal number of clusters and analysed the clinical characteristics of each subgroup. RESULTS: We ultimately identified three subgroups. In cluster 1, younger patients predominantly exhibited the highest risk of coronary artery aneurysm and the lowest rate of intravenous immunoglobulin resistance. Cluster 2 was characterised by high inflammatory markers and a lowered risk of coronary artery aneurysm. Cluster 3 was characterised by liver involvement, with significant elevations in liver enzymes, gamma-glutamyl transferase and total bilirubin. We found a positive correlation between the ratio of the rising trend and intravenous immunoglobulin resistance. Cluster 1 and cluster 3 shared similarities with the previously identified younger age subgroup and liver subgroup, respectively, whereas cluster 2 was unique to our study. CONCLUSIONS: Our study preliminarily validated a previous KD subgroup study and established a KD subgroup model in China.


Subject(s)
Immunoglobulins, Intravenous , Mucocutaneous Lymph Node Syndrome , Humans , Mucocutaneous Lymph Node Syndrome/epidemiology , Mucocutaneous Lymph Node Syndrome/diagnosis , Mucocutaneous Lymph Node Syndrome/blood , China/epidemiology , Male , Retrospective Studies , Female , Child, Preschool , Infant , Child , Immunoglobulins, Intravenous/therapeutic use , Cluster Analysis , Coronary Aneurysm/epidemiology , Coronary Aneurysm/immunology , Principal Component Analysis
8.
Arch Dis Child ; 2024 Oct 18.
Article in English | MEDLINE | ID: mdl-39379139

ABSTRACT

OBJECTIVE: IgA vasculitis (IgAV) is the most frequently experienced subtype of vasculitis seen in children. Most children fully recover, however, complications including chronic kidney disease are recognised. The aim of this project was to use a best available evidence, group agreement, based approach to develop national recommendations for the initial management of IgAV and its associated complications. METHODS: A fully representative multiprofessional guideline development group (GDG), consisting of 28 members, was formed and met monthly. Graded recommendations were generated using nationally accredited methods, which included a predefined scope, open consultation, systematic literature review, evidence appraisal, review of national or international guidelines and a period of open consultation. Audit measures and research priorities were incorporated. RESULTS: The IgAV GDG met over a 14-month period. A total of 82 papers were relevant for evidence synthesis. For the initial management, four topic areas were identified with five key questions generating six graded recommendations related to classification, specialist referral and musculoskeletal involvement. For the associated complications, five topic areas with 12 key questions generated 15 graded recommendations covering nephritis, gastrointestinal and testicular involvement, atypical disease and follow-up. Open consultation feedback was incorporated. The guidelines were endorsed by the UK Kidney Association and Royal College of Paediatrics and Child Health and are available online. CONCLUSION: Despite IgAV being a rare disease with limited evidence, a national standardised approach to the clinical management for children and young people has been achieved. This should unite approaches to care and act as a foundation for improvement.

9.
Clin Anat ; 2024 Oct 01.
Article in English | MEDLINE | ID: mdl-39352060

ABSTRACT

Over the past few decades, researchers and clinicians have dedicated significant attention to fascial tissues. Current interest focuses on their anatomical and pathophysiological features. Breakthroughs in ultrasound (US) and magnetic resonance imaging (MRI) have enhanced our ability to study the dynamics and alterations of the tissue structures. However, a microscopic perspective is also essential for a comprehensive understanding of some pathologies of the fasciae. The aim of this study was to investigate, using a cadaveric study: (1) the ease of visualization of the landmarks used for the US-guided fascial core needle biopsy (CNB); (2) the consistency and accuracy of needle placement inside fascial layers using US guidance and confirmed by histological examination; (3) inter-rater reliability. We assessed the feasibility of US-guided CNB in different topographical regions of human cadavers: the thoracolumbar fascia (TLF), fascia lata (FL), and crural fascia (CF). The results, confirmed by histological examination, revealed no significant difference in needle placements between the in-plane approaches in the long and short axes for all locations and fasciae studied (long axis: 91.88%; short axis: 96.22%); p > 0.05. US-guided core needle biopsy with the in-plane approach is feasible, consistent and reliable. It could provide most or all of high-quality fascial tissue samples required for pathological examination. It could also reveal changes in fascial pathologies, capturing the exact site of pathology thanks to US guidance, in particular in patchy diseases such as eosinophilic fasciitis.

10.
Rheumatol Adv Pract ; 8(4): rkae112, 2024.
Article in English | MEDLINE | ID: mdl-39376964

ABSTRACT

Objectives: The last major UK survey of medical undergraduate rheumatology teaching was 25 years ago. This study aimed to describe current teaching practice, the perceptions of teachers and students and their engagement with Versus Arthritis teaching resources and future challenges and opportunities. Methods: Electronic surveys were distributed by e-mail and/or social media to relevant teachers and students identified within all 37 UK medical schools. Results: A total of 34/37 (91%) teacher and 30/37 (81%) student surveys were returned. Compared with the last survey, the proportion of schools delivering rheumatology-identifiable teaching has fallen from 100% to 86% and the mean number of teaching days from 30 to 10. Rheumatology teaching is now more dispersed throughout the curriculum. Students preferred active learning methods such as simulation and expert patient teaching, while teachers preferred small-group teaching, online learning and lectures. The Versus Arthritis resources appeared underutilized by students but were considered useful. Most students thought rheumatology careers were not promoted within their medical school. Conclusion: A decrease in dedicated rheumatology teaching time was noted since the last survey 25 years ago. Greater promotion of rheumatology as a speciality and future career is required to maintain its professional identity and prevent marginalization.

11.
J Investig Med High Impact Case Rep ; 12: 23247096241286379, 2024.
Article in English | MEDLINE | ID: mdl-39374108

ABSTRACT

Systemic lupus erythematosus (SLE) is an autoimmune disease targeting multiple organs through immune complexes. Pericarditis and pericardial effusions are often encountered; however, cases of biventricular failure are notably less common in the setting of SLE. We report a 43-year-old male patient presenting with cardiac tamponade with biventricular failure in SLE. Our patient with known SLE, not on medication, presented with exertional dyspnea. He had decreased heart sounds, jugular venous distention, and right-sided inspiratory crackles on physical examination. On further investigation, he was found to have a reduced ejection fraction (EF) of 10% to 15% and moderate pericardial effusion. He was treated with immunosuppressive therapy and goal directed medical theraphy (GDMT), with improvement in his EF to 58% 3 months later. This case demonstrates the importance of early recognition of SLE-induced tamponade and the vitality of prompt medical therapy to reduce any further cardiac deterioration.


Subject(s)
Cardiac Tamponade , Heart Failure , Lupus Erythematosus, Systemic , Humans , Cardiac Tamponade/etiology , Lupus Erythematosus, Systemic/complications , Male , Adult , Heart Failure/etiology , Heart Failure/complications , Pericardial Effusion/etiology , Echocardiography , Immunosuppressive Agents/therapeutic use
12.
BMJ Open ; 14(10): e090360, 2024 Oct 07.
Article in English | MEDLINE | ID: mdl-39375185

ABSTRACT

INTRODUCTION: First steps for knee osteoarthritis (OA) is a cluster randomised implementation trial examining the effect of an educational reminder message included in knee X-ray reports on the proportion of people subsequently referred to exercise professionals for their knee OA. Evaluating the processes supporting the completion of the study and the efficacy of the reminder message is essential to interpreting the outcomes of the study and aiding translation into practice. METHODS AND ANALYSIS: We will conduct a concurrent process evaluation throughout the current study using a previously published framework for examining cluster randomised trials. This framework divides processes into those occurring at the cluster level and those at the target population level. For the current study, the cluster level is within radiology clinics. The target population is people with newly diagnosed radiologically evident, structural knee OA. A mixed methods design, incorporating survey data, administrative records, field notes and semi-structured interviews with representatives from radiology clinics and people with knee OA, will evaluate these processes. The focus of the evaluation will be recruitment and response processes of the radiology clinics and delivery and response processes for the people with knee OA. We will also describe the context and explore how the nudge theory of behavioural change influences the outcome of the study. ETHICS AND DISSEMINATION: The study protocol, inclusive of the process evaluation, was approved by Macquarie University Human Research Ethics Committee (#520221190343842). Findings will be disseminated through national and international conferences, national industry stakeholders and patient advocacy groups to reach all levels of healthcare. Staff at radiology clinics and people with knee OA involved in interviews provide written, informed consent to participate in the process evaluation. Specific findings will be incorporated into training modules aimed at radiology clinics and will be developed by our industry partners. TRIAL REGISTRATION: Prospectively registered with the Australian New Zealand Clinical Trials Registry (ACTRN12622001414707p). Registration occurred in December 2022.


Subject(s)
Exercise Therapy , Osteoarthritis, Knee , Humans , Osteoarthritis, Knee/therapy , Exercise Therapy/methods , Randomized Controlled Trials as Topic , Process Assessment, Health Care , Reminder Systems , Health Services Accessibility
13.
Cureus ; 16(9): e68844, 2024 Sep.
Article in English | MEDLINE | ID: mdl-39381488

ABSTRACT

Immunoglobulin G4-related disease (IgG4-RD) is a rare immune-mediated disease affecting multiple organs and tissues. There is often the presence of elevated serum Ig4 subtype with histological evidence of lymphoplasmacytic infiltration, fibrosis, and phlebitis. The mainstay of treatment is steroid therapy. This case report is based on a 24-year-old man with IgG4-related type 1 autoimmune pancreatitis (AIP) who also had elevated serum IgG4 subclass and histological features in keeping with IgG4-RD. The main complaints were dry cough, nasal congestion with sneezing, sore throat, and fever. Necessary investigations were performed and based on the International Consensus Diagnostic Criteria, the diagnosis of AIP type 1 was confirmed, which is a pancreatic manifestation of IgG4-RD. He was subsequently treated with prednisolone and azathioprine and is showing a good response to the treatment.

14.
Reumatologia ; 62(4): 259-265, 2024.
Article in English | MEDLINE | ID: mdl-39381729

ABSTRACT

Introduction: Non-traumatic spinal cord injury (NTSCI) represents a medical-surgical emergency. In Burkina Faso, limited data exist on the etiological profiles of this syndrome in rheumatology. This study aimed to describe the etiological profile of NTSCI in the Rheumatology Department of the University Hospital Center of Bogodogo (CHU-B). Material and methods: This was a cross-sectional, retrospective study with descriptive and analytical aims, conducted from March 1, 2017, to December 31, 2023, in the Rheumatology Department of CHU-B. Patients diagnosed with non-traumatic spinal cord compression syndrome during hospitalization were included. Results: The frequency in the Rheumatology Department of NTSCI was 2.94%, accounting for 104 patients. There were 68 men (65.38%), with a sex ratio of 1.88. The average age of the population was 57.91 years. All patients experienced back pain, with a lumbar location in 77 patients (74.04%). The average duration of the motor deficit was 2.97 months. A total of 3,532 patients were admitted to the conventional hospitalization unit of the Rheumatology Department at the CHU-B from March 1, 2017, to December 31, 2023. Among these, 104 patients had NTSCI, yielding a frequency of 2.94%. Spinal MRI was performed in 58 patients (55.77%), and the compression was extradural in 76.92% of cases (n = 80). The etiologies identified were Pott's disease in 32 patients (30.77%), followed by spinal metastases in 22 patients (21.15%). Twenty-nine patients (27.89%) experienced complications related to prolonged bed rest. No factor was significantly associated with the recovery of the motor deficit. Conclusions: Non-traumatic spinal cord injury is relatively rare in rheumatological practice in Ouagadougou. The etiology is predominantly Pott's disease, which confirms the geographical distribution of NTSCI causes.

15.
BMJ Mil Health ; 2024 Oct 08.
Article in English | MEDLINE | ID: mdl-39384221

ABSTRACT

Musculoskeletal injury (MSKI) is the most common reason for short-term occupational limitation and subsequent medically related early departure from the military. MSKI-related medical discharge/separation occurs when service personnel are unable to perform their roles due to pain or functional limitations associated with long-term conditions, including osteoarthritis (OA). There is a clear link between traumatic knee injuries, such as anterior cruciate ligament or meniscal, and the development of post-traumatic OA (PTOA). Notably, PTOA is the leading cause of disability following combat injury. Primary injury prevention strategies exist within the military, with interventions focused on conditioning, physical health and leadership. However, not every injury can be prevented, and there is a need to develop secondary prevention to mitigate or reduce the risk of PTOA following an MSKI. Two international collaborative groups, OPTIKNEE and OA Action Alliance, recently produced rigorous evidence-based consensus statements for the secondary prevention of OA following a traumatic knee injury, including consensus definitions and clinical and research recommendations. These recommendations focus on patient-centred lifespan interventions to optimise joint health and prevent lost decades of care. This article aims to describe their relevance and applicability to the military population and outline some of the challenges associated with service life that need to be considered for successful integration into military care pathways and research studies.

16.
BMJ Open ; 14(10): e087778, 2024 Oct 01.
Article in English | MEDLINE | ID: mdl-39353697

ABSTRACT

INTRODUCTION: Physiotherapists provide non-pharmacological conservative treatment for osteoarthritis (OA) using a wide spectrum of interventions. Previous surveys have identified global physiotherapy OA management practices. However, no review to date summarises the scope and findings of these studies. This article describes a scoping review protocol to map the surveys investigating physiotherapeutic interventions offered to patients with OA to identify the treatment interventions currently used for OA, the physiotherapists' attributes and organisational practice factors potentially associated with the choice of these interventions and to detect knowledge-practice gaps in the provision of physiotherapeutic interventions in OA treatment, contributing to guiding future research on this topic. METHODS AND ANALYSIS: This is a protocol for a scoping review that will be based on Arksey and O'Malley scoping review methodology and the methodological guidance for conducting scoping reviews published by the Joanna Briggs Institute (JBI). Relevant articles will be searched using the following databases: Medline (PubMed), Embase, Web of Science and Google Scholar. Surveys studies addressing physiotherapeutic interventions offered to patients with OA will be included. Study will be selected through title/abstract and full-text screening stages and data will be extracted using an instrument based on the model available in the JBI Manual. The findings will be summarised using descriptive statistics according to our research questions. This review will be reported in accordance with the Preferred Items for Systematic Reviews and Meta-Analyses for Scoping Reviews. ETHICS AND DISSEMINATION: Ethical approval is not required for this scoping review. Review findings will be disseminated through peer-reviewed publications, scientific conference presentations and scientific meetings. STUDY REGISTRATION: This protocol has been registered with the Open Science Framework (DOI 10.17605/OSF.IO/J3RBT).


Subject(s)
Osteoarthritis , Physical Therapy Modalities , Research Design , Humans , Osteoarthritis/therapy , Review Literature as Topic , Surveys and Questionnaires
17.
Front Med (Lausanne) ; 11: 1456063, 2024.
Article in English | MEDLINE | ID: mdl-39359917

ABSTRACT

Behçet's syndrome (BS) is a rare chronic multi-systemic inflammatory disorder that usually involves adults between third and fourth decades of life, while pediatric and juvenile onset are relatively rare. BS young patients (YP) often develop a full-blown disease late after onset, requiring careful diagnostic workup and regular follow-up while they grow up. In this regard, the purpose of transitional programs is to ensure continuous high-quality care to YP with chronic conditions, providing them with the skills necessary to become independent and empowered adults able to chronically self-manage their disease. EULAR/PReS released the first set of standards and recommendations for transitional care (TC) of YP with juvenile-onset rheumatic diseases, but the appropriate timing for transition, the tools to evaluate patients' readiness, and indicators of transition plans effectiveness still need to be identified. Although little is known regarding TC in BS, it is easy to assume that BS YP will benefit from developmentally and disease-specifically appropriate transition plans, which may promote continuity of care, improve perceived quality of life and prevent poor disease outcomes. This perspective article discusses the key concepts and the goals of TC, addressing the potential challenges and opportunities of TC for YP with BS in clinical practice.

18.
J Pak Med Assoc ; 74(10 (Supple-8)): S176-S180, 2024 Oct.
Article in English | MEDLINE | ID: mdl-39434298

ABSTRACT

Objective: To find the relationship between vitamin D deficiency and the activity of systemic lupus erythematosus in Iraqi patients. METHODS: The case-control study was conducted at Baghdad Teaching Hospital, Baghdad, Iraq, from July to October 2018, and comprised systemic lupus erythematosus patients regardless of age and gender visiting the Rheumatology outpatient clinic. Serum levels of complement protein 3, complement protein 4, anti-doublestranded deoxyribonucleic acid and 25-hydroxy vitamin D were estimated. Based on disease activity scores, patients were divided into moderate activity group SLE-M and severe activity group SLE-S. Healthy subjects matched for age and gender were also enrolled as the control group. Data was analysed using Graph Pad Prism 5.0. RESULTS: Of the 150 subjects, 62(41.3%) were in SLE-S group, 38(25.3%) in SLE-M and 59(33.3%) in the control group. Among the patients, 97(97%) were females and 3(3%) were males, with a female-to-male ratio of 32:1. The patients' age range was <10-≥50 years, while the control group consisted of 2 (4%) males and 48 (96%) females with an age range of <10-≥50. The mean levels of serum complement protein 3, complement protein 4 and vitamin D levels were significantly lower in the patient groups compared to the controls (p<0.05). CONCLUSIONS: Systemic lupus erythematosus patients suffered from either vitamin D deficiency or insufficiency, and low vitamin D levels were found related to disease activity.


Subject(s)
Complement C3 , Complement C4 , Lupus Erythematosus, Systemic , Vitamin D Deficiency , Vitamin D , Humans , Lupus Erythematosus, Systemic/blood , Lupus Erythematosus, Systemic/epidemiology , Lupus Erythematosus, Systemic/complications , Female , Male , Iraq/epidemiology , Case-Control Studies , Adult , Vitamin D Deficiency/epidemiology , Vitamin D Deficiency/blood , Vitamin D Deficiency/complications , Vitamin D/blood , Vitamin D/analogs & derivatives , Complement C3/analysis , Complement C3/metabolism , Adolescent , Complement C4/metabolism , Complement C4/analysis , Middle Aged , Young Adult , Child , Severity of Illness Index
19.
J Pak Med Assoc ; 74(10 (Supple-8)): S210-S213, 2024 Oct.
Article in English | MEDLINE | ID: mdl-39434306

ABSTRACT

OBJECTIVE: To assess the effect of treatment on interferon-alpha titer in the serum of female patients with systemic lupus erythematosus. METHODS: The case-control study was conducted at the Rheumatology Unit of Baghdad Teaching Hospital from January 2022 to April 2022 and comprised female systemic lupus erythematosus patients and healthy controls matched for age. The treated patients formed group A, while the untreated patients were placed in group B, and the controls were in group C. The disease severity status was confirmed using the systemic lupus erythematosus disease activity index. The serum level of interferon-alpha was determined using the enzyme-linked immunosorbent assay. Data was analysed using SPSS 21. RESULTS: Of the 150 subjects, 5033.3%) were in each of the 3 groups. The mean age of patients was 32.3±9 years while that of the controls was 33.0±11 years. Erythrocyte sedimentation rate, creatinine and aspartate aminotransferase were significantly higher in patient groups compared to the controls (p<0.05), while no-significant difference was noted between the treated and untreated groups (p>0.05). Interferon-alpha level was significantly high in group B compared to group A (p=0.037). The increase was more pronounced in those aged ≤50 years (p<0.05). Interferon-alpha was significantly high in group B patients with a disease duration of <1 year compared to group A (p=0.01). Interferon-alpha showed a significant increase in group B compared to the group A in patients with inactive systemic lupus erythematosus disease activity index and with family history (p<0.05). CONCLUSIONS: Treatment had a direct impact on interferon-alpha protein level in the serum of female patients of systemic lupus erythematosus.


Subject(s)
Interferon-alpha , Lupus Erythematosus, Systemic , Humans , Lupus Erythematosus, Systemic/drug therapy , Lupus Erythematosus, Systemic/blood , Female , Case-Control Studies , Interferon-alpha/therapeutic use , Adult , Aspartate Aminotransferases/blood , Blood Sedimentation , Creatinine/blood , Middle Aged , Young Adult , Severity of Illness Index
20.
BMJ Case Rep ; 17(10)2024 Oct 21.
Article in English | MEDLINE | ID: mdl-39433394

ABSTRACT

Polyarteritis nodosa (PAN) is a rarely seen vasculitis that often affects small-sized and medium-sized arteries. PAN can present with diverse clinical features depending on the organs involved, including potential cardiac involvement. Typical cardiac manifestations of PAN in the paediatric age group include pericarditis, valvular heart disease and coronary artery aneurysms. In contrast to PAN, Kawasaki disease (KD) is often associated with coronary artery abnormalities. Herein, we report a case of a young boy presented with KD-like features, including a coronary artery aneurysm confirmed by an echocardiogram (ECHO) and eventually diagnosed as PAN. The patient was treated with steroids, methotrexate and tocilizumab with improvement of the symptoms. Repeated ECHO revealed the resolution of the coronary artery aneurysms. Our paper highlights a rare presentation of PAN mimicking KD presentation. Physicians should consider PAN in chronic or severe courses of KD or coronary artery aneurysms.


Subject(s)
Coronary Aneurysm , Echocardiography , Mucocutaneous Lymph Node Syndrome , Polyarteritis Nodosa , Humans , Polyarteritis Nodosa/diagnosis , Polyarteritis Nodosa/drug therapy , Polyarteritis Nodosa/complications , Polyarteritis Nodosa/diagnostic imaging , Mucocutaneous Lymph Node Syndrome/diagnosis , Mucocutaneous Lymph Node Syndrome/complications , Mucocutaneous Lymph Node Syndrome/drug therapy , Male , Diagnosis, Differential , Coronary Aneurysm/diagnostic imaging , Coronary Aneurysm/diagnosis , Methotrexate/therapeutic use , Antibodies, Monoclonal, Humanized/therapeutic use
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