bluuebunny/crossref_metadata_2025_split huggingface dataset:
P011 Time to diagnosis for different types of inflammatory arthritis
Authors: Jeyaratnam Sangeeta , Fish Sarah , Horton Sarah , Madan Ayesha , Rao Chandini , MacPhie Elizabeth 
Published: April 2024

Abstract

Background/Aims
Timely diagnosis of inflammatory arthritis (IA) is crucial for optimising patient outcomes. However, it is appreciated that some types of inflammatory arthritis may be more challenging to diagnose as they present very differently. The National Early Inflammatory Arthritis Audit (NEIAA) provides the opportunity for rheumatology services to benchmark the care they provide. This project used NEIAA to investigate if there is a difference in time to diagnosis for rheumatoid arthritis (RA), psoriatic arthritis (PsA) and axial spondyloarthritis (AxSpA).

Methods
Lancashire and South Cumbria Foundation Trust (LSCFT) recruited patients diagnosed with an IA to NEIAA over a five-year period (May 2018 to March 2023). Information on demographics, duration of symptoms prior to referral, GP referral time and rheumatology waiting time were analysed.

Results
A total of 393 patients with either RA, PsA or AxSpA were included in the analysis. There was a higher proportion of patients from minority ethnic backgrounds in the AxSpA group. The ratio of female to male was &gt;1 in RA and PSA but approximately 1:1 in AxSpA. The mean ages of patients in the RA, PsA and AxSpA groups were 60, 49 and 40 years, respectively. Table 1: Difference in time to diagnosis for the different types of IA, highlights the median time to diagnosis. Median was used due to outliers in data. Patients with AxSpA had the longest average duration of symptoms prior to presenting to the GP, with nearly 80% having symptoms for over a year. GP referral time was significantly shorter in the AxSpA group. Time to first rheumatology appointment was similar across the three groups.

Conclusion
There is a difference in time to diagnosis for the different types of IA. Further work is needed to understand why patients with AxSpA are presenting so late to their GP before being referred. We recognise the importance of exploring whether patients with AxSpA have been referred to other specialities or healthcare professionals. Investment in clinical triage and education sessions for primary care may help explain these results. More focus is needed on raising public awareness locally.

Disclosure
S. Jeyaratnam: None. S. Fish: None. S. Horton: None. A. Madan: None. C. Rao: None. E. MacPhie: None.
P015 Nodular skin rash associated with relapsing polychondritis, a challenging diagnostic journey to VEXAS syndrome
Authors: Gunawardena Methsala , Yalakki Leena , Moitra Rupak , Williams Emma L , Wong Henna , D'Cruz David 
Published: April 2024

Abstract

Background/Aims
VEXAS syndrome, first described in 2020, is a haemato-inflammatory disease caused by a mutation in the gene-UBA1.This syndrome can coexist with or without Myelodysplastic syndrome. What makes this case interesting is its long and challenging diagnostic journey and the involvement of different clinical specialties such as dermatology, ophthalmology, rheumatology, respiratory, haematology, and stroke teams. Ultimately, two years after the initial presentation, this appeared to be the first case of VEXAS with confirmed genetic mutation in the region. VEXAS being a relatively new entity, treating clinician/s were not aware of the possibility initially. Therefore, spreading awareness regarding the clinical picture of VEXAS is prudent. This case report highlights rheumatological and dermatological manifestations of the disease to aid in the timely diagnosis and management of future cases.

Methods
We report a 74-year-old previously healthy male presented in 2021 with a rash on his legs, arms, and torso since October 2019. It was progressive, painful, palpable, erythematous, non-pruritic, intermittent rash, cleared with hyperpigmentation. He also had oligoarticular arthritis, constitutional symptoms, recurrent episodes of polychondritis, uveitis, bibasal lung fibrosis, and an ESR and CRP above 100. He also had lymphadenopathy above the diaphragm, but the biopsy only showed necrotic material, and the bone marrow biopsy did not show any features of haematological malignancy. After excluding infections, he was started on high-dose oral prednisolone for relapsing polychondritis. The skin and inflammatory markers improved immediately following high doses of steroids. However, whenever steroids were weaned down, even with the help of Methotrexate, his skin was flaring. He also had unprovoked DVT and, while on anticoagulation, developed occipital infarction. He had extensive skin involvement when he had to stop Methotrexate following a chest infection.

Results
The skin biopsy was inconclusive. There was no significant cytopenia but persistent macrocytosis even after stopping Methotrexate. As a result, he underwent a repeat bone marrow biopsy, which showed Vacuoles and gene UBA1 mutation in 2023.

Conclusion
The heterogeneity and complexity of the disease are due to multi-organ involvement, including skin, joints, eyes, lungs, blood vessels, and cartilage, simultaneously or cumulatively. Skin involvement in VEXAS can also be vast, ranging from neutrophilic dermatosis, erythema nodosum-like lesions, leukocytoclastic, or urticarial vasculitis. Cutaneous manifestations develop in the majority with VEXAS syndrome and can be the first presenting symptom in this new rare syndrome.

Disclosure
M. Gunawardena: None. L. Yalakki: None. R. Moitra: None. E.L. Williams: None. H. Wong: None. D. D'Cruz: None.
P019 Comparison of newly proposed chronic nonbacterial osteomyelitis classification criteria to previous diagnostic criteria
Authors: Patel Aarat M , Lee Kelley S , Kim Ashley J 
Published: April 2024

Abstract

Background/Aims
Chronic nonbacterial osteomyelitis (CNO) is an aseptic autoinflammatory bone disease of unknown etiology. This diagnosis can be delayed due to the non-specific nature of symptoms and normal imaging studies. New classification criteria were proposed in 2022. This study seeks to validate these new classification criteria by comparing them to previous diagnostic criteria in a cohort of known CNO patients.

Methods
This is a retrospective case-series of pediatric patients diagnosed with CNO (n = 39) between 2012 and 2023 in Virginia, USA. The newly proposed classification criteria (Table 1) was applied to this cohort. The entry criteria of chronic bone pain, age &lt;18 years, abnormal radiograph and/or MRI were used prior to applying the newly proposed classification criteria. The 2008 Jansson diagnostic criteria and 2016 Bristol diagnostic criteria were also applied to the cohort for comparison and further validation. The clinical manifestations, laboratory results, and imaging studies that lead to a diagnosis of CNO were reviewed.

Results
The average age of diagnosis was 10 (range 2-17) years-old with the average time between onset of symptoms to confirmed clinical diagnosis being 24 (range 1-120) months. The most common presenting symptoms were bone pain (100%) and arthritis (49%). Evidence of radiographic abnormalities leading to CNO diagnosis was found with regional MRI (64%), bone scan (44%) with some requiring full body MRI (31%). Only one patient did not meet the new classification criteria (1/39; 3%) because of age &lt;3 years, fever and elevated ESR which together gave less than the required 55 points for criteria fulfillment. One different patient (1/39; 3%) did not meet both the Bristol and Jansson criteria because of unifocal disease and lack of bone biopsy.

Conclusion
This retrospective chart review validates the newly proposed classification criteria for CNO. Older diagnostic criteria (Jansson and Bristol) both require advanced imaging and at times bone biopsy for fulfillment. We conclude that the newly proposed criteria is easy to apply even in the absence of a bone biopsy or advanced imaging studies. The newly proposed criteria will aid in the development of a homogeneous cohort for future studies about CNO.

Disclosure
A.M. Patel: Member of speakers’ bureau; ABBVIE, AMGEN, SANOFI. K.S. Lee: None. A.J. Kim: None.
P028 Progressive multifocal leukoencephalopathy in a patient with systemic lupus erythematosus
Authors: Babla Deep , Suleman Yasir , Whittington Ashley , Hamdulay Shahir 
Published: April 2024

Abstract

Background/Aims
Progressive multifocal leukoencephalopathy (PML) is a demyelinating disease that develops following the reactivation of the John Cunningham virus (JCV) that in rare circumstances can occur in autoimmune disorders such as systemic lupus erythematosus (SLE). We present a 75-year-old female with SLE who had been treated with immunosuppressive therapy (mycophenylate and steroids) for one year.

Methods
A 75 year-old female presented with sub-acute deterioration of physical and cognitive function over the course of a few months including memory loss, ataxia, dysgraphia, word finding difficulty, confusion, low mood and reduced oral intake. Neurological examination revealed receptive and expressive aphasia, gaze fixation, unilateral weakness, brisk reflexes with positive plantar reflex and rigidity. A diagnosis of PML was made due to the detection of JC virus in the cerebrospinal fluid (234 copies/ml) and brain MRI findings consistent with the disease. Haematology results showed a profound lymphopenia (0.1x109 ). Mycophenolate was held and she was commenced on increasing doses of Mirtazapine up to 45mg. Clinical review at four months found marked improvement in her cognitive and physical abilities. Repeat CSF analysis found JC virus to be undetectable.

Results
PML has a 20-fold greater incidence in SLE when compared with the general population. Lymphopenia is a common finding in patients with SLE and has been found to correlate with both the risk and severity of opportunistic infections. The cause of this can be multifactorial and immunosuppressive drugs are often implicated however SLE in isolation can cause severe CD4+ lymphopenia leading to an increased risk of PML reactivation. This case demonstrates the importance of monitoring lymphocyte counts in SLE. Current guidance suggests adjusting regimes of immunosuppressive therapy if lymphocyte counts fall below 1x109 however further guidance on its management may be warranted. The heterogenous manifestation of SLE can make differentiating between the neurological syndromes associated with rheumatic disease and PML challenging. Given the management of PML is vastly different to that of SLE in regards to introduction or withdrawal of immunosuppressive therapies, there should be a high index of clinical suspicion in the early stages of investigation. In vitro studies have explored the possibility of mirtazapine (a 5-HT2A antagonist) providing a low-risk treatment option for those with SLE and this warrants further research into its efficacy in treating or preventing PML in this cohort. Our patient had improved symptomology after cessation of mycophenolate and the commencement of Mirtazapine.

Conclusion
PML is a rare complication of SLE but should be considered in patients admitted with neuropsychiatric presentations, the use of Mirtazapine may provide a low-risk treatment strategy.

Disclosure
D. Babla: None. Y. Suleman: None. A. Whittington: None. S. Hamdulay: None.
P039 A systematic review and meta analysis of anti rheumatic drugs and pneumococcal vaccine immunogenicity in inflammatory arthritis
Authors: Nagra Deepak , Bechman Katie , Adas Maryam , Yang Zijing , Alveyn Edward , Patel Samir , Russell Mark , Norton Sam , Wincup Chris , Baldwin Christopher , Galloway James 
Published: April 2024

Abstract

Background/Aims
Pneumococcal pneumonia is an important cause of morbidity and mortality amongst patients with inflammatory arthritis. Vaccination is recommended by the National Institute of Clinical Excellence (NICE) but it remains unclear how vaccine efficacy is impacted by different immunosuppressive agents. Our objective was to compare the chance of a seroconversion following vaccination against pneumococcus in patients with inflammatory arthritis to the general population as well as to compare the chance of seroconversion across different targeted therapies.

Methods
We searched MEDLINE, Embase and the Cochrane library databases from inception until 20th June 2023. We included randomised controlled trials and observational studies. Aggregate data were used to undertake pairwise meta-analysis. Our primary outcome of interest was vaccine seroconversion. We accepted the definition of serological response reported by the authors of each study.

Results
20 studies identified in the systematic review (2,807 patients) with 10 reporting sufficient data to be included in the meta-analysis (1,443 patients). The odds of seroconversion in patients receiving targeted therapies, relative to the general population, was 0.61 (95% CI 0.35 to 1.08). The reduced odds of response was skewed strongly by the effects of abatacept and rituximab with no difference between patients on TNF inhibitors or IL6 inhibition and healthy controls. In the analysis comparing within inflammatory arthritis populations the findings remained consistent with rituximab having the strongest negative impact on vaccine response. TNF inhibition monotherapy associated with a greater odds of vaccine response compared with methotrexate (2.25 (95% CI 1.28 to 3.96)). JAK inhibitor studies were few in number and did not present comparable vaccine response endpoints to include in meta-analysis. The information available does not suggest a significant detrimental effects of JAK inhibitor on vaccine response.

Conclusion
This updated meta-analysis confirms that for the majority of patients with inflammatory arthritis, pneumococcal vaccine can be administered with confidence that it will achieve comparable seroconversion rates to the healthy population. Patients on rituximab were the group least likely to achieve a response and further research is needed to explore the value of multiple course pneumococcal vaccination schedules in this population.

Disclosure
D. Nagra: Honoraria; Abbvie, Galapagos, UCB. K. Bechman: None. M. Adas: None. Z. yang: None. E. Alveyn: None. S. Patel: None. M. Russell: None. S. Norton: None. C. Wincup: None. C. Baldwin: None. J. Galloway: None.
P042 The impacts of rheumatic diseases in the workplace and employment
Authors: Beesley Richard P , Beesley Rebecca M , Middleton Kate , McBride Andrea 
Published: April 2024

Abstract

Background/Aims
Patients with rheumatic and musculoskeletal conditions report a range of impacts on their personal life, family, education and employment. Whilst reasonable adjustments are required in law to provide sufficient support to people with chronic health conditions, these are not always forthcoming or adequate to mitigate the impacts of disease on employment.
This collaborative study between three patient organisations explored the impact of disease on employment in a UK population.

Methods
Adults in the UK aged 18+ who had any diagnosis of a musculoskeletal or rheumatic condition were invited to complete an online survey. Respondents provided information about their diagnosis, experiences and attitudes across a range of themes and topics, including work and employment.

Results
In total, 532 respondents completed the survey (64% female; 68% White ethnicity). Overall, 371 (77%) reported that their arthritis affected them at work or in their daily duties and responsibilities, with a higher proportion of females experiencing this (82% compared to 65% of males, p&lt;.001). The majority reported fatigue (75%) and impacts on their ability to do household chores (63%), and around a third had their choice of job limited (33%) or have had to reduce their working hours (30%) due to their arthritis, and 29% found their arthritis has not been recognised in employment. Overall, 16% reported their employer was not supportive when they needed to attend medical appointments, and 53% had found their arthritis affected them financially through having to reduce their hours at work, taking unpaid time off, paying to travel to appointments, paying for their prescriptions, or other reasons.

Conclusion
Arthritis brings a substantial burden to patients, including in their access to, and experiences of, the workplace. Whilst many patients have developed their own ways to circumvent the barriers they experience (such as choosing employment that is more accessible to them, reducing hours, or changing roles), these choices can impact them financially. Increased awareness of the impacts, and improved understanding, amongst employers, coupled with better support for patients to access employment and for employers to access support and guidance, may help reduce barriers and improve experiences for patients with arthritis.

Disclosure
R.P. Beesley: None. R.M. Beesley: None. K. Middleton: None. A. McBride: None.
P068 The Use of Virtual Reality Headsets instead of Entonox for Paediatric Patients undergoing Steroid Joint Injections or Venepuncture / Cannulation
Authors: Lee Helen C , Leach Samantha , Hawley Daniel , Barker Nicki 
Published: April 2024

Abstract

Background/Aims
Entonox has traditionally been used for patients with Juvenile idiopathic arthritis (JIA) who require intra-articular joint injections (IAIs). However, Entonox is now the third most misused drug especially in school age children and young people (CYP). Entonox also has an environmental impact. In addition, Entonox has side-effects including euphoria, disorientation, nausea and vomiting. Recently there has been increased awareness of the risks of Vitamin B deficiency associated with prolonged Entonox use which can lead to neurological toxicity. These factors have led us to consider virtual reality (VR) devices as an alternative for patients requiring IAIs. The VR headset we used is a class one medical device that can provide both reduction in anxiety levels and pain relief in patients needing anxiety-provoking or painful procedures such as IAIs. Aims: To trial the use of VR devices in a Paediatric Rheumatology Service and to assess effectiveness in reducing anxiety and pain when used during IAIs and cannulation / venepuncture.

Methods
A pragmatic pilot study of VR headset use was carried out from November 2022 until September 2023. All CYP undergoing IAIs without GA were offered either Entonox sedation or VR headset distraction. Each CYP using the VR headset was asked to score anxiety and pain levels before and after the procedure using a Likert scale. Patient demographics were also collected (age and gender).

Results
A total of 42 patients attended for IAI’s during the study window between the ages of 7 and 16. Thirty-three opted to use the VR headset. Nine opted for Entonox. An additional 22 patients opted to use the VR headset for venepuncture / cannulation. The 64 users of the VR headset reported an average 47.8% reduction in anxiety and a 0.8% reduction in pain.

Conclusion
The study demonstrated that the use of a VR headset reduces anxiety in CYP having painful procedures. A minimal reduction in pain however was reported. This may be due to some patients having low pre procedural pain scores. The terms of the headset loan limited the questions that could be set for data collection. It has therefore not been possible to separate results from children who underwent IAIs or venepuncture / cannulation. We also did not collect comparative scores from the Children and young people who chose to use Entonox. Funding has now been secured for the VR headset and we are carrying out a prospective evaluation, including feedback from patients and staff, into the role of VR devices in managing situations involving procedural anxiety.

Disclosure
H.C. Lee: None. S. Leach: None. D. Hawley: None. N. Barker: None.
P069 Moving more with Axial Spondyloarthritis - a short survey to help design future exercise studies
Authors: Roberts Matthew , Qooja Sepehr , Moorthy Arumugam , Bishop Lettie 
Published: April 2024

Abstract

Background/Aims
Axial Spondyloarthritis (AxSpA) is an inflammatory arthritis affecting 0.5-1.0% of people in the UK. Physical activity guidance for people living with AxSpA from the National Institute for Health and Care Excellence is vague. Individualised, structured aerobic exercise is recommended without any direction towards exercise type, frequency, intensity, or acknowledgement of physical limitations to an individual’s environment which may prevent exercise participation. Consequently, the proportion of people with AxSpA meeting the World Health Organisation physical activity recommendations (&gt;150 minutes of moderate-intensity exercise per week) is significantly lower than the general population. Therefore, a short survey was administered to people with AxSpA to understand types of physical activities they would like to participate in, and any common barriers to exercise participation.

Methods
The National Axial Spondyloarthritis Society (NASS) invited subscribers to their e-newsletter with lived experience to complete a short online survey. Respondents were asked ‘which of the following activities would you like to include as part of your usual lifestyle?’ ‘What would prevent you from taking part in the activities above?’ and ‘Do you already include any of the physical activities in your usual lifestyle?’ Respondents were allowed to choose multiple options from a range of activities and barriers.

Results
266 people living with AxSpA (68% women, 31% men, 1% non-binary, 94% white British/Irish) responded. Walking was most selected for activities respondents would like to be included as part of their lifestyle (83%), followed by stretching/flexibility exercises (61%), swimming (46%), weight training and resistance exercise (41%), and cycling (33%). Only eight respondents (3%) stated hydrotherapy which is traditionally advocated as exercise therapy in AxSpA. Worries about pain and flare ups (57%), fatigue (45%), time (39%), cost (27%), not knowing enough about how much activity to do (22%), and lack of available facilities (20%) were common barriers.

Conclusion
To maximise reach and benefit, exercise guidance needs to relate to exercise that people with AxSpA would like to or already do; the benefits of exercise can only be realised if people want to participate. The responses emphasise the need for guidance relating to inclusive models of exercise. This includes exercise such as walking that is flexible and adaptable to limitations of time and does not require additional cost, equipment, or facilities. The results also highlight the importance of including outcome assessments of pain, flare frequency, and fatigue.

Disclosure
M. Roberts: None. S. Qooja: None. A. Moorthy: Consultancies; AM has received speaker fees from Lilly, Novartis, Galapagos, and UCB. L. Bishop: Grants/research support; NCB has received funding from NASS..