bluuebunny/crossref_metadata_2025 huggingface dataset:
P197 Evaluating the safety and factors associated with treatment cessation in Takayasu arteritis
Authors: Maughan Robert T , Porter Andrew , Dahanayake Chanaka , Ianonne Claudia , Alapat Ritu , Pericleous Charis , Youngstein Taryn A , Mason Justin
Published: April 2023

Background/Aims

Immunosuppression in Takayasu arteritis (TA) reduces the risk of arterial disease progression. However, long-term use of glucocorticoids (GC) and other agents carries risk, particularly as patients age. GC tapering practice can vary and there are no guidelines on the safety of treatment cessation due to a lack of data. This study describes the cessation of treatment in a large single-centre cohort and evaluates the baseline, clinical and treatment parameters associated with successful treatment stop.

Methods

The Imperial College Takayasu Arteritis Cohort (UK) was reviewed retrospectively; 158 patients, median follow-up 8.4 [5-13.6] years. Patients with data available within 1 year of audit (31/03/2021) were analysed. Treatment was defined as > 6 months of GC, csDMARD or biologic medication and cessation as stopping treatment for >1 year. Cessation safety was assessed by serial angiography (whole aorta MRI or CT) and NIH disease activity scoring. Baseline demographic, clinical and treatment parameters as well as initial treatment responses were compared for cessation and non-cessation cases. Data are median [IQR], comparisons are non-parametric for continuous variables.

Results

Of 129 (82%) cases requiring treatment, 107 (83%) remained in care. 29 (27.1%) cases of cessation were identified, time off-treatment was 3.6 [2.5-8.2] years. For 25 (86%) cessation cases, treatment withdrawal was due to sustained quiescence. Infection, non-adherence and tolerance accelerated cessation in 4 cases. Long-term inactivity was confirmed by disease activity scoring in all cases and by angiography where available (23 cases). No instances of treatment restart were observed. Baseline demographic and disease severity parameters were similar in cessation and non-cessation patients (Table). Cessation cases had lower GC doses after 2 years of treatment and a trend towards lower disease activity, suggesting better responses. Relatedly, biologic administration (reserved for refractory disease) was markedly lower in the cessation group. Finally, cessation cases had earlier treatment start years, were older at audit and had an increased proportion of Numano Type V classification.

Conclusion

In TA, complete treatment cessation is feasible and safe in a proportion of cases. Initial treatment response, treatment duration and Numano type V arterial involvement are potential predictors of successful treatment cessation and may aid future stratification strategies after validation.

Disclosure

R.T. Maughan: None. A. Porter: None. C. Dahanayake: None. C. Ianonne: None. R. Alapat: None. C. Pericleous: None. T.A. Youngstein: None. J. Mason: None.
P200 The relationship between patient and physician global assessment in patients with anti-neutrophil cytoplasmic antibody associated vasculitis
Authors: Bhonsle Ajinkya , Coughlan Tim , Graven Rachel , Bussa Paula , Gingold Michael , Polkinghorne Kevan , Ryan Jessica , Kitching A Richard
Published: April 2023

Background/Aims

Patients with anti-neutrophil cytoplasmic antibody associated vasculitis (AAV) often have reduced health-related quality of life (HRQoL) as assessed by the SF-36 questionnaire. Global assessment provides a patient and physician’s view on the patient’s overall health and wellbeing. The aim of this project was to examine the level of agreement between the patient and physician global assessment (PtGA and PhGA) in patients with AAV.

Methods

Patients attending our dedicated tertiary vasculitis clinic were recruited into this prospective study. PtGA and PhGA scores, out of 100, were assessed via a visual analog scale, with higher scores representing better health. SF-36 assessed HRQoL. Discordance was defined as an absolute difference of ≥ 20 between PhGA and PtGA. Active disease was defined as a BVAS of ≥ 1.

Results

Ninety-seven patients (mean age 58 years, 46% male) participated. Forty-three (44%) patients had active disease. The majority (88%) were on immunosuppressive therapy with renal (79.4%), lung (42.3%) and sino-nasal (40.2%) being the most commonly involved systems. The mean (SD) PtGA and PhGA was 63.4 (22.2) and 60.8 (24.8) respectively (t = 2.63, p = 0.0049). Mean (SD) PtGA in active versus inactive disease was 59 (22.8) and 66 (21.5) respectively (t = 1.49, p = 0.07). PhGA scores were higher than PtGA in 44 patients with an overall mean difference of + 13.7 (95% confidence interval [CI] 9.97, 17.46, p < 0.001). PtGA scores were higher than PhGA in 48 patients (mean difference +17.9, 95% CI 22.6, 13.3, p < 0.001). Scores were discordant in 26 patients, with PhGA>PtGA (negatively discordant) in 10 (38%). Mean (SD) PhGA in discordant patients versus non-discordant patients was 47.5 (24.7) versus 65.7 (23.1) respectively (t = 1.71, p = 0.001). Mean (SD) age in positively discordant versus negatively discordant patients was 58.8 (17.3) versus 69.1 (8) respectively (t = 1.71, p = 0.026). The percentage of patients with active disease (n=number of patients) in positively discordant versus negatively discordant patients was 50% (n = 8) versus 30% (n = 3) respectively (p = 0.008). Bland-Altman plots showed no systematic bias as global scores increased.

Conclusion

Overall, physicians did not overestimate patients' global health when using the global assessment tool. Discordance rates were 26%, within which physicians were likelier to assess the patient's global health as lower than patients did themselves. Positively discordant patients (PhGA<PtGA) were likelier to have active disease and were younger on average.

Disclosure

A. Bhonsle: None. T. Coughlan: None. R. Graven: None. P. Bussa: None. M. Gingold: None. K. Polkinghorne: None. J. Ryan: None. A. Kitching: None.
E002 Management of adults with systemic lupus erythematosus (SLE) in 2021: a multi-centre audit within Wales as part of an ongoing quality improvement exercise
Authors: Wright Sarah E , O'Beirn Patrick , Rhys-Dillon Ceril
Published: April 2023

Background/Aims

Significant changes to working practices within rheumatology have occurred since the beginning of the COVID-19 pandemic: virtual consultations have now become commonplace. This audit was undertaken as part of ongoing quality improvement work to assess the standards of care provided to patients with SLE attending rheumatology departments across Wales. Care was audited against the standards outlined in the 2018 British Society of Rheumatology guideline for the management of adults with SLE. Results were compared with the previous multi-centre audits of SLE care in Wales and the UK. We were interested to see whether changes to working practices had significantly affected the care of patients with SLE across Wales.

Methods

Rheumatology services in Wales were invited via email to participate. Data collection occurred between June and August 2021. Data was collected via online questionnaire, held securely by the Dudley Group NHSFT. Unit questionnaires were submitted by a lead clinician in each centre. Five centres across Wales submitted data from 62 clinical encounters with patients with SLE.

Results:

Conclusion

Results of this multi-centre audit do not demonstrate any widespread significant changes in care for patients with SLE in Wales between 2019 and 2021, despite 48% of consultations audited being undertaken virtually. Blood pressure and urinalysis were not consistently measured in 2021, likely secondary to the increased prevalence of virtual consultations. Group education has been undertaken as to the importance of facilitating observations in the virtual environment and centres have shared their experiences of how they tackle this challenge locally.

Disclosure

S.E. Wright: None. P. O'Beirn: None. C. Rhys-Dillon: None.
E006 An audit showing the successful operation of a nurse-led gout clinic during the COVID-19 pandemic
Authors: McWilliams Lesley-Ann , Stewart Roger G A
Published: April 2023

Background/Aims

Research has shown nurse-led gout clinics provide better outcomes compared to usual care. This District General Hospital set up a pilot nurse-led gout clinic in autumn 2019. This aimed to improve patients’ understanding of their condition, achieve better control of serum uric acid levels (SUA), reduce flares and prevent Emergency Department attendances.

Methods

A modified clinic protocol, closely modelled on BSR guidance was agreed within the department. With consultant supervision, one nurse specialist provided a mix of in-person and telephone appointments. Targets were set aiming for SUA <360µmol/L for most patients and <300µmol/L for those with erosive change or tophi. All patients were offered prophylaxis. Patients required a rheumatologist’s diagnosis of gout or crystal confirmation for enrolment. Exclusion criteria were significant renal or hepatic derangement. Within 3 months of the service starting SARS-CoV-2 impacted the operation of healthcare worldwide and led to the closure of routine outpatient clinics in Northern Ireland. A decision was made to switch the gout clinic to run entirely by telephone. Blood testing was facilitated through primary care and phlebotomy hubs.

Results

Over a 19-month period, 78 patients were treated and audited through this clinic: 69 men and 9 women. Average age was 57, mean SUA 509 µmol/L at referral and 322 µmol/L on discharge. 69 patients received allopurinol and 9 received febuxostat. No patients required uricosuric drugs. All patients were offered and agreed to take prophylaxis with a majority (85.8%) remaining on it for 3-6 months. Patients required a mean of 3.38 appointments prior to discharge from the clinic. The mean dose of urate lowering therapy on discharge was 315.9mg allopurinol and 93.3mg febuxostat. 95% experienced ≤2 flares during their enrolment in the clinic with no patients requiring Emergency Department attendance due to gout flare.

Conclusion

The nurse-led gout clinic was well received by patients and was effective as a telephone service during the pandemic when so many services were stood down. The clinic was able to continue to provide education, deliver effective reductions in uric acid as well as reduce incidence of flares and Emergency Department attendances. Lower doses of urate lowering therapy than expected were needed to achieve target. A small number of patients were discharged prior to enrolment for initial non-engagement which may have been exacerbated by the lack of face-to-face appointments. Our COVID-19 model did struggle with those patients needing an interpreter. In-person initial appointments have since been restarted; however, a greater proportion of reviews will continue to be offered by telephone given the unexpected success of the model. This audit showed that a nurse-led gout clinic can run successfully, even during a pandemic with a significant reliance on telephone consultations.

Disclosure

L. McWilliams: None. R.G.A. Stewart: None.
E009 A review of MRI indications and outcomes to identify a clinical predictor for joint inflammation
Authors: Barry Lucinda , Roberts Abigail , Menon Bina , Subesinghe Sujith
Published: April 2023

Background/Aims

Due to the nature of symptoms there is often a delay in diagnosing axial spondyloarthritis (AxSpA), the median delay from initial symptoms is over 8 years. A Rheumatology IBP (inflammatory back pain) service can reduce this to 3 years. MRI is highly sensitive for recognising early spine and sacroiliac joint (SIJ) changes. The objective of this project was to evaluate the value of spinal and SIJ MRI requests in a rheumatology department to try and identify a clinical predictor of MRI inflammation.

Methods

A retrospective review of 107 patients who underwent a spinal and SIJ MRI. An assessment of the clinical indications, MRI findings, and the management impact. Numerous parameters were assessed for each patient, including appointment type (face-to-face vs telephone), referrer grade, AxSpA ASAS criteria, CRP, HLA-B27 status, MRI result and management impact.

Results

41.25% of patients who had ≥2 AxSpA features had inflammation on MRI. However, only 11.54% of patients with ≤1 AxSpA feature had inflammation on MRI. ≥2 AxSpA features was a significant predictor for MRI inflammation, with a p-value of 0.00545 and sensitivity 91.67%. Additionally, just 33% of patients had HLA-B27 status documented, providing scope for improvement when using AxSpA features as a predictor. An active BASDAI score was not found to be a predictor of MRI inflammation. Additionally, there was no statistical difference in the MRI findings following face-to-face or telephone consultations; 35.53% had inflammation on MRI compared to 32.26%, respectively.

Conclusion

≥2 AxSpA features is a statistically significant predictor of active MRI inflammation. This could be used to support a new triage assessment pathway for inflammatory back pain referrals to rheumatology.

Disclosure

L. Barry: None. A. Roberts: None. B. Menon: None. S. Subesinghe: None.
E023 Rheumatoid vasculitis presenting in the absence of rheumatoid arthritis
Authors: Cromie David A J , Stewart Sarah K , Wright Gary D
Published: April 2023

Background/Aims

Rheumatoid vasculitis (RV) is an extra-articular manifestation of rheumatoid arthritis (RA) which is usually seen in long-standing severe disease, affecting approximately 1-5% of the patient population. It involves small- to medium-sized arteries and has a high mortality rate of 40% at 5 years post-diagnosis.

Methods

In this report we describe a case of a 58-year-old male with no significant past medical history other than previous heavy smoking, who presented with a several week history of weakness, paraesthesia, numbness and pain affecting both hands and feet. He had also experienced weight loss of 12lbs in a short period, night sweats, and arthralgia in his hands, along with a six-week history of Raynaud’s phenomenon.

Results

Examination revealed nailfold infarcts but there was no evidence of synovitis and this was subsequently confirmed to be absent on ultrasound of the small joints of the hands and feet. Investigations showed: CRP 79, ESR 52, rheumatoid factor >130 and anti-CCP >733. Cryoglobulins were not detected, complement was normal and neuropathy screen bloods including viral and autoantibody screens were otherwise unremarkable. During admission he developed nodular lesions over the extensor aspect of his forearms bilaterally, consistent with rheumatoid nodules. These evolved over a period of four days. He also developed a vasculitic rash over the medial malleolus of his left foot. Nerve conduction studies demonstrated mononeuritis multiplex and PET CT showed evidence of small vessel vasculitis. CT chest, abdomen and pelvis identified a 3cm hypodense lesion within the tail of the pancreas, subsequently found to be a benign lymphoepithelial cyst on biopsy. Treatment with intravenous methylprednisolone for three days resulted in a rapid improvement in both motor and sensory function of his limbs. On discharge, he was commenced on oral methotrexate and a reducing course of prednisolone, starting at 30mg daily. The clinical picture was highly suggestive of RV in the absence of an established inflammatory arthritis. When reviewed three months post-discharge he reported continuing improvement of his symptoms; the nodules had regressed in size and almost disappeared.

Conclusion

To our knowledge, only seven cases have been reported in the literature of a patient with RV at initial diagnosis of RA and two cases in which a diagnosis of RV preceded the diagnosis of RA. Due to its high mortality and morbidity we would like to increase awareness amongst clinicians of this unusual vasculitic form of clinical presentation of RA.

Disclosure

D.A.J. Cromie: None. S.K. Stewart: None. G.D. Wright: None.
E036 Arthritis in East Africa: an observational study
Authors: Kuo Catherine , Black Louisa , Kelly Clive
Published: April 2023

Background/Aims

The burden of musculoskeletal (MSK) disease in East Africa remains largely unknown. Early evidence from Tanzania suggests that it may have a substantial adverse impact on health care outcomes, with both social and economic consequences. Limited data on prevalence and case mix of MSK disorders is presently available in East Africa. Data is urgently required to facilitate the planning, provision and funding of the services needed to meet population requirements here. The need is likely to be greatest among the poorer and most isolated members of the community. We describe the case mix of MSK disorders we encountered among people across five regions who had no previous access to clinical care in East Africa.

Methods

Over a four-week period in 2022, we undertook a series of clinics in five separate locations across impoverished areas of Zambia and Kenya. These ranged from city slums to isolated rural communities. We recorded demographic features for every consultation, along with our diagnosis and intervention. We calculated the percentage of people consulting with MSK issues and describe the case mix of MSK disorders, along with interventions provided.

Results

We completed a total of 1089 community consultations in Zambia and Kenya. Of these, a total of 271 people (24.9%) reported primary MSK issues. This population was mainly female (66%) and had a median (range) age of 58 (13-90) years. The percentage of patients who consulted with MSK issues were significantly influenced by geographic location, rising from 8% in urban areas to 52% in the most rural sites. The commonest MSK diagnoses we made at first consultation were osteoarthritis (49.8%), mechanical low back pain (27.7%) and soft tissue rheumatism (10.0%), with 3.0% having evidence of inflammatory joint disease (IJD). Therapeutic intervention was provided in 62.3% and procedures were performed in 11.4%.

Conclusion

MSK disease appears to be a common reason for seeking medical intervention in East Africa, especially among older females who have accumulated a significant mechanical burden from physical exertion and childbearing. Therapeutic intervention was frequently required, although smaller numbers had evidence of IJD. Increased awareness of the burden of MSK disease on poorer populations is necessary. Training programs in rheumatology are urgently required to ensure that care pathways are established with adequate funding and regular rapid access to ensure the provision of appropriate support and intervention.

Disclosure

C. Kuo: None. L. Black: None. C. Kelly: None.
E043 Work resources for people with rheumatic and MSK conditions
Authors: Mishra Nandini , Macfarlane Gary , Wainwright Elaine , Jenkins Caron , McAllister Maureen , Walker-Bone Karen , Hollick Rosemary
Published: April 2023

Background/Aims

Supporting people with health conditions to remain in work is a UK government priority. We aimed to understand what resources are available from national charities for people with rheumatic and musculoskeletal conditions (RMDs), where people look for information about their condition, how helpful they find information, and identify areas for improvement.

Methods

National RMD charities were identified, and individual charity websites searched for information on work, and the frequency and extent to which these included key topics. Ten patient contributors provided feedback on the usefulness of topics covered and any gaps. Questions on type and experiences of information accessed were included in a UK cross-sectional web-based survey of people who reported a RMD diagnosis. The survey was co-designed with patient partners and disseminated to charities and social media channels between 30/8/21-26/11/21.

Results

18 national RMD charities were identified, and available work information summarised (Table 1). There was significant variation in work information provided, with limited internal signposting between charities to those with more comprehensive resources. External signposting to NHS and government resources was variable and differences in work policy across devolved nations often not acknowledged. 859 people completed the survey. Charity websites, clinical services and friends/family were common sources of information. Compared to people with inflammatory RMDs, those with non-inflammatory RMDs were more likely to be employed/not working because of illness (76.9% v. 64%) and more likely to report difficultly accessing information about their condition (63.8% v. 36.4%). Our patient partners found navigating work information challenging. Improvements suggested included using simpler language and signposting to ‘bona fide’ information. Patients wanted positive patient stories, help with ‘soft skills’ e.g., how to have constructive conversations with their employer, and better employer training.

Conclusion

Charities have a key role in providing information but could improve signposting between charity websites and to external resources, including positive patient vignettes, and clearer language. People with non-inflammatory conditions feel their information needs are not being met. This provides important guidance to inform development of resources to support people with RMDs to work.

Disclosure

N. Mishra: None. G. Macfarlane: None. E. Wainwright: None. C. Jenkins: None. M. McAllister: None. K. Walker-Bone: None. R. Hollick: None.
E064 A rare hereditary haemochromatosis type three (3) related arthritis
Authors: Umair Hafiz Muhammad , Kazmi Muhammad Farooq
Published: April 2023

Background/Aims

Arthritis is a common manifestation in hereditary haemochromatosis (HHC). Haemochromatosis arthropathy frequently involves small joint of hands especially the metacarpo-phalangeal joints (MCPJs) and hence is one of the differentials when assessing for rheumatoid arthritis, psoriatic arthritis along with osteoarthritis but requires a high index of suspicion. We report a case of Haemochromatosis arthropathy where the arthropathy was due to type 3 hereditary haemochromatosis (HHC) which is a much rarer form and requires specialist genetic testing.

Methods

A 38-year-old lady was referred with abnormal iron profile and ten-month history of small joint arthralgia mainly affecting the index and middle finger in right hand with occasional swelling. She had a normal body mass index with minimal alcohol intake. Clinical examination showed low grade synovitis in index finger metacarpo-phalangeal joint (MCPJ). Blood investigations for Rheumatoid arthritis and connective tissue diseases were negative with normal CRP & ESR, Ferritin was slightly raised at 236 and transferrin saturation was 100% on two successive occasions, LFTs were normal. Xray of hands showed slight narrowing of 2nd MCPJ and US hands showed thickened synovium but no erosions or Doppler activity in 2nd MCPJ.

Results

Based on these, possible hereditary haemochromatosis (HHC) arthropathy was considered. By that time, she had been seen by the hepatology service where no evidence of iron deposition in other organs was found. Surprisingly her genetic testing for hereditary haemochromatosis (HHC) (Cys282Y/H63D in HFE gene) was negative resulting in most common HFE-related HHC excluded. Iron studies were repeated which were still raised, hence after discussion with hepatologist and biochemist the plan was made to investigate for rarer form of hereditary haemochromatosis (HHC), genetic tests showed that she had TFR2 variant mutation which would be consistent with type 3 hereditary haemochromatosis (HHC), which is inherited in autosomal recessive pattern. Venesections were started with ferritin levels improving to less than 50 with plans to monitor arthralgia to see if there is any improvement over time.

Conclusion

Metacarpo-phalangeal joints (MCPJs) involvement is common in various arthropathies. This case highlights the importance of considering other, much rarer forms of hereditary haemochromatosis (HHC) as treatment will help in preventing iron deposition in other organs like liver, heart, pancreas etc. These conditions are very rare and there is need to create awareness of genetic testing as hereditary haemochromatosis (HHC) arthropathy does present not uncommonly to rheumatology services and requires dedicated non-HFE molecular panel.

Disclosure

H. Umair: None. M. Kazmi: None.
E073 Attitudes to technology-supported rheumatoid arthritis care, questionnaire study: opportunities for technology to improve RA care
Authors: MacBrayne Amy , Soyel Hamit , Marsh William , Fenton Norman , Pitzalis Costantino , Curzon Paul , Humby Frances
Published: April 2023

Background/Aims

Rheumatoid arthritis (RA) outcomes have significantly improved with the treat-to-target paradigm, however this necessitates intensive monitoring. Demand outstrips capacity in rheumatology services, making Mobile Health (mHealth) an attractive prospect. However, software developers often design without understanding the needs of ultimate users, solving non-existent problems. EULAR’s ‘Points to Consider’ for remote care specifies that interventions “should be developed in collaboration with all stakeholders including the healthcare team … and people with RMDs” as an overarching principle. This study sought to understand existing challenges for end-users, and how technology might support these.

Methods

Questionnaires were devised collaboratively between Rheumatologists and Computer scientists, informed by discussions with an RA patient group. People >18 years old with a diagnosis of RA completed paper surveys (utilising likert scales, ranking preferences, and tick-box answers) whilst awaiting appointments at Mile End Hospital (Barts Health NHS Trust). Clinicians were recruited to complete an online survey via mailing lists from multiple centres across London & South-East England, from October 2019-March 2020. The study was registered (CMPS ID [43816]). Ethical approval was obtained through the Bloomsbury Research and Ethics Committee (19/LO/1345). This work forms part of a larger mixed-methods study funded by the PAMBAYESIAN project grant (EP/P009964/1).

Results

109 people with RA returned paper questionnaires, 41 clinicians completed the online survey. Pain management, flares, and medications were identified as key challenges by both groups, however patients ranked lifestyle changes much higher than clinicians, who considered disease acceptance and understanding more significant. Considering opportunities; people with RA rated accessing their rheumatologist as the most useful potential feature (72.9%), followed by joint count body maps (59.8%), setting the agenda for appointments (52.3%), information about RA (45.8%) and support with drug monitoring (45.8%). Clinicians rated medication support most useful(96.8%), followed by information about RA (87.1%), agenda setting (87.1%) and flare management (83.9%).

Conclusion

People with RA and their clinicians identify flares, pain, and medication management as key challenges in the disease, although clinicians may underestimate the impact upon patients’ lifestyles. Medication support, information giving, and agenda setting for appointments.

Disclosure

A. MacBrayne: None. H. Soyel: None. W. Marsh: None. N. Fenton: None. C. Pitzalis: None. P. Curzon: None. F. Humby: None.
E079 Preliminary analysis of autoantibody profiles of autoimmune inflammatory myopathies: study from a single North Indian tertiary care centre
Authors: Dhuria Shahzeene , Khatri Prabin , Negalur Natasha , Telang Kaustubh , Ghosh Shounak , Yadavalli Durgarao , Singal Vinay , Gupta Rajiva
Published: April 2023

Background/Aims

Studies in autoimmune inflammatory myositis (AIM) have shown a strong role of autoantibodies in the diagnosis and prognosis of patients with myositis. This ongoing study shares the preliminary data of 139 patients with AIM from a North Indian tertiary care centre. This prospective, observational study was conducted at a 1250-bedded hospital to assess the prevalence of myositis-specific antibodies (MSA) and myositis-associated antibodies (MAA) in patients from the Indian subcontinent with AIM and the correlation of these antibodies with clinical features.

Methods

From November 2016 to September 2022, patients with AIM (satisfying the Bohan and Peter criteria of 1975) attending the Rheumatology and Clinical Immunology Department of Medanta Hospital were included after taking their informed consent, and divided into subgroups as dermatomyositis (DM), polymyositis (PM), CTD-associated myositis (CTD-M), cancer-associated myositis (CAM), and juvenile myositis (JM). The Institutional Ethics Committee approved the study. Clinical data and sera from patients were collected and analysed.

Results

The study included 139 individuals with a mean age of 45.4 years. Fifty-two had DM, 49 had PM, 25 had CTD-M, 6 had CAM, and 7 had JM. ANA positivity was found in 67.70%, MSA in 22.3%, MAA in 25.4%, and both in 12.3%. Antibodies against Mi-2 were identified in 13 (28.9%) patients, Jo-1 in 14 (32.50%), PL-7 in 3 (6.7%), PL-12 in 4 (8.9%), SRP in 9 (20%), and MDA-5 and NXP2 in 1 (2.2%) patient each. Antibodies against Ro were found in 28 (57.1%) of the patients, RNP in 8 (16.3%), and PM-Scl in 9 (18.4%). Mi-2 antibodies were mostly identified in DM patients and were significantly associated with skin rash. SRP positivity was mostly found mainly in PM. Raynaud's and ILD were linked to the presence of Jo-1 and Ro antibodies. Malignancy screening was negative in NXP2 and TIF1γ antibody-positive patients.

Conclusion

MSA was found in 22.3% of cases and MAA in 25.4%. Mi-2 antibodies were linked to rash, but none had ILD, whereas Jo-1 antibodies were linked to mechanic hands, arthritis, and ILD. We aim to gather more strong results in the future as additional patients are recruited in this continuing trial.

Disclosure

S. Dhuria: None. P. Khatri: None. N. Negalur: None. K. Telang: None. S. Ghosh: None. D. Yadavalli: None. V. Singal: None. R. Gupta: None.
E085 Investigating the relationship between daily disease impact scores and mood in patients with psoriatic arthritis using a smartphone application: a novel use of the PsAID-9 questionnaire
Authors: McGagh Dylan , McGowan Niall , Wu Yuhao , Hinds Chris , Saunders Kate E A , Coates Laura C
Published: April 2023

Background/Aims

Psoriatic arthritis (PsA) is a form of inflammatory arthritis affecting up to 30% of those with psoriasis. It has a considerable impact on patients’ functional capacity and quality of life. Studies have shown an increased prevalence of depression in patients with PsA which can contribute to reduced likelihood of disease remission. Smartphone applications have been developed to monitor symptoms in patients with rheumatic diseases. We used experience sampling to assess the relationship between daily PsA symptom burden using the PsAID-9 questionnaire and mood components utilising the validated Mood Zoom (MZ) questionnaire.

Methods

Participants (n = 25) with PsA aged ≥18 and <80 years, meeting the CASPAR criteria were recruited from rheumatology clinics at a single centre. At baseline, disease impact was assessed using the Psoriatic Arthritis Impact of Disease-9 (PsAID-9) questionnaire. Participants used the Mezurio smartphone application daily for 28 days to collect data on the PsAID-9 and Mood Zoom questionnaires. MZ comprises 10-items assessing mood symptoms on a 5-point Likert scale. The relationship between daily fluctuations in PsA symptoms and each mood component was assessed using linear mixed-effect (LMER) models. Likelihood ratio testing and Chi-square tests were used to identify statistical significance. Each model controlled for gender and PsAID-9 remission at baseline.

Results

Mean (SD) age was 49.6 (12.05) years. Sixteen had co-morbid psoriasis. Mean (SD) PsAID-9 scores were 3.28 (1.92). There was a bi-directional association between the mood components of MZ and total PsAID-9 score for each day, as shown in Table 1.

Conclusion

This study has demonstrated a relationship between daily fluctuations in disease burden and mood. This is the first study to pilot a daily assessment of disease impact versus traditional longer interval collections. Whilst these data demonstrate an association between mood and disease symptoms, they do not allow us to make inferences on the causal effect of mood on symptoms and vice versa. Further research into the relationship between mood and disease burden in PsA are warranted.

Disclosure

D. McGagh: None. N. McGowan: None. Y. Wu: None. C. Hinds: None. K.E.A. Saunders: None. L.C. Coates: None.
E093 The effect of new classification criteria on the diagnosis of large vessel vasculitis
Authors: Mukhtyar Chetan B , Beadsmoore Clare , Coath Fiona , Ducker Georgina , Sisson Katherine , Watts Richard A
Published: April 2023

Background/Aims

Giant cell arteritis and Takayasu arteritis are the two recognised forms of large vessel vasculitis. Clinically there is recognition of large vessel vasculitides which are not easily classified into the two forms. Classification criteria have been proposed since 1990 and have been updated in 2022. Classification criteria should be able to minimise the number of cases that are unclassifiable, and the number of cases that are classified into more than one set. We test both sets of criteria to test their performance in our cohort.

Methods

In our centre, between January 2011 to December 2020, 270 individuals were diagnosed as having large vessel vasculitis by virtue of having either a positive biopsy, ultrasonography, or positron emission tomography. The 1990 and the 2022 classification criteria were applied to our cohort.

Results

The mean age was 74. 181 were female. The results are as in Table 1. Using the 1990 criteria, 2 individuals met both classifications and 30 did not meet either. Using the 2022 criteria, 3 individuals met both classifications and 24 did not meet either. The numbers classified as having Takayasu arteritis has reduced from 13/270 to 2/270. The numbers classified as giant cell arteritis have increased from 225/270 to 241/270.

Conclusion

The new classification criteria have expanded the phenotype recognised as giant cell arteritis by mainly including previously unclassified individuals, and narrowed the phenotype recognised as Takayasu mostly by declassifying it as not meeting any criteria. Overall, the new criteria improve classification for 5/270 (1.8%) cases.

Disclosure

C.B. Mukhtyar: None. C. Beadsmoore: None. F. Coath: None. G. Ducker: None. K. Sisson: None. R.A. Watts: None.
OPTIS CELLULAR TECHNOLOGY LLC v APPLE RETAIL UK LTD (TRIAL B)
Authors:   Patents Court
Published: April 2023

H1 Patents — European Patents — Telecommunications — Standards essential patents — Infringement — Essentiality — Validity — Common general knowledge — Construction — Scope of Protection — Anticipation — Anticipation by equivalence — Obviousness — Workshop modifications — Proprietary estoppel — Acquiescence —”Inaction or silence — Reliance — Detriment — Telecommunications standards development processes and procedures — ETSI IPR policy — Construction — French law — Timing of declarations of essentiality — “Patent ambush” — Bona fide purchaser for value without notice
Local fat content determines global and local stiffness in livers with simple steatosis
Authors: Li David , Janmey Paul A. , Wells Rebecca G.
Published: April 2023

Fat accumulation during liver steatosis precedes inflammation and fibrosis in fatty liver diseases, and is associated with disease progression. Despite a large body of evidence that liver mechanics play a major role in liver disease progression, the effect of fat accumulation by itself on liver mechanics remains unclear. Thus, we conducted ex vivo studies of liver mechanics in rodent models of simple steatosis to isolate and examine the mechanical effects of intrahepatic fat accumulation, and found that fat accumulation softens the liver. Using a novel adaptation of microindentation to permit association of local mechanics with microarchitectural features, we found evidence that the softening of fatty liver results from local softening of fatty regions rather than uniform softening of the liver. These results suggest that fat accumulation itself exerts a softening effect on liver tissue. This, along with the localized heterogeneity of softening within the liver, has implications in what mechanical mechanisms are involved in the progression of liver steatosis to more severe pathologies and disease. Finally, the ability to examine and associate local mechanics with microarchitectural features is potentially applicable to the study of the role of heterogeneous mechanical microenvironments in both other liver pathologies and other organ systems.
FURIN suppresses the progression of atherosclerosis by promoting macrophage autophagy
Authors: Chen Hongping , Zhang Lihui , Mi Shaohua , Wang Hua , Wang Chunxiao , Jia Wenjuan , Gong Lei , Dong Haibin , Xu Bowen , Jing Yanyan , Ge Peipei , Pei Zhigang , Zhong Lin , Yang Jun
Published: April 2023

FURIN, a member of the mammalian proprotein convertases (PCs) family, can promote the proteolytic maturation of proproteins. It has been shown that FURIN plays an important role in the progression of atherosclerosis (AS). Current evidence indicates that autophagy widely participates in atherogenesis. This study aimed to explore whether FURIN could affect atherogenesis via autophagy. The effect of FURIN on autophagy was studied using aortic tissues from aortic dissection patients who had BENTALL surgery, as well as macrophages and ApoE−/− mice. In atherosclerotic plaques of aortic tissues from patients, FURIN expression and autophagy were elevated. In macrophages, FURIN‐shRNA and FURIN‐overexpression lentivirus were used to intervene in FURIN expression. The results showed that FURIN overexpression accelerated LC3 formation in macrophages during the autophagosome formation phase. Furthermore, FURIN‐induced autophagy resulted in lower lipid droplet concentrations in macrophages. The western blot revealed that FURIN regulated autophagy via the AMPK/mTOR/ULK1/PI3KIII signaling pathway. In vivo, FURIN overexpression resulted in increased macrophage LC3 formation in ApoE−/− mice atherosclerotic plaques, confirming that FURIN could inhibit the progression of AS by promoting macrophage autophagy. The present study demonstrated that FURIN suppressed the progression of AS by promoting macrophage autophagy via the AMPK/mTOR/ULK1/PI3KIII signaling pathway, which attenuated atherosclerotic lesion formation. Based on this data, current findings add to our understanding of the complexity of AS.
Fostering Adaptive Expertise Through Simulation
Authors: Clarke Samuel O. , Ilgen Jonathan S. , Regehr Glenn
Published: April 2023

Technology-enhanced simulation has been used to tackle myriad challenges within health professions education. Recently, work has typically adopted amastery learningorientation that emphasizes trainees’ sequential mastery of increasingly complex material. Doing so has privileged a focus on performance and task completion, as captured by trainees’ observable behaviors and actions. Designing simulation in these ways has provided important advances to education, clinical care, and patient safety, yet also placed constraints around how simulation-based activities were enacted and learning outcomes were measured. In tracing the contemporary manifestations of simulation in health professions education, this article highlights several unintended consequences of this performance orientation and draws from principles ofadaptive expertiseto suggest new directions. Instructional approaches grounded in adaptive expertise in other contexts suggest that uncertainty, struggle, invention, and even failure help learners to develop deeper conceptual understanding and learn innovative approaches to novel problems. Adaptive expertise provides a new lens for simulation designers to think intentionally around how idiosyncrasy, individuality, and inventiveness could be enacted as central design principles, providing learners with opportunities to practice and receive feedback around the kinds of complex problems they are likely to encounter in practice. Fostering the growth of adaptive expertise through simulation will require a fundamental reimagining of the design of simulation scenarios, embracing the power of uncertainty and ill-defined problem spaces, and focusing on the structure and pedagogical stance of debriefing. Such an approach may reveal untapped potential within health care simulation.
Postural Orthostatic Tachycardia Syndrome After COVID-19: A Systematic Review of Therapeutic Interventions
Authors: Abbate Gerardina , De Iulio Beatrice , Thomas Georgia , Priday Anna , Biondi-Zoccai Giuseppe , Markley Roshanak , Abbate Antonio
Published: April 2023

Postural orthostatic tachycardia syndrome (POTS) is a clinical syndrome of inappropriate increase in heart rate on standing that has been recently also associated with Coronavirus Disease 2019 (COVID-19) as part of the postacute sequelae of COVID-19 (PASC) or long-COVID. We herein aimed to systematically review reported cases of POTS after COVID-19 and determine the characteristics of the subjects, the diagnostic approach used, and the treatment strategies. We searched the literature according to the following criteria: (1) diagnosis of POTS according to standard definition; (2) timely association with a probable or definite diagnosis of COVID-19; and (3) a description of the individual subject(s). We identified 21 reports meeting criteria between March 2020 and September 2022, including 68 subjects (51 females and 17 males, 3:1 ratio) with a mean age of 34 ± 12 years, with reports deriving from the United States, Norway, Sweden, Israel, Ireland, United Kingdom, Singapore, and Japan. Most cases had mild COVID-19 symptoms. The most common POTS symptoms were palpitations, chest pain, lightheadedness, and debilitating fatigue. The diagnosis was established by means of head-up tilt table or active stand test. Nonpharmacologic treatments (fluids, sodium intake, and compression stockings) were virtually always used, but largely ineffective. Subjects received different treatments, the most common being beta-adrenergic blockers (ie, propranolol), mineral corticosteroids (ie, fludrocortisone), midodrine, and ivabradine. Symptoms tended to improve over time, but most patients remained symptomatic for several months. In conclusion, POTS after COVID-19 is a clinical condition affecting young individuals, and disproportionately young women, occurring as part of PASC—long-COVID, often debilitating, which can be easily diagnosed with a thorough clinical assessment and measuring changes in orthostatic heart rate and blood pressure. POTS after COVID-19 seems to be poorly responsive to nonpharmacological treatments but with symptoms improving with pharmacological interventions. Given the limited data available, additional research is urgently needed with respect to its epidemiology, pathophysiology, and treatments.
A Novel Dissection Method of the Internal Mammary (Thoracic) Artery: Anastomotic Vessel of the DIEP Flap
Authors: Sakai Shigeki , Yuzaki Ikki , Nagashima Hayato , Kajita Hiroki , Kiuchi Tomoki , Ishii Naohiro , Matsuzaki Kyoichi , Takatsume Yoshifumi , Kishi Kazuo
Published: April 2023

Deep inferior epigastric perforator flaps are commonly used for breast reconstruction using autologous tissue. For such free flaps, the internal mammary artery provides stable blood flow as the recipient for anastomosis. We report a novel dissection method of the internal mammary artery. First, the perichondrium and costal cartilage of the sternocostal joint are dissected with electrocautery. Then, the incision on the perichondrium is extended along the cephalic and caudal ends. Next, this C-shaped superficial layer of perichondrium is elevated from the cartilage. The cartilage is incompletely fractured with electrocautery, with the deep layer of perichondrium intact. Then, the cartilage is completely fractured by leverage and removed. The remaining deep layer of perichondrium is incised at the costochondral junction and shifted aside, revealing the internal mammary artery. The preserved perichondrium creates a rabbet joint to protect the anastomosed artery. This method not only enables a more reliable, safer dissection of the internal mammary artery, but also allows reusage of the perichondrium as underlayment in the setting of anastomosis, and coverage for the incised rib edge, protecting the anastomosed vessels.
Changes in Education and Consistency Needed in Recurrent Urinary Tract Infection Care: Patient and Expert Physician Perspectives
Authors: Sadun Taylor , Scott Victoria C. S. , Ackerman A. Lenore , Anger Jennifer T. , Kim Ja-Hong
Published: April 2023

Purpose:

This study seeks to identify barriers to care and possible interventions to improve the treatment of recurrent urinary tract infections.

Materials and Methods:

Focus groups of twenty-nine women with recurrent urinary tract infections and interviews of fifteen expert physicians subspecializing in female pelvic medicine and reconstructive surgery were performed to assess the current management paradigm. The data were analyzed using the grounded theory methodology. Qualitative studies of physicians and their patients with recurrent urinary tract infection were analyzed in parallel, elucidating areas of convergent and divergent thought and allowing the investigators to identify pertinent areas for clinical improvement.

Results:

Qualitative analysis of the transcripts identified 3 preliminary themes: patients harbored fear; patients often received fragmented and inconsistent care for acute urinary tract infections; and patients and experts were both frustrated by limited management options and guidance for a chronic problem. Emergent themes included the need for increased patient and physician education in urinary tract infection pathophysiology and treatment and the need for a uniform multidisciplinary approach by experts and primary care physicians.

Conclusions:

We have identified 2 critical needs in the care of women who suffer from recurrent urinary tract infections. These deficiencies are ameliorated with clear delineation of physician roles in patient management by urologists, primary care physicians, and infectious disease specialists. Our data support the concept that a team-based approach that best uses the strengths of each specialty would dramatically improve both the outcomes and experiences of women suffering with this debilitating chronic condition.
An Immunohistochemical Study of Breast Cancer Brain Metastases: The Role of CD44 and AKT in the Prognosis
Authors: Martins Gama João , Caetano Oliveira Rui , Teixeira Paulo , Silva Fátima , Abrantes Carlos , Figueiredo Paulo , Simões Sandra , Rebelo Olinda , Cipriano Maria Augusta
Published: April 2023

Breast cancer is a major health burden, and up to one-third of patients with breast cancer develop brain metastases, which are linked to a very poor prognosis. Few biomarkers are available to predict the prognosis of patients with metastases. Assessment by immunohistochemistry may be used as a tool to predict the behavior of these tumors. A retrospective transversal study including 114 patients (diagnosed between 2000 and 2016) with breast cancer brain metastases was carried out using archival biological material from 114 patients with breast cancer brain metastases. Expression of CD44, HER2, ER, PR, CA9, PDL-1, CD133, ALDH1, PTEN, AKT, PI3K, and AR markers was assessed by immunohistochemistry. The overexpression of CD44 and AKT was associated with worse overall survival (P=0.047 and P=0,034, respectively), on univariate analysis, in the cohort of parenchymal and bone metastases; the impact of AKT expression was also evident in the parenchymal cohort on uni (P=0.021) and multivariate analysis (P=0.027). The remaining markers did not exhibit a statistical correlation. Immunohistochemistry markers such as CD44 and AKT may have a prognostic impact on survival in patients with breast cancer brain metastases. The conjugation with other markers may help with the stratification of patients and therapy.