bluuebunny/crossref_metadata_2025_split huggingface dataset:
E023 Rheumatoid vasculitis presenting in the absence of rheumatoid arthritis
Authors: Cromie David A J , Stewart Sarah K , Wright Gary D 
Published: April 2023

Abstract

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 &gt;130 and anti-CCP &gt;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

Abstract

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

Abstract

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

Abstract

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

Abstract

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 &gt;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

Abstract

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

Abstract

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 &lt;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

Abstract

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.
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

AbstractFat 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.