bluuebunny/crossref_metadata_2025 huggingface dataset:
P052 Case series: a rare form of calcinosis in patients with systemic sclerosis-myositis overlap
Authors: Achieng Sheilla , Samaranayaka Muditha , Herrick Ariane L
Published: April 2022

Background/Aims

Calcinosis is a well described entity that occurs in systemic sclerosis (SSc) and dermatomyositis. In patients with SSc, calcinosis typically occurs over pressure points but very rarely, widespread calcinosis may occur, presenting in an extensive pattern. We present a case series of four patients with SSc with this rarer form of diffuse calcinosis with the aim of raising awareness of this poorly recognised pattern of debilitating disease.

Methods

Four cases of SSc with extensive calcinosis were identified from a cohort of 461 patients with SSc who had attended a tertiary referral centre over the preceding 3 years, and their case notes reviewed.

Results

Demographic and clinical features of the four patients are summarised in Table 1. All four patients had the diffuse cutaneous subtype of SSc in the context of 102 patients [22%] of the entire SSc cohort of 461 patients, and all had overlapping features of myositis. Two were anti-Scl 70 antibody positive and the other two were anti-PMScl- positive. Widespread calcinosis was evident in all four patients in a pattern akin to ‘sheets’ of calcified deposits, very different to the lobular pattern of disease that has been well-described in the literature. Development of calcinosis occurred between one and six years of the onset of SSc, and in one patient the calcinosis discharged through skin at multiple sites. In all patients, treatment included mycophenolate mofetil. Treatments tried for the calcinosis included intravenous sodium thiosulfate in two patients (on the basis of anecdotal reports), but without significant clinical improvement.

Conclusion

It is well recognised that dystrophic calcification in SSc usually associates with disease duration. In our case series, however, all four patients presented with calcinosis very early on in the course of their disease and another striking point was that all had overlapping features of diffuse cutaneous SSc and myositis. Awareness of this widespread pattern of disease (and its associations) is lacking, highlighting a need for further research into the pathophysiology of this rare phenotype of debilitating calcinosis to identify future targeted treatments.

Disclosure

S. Achieng: None. M. Samaranayaka: None. A.L. Herrick: None.
P065 Post COVID-19 vaccination inflammatory arthritis: a case series of patients at a district general hospital
Authors: Fordham Sarah E , Achilleos Katerina M , Bhagat Shweta S , Sykes Mark P
Published: April 2022

Background/Aims

Vaccination against severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) has undoubtedly changed the course of the pandemic and lessened hospital admissions and death as a result of the aforementioned virus. Reactive arthritis has been reported with other vaccinations, for example influenza, and now it seems patients are presenting with similar symptoms post SARS-CoV-2 vaccination, the first human mRNA vaccine to be used against a virus. There are occasional case reports in the literature of patients developing various rheumatological conditions post COVID-19 vaccination including reactive arthritis and polymyalgia rheumatica. Currently the Summary of Product Characteristics (SmPC) for Pfizer BioNTech, Moderna and AstraZenica vaccinations only list myalgia and arthralgia as side effects in the musculoskeletal and connective tissue disorders section. Our aim was to collect data in a prospective manner on patients who presented to clinic with a new rheumatological condition which was believed to have been triggered by receiving a COVID-19 vaccination and their progress was followed over time.

Methods

Data have been collected prospectively from patients who have presented to the West Suffolk NHS Foundation Trust rheumatology department with symptoms which are thought to be attributable to COVID-19 vaccination over the last five months.

Results

Nineteen patients had a new inflammatory condition that appeared to have been triggered by the COVID-19 vaccination. The mean age of onset of symptoms is 65.2 years (range 22-85 years) with the mean number of days between vaccine and symptom onset of 9.6 days. 89% presented within 14 days of vaccination. Eleven patients received the Pfizer BioNTech vaccine and 6 the AstraZeneca vaccine. Seven patients presented after their first vaccine and seven after their second. Three had symptoms after both. The most common presentation was a small joint polyarthritis (8 patients) and polymyalgia rheumatica type symptoms (5). All but one were seronegative. So far, outcomes have varied between self-resolution or conservative management (6 patients), short- and long-term prednisolone requirement (6) and initiation of disease modifying anti-rheumatic drugs (5).

Conclusion

The emergence of post-COVID-19 vaccine inflammatory arthritis appears to be a new condition which rheumatologists will be required to treat. With the introduction of booster vaccinations and third doses this phenomenon is likely to be seen in clinic for the foreseeable future. Further data are required in order to guide best treatment options and enable better prognostic indicators.

Disclosure

S.E. Fordham: None. K.M. Achilleos: None. S.S. Bhagat: None. M.P. Sykes: None.
P077 Developing a better explanation of osteoarthritis: results from a conjoint analysis of patient preferences
Authors: Paskins Zoe , Nicholls Elaine , Grossmann Heiko , McRobert Cliona , Peat George , Shivji Noureen , Bartlam Bernadette , Croft Peter , Jinks Clare , Maddison John , Main Chris , Quicke Jonathan , Porcheret Mark , Protheroe Joanne , Cottrell Elizabeth
Published: April 2022

Background/Aims

Despite the negative impact of osteoarthritis (OA) and existence of evidence-based guidelines, many patients and professionals lack clarity about the nature of OA and effective treatment strategies. This project aims to improve OA explanations in consultations and investigate the extent to which different explanation statements impact on intention to self-manage OA.

Methods

Participants registered at four general practices, aged ≥45 years, with a recorded consultation for OA in the previous two years were mailed a survey. The survey included eight pairs of potential OA explanation statements for participants to select the explanation that would most help them to self-manage their OA, alongside questions on socio-demographics, OA symptoms, comorbidity and health literacy. The OA explanations were designed using a partial-profile choice-based conjoint analysis (profile strength 4, comparison depth 3) from a set of 11 theoretically informed key attributes (Table 1). Each attribute contained two statements: one representing current information sources, and one a newly designed statement from our previous co-design work with patients and stakeholders.

Results

The survey response rate was 22% (428/1980) (average age = 65 years [SD = 10]; 66% female). The newer statement was preferred to the existing statement for 10 of the 11 statements (indicated by a positive regression coefficient) and 8 of these differences were statistically significant (p < 0.05) (Table 1). Sensitivity analyses (e.g. to adjust the model to allow for within person correlation of response, and to test for 2-way interactions between model attributes) did not change the findings from the primary model.

Conclusion

Patients with OA preferred the newer statements, with one exception (causes). The preferred statements have been discussed with the project’s Patient Advisory Group and the statements combined into a written leaflet and animation, and further assessed using Flesch reading ease and Flesch Grade level. Our conjoint analysis has provided evidence about what explanation statements patients with OA prefer and improve likelihood of self-management. The next step is to evaluate the leaflet and written animation in think-aloud qualitative interviews before refining the OA explanation and widespread dissemination.

Disclosure

Z. Paskins: None. E. Nicholls: None. H. Grossmann: None. C. McRobert: None. G. Peat: None. N. Shivji: None. B. Bartlam: None. P. Croft: None. C. Jinks: None. J. Maddison: None. C. Main: None. J. Quicke: None. M. Porcheret: None. J. Protheroe: None. E. Cottrell: None.
P085 NRAS New2RA Right Start for people newly/recently diagnosed with RA
Authors: Bosworth Ailsa M , McNicol Iain
Published: April 2022

Background/Aims

The National Rheumatoid Arthritis Society (NRAS) follows best practice, evidence-based standards in all we do. Whilst huge strides have been made in the diagnosis and treatment of Rheumatoid Arthritis (RA), the impact on quality of life can be significant and for many, RA remains hard to come to terms with. Anxiety and depression are frequent co-morbidities seen in RA, particularly in the early stages. This can impede people’s ability to acquire knowledge about their disease together with supported self-management skills and coping strategies. The aim of Right Start is to improve outcomes of the newly/recently diagnosed with RA through a framework of emotional, educational and peer support, and access to high quality supported self-management resources tailored to individual need.

Methods

Right Start involves a simple, 4-step process: • a call (up to 1 hr) with a member of our helpline. • 1:1 peer support from trained volunteers with RA, 24/7 online community support • a tailored package of hard copy information with e-links of interest sent by post • further follow up available by helpline and individual peer support Right Start enables health professionals to meet their responsibilities against NICE Quality Standard 33, Statement 3, on which they are audited through National Early Inflammatory Arthritis Audit (NEIAA), and the EULAR Recommendations for implementation of self-management strategies in inflammatory arthritis.

Results

Since launch at BSR 2019, 71 rheumatology units have referred over 400 patients to this service. Approx. 1/3rd couldn't be contacted and after 3 attempts, a letter and newly diagnosed pack are sent, inviting contact at future date. Anecdotally people are highly satisfied with this service and a number of units are referring multiple patients. To gather empirical data on the impact of the service NRAS has partnered with Manchester University to undertake an Enhanced Right Start pilot in 5 UK rheumatology units involving the use of validated patient reported outcome and experience measures from the users recruited, and quantitative and qualitative data from the health professionals. The pilot is due to commence before end 2021. Meantime, referrals continue to grow.

Conclusion

The NHS rheumatology workforce is in crisis and Right Start enables professionals to offer evidence-based education and supported self-management resources to optimise patient care. A Nurse Consultant (West Middx.) said of the service: “I have found Right Start to be an excellent resource for people who have recently been diagnosed with RA. When time is limited time in clinic, a referral provides the patient with tailored information which complements the education I have been able to provide. Feedback from patients has been positive particularly relating to the phone calls with the NRAS helpline team and the NRAS volunteer with RA. I would definitely recommend the service.”

Disclosure

A.M. Bosworth: None. I. McNicol: None.
P086 Patient perceptions of musculoskeletal ultrasound as an educational tool in a rheumatology podiatry clinic
Authors: Newcombe Lisa , Tougher John , Kelleher Nancy , Woodburn Jim , Barn Ruth
Published: April 2022

Background/Aims

Emerging evidence suggests musculoskeletal ultrasound (MSUS) may benefit patient understanding of their inflammatory arthritis and medication adherence. The overall objective was to explore patient perceptions of MSUS when used as a patient education tool in relation to foot problems in people with rheumatic and musculoskeletal diseases (RMD).

Methods

Consecutive patients attending a rheumatology podiatry clinic at the Queen Elizabeth University Hospital Podiatry Department, Glasgow, were invited to participate. To gather information on how useful MSUS was for patient understanding of their foot problems, participants completed a brief questionnaire before and after their MSUS scan. MSUS assessment of affected foot structures was conducted by a single podiatrist with MSUS training (LN) using an Esaote MyLabAlpha portable US machine. Participants were shown both grayscale and power Doppler MSUS still images with/without video clips and caliper measurements depending on the presenting complaint. Anatomical foot models were used to orientate patients to foot anatomy and corresponding MSUS images.

Results

A total of 43 (12 male: 31 female) participants were recruited with a mean (SD) age of 50 (12) years and median disease duration (IQR) of 6 (1,10) years. The most common RMD was spondylarthopathy (30%) followed by RA (23%). In this group, the rearfoot region (35%) was most frequently involved. The most frequently affected structure was tendons (30%) with multiple structures affected in 19%. A range of mechanical and inflammatory MSUS diagnoses were made. 56% of participants had not previously seen MSUS of the joints or tendons, with the remaining 44% unsure. 93% reported the MSUS scan improved understanding of their foot pain and 98% reported it will make them more likely to adhere to podiatry treatments and advice [Table 1]. 98% also reported that MSUS was more useful than other educational resources for understanding their foot pain. Still images were selected most frequently as the method that made the most sense to participants.

Conclusion

This study provides preliminary evidence of the potential value of MSUS as an educational tool in podiatry, with potential to enhance adherence with podiatry interventions and rehabilitation programmes. Further work using a follow up design is required.

Disclosure

L. Newcombe: None. J. Tougher: None. N. Kelleher: None. J. Woodburn: None. R. Barn: None.
P091 Who do we risk leaving behind? A survey of digital access and e-health literacy in people with inflammatory conditions
Authors: Hider Samantha , Muller Sara , Gray Lauren , Manning Fay , Brooks Mike , Heining Dominic , Menon Ajit , Packham Jonathan , Raghuvanshi Subhra , Roddy Edward , Ryan Sarah , Scott Ian , Paskins Zoe
Published: April 2022

Background/Aims

The COVID-19 pandemic led to an overnight shift in healthcare delivery and rapid uptake of digital technology. Such approaches risk digital exclusion for people without access to or not confident in their use of technology. We sought to examine digital access and e-health literacy in people with inflammatory conditions.

Methods

People (n = 2,024) were identified from their electronic health record and invited to participate in a survey, using SMS and postal approaches. Data were collected on age, gender, self-reported arthritis diagnosis (RA, PsA, AS, SLE and other), access to an internet-enabled device and frequency of internet access, health literacy (single-item literacy screener) and self-perceived e-health literacy (eHEALs). Ethical approval was obtained (Ref 21/PR/0867).

Results

639 people completed the survey, of whom 287 (44.9%) completed it online. Mean (sd) age was 64.5 (13.1) years and 384 (64.7%) were female. 98.3% were white. Approximately 20% of people did not have access to an internet enabled device (Table). 93 (15.3%) of patients reported never accessing the internet, this proportion was higher in people with RA. Approximately 19% had low health literacy. In those reporting internet use, eHealth literacy was moderate. The most popular options for accessing arthritis advice were telephone helplines or appointments.

Conclusion

Low health literacy, lack of digital access and low reported internet use was common, especially in people with RA, leading to high use of telephone advice and rheumatology appointments. Digital roll-out needs to take account of people requiring extra support to enable them to access care or risks excluding many patients with inflammatory conditions.

Disclosure

S. Hider: None. S. Muller: None. L. Gray: None. F. Manning: None. M. Brooks: None. D. Heining: None. A. Menon: None. J. Packham: None. S. Raghuvanshi: None. E. Roddy: None. S. Ryan: None. I. Scott: None. Z. Paskins: None.
P113 The prevalence of depression and anxiety at first presentation to rheumatology outpatients
Authors: Arumalla Nikita , Littlewood Rebecca , Chen Winnie , Garrood Toby
Published: April 2022

Background/Aims

Depression and anxiety are more common in patients with rheumatic diseases and have been associated with higher levels of fatigue, pain and levels of physical disability. Although this has been described in distinct rheumatic conditions, the burden of psychological morbidity across the diseases presenting to rheumatology outpatients is not as well known. The prevalence of anxiety and depression in the UK was reported to be 5.9% and 3.3% respectively. Here, we report rates of depression and anxiety in a cross-section of new patients referred to rheumatology outpatients.

Methods

All new patients referred to a rheumatology outpatient clinic in London, UK for a 10-week period in February to May 2019 received a digital pre-appointment questionnaire which included Patient Health Questionnaire (PHQ8) and Generalised Anxiety Disorder Questionnaire (GAD7) questionnaire for detecting depression and anxiety respectively. A retrospective analysis was performed to assess the prevalence of significant depression and/or anxiety in various rheumatic diseases, defined as PHQ8 or GAD7 ≥ 10, signifying at least moderate depression or anxiety respectively.

Results

464 patients completed the questionnaire, 338 female (73%), with a mean ± SD age of 44.3 ± 13.6 years. 41.2% of patients screened positive for depression and 26.5% for anxiety, with 24.4% screening positive for both. Of the patients screening positive for anxiety, 91.9% were also depressed. The four most prevalent conditions were rheumatoid arthritis (RA, 8.6%), osteoarthritis (OA, 15.3%), psoriatic arthritis (PsA, 8.4%) and fibromyalgia (FM, 24.4%). The prevalence of at least moderate depression and/or anxiety in RA, OA, PsA and FM was 22.5%, 35.2%, 46.1% and 77% respectively. Logistic regression determined odds ratios relative to RA for significant depression and/or anxiety as follows: OA 1.89 (95% CI 0.76-4.67), PsA 2.75 (1.02-7.41), FM 11.80 (4.91-28.36).

Conclusion

The burden of psychological illness is high across the population of patients presenting to rheumatology outpatients. Our findings show significant prevalence of psychological illness at first presentation. Early identification of psychological distress is important as it can predict adverse long-term outcomes in patients with rheumatic conditions. These results justify an integrated approach between rheumatology and mental health services.

Disclosure

N. Arumalla: None. R. Littlewood: None. W. Chen: None. T. Garrood: None.
P120 A collaborative approach to rolling out advice and guidance
Authors: MacPhie Elizabeth , Horton Sarah , Madan Ayesha , Rao Chandini , Fish Sarah
Published: April 2022

Background/Aims

Advice and Guidance (A&G) is a key priority for out-patient transformation, provides primary care with an immediate specialist opinion and negates patients waiting several months to see a specialist in clinic. During the last year we have taken incremental steps to embed and promote A&G. The importance of seeking the views of primary care colleagues has been paramount to increasing engagement and understand any unintended consequences.

Methods

A&G was switched on through e-Referral Service in July 2019. Information was provided as to what was appropriate for A&G. In December 2020 an alternative A&G system was rolled out providing an opportunity to promote the use of A&G and we started to redirect appropriate referrals at triage. In July 2021, we took a further step to return referrals where appropriate with an immediate specialist opinion. Both A&G systems record activity and a spreadsheet is kept of all triage outcomes. The new A&G system collects feedback from primary care. There have been regular opportunities to speak to primary care colleagues.

Results

During the first year of implementation of A&G we received on average three queries a week. Following the roll-out of the new A&G system we saw a steady rise in the number of queries. In April to June 2021, received 56 direct A&G queries. Of 669 referrals received, 33 were redirected to A&G. In July to September 2021, we received 58 direct A&G queries. Of 549 referrals received, 12 were redirected to A&G and 19 were returned with immediate advice. Routine waiting times have reduced from 12 to 7 weeks over the last 12 months. Feedback from primary care regarding 169 A&G queries has been very positive with 98% finding advice helpful. In partnership with the CCG we have developed a Top Tips Guide for Primary Care and delivered an evening workshop to promote the use of A&G. Feedback from GPs resulted in a letter being sent to patients when we respond to a referral with immediate advice and a leaflet is being developed to help patients understand the triage process and the different means by which a specialist opinion may be provided.

Conclusion

A&G has most importantly provided patients with an immediate specialist opinion and reduced the waiting times for those patients who still need to be assessed in clinic. Small incremental changes have allowed the team to appropriately job plan the workload and colleagues in primary care adapt to a new way of working. Regular discussions with primary care have helped understand any frustrations and to adopt a solution-focused approach. We recognise the importance of involving patients in the discussion, supporting them to understand an alternative way of working and change their mind-set on how specialist advice can be delivered.

Disclosure

E. MacPhie: Other; EM is the secretary of the North West Rheumatology Club, meetings have been supported by UCB, MSD, Abbvie and Lilly. S. Horton: None. A. Madan: None. C. Rao: None. S. Fish: None.
P126 GCA Hospital Standards (GHOST) - making a map of specialised services for the care of giant cell arteritis across England
Authors: Coath Fiona L , Bukhari Marwan , Ducker Georgina , Griffiths Bridget , Hamdulay Shahir , Hingorani Melanie , Horsbrugh Candy , Jones Colin , Lanyon Peter , Mackie Sarah , Mollan Susan , Mooney Janice , Nair Jagdish , O’Sullivan Eoin , Patil Ajay , Robson Joanna , Saravanan Vadivelu , Whitlock Madeline , Mukhtyar Chetan
Published: April 2022

Background/Aims

The objective of this project is to map services essential to delivering high quality care in giant cell arteritis (GCA) across England, identifying gaps in provision and thereby help to remove inequalities. To do this however, there must first be agreement on what these best practice services and standards are.

Methods

A steering committee was formed comprising 18 expert representatives from the 13 clinical regions in England, including rheumatology, ophthalmology, allied health professional and patient representation. A modified Delphi process was commenced with each member initially providing five aspects of service they felt were essential for best practice GCA care. From the 90 answers, common themes were identified by creation of a word cloud and then condensed into domains of practice. These domains were then ranked by each member in order of perceived importance. The top 10 domains taken forward for further review were clinical pathways, patient access, Rheumatology involvement, Ophthalmology involvement, ultrasonography provision, temporal artery biopsy provision, PET-CT scan provision, glucocorticoid treatment, patient education and multi-disciplinary team working. Domains identified as separate areas but not quite making it into the top 10 were Tocilizumab provision, audit and governance and research. With the latter two in particular, it was felt these are overarching principles which should run through all aspects of clinical work. Group consultation was undertaken to discuss the relevant aspects, and from this, three quality metrics and one summary statement were devised for each domain. Rheumatology and Ophthalmology provision were amalgamated, as it was felt these were equally as important, with similar requirements. On the first pass of voting all except ‘patient access’ achieved over 75% agreement amongst the steering committee members. After group consultation and amendment, ‘patient access’ also achieved the minimum 75% agreement cut-off. The final statements can be seen in the table below.

Results:

Conclusion

By devising specific quality metrics in addition to the recommendation statements above, it is envisaged these standards can be easily used as an audit tool to identify gaps and development needs in GCA services.

Disclosure

F.L. Coath: None. M. Bukhari: None. G. Ducker: None. B. Griffiths: None. S. Hamdulay: None. M. Hingorani: None. C. Horsbrugh: None. C. Jones: None. P. Lanyon: None. S. Mackie: None. S. Mollan: None. J. Mooney: None. J. Nair: None. E. O’Sullivan: None. A. Patil: None. J. Robson: None. V. Saravanan: None. M. Whitlock: None. C. Mukhtyar: None.
P133 NICE Guidance 41: Is it applicable to a real-world cohort of cervical spine fractures in older people?
Authors: Mony Damien , Gilbert Ffion
Published: April 2022

Background/Aims

Current guidance for the assessment and management of cervical spine fractures (CSFracs) in the UK is governed by NICE Guidance 41 (NG41). The guidance is based on the Canadian C-Spine Rule (CCR) which dictates whether spine imaging is necessary following trauma. There is little research into the reliability of NG41, notably on low energy impacts resulting in CSFracs in the elderly. The consequence of weaknesses in NG41 may result in delayed or missed CSFracs. Therefore, this report will deconstruct NG41 to identify potential areas of improvement.

Methods

Data were collected from medical notes on mechanism of injury and self-reported symptoms at The Royal Devon and Exeter Hospital in 84 patients >50 years old with confirmed CSFracs over a 4-year period. Quantitative and thematic analysis of data was used to compare with this cohort with NG41. The comparison focussed on age, symptoms and the mechanism of injury.

Results

Of the 84 patients, 32.1% were reported as having a dangerous mechanism of injury. 74.6% of patients aged ≥65 were considered low risk for CSFracs. Neck pain was reported by 74.5% of patients. Only 6.0% presented with paraesthesia as a predominant symptom.

Conclusion

The significant proportion of patients aged ≥65 considered low risk for CSFracs emphasises changes are required to NG41 to include low energy impacts in the guidance. Age remains to have the strongest correlation with CSFracs. Neck pain is the predominant symptom in the data but has no mention in NG41. Larger scale research and modified guidance testing is required before changes to NG41 can be made.

Disclosure

D. Mony: None. F. Gilbert: None.
P141 What are the predictors of fragility fracture in patients with low body mass index: an observational cohort study
Authors: Sharma Seema D , Bukhari Marwan
Published: April 2022

Background/Aims

Low body mass index (BMI) is known to be associated with increased risk of fracture and therefore measurements of height and weight contribute towards the Fracture Risk Assessment Tool (FRAX) score, an estimation tool used to calculate approximate risk of fracture to guide therapy decisions. However, data is lacking on which measurement of bone mineral density is best in those that are underweight. Using a large population of patients with low BMI, we aimed to determine which measurement of bone mineral density (BMD) is the most accurate predictor of fragility fracture in this group of patients. We also aimed to explore the relationship of other traditional risk factors for fracture in this subpopulation.

Methods

Data on risk factors for osteoporosis and fragility fractures was collected on patients presenting for BMD estimation at a district general hospital in North West England between June 2004 and October 2016. All analysis was performed on those with low BMI, defined as < 18.5kg/m2. Firstly, univariate logistic regression was performed to assess risk of fracture using BMD for lumbar spine, femoral neck and total hip; as well as smoking, alcohol use, presence of rheumatoid arthritis, history of steroid use and history of hip fracture in a parent. Risk factors were defined as per FRAX. This was followed by multivariate regression of available risk factors, using the best BMD predictor identified from univariate analysis. All models were controlled for sex and age at first scan. Results for continuous variables are reported as beta coefficients and for categorical variables as odds ratios. All results are reported with 95% confidence intervals. Area under ROC curve for each model was calculated.

Results

921 of 35,759 patients had a BMI of under 18.5 and were included in analysis. Of these, 88.4% were female, mean age at time of scan was 58.6 years. Results are shown in the table.

Conclusion

In those with low BMI, BMD of left total hip was the best predictor of fragility fracture. Smoking, alcohol use and history of hip fracture were also associated, but previous steroid use and diagnosis of rheumatoid arthritis was not.

Disclosure

S.D. Sharma: None. M. Bukhari: None.
P146 Hepatitis E infection in rheumatic disease: no evidence of chronicity
Authors: Zollinger-Read Caroline A , Raza Mohammad , Kazmi Muhammad F
Published: April 2022

Background/Aims

Hepatitis E virus [HEV] is the most common cause of acute hepatitis in developing countries but rare in the industrialised world. In the vast majority it causes an acute self-limiting infection which is often asymptomatic. It is usually detected due to raised transaminases in liver function tests [LFTs] which are checked as part of DMARD monitoring. There is increasing literature that immunocompromised patients with human immunodeficiency virus or transplant recipients are at risk of developing chronic HEV infection, which can progress to liver failure. It is unclear that immunosuppressed patients who have a rheumatological conditions have similar issues, which we have looked into locally. Chronic infection is defined as having detectable RNA for over 3 months.

Methods

Retrospective review of patients attending rheumatology outpatient clinic for blood monitoring, who had positive Hepatitis E serology between 2010 and 2021.

Results

We identified nine patients attending the rheumatology clinic in Sheffield, confirmed as having acute infection with HEV as Hep E IgM antibody was positive. They were all on immunosuppression and hence having regular monitoring bloods and were receiving either prednisolone, DMARDS or biologics or in combination. There were five males and four females, with an age range of 38 to 71 years. Eight patients had acutely abnormal LFTs with a peak ALT [normal <41 IU/L] ranging from 59 to 1651 while in one the LFTs remained normal. Eight out of nine patient's LFTs recovered to baseline over a period ranging from 3 to 6 weeks (one moved out of area). Five patients had HEV RNA tested after the positive IgM. Two patients had HEV RNA detected initially, the rest had undetectable RNAs. Repeat RNA testing 2 months later was undetectable in both patients, suggesting no chronic HEV infection in all patients. No patients were given HEV antiviral treatment. Almost all patients had their immunosuppressant treatment paused, which was recommenced once their LFTs normalised. In all patients LFTs have remained normal even after 5 years with no evidence of reactivation in spite of ongoing immunosuppression. We plan to check HEV RNA if the LFTs were to become abnormal.

Conclusion

Our case series raises awareness that rheumatologists should consider HEV as a differential in patients with a marked unexpected rise in transaminases. We observed that all our patients cleared HEV infection and there was no evidence of chronicity even with ongoing immunosuppression, which is different from Hepatitis B & C. We recommend that immunosuppressant treatment should be stopped initially following HEV infection and reintroduced once LFTs have normalised and HEV RNA is undetectable.

Disclosure

C.A. Zollinger-Read: None. M. Raza: None. M.F. Kazmi: None.
P147 Venous fibrosis and stenosis in IgG4-related disease
Authors: Baslas Rohit , Condurache Dorina , Greenstein David , Patrone Lorenzo , Hamdulay Shahir
Published: April 2022

Background/Aims

IgG4-related disease (IgG4-RD) is a multi-system fibro-inflammatory disease. Common presentations involve salivary and lacrimal glands, orbital disease, autoimmune pancreatitis, retroperitoneal fibrosis and tubulointerstitial nephritis. The main histopathological features are a dense, polyclonal, lymphoplasmacytic infiltrate rich in IgG4+ plasma cells, storiform fibrosis and obliterative phlebitis. Cases of retroperitoneal fibrosis resulting in venous compression with DVT are well described, however to our knowledge venous fibrosis and stenosis confirmed with IVUS in IgG4-RD RPF has never been described.

Methods

An 82-year-old lady presented with bilateral lower leg oedema. She had a past diagnosis of IgG4 disease made on the basis of retroperitoneal fibrosis (confirmed on PET-CT), raised serum IgG4 levels (IgG4 2.38; normal 0.04-0.86 g/l) a supportive tissue biopsy (fibro-lymphocytic tissue with non-diagnostic IgG4 plasma cells) and raised inflammatory markers (CRP 104, ESR 120). Disease responded well to prednisolone and azathioprine, resulting in regression of RPF. She presented with left leg pitting oedema 4 months after presentation which progressed to chronic leg venous ulceration. D-dimer testing, albumin, liver function, and renal function tests were normal. There were no signs of lymphoedema. Venous Doppler studies of the left lower leg showed a patent and compressible left common femoral, superficial femoral and popliteal vein excluding a DVT and Baker’s cyst. A repeat CT pelvis showed no compressive mass. An intravascular ultrasound showed that the wall of the IVC was stiff and of dampened amplitude with respiratory motion. There was also increased echogenicity of the left common and external iliac vein wall with significant stenosis of the distal left common iliac vein and the external iliac veins. On the right side, there was increased echogenicity of the right common iliac vein, but the lumen was well preserved.

Results

These findings indicated fibrosis of the IVC wall and left common iliac vein with resulting venous stenosis. The patient underwent bilateral iliac vein stenting with improvement of leg swelling and eventual healing of the venous ulcer.

Conclusion

This case demonstrates that venous fibrosis and stenosis may occur in the absence of compressive lesions leading to leg swelling. Further diagnostic information may be identified by IVUS and radiological intervention with venous stents may be required.

Disclosure

R. Baslas: None. D. Condurache: None. D. Greenstein: None. L. Patrone: None. S. Hamdulay: None.
P151 Assessing the impact of a family day for young people and their families living with juvenile dermatomyositis in the Northwest of England
Authors: Cuthbert Verna J
Published: April 2022

Background/Aims

JDM is a rare, autoimmune disease affecting primarily the muscles and skin. There are approximately 2-4 cases per million children and young people. JDM can have a huge impact on the lives of the young people, their siblings and families and many young people and families have never met others with the condition. Aims: To organise the first family day for young people and families living with JDM in the Northwest of England, and to assess the impact of the day. For Young people and their families to feel less isolated living with JDM; for young people and siblings to get to take part in an activity to build their confidence; for young people and their families to receive information about JDM and living with the condition.

Methods

Invitations were sent out to young people and families with JDM who attended Alder hey Children’s Hospital, Manchester Royal Children’s Hospital, Sheffield Children’s Hospital and Leeds Children’s Hospital. The family day was organised outside of a hospital setting at the Chill Factor in Manchester. The young people were invited to enjoy snow activities, a science workshop, arts and crafts and soft play, as well as enjoy a fabulous lunch! A programme of speakers was organised and included a specialist nurse from GOSH discussing her current JDM research; a Manchester patient talked about the lived experience of having JDM and entering adulthood. A question and answer session was organised for young people and families to ask questions to a multi-professional panel.

Results

Feedback from questionnaires filled out on the day and email responses suggest the young people and families found the day beneficial and benefited from meeting up with other families and sharing experiences. The feedback suggested they found the education sessions and tips gained helpful and would aid management of the condition. Feedback suggested that the young people and their siblings had fun! Meeting other young people with the same condition was frequently mentioned in the feedback as very beneficial and helped to reduce feelings of isolation. Feedback suggests that the majority of young people and families would like to keep in touch with other JDM families.

Conclusion

The feedback received from the families and young people suggested that the aims of the day were met and there was considerable interest in further such events in the Northwest and/or keeping in touch with other families living with JDM.

Disclosure

V.J. Cuthbert: None.
P156 Multidisciplinary paediatric rheumatology clinics
Authors: Teoh Lisa
Published: April 2022

Background/Aims

Three years ago, long pending lists and poor engagement/accessibility were affecting JIA management. Physiotherapy was often not done within two weeks of joint injections. There were a few children with fixed flexion deformities.

Our aims were: timely and easy access to good multidisciplinary care; regular follow-up for children with JIA; timely physiotherapy after joint injections; holistic care through hospital and community.

Methods

1) Shared database to ensure all children received appropriate follow-up; 2) joint clinics with paediatrician, physiotherapy and occupational therapy; 3) therapy follow-up in the community; 4) handbook for families including team contact details.

Results

Improved service outcomes:

Improved clinical outcomes:

Conclusion

Working together in a joint approach has made us more efficient and effective. Winning the trust and engagement from our children and families over the last few years has made our work rewarding and led to improved clinical results.

We hope to be able to continue working in this way by gaining recognition from the trust of the value this adds for families.

Disclosure

L. Teoh: None.
P164 New JIA-at-NRAS resource Medicines in Juvenile Idiopathic Arthritis
Authors: Wilson Debbie L
Published: April 2022

Background/Aims

Biologics have been commissioned by the NHS for use to treat JIA since 2014. Many of the original biologics that were first developed have since lost exclusivity, allowing other manufacturers to produce highly similar versions (biosimilars). This difference between the originator and the biosimilar has caused confusion within the patient community, especially in how it has been communicated to them. Parents found the concept of biosimilars confusing and wanted more information about this, recommending that written material be provided. Previous conversations with healthcare professionals, parents and young people living with JIA had also found there was a lack of written information on all JIA medications so we looked into how this gap could be filled.

Methods

Knowing there was a need for this information, a focus group was convened with young people with JIA, parents of children with JIA and healthcare professionals. From this focus group and separate conversations with healthcare professionals it very quickly became apparent a resource not only covering medications for JIA but detailing the wider JIA journey was needed. This resource would need to cover each step from diagnosis to a young person taking control of their disease management. It would also cover how the complexity of the immune system means that some children/young people respond differently to medication; help them to learn about the unpredictability of the disease and to take control of managing their disease, particularly as they move onto adult rheumatology services. NRAS worked collaboratively with health writer and healthcare professionals to make sure this new resource covered all the topics that were discussed at the focus group and detailed all the medications used to treat JIA, including information on the issue of differentiating between biologics and biosimilars.

Results

The Medicines in JIA booklet is now freely available to all parents, young people and healthcare professionals from the jia.org.uk website. The addition of visuals makes the information easier to navigate, clearer to understand and helps to illustrate the JIA journey.

Conclusion

The feedback NRAS has received from both healthcare professionals and parents has been very positive: “We think that they’re absolutely fantastic, they cover everything, and I’m so pleased I’ve got these to give to parents and children.” Paediatric Rheumatology Nurse Haywood Hospital “This is brilliant and much needed. Great piece of work much appreciated by all of us”. Lead Nurse Nottingham Children and Young People’s Rheumatology Service “This has been my absolute saviour! It gives you support on administering the medicines and made me feel right at ease after reading it”. Parent of child with JIA

Disclosure

D.L. Wilson: None.
P167 COVID-19 perniosis, a differential diagnosis to consider in adolescent patients presenting with chilblain-like lesions
Authors: Gak Nataliya , Choida Varvara , Leandro Maria , Sen Debajit , Ciurtin Coziana , Fisher Corrine
Published: April 2022

Background/Aims

Chilblain-like lesions (perniosis) have been reported frequently during COVID-19 pandemic in children and adolescents with no history of exposure to cold temperatures or underlying autoimmune conditions. Patients with these skin changes reported mild COVID-19 symptoms or previous contact with confirmed COVID-19 cases before they became symptomatic. In the majority of cases, a causal relationship between SARS-CoV-2 infection and chilblain-like lesion has not been proven.

Methods

Retrospective review of patients with chilblain-like lesions, possibly secondary to SARS-CoV-2 infection, presenting to a tertiary Adolescent Rheumatology service between January and August 2021.

Results

We identified five, male, adolescent patients (mean age, 16 years old) who presented with new onset of chilblain-like lesions affecting fingers, toes and heels in December 2020, which coincided with the peak of second wave of COVID-19 infection. One month prior to skin changes occurrence, 3 out of 5 patients experienced mild respiratory COVID-19-like symptoms and the rest of the patients were asymptomatic but were in contact with COVID-19 positive cases following outbreaks in schools. 1 of 3 symptomatic patients had a positive COVID-19 PCR test prior to skin manifestations. 2 out of 4 patients with heel lesions had deep, full thickness skin loss heel ulcers and 2 of 5 patients had superficially ulcerated lesions on a finger and toes, respectively, resulting in inability to attend school. None of the patients had any other symptoms or signs to suggest an underlying autoimmune connective tissue disorder. Demographics, clinical features and serological data are summarised in Table 1. One patient underwent a biopsy of heel ulcer which was histologically consistent with perniosis. In two patients (40%) chilblain like lesions resolved spontaneously within 2 months. Three patients (60%), with progressive ulcerated lesions, required various combinations of treatments with aspirin, calcium channel blockers (nifedipine), topical or oral steroids and hydroxychloroquine with complete resolution of symptoms within 6 months.

Conclusion

Chilblain-like lesions, including heel involvement associated with mildly symptomatic COVID-19 infection, have been reported before. Our mini-case series raises awareness of ulcerating chilblain like lesions possibly secondary to COVID-19 in adolescent patients, which require early recognition and instigation of treatment leading to better patient’s outcomes.

Disclosure

N. Gak: None. V. Choida: None. M. Leandro: None. D. Sen: None. C. Ciurtin: None. C. Fisher: None.
P168 An enriched population of tissue-resident CD8 memory T cells in young people with juvenile idiopathic arthritis recapitulate findings from mouse models of inflammatory arthritis flares
Authors: Bolton Chrissy , Rich-Griffin Charlotte , Dendrou Calliope , Brown Chrysothemis , Al-Mossawi Hussein , Croft Adam , Wedderburn Lucy
Published: April 2022

Background/Aims

Asymmetrical joint inflammation at presentation and during flares of juvenile idiopathic arthritis (JIA) implicates resident cell populations and the microenvironment in disease. Persistent CD8+ tissue-resident cell populations have been identified from murine models, capable of priming the joint towards an exaggerated inflammatory response, which resist systemic treatment. Clonally-expanded enriched populations of these tissue-resident CD8+ memory T cells (TRM) have been identified in synovial fluid of children with JIA.

Methods

We integrate and compare scRNA-seq findings from paired synovial fluid mononuclear cells and blood mononuclear cell (PBMC) samples from children with oligoarticular JIA (n = 2), adults with psoriatic arthritis (n = 3) and blood from healthy adult volunteers (n = 4).

Results

A number of T lymphocyte and myeloid cell populations demonstrated enrichment amongst synovial fluid mononuclear cells (compared to paired PBMCs) that corresponded across arthritic subtypes. A mean of 2.98% of T cells in synovial JIA samples had the CD69+ITGAE+CD8A+ tissue-resident signature compared to a mean of 0.37% in paired JIA PBMCs and 0.24% in other sample types (P <.00001, vs synovial psoriatic samples). These cells recapitulated previously observed traits of tissue-resident CD8+ T cells, including high expression of transcription factor RUNX3, granzymes and the FABP5 fatty acid transporter. In mouse models the chemokine CCL5 was critical for leukocyte recruitment to mediate inflammatory flares and indeed, JIA synovial fluid samples exhibited stronger expression of CCL5 in TRM. Additionally, transcripts for cytokines involved in CD8+ tissue-resident T cell regulation and maintenance were specifically enriched in JIA synovial samples compared to peripheral blood, including IL-15, IL-12, IFN-γ; suggesting an environment conducive to the persistence of this population.

Conclusion

We confirm the existence of previously-described cell populations in synovial fluid from JIA patients and identify the enrichment of CD8 T cells with a tissue-resident signature. Further study is required to identify the significance or functional implications of these populations.

Disclosure

C. Bolton: None. C. Rich-Griffin: None. C. Dendrou: None. C. Brown: None. H. Al-Mossawi: None. A. Croft: None. L. Wedderburn: Consultancies; L.R.W. receieved a consultancy fee from Pfizer unrelated to this work. Grants/research support; L.R.W. declares support from AbbVie, GSK, Pfizer, Sobi, and UCB to the CLUSTER Consortium.