bluuebunny/crossref_metadata_2025 huggingface dataset:
P162 Socio-economic status in patients with juvenile idiopathic arthritis in the United Kingdom
Authors: Tan Yvonne , Costello Ruth , Hyrich Kimme , Humphreys Jenny
Published: April 2022

Background/Aims

Juvenile idiopathic arthritis (JIA) is a chronic inflammatory condition affecting children and young people (CYP) with onset <16 years, and may be associated with long-term joint damage and disability if treatment is delayed. CYP with JIA from low socio-economic status (SES) have been shown to have poorer performance in school activities and self-reported functional assessments compared to those from higher SES groups, however SES association with incidence of JIA has rarely been explored. Therefore, this study aimed to investigate the association between socio-economic status and incident JIA in England.

Methods

CYP <16 years with a first JIA diagnostic code were identified in the Clinical Practice Research Datalink (CPRD) between the years 2000 and 2018, using a pre-defined codelist and were validated through linkage to Hospital Episode Statistics. Age- and gender-matched controls were identified at a ratio of 4:1. SES was defined using quintiles of indices of multiple deprivation (IMD). Descriptive analyses compared baseline characteristics (age, gender, age of first JIA code, IMD, region) in cases and controls. Logistic regression investigated the association between IMD quintiles in JIA cases compared to controls, adjusting for baseline characteristics, and linear regression analysed the association between age at first JIA code and IMD quintile.

Results

There were 1649 cases and 4748 controls included in this study. Most cases (1048, 64%) were female with median (interquartile range (IQR)) age at first JIA code 8.49 (4.20-12.05) years. Amongst cases, males were older at first JIA code, median (IQR) 9.80 (6.09-12.68) years, compared to females 7.55 (3.45-11.70) years. In both cases and controls, the proportion of CYP was highest in the most deprived quintile (IMD 1), 375/1649 (22.7%) and 1108/4738 (23.4%) respectively, and proportions remained similar in the remaining quintiles. There was no association identified between IMD quintile and occurrence of JIA, the multivariable odds ratio (95% CI) comparing most and least deprived groups was 0.98 (0.83-1.17). However, amongst cases there was an association between older age at diagnosis and higher SES [ß(95%CI) 0.15 years (0.01-0.29)] such that CYP in the IMD 5 quintile (highest SES) were on average 8 months older at their first JIA code compared to CYP in IMD 1.

Conclusion

This study found CYP from higher socio-economic groups appeared to be diagnosed at an older age than those from the least deprived backgrounds. Factors such as later presentation to healthcare after disease onset, delays in referrals to specialist care, delays in diagnosis by specialist care or differences in population demographics across IMD quantiles may all contribute to the older age of diagnosis in high SES group. Further research is required to explore these factors.

Disclosure

Y. Tan: None. R. Costello: None. K. Hyrich: None. J. Humphreys: None.
P163 The cytokine horror: Kawasaki disease shock syndrome complicated with macrophage activation syndrome
Authors: Rajagopala Lavanya , Jagoda Jayathri , Gunatilaka Mahesh L
Published: April 2022

Background/Aims

Kawasaki disease (KD) is an acute febrile systemic vasculitis of unknown aetiology, affecting medium-sized vessels. It often occurs in children less than 5 years old. During the acute phase of the disease, less than 5% of children develop hypotension and shock. This severe form is called Kawasaki Disease Shock Syndrome (KDSS). Macrophage activation syndrome (MAS) is a heterogenous syndrome complex encompassing a broad spectrum of clinical features. MAS associated with KDSS is an extremely rare life threatening entity that warrants a high index of clinical suspicion, prompt and proper management.

Methods

We present our experience in the dilemmas and challenges faced in the diagnosis and management of KDSS complicated with MAS in a poor resource setting. It is our first experience of encountering MAS in KDSS.

Results

A 9-month-old baby with fever and a maculopapular rash involving palms and soles for 5 days duration was transferred from a peripheral hospital. A preliminary diagnosis of acute Kawasaki disease was made with the classical features that included the rash, cracked lips, pedal oedema, BCG scar inflammation, perianal excoriation and irritability. The fever did not settle following standard therapy that included three doses of intravenous immunoglobulins 2g/kg, aspirin and low dose dexamethasone. The child quickly began to decompensate. She developed cold clammy skin with hypotension on the 8th day of the illness. There was evidence of hepatosplenomegaly with transaminitis. 2D Echo revealed normal coronary vasculature without any dilatations or strictures. However, there was evidence of myocarditis supported by diffuse T inversions in the anterolateral leads and a highly positive troponin titre. Investigations revealed aa leucocytosis (22,100/dl), an abrupt drop in platelet count (350,000/dl to 80,000/dl), red blood cells, haemoglobin (11g/dl to 8g/dl) and erythrocyte sedimentation rate 95mm/hr to 50mm/hr. Hyperferritinemia, hypertriglyceridemia and hypofibrinogenemia supported the diagnosis of MAS. Blood culture was sterile. Initial bone marrow biopsy was inconclusive. High dose dexamethasone 10mg/m2/day and cyclosporine 2mg/kg/day was initiated. The circulatory collapse was managed with fluid boluses and a low dose adrenaline infusion. Subsequently there was dramatic clinical improvement and her cell counts and transaminases normalised on day 15.

Conclusion

We emphasise the importance of early recognition of refractory MAS in pediatric populations with rheumatic diseases and we suggest early initiation of cyclosporin as a rapid and effective treatment option in the poor resource settings where the ideal management with anakinra is beyond the realms of possibility.

Disclosure

L. Rajagopala: None. J. Jagoda: None. M.L. Gunatilaka: None.
P171 Reasons for stopping biologic and non-biologic disease-modifying treatments in children and young people managed by a tertiary paediatric rheumatology centre
Authors: Hartley Karen , Sen Ethan S
Published: April 2022

Background/Aims

The Paediatric Rheumatology Department at Newcastle upon Tyne Hospitals looks after children and young people (CYP) across the North East and Cumbria region. The centre treats around 220 patients (250 active prescriptions) using homecare services to deliver injectable medicines. These prescriptions are for five medicines: methotrexate, adalimumab, anakinra, tocilizumab and etanercept. This project aimed to analyse the reasons treatments were stopped and identify potentially-modifiable factors to optimise treatment.

Methods

Patients were identified from the departmental database. Cases were included if they stopped one of the 5 subcutaneous treatments during the period 1st July 2020 to 30th June 2021. Demographic details, the underlying diagnoses and reasons for stopping treatment were extracted from electronic patient records.

Results

During the 12-month period analysed, 109 prescriptions were stopped. This represented 104 unique patients (56% female). The commonest indications for treatment were juvenile idiopathic arthritis in 80% (87/109), idiopathic uveitis in 11% (12/109) and autoinflammatory diseases in 5.5% (6/109). The following treatments were stopped: methotrexate in 47 patients, adalimumab in 43, etanercept in 7, tocilizumab in 6 and anakinra in 6. The reasons for stopping prescriptions are shown in Table 1.

After excluding 17 cases whose prescriptions were taken over by another unit, 92 prescriptions were stopped for clinical reasons. Of these, 28% were stopped due to side effects. All 19 patients who suffered nausea or vomiting, and all 5 patients with abnormal liver function tests, were receiving methotrexate. The 2 patients who stopped treatment due to neutropenia were both receiving tocilizumab. Almost 23% (21/92) of prescriptions were stopped due to inefficacy. All cases were switched to another biologic drug.

Conclusion

A substantial proportion of subcutaneous disease-modifying treatments were stopped due to inefficacy or side effects. Stopping and switching treatments represents a period of uncertainty for CYP and their families, with potential impact on disease control and quality of life. Management could be improved by a stratified medicine approach, using biomarkers to predict patients at highest risk of side effects or most likely to respond to a particular treatment. This is a key aim of current research.

Disclosure

K. Hartley: None. E.S. Sen: None.
P172 NRAS Young Voices Panel
Authors: Wilson Debbie L
Published: April 2022

Background/Aims

NRAS understands that inflammatory arthritis affects people of all ages. In early 2020 NRAS decided that a voice of the JIA community was needed to inform and provide guidance on a framework for JIA services at NRAS. The group would also empower younger people with RA, who are often frustrated by the perception that the condition “only affects elderly people”.

Methods

NRAS recruited a group of young people with lived experience of JIA and RA to reflect the target population of the JIA service age range. The group were drawn from diverse backgrounds and now it includes representation from the Cabinet office, health care professionals and experts in youth engagement and digital marketing together with PhD students and creative professionals.

Results

Throughout the last 12 months the Young Voices Panel have developed and prioritised the service framework for young people with inflammatory arthritis and JIA-at-NRAS. One of the main priorities was peer-to-peer support and now having the JIA Parents online group we can offer this to parents, alongside our other new JoinTogether online groups as well (e.g. 18 to 35year olds, Working with inflammatory arthritis, Parenting with inflammatory arthritis and Exercise and Back to Sport with inflammatory arthritis). The Young Voices have shared their stories and experiences in Facebook Live sessions during RAAW, Wear Purple for JIA in November 2020 and recently for the new #WearPurpleForJIA Wellbeing Week in June 2021. Some of the Young Voices were also part of the focus group for the new Medicines in JIA booklet which has now been launched. They spoke with parents during our zoom event Coping with JIA during COVID-19 in March to show parents that their children can thrive in life whilst learning to manage to the ups and downs that RA/JIA has. They also shared their experiences with young people at the young person’s event Navigating your future with inflammatory arthritis in April discussing how to tell friends and partners about their disease and where to find support at university and explain to employers about the conditions. They have also helped NRAS focus on what young people may want from the new JIA digital membership and participated in raising awareness for young people with JIA/RA podcasts and articles for Enable magazine.

Conclusion

The Young Voices Panel have been an integral part of NRAS and will continue to develop the service framework and be the voice of young people with JIA/RA. Feedback from events: “Thank you for sharing your stories and experiences, you are inspirational”. “I am so glad that young people are getting of a voice with regard to being diagnosed with arthritis”.

Disclosure

D.L. Wilson: None.
P184 Are there any ethnic differences in the response to Janus Kinase Inhibitor therapy for the treatment of rheumatoid arthritis?
Authors: Jubber Ameen , Hussain Wajith H. Z , Moorthy Arumugam
Published: April 2022

Background/Aims

Baricitinib is an oral, synthetic Janus Kinase inhibitor. It has become a commonly used drug in the treatment of rheumatoid arthritis (RA), both as combination therapy and monotherapy. Previous studies have compared drug efficacy in different ethnicities, but no studies have compared the efficacy of baricitinib for the treatment of RA in different ethnicities. Given the large South Asian population in Leicestershire, we reviewed our cohort of RA patients on baricitinib to see whether there is any difference in drug response rates between the Asian and Caucasian cohorts.

Methods

This was a retrospective study. Patients included were those under the care of rheumatology at University Hospitals of Leicester (UHL) with a diagnosis of RA and either receiving baricitinib, or had received it in the past. Data was collected using the UHL IT systems, clinic letters, and pharmacy records. In addition to ethnicity, we reviewed patient age, gender, concurrent DMARDs used, previous biologics used, baseline and post-treatment DAS28, dropout from therapy, baseline biochemical assays (anti-CCP and RF status) and radiographic findings. Independent T-test was used to compare continuous data and the Pearson Chi-Square test was used to compare categorical data. SPSS was used to analyse the results.

Results

120 patients were included in the analysis and data was analysed with PFA. There was no statistically significant difference in the mean DAS28 at baseline (Asian 5.17 vs Caucasian 4.65, p value 0.107) and post-treatment (Asian 2.83 vs Caucasian 3.33, p value 0.404) (see table 1). Comparing both ethnicities, there was no statistically significant difference in previous biologics used, the presence or absence of anti-CCP and RF, and radiographic findings of erosions. Limitations of the study include the low proportion of post treatment DAS28 values recorded and absence of data comparing primary or secondary biologic failure.

Conclusion

This is the first study of its kind and found no significant difference in baricitinib response between the Asian and Caucasian cohorts. Future studies are needed to evaluate this subject further. Such data is important as it can contribute to a body of evidence that may in future help inform clinical decision making.

Disclosure

A. Jubber: None. W.H.Z. Hussain: None. A. Moorthy: None.
P189 A longitudinal study of psychological predictors of response to adalimumab in patients with rheumatoid arthritis
Authors: Raza Nahrin , Nair Nisha , Plant Darren , Hyrich Kimme , Morgan Ann W , Isaacs John , Wilson Anthony G , Barton Anne
Published: April 2022

Background/Aims

The treatment of rheumatoid arthritis (RA) has improved since the introduction of targeted biological disease-modifying antirheumatic drugs (bDMARDs), such as adalimumab. However, no treatment options for RA are universally effective. Psychological factors may have an important role in treatment response. Published data reported that pre-treatment psychological variables such as an increased treatment necessity belief correlated with better adherence to adalimumab in RA patients. This current study aimed to investigate whether psychological factors correlate with change in 4V-DAS28-CRP as well as subcomponents of the score in adalimumab-treated RA patients.

Methods

Psychological, clinical and demographic data and DAS28 scores were collected pre-treatment, and at 3,6, and 12-months post-treatment, as part of a prospective national study, BRAGGSS. Sixteen pre-treatment clinical and psychological variables were tested for association with treatment response at 3 and 12 months. Multiple linear regression models were analysed and a Bonferroni corrected p-value of < 0.003 was applied at each interval to assess significance.

Results

Data was available for 501 patients at 3 months follow up. Pre-treatment Beliefs about Medicine Questionnaire (BMQ) scores with regards to treatment necessity (P = 0.001), and two domains of the Illness Perception Questionnaire (IPQ), which were treatment belief and treatment control (P = 0.0001), correlated with the change in DAS28 by 3 months (Table 1). At 12 months, data were available for 362 patients. The treatment belief domain of the pre-treatment Illness Perception Questionnaire correlated with the change in tender joint count (P = 0.001) (Table 1). At 12 months no other sub-components correlated with the psychological variables.

Conclusion

Pre-treatment scores with regards to Illness Perception and Beliefs about Medicine correlated with 4V-DAS28-CRP response at 3 months. Of the subcomponents of DAS28, the psychological variables showed the highest correlation with tender joint count; as the tender joint count receives the highest weighting in the 4 variable DAS28 score, this may confound the assessment of treatment response. It will be necessary to undertake further analyses to assess the predictive value of these potentially modifiable psychological variables identified, but to also understand factors contributing to these psychological scores, such as the role of severe disease itself on psychological beliefs.

Disclosure

N. Raza: None. N. Nair: None. D. Plant: None. K. Hyrich: None. A.W. Morgan: None. J. Isaacs: None. A.G. Wilson: None. A. Barton: None.
P190 Unsupervised automated clustering of mass cytometry data identifies unique CD4+ T cell subsets in rheumatoid arthritis
Authors: Mulhearn Ben , Marshall Lysette , Sutcliffe Megan , Barton Anne , Raychaudhuri Soumya , Viatte Sebastien
Published: April 2022

Background/Aims

Treating rheumatoid arthritis (RA) continues to rely heavily on a trial-and-error approach. The potential of deep immunophenotyping of peripheral blood remains to be proven in tailoring treatment to an individual’s biological variability. Here, we aim to compare the analysis of single-cell data using an unsupervised automated clustering approach with conventional biaxial gating to identify T cell imbalances RA.

Methods

Unstimulated and stimulated peripheral blood mononuclear cells from 10 RA and 10 healthy controls (HC) were immunophenotyped by mass cytometry (CyTOF) using a T-cell panel. Firstly, a conventional biaxial gating strategy and standard definitions of Th cell subsets were applied to compare subset frequencies between cases and controls. Secondly, publicly-available unsupervised analytical workflows [1] were used to automatically define cell clusters hypothesis-free. Their differential abundance between RA and HC was assessed with Mixed-effects modelling of Associations of Single Cells (MASC)[2].

Results

Applying unsupervised automated clustering algorithms clearly identifies unusual marker combinations and a Th1/Th17 : Th2/Treg imbalance in RA in this small sample size, while conventional analytical techniques fail to do so. Cell clusters, including a classical pro-inflammatory Th1-like (IL-2+ T-bet+) subset and a novel IL-17+ T-bet+ subset were significantly enriched in RA compared to HC (odds ratio 4.9, P = 6.6 x 10-4; odds ratio 5.5, P = 1.1 x 10-2). In contrast, a FoxP3+ IL-2+ HLA-DR+ cluster was amongst those reduced in RA (odds ratio 0.2, P = 2.0 x 10-7).

Conclusion

Automated clustering techniques are more powerful than conventional analytical methods when analysing high-dimensional single-cell data. A hypothesis-free approach allows identification of novel cell clusters with unexpected marker combinations, such as T-bet and IL-17, which might otherwise be overlooked with biaxial gating.

References[1] Robinson MD et al. 2017. F1000Research.[2] Fonseka CY et al. 2018. Sci. Transl. Med.

Disclosure

B. Mulhearn: None. L. Marshall: None. M. Sutcliffe: None. A. Barton: None. S. Raychaudhuri: None. S. Viatte: None.
P206 A survey to explore how our rheumatoid patients used the British Society for Rheumatology electronic patient-reported outcome measure platform
Authors: Fish Sarah J , Brazendale Jane , Greenall Carol , MacPhie Elizabeth
Published: April 2022

Background/Aims

Patient-reported outcome measures (PROMs) engage and empower patients, providing a powerful tool in understanding the impact of healthcare interventions. Where PROMs are embedded in clinical practice, trends can be recognised, informing treatment decisions. In August 2020, the British Society for Rheumatology launched its ePROM platform; in March 2021 recruitment of our Rheumatoid arthritis (RA) patients commenced. Six months later, we were keen to explore patient engagement, confidence completing self-reported DAS28 and determine if patients viewed ePROMs as enhancing their care.

Methods

The ePROMs platform was discussed with patients with stable RA. To date 88 RA patients are registered, each was sent an immediate DAS28 followed by six monthly DAS28’s. In September 2021 a survey was created on SurveyMonkey® and emailed to patients registered. The survey gathered: age, employment status, disease duration and treatment pathway, previous use of apps to record DAS28, utilisation of ePROMs to record DAS28, response times to DAS28 completion requests, engagement with DAS28 patient video before completion of DAS28, time taken to complete DAS28 electronically, confidence assessing own joints, experience of ePROMs influencing patient involvement in own care, benefits of ePROMs for patient, suggestions for improvement.

Results

The response rate was 23.8% (21), with 42.8% (9) of respondents having completed a DAS28 ePROM. See table of ePROM survey results. The majority of respondents were 65+ years old with 10+ years’ disease duration and unemployed/retired. Under half had completed a self-reported DAS28, of those over 70% had viewed the patient video and over 90% felt confident completing the DAS28, with 90% feeling that ePROMs increased their involvement in care. Patients commented that with ePROMs they feel well connected to the team. One patient reported, ‘Exact answers are given. I feel face to face, l say that I feel fine when I don't’.

Conclusion

Only 42.8% of our RA patient respondents have engaged with the ePROMs platform and completed a DAS28, of which 90% of patients were confident completing a self-reported DAS28. For respondents who have engaged with ePROMs, their experience has been positive and empowered them to feel involved in their care.

Disclosure

S.J. Fish: None. J. Brazendale: None. C. Greenall: None. E. MacPhie: Other; EM is the North West Rheumatology Club secretary and meetings have been sponsored by MSD, UCB, Abbvie and Lilly.
P209 Subgroups of refractory rheumatoid arthritis and difficult to treat features highlight differences in comorbidity and smoking history - a single-centre observational study
Authors: Rogers George , Tan Yvonne , Shukla Rudresh , Gorodkin Rachel , Parker Ben , Bruce Ian , Humphreys Jennifer , Barton Anne , Hyrich Kimme , Bruce Ellen , Ho Pauline , Buch Maya H
Published: April 2022

Background/Aims

Limited therapeutic efficacy and ‘difficult to treat’ (D2T) characteristics mean some rheumatoid arthritis (RA) patients cycle through multiple therapies to achieve their treatment targets. The aim of this study was to evaluate for subgroups of refractory RA (RefRA) in a real-world population and describe D2T characteristics.

Methods

Patients on their third or more targeted therapy were identified from the electronic health record (EHR) at Manchester Royal Infirmary. Demographic and clinic information were obtained. Raised disease activity was identified as DAS28ESR>3.2. Findings of power Doppler joint synovitis on ultrasound (PDUS) if undertaken within a month of clinical assessment. These data were used to categorise patients as raised disease activity and PD positive (persistent inflammatory RefRA, PIRRA) or raised disease activity in the absence of PD (non-inflammatory RefRA, NIRRA). Descriptive analyses were used to describe the characteristics in the two groups. Missing data were counted as negative observations.

Results

82 patients who were on a third advanced therapy or higher were identified. 41/82 (50%) had raised disease activity and 32/41 (78%) had MSUS performed. 18/32 (56%) of these were PIRRA and 14/32 (44%) were NIRRA. Table 1 details demographic and clinical characteristics in the two groups as well as the controlled RefRA population.

The PIRRA group had more erosive disease [13/18 (72%) PIRRA vs 8/14 (57%) NIRRA] and smoking history [9/18 (50%) of PIRRA vs 5/14 (35%) of NIRRA]. The PIRRA group also had greater levels of co-morbidity but previous bDMARD toxicity was comparable between the two groups.

Whilst the DAS28ESR was higher in the PIRRA group, there were only marginal differences in the tender and swollen joint counts and CRP compared to NIRRA.

The controlled RefRA population as expected showed notably lower DAS28-ESR and individual components although more similar to the PIRRA group with regards to comorbidity.

Conclusion

Just over half of the ref-RA cohort with raised disease activity had MSUS-detected synovitis and may be regarded as PIRRA. The PIRRA group had higher disease activity although joint counts and even acute phase were comparable. Higher smoking history and comorbidity appear to distinguish PIRRA and NIRRA in this cohort.

Disclosure

G. Rogers: None. Y. Tan: None. R. Shukla: None. R. Gorodkin: None. B. Parker: None. I. Bruce: None. J. Humphreys: None. A. Barton: None. K. Hyrich: None. E. Bruce: None. P. Ho: None. M.H. Buch: None.
P225 A case of PL-7 positive amyopathic dermatomyositis associated with autoimmune liver disease
Authors: Lee Martin A , Scott-Lang Victoria , Raja Kaiser , Sahu Sharmishta
Published: April 2022

Background/Aims

Amyopathic dermatomyositis (ADM) is a variant of dermatomyositis (DM) characterized by the presence of pathognomonic skin features of dermatomyositis but without clinical muscle weakness. ADM is believed to account for about 20% of cases of DM.

Methods

We present the case of a 40-year-old lady who presented in May 2020 with a 6-week history of periorbital oedema and a heliotrope rash. She had a past medical history of rectosigmoiditis diagnosed on colonoscopy in 2018 managed with mesalazine 2g once a day. Blood tests revealed a transaminitis (ALT 372 u/l, AST 201 u/l). Full blood count was normal as were inflammatory markers (ESR 4 mm/hr, CRP 2 mg/dl) and renal function (creatinine 63.9 umol/l). CK was normal (72 u/l). A non-invasive liver screen including hepatitis B and C, caeruloplasmin, alpha-fetoprotein and anti-mitochondrial antibodies was negative. A liver ultrasound was reported as normal. An ANA was positive and an ENA screen revealed positive RNP-68, S/RNP and anti-smooth muscle antibodies. Atypical pANCA antibodies were also positive. A myositis antibody screen revealed a positive PL-7 antibody. A skin biopsy of the affected area around the eye demonstrated prominent dermal periadnexal and perivascular chronic inflammation composed mainly of lymphocytes and plasma cells consistent with a connective tissue disease. A liver biopsy confirmed autoimmune liver disease (AILD). Tumour markers (CEA, CA 125 and CA 19-9) were normal and a CT scan of the chest, abdomen and pelvis to rule out occult malignancy was normal. Full lung function tests including transfer factor were normal. A diagnosis of PL-7 positive ADM with associated AILD was made.

Results

The patient’s ADM and AILD were successfully treated with a reducing course of budesonide (6mg three times daily for 4 months reduced to 3mg twice daily for four months then 3mg daily for 3 months) and mycophenolate mofetil 720mg twice daily. Currently, 16 months after her initial presentation, the patient’s ADM and AILD are in remission and she remains on mycophenolate mofetil 720mg twice daily.

Conclusion

Anti-PL-7 is one of a number of anti-aminoacyl tRNA synthetase (ARS) antibodies that are associated with DM, polymyositis (PM) and ADM. Other anti-ARS antibodies include Jo-1, PL-12, EJ, OJ, KS, ZO and YRS/HA. Antibodies to PL-7 have been reported to be associated with milder muscle involvement than antibodies to Jo-1. Antibodies to PL-7 have been associated with severe interstitial lung disease (ILD). An association between AILD and inflammatory myositis has been reported in the literature but only in a handful of case reports. To the best of our knowledge however, AILD has not previously been reported in PL-7 positive DM, PM or indeed ADM.

Disclosure

M.A. Lee: None. V. Scott-Lang: None. K. Raja: None. S. Sahu: None.
P246 Revision to musculoskeletal domain of BILAG-2004 index to incorporate ultrasound findings
Authors: Sandler Robert D , Vital Edward M , Prabu Athiveer , Riddell Claire , Mahmoud Khaled I , Teh Lee-Suan , Edwards Christopher J , Yee Chee-Seng
Published: April 2022

Background/Aims

The BILAG-2004 index grades musculoskeletal (MSK) inflammation as mild (C), moderate (B) and severe (A), based on clinical features. In a recent study, 27% of SLE patients with MSK pain have ultrasound (US) synovitis without swelling, and respond better to therapy than those with normal ultrasound. We aimed to revise the MSK domain of the BILAG-2004 index to improve its utility in modern practice.

Methods

An MSK sub-committee reviewed the existing BILAG-2004 MSK definitions and glossary, then re-analysed data from recent studies to propose revised definitions.

Results

The new definitions/changes are: (i) BILAG-C arthralgia (if US is not performed) is defined as a classical inflammatory symmetrical small-joint distribution of symptomatic joints on clinical assessment, based on the features most associated with US inflammation in a previous study. If US is performed, all previous BILAG-C patients are reclassified: (ii) BILAG-B now includes patients without swelling if they have significant synovitis/tenosynovitis on US in symptomatic joints/tendons; (iii) patients with arthralgia without swelling are scored as BILAG-D instead of C, if US of the symptomatic joints has been performed and is normal; (iv) a more detailed definition of functional impairment is provided for BILAG-A. Data from 221 symptomatic SLE patients (BILAG-A-C) in two recent studies were re-analysed in the light of the above changes. 121/221 (54.8%) were graded clinically as having arthralgia / myalgia (BILAG-C) on BILAG-2004 . Of these, 53/121 (43.8%) had evidence of synovitis (GS > 1 or PD > 0) or tenosynovitis (GS > 0 or PD > 0) on US. The effect on grading if US were performed on all BILAG C cases is shown in table 1.

Conclusion

Future versions of the BILAG index will allow the use of US to classify patients who are graded as mild inflammatory arthritis (BILAG-C) to moderate (BILAG-B) or non-inflammatory pain (BILAG-D/E). Although US is not required for all assessments it may be used when there is diagnostic uncertainty. This is in keeping with clinical practice in inflammatory arthritis care, and similar to use of other investigations (e.g. renal biopsy) that contribute to BILAG assessments of other systems.

Disclosure

R.D. Sandler: None. E.M. Vital: None. A. Prabu: None. C. Riddell: None. K.I. Mahmoud: None. L. Teh: None. C.J. Edwards: None. C. Yee: None.
P262 The occurrence and characteristics of severe pain in patients with axial spondyloarthritis
Authors: Jones Gareth T , Rotariu Ovidiu , Dean Linda E , MacDonald Alan G , Macfarlane Gary J
Published: April 2022

Background/Aims

Pharmacological management of axial spondylarthritis (axSpA) seeks to control inflammation. Even if successful, available evidence suggests that many patients continue to experience pain. The aim of the current study was to determine the prevalence and characteristics of severe pain among persons with axSpA.

Methods

The Scotland Registry for Ankylosing Spondylitis (SIRAS) collected clinical and patient-reported data from adults seen in secondary care in Scotland with a clinical diagnosis of ankylosing spondylitis. Questionnaires asked about severe pain (high pain intensity; high pain interference; and extreme/unbearable pain), lifestyle, and various aspects of health. The relationship between severe pain and clinical/patient-reported factors was assessed using logistic regression.

Results

929 participants had pain data available (73% male; median symptom duration 20yrs). High pain intensity and pain interference were more common (57% and 42%) than extreme/unbearable pain (11%). Prevalence did not differ with age, although women were less likely to report severe pain than men (Odds Ratios (ORs) 0.56-0.61) as were those with longer duration of education, and those from more affluent areas. The odds of severe pain increased with every 1 unit increase in BASFI (ORs 1.44-1.56). Strong associations were also seen with disease activity, spinal mobility, fatigue, poor sleep, and worse quality of life.

Conclusion

In axSpA, severe pain is common, with a clear socio-economic gradient and major impact on quality of life. Rheumatologists need to be aware of the large unmet need in terms of pain management in this patient group with around 1 in 9 patients reporting extreme/unbearable pain.

Disclosure

G.T. Jones: Grants/research support; Research grant income for biologics registers in axSpA and PsA from the British Society for Rheumatology. The Society receives income from AbbVie, Pfizer, UCB and Amgen to support these registers, current analysis funded by GlaxoSmithKline who were provided with a copy of the results prior to submission. O. Rotariu: None. L.E. Dean: None. A.G. MacDonald: None. G.J. Macfarlane: Grants/research support; Research grant income for biologics registers in axSpA and PsA from the British Society for Rheumatology. The Society receives income from AbbVie, Pfizer, UCB and Amgen to support these registers, current analysis was funded by GlaxoSmithKline who were provided with a copy of the manuscript prior to submission.
P285 The utility of FibroScan in the monitoring of methotrexate therapy in inflammatory arthritis patients
Authors: Amin Mohammad H , Justice Elizabeth
Published: April 2022

Background/Aims

Methotrexate (MTX) is a drug used to treat inflammatory arthropathies. A significant side effect of MTX is liver fibrosis. There are numerous ways to screen for liver fibrosis, liver biopsy being the gold-standard, but its invasive nature and accompanying adverse effects limits its utility as a screening tool. Guidelines advise the use of liver function tests, yet liver enzymes and synthetic liver function may be normal even in advanced disease. This leaves the question of the best screening tool for liver fibrosis unanswered. This data collection seeks to explore the utility of FibroScan in the screening of liver fibrosis in rheumatoid arthritis and psoriatic arthritis patients taking long-term methotrexate.

Methods

A hospital-based survey was conducted where electronic records of rheumatoid and psoriatic arthritis patients, who were referred for FibroScan due to being on long-term methotrexate therapy, were reviewed. Long-term MTX therapy was defined as MTX use of greater than ten years. The following data were collected: demographics, BMI, MTX duration, results of FibroScan, and impact on management. The primary outcome was to determine whether FibroScan altered the patients’ management as this has not been tested in the literature previously. ‘Change of management' was defined as reduction of MTX dose or cessation of MTX therapy. Three secondary outcomes were assessed. Firstly, relationship of BMI (body mass index) with FibroScan result. FibroScan result consisted of median elastography and controlled attenuation parameter (CAP). Secondly, relationship of BMI with impact on management. Finally, relationship of MTX therapy duration with impact on management.

Results

A total of 16 patients were identified. With regards to primary outcome, 29.4% of patients had their management changed secondary to FibroScan. In terms of secondary outcomes, positive correlations were found between BMI and median elastography and CAP. Mean BMI was greater in patients who had their management changed. Patients whose MTX was stopped had a greater mean MTX therapy duration.

Conclusion

These data show the utility of FibroScan to screen for liver fibrosis in patients on long-term MTX therapy. It recommends that practitioners consider FibroScan in patients who have been taking MTX for longer than 10 years, especially if they have associated risk factors such as high BMI.

Disclosure

M.H. Amin: None. E. Justice: None.
P287 The impact of restricted access to targeted therapies for rheumatoid arthritis on rheumatologists and the service they can provide
Authors: Walker David , Robinson Sandra
Published: April 2022

Background/Aims

Just under half of the clinical commissioning groups (CCGs) in England restrict the number of targeted therapies that can be prescribed for rheumatoid arthritis (RA) before an individual funding request (IFR) is required. We were interested to explore the impact of this on rheumatology services and rheumatologists themselves.

Methods

A national survey of rheumatologists who prescribe high-cost drugs was developed to explore prescribing restrictions by CCGs, the impacts on the service and the strategies adopted to mitigate this. Results from the survey were then used to develop an interview schedule for semi structured interviews with five rheumatologists to explore this in more detail.

Results

A total of 53 rheumatologists started the survey. 23 participants (44%) were restricted in their use of high-cost drugs. Restrictions were mainly by total number rather than mode of action. For 46, (88%) restrictions were communicated through local guidelines. 41% had additional local restrictions either through their pharmacy (5%), local pathways (10%) or a multi-disciplinary team meeting (MDT; 27%). 51% routinely discussed high-cost drugs with colleagues, with 21% discussing only complicated patients. The effect on the service was rated: a lot = 43%; moderately =24%; and a little or not = 7%. 95% said it affected their practice e.g., persisting with ineffective therapy. Blueteq was used by 69%; 29% were negative or very negative about it, 29% were neutral, and 23% were positive about it. 19% had it completed by someone else. Strategies for mitigating the restrictions included: 43% of participants had used a foot instead of a hand in the DAS28; 68% had changed diagnostic category to justify treatment; 51% had negotiated a local pathway. Three themes emerged from the semi-structured interviews. Theme 1) Persistence with partially effective therapy was used to avoid running out of options. There was genuine distress about the poor service that was being delivered. Theme 2) IFRs do not work but negotiating alternative pathways with the CCG can. Theme 3) Bluetec is an extra burden in the process but is OK if you do not have to fill it in yourself.

Conclusion

Restricting the number of targeted therapies that can be prescribed for RA has a detrimental effect on the service and the prescribers involved which often results in persistence with partially effective therapies. Continuing partially effective therapies is not satisfactory if there may be more effective therapies available.

Acknowledgements: On behalf of the EVA-RA Joint Working Group.

Disclosure

D. Walker: Consultancies; Galapagos, Eli Lilly. S. Robinson: None.
P289 The effectiveness of interventions to reduce psychological distress in patients with autoimmune rheumatic conditions: a systematic review of effectiveness
Authors: Hulley-Mills Jamie , Sliverthorne Christine , Jones Bethan , Hooper Jo , Robson Joanne , Dures Emma , Daniels Jo , Thompson Miles , Ndosi Mwidimi
Published: April 2022

Background/Aims

Autoimmune rheumatic conditions are multisystem chronic disorders associated with increased psychological distress. Consequences include poor medication adherence and high levels of disease activity. Psychological interventions may reduce distress and change health behaviours. We aimed to determine the effectiveness of interventions for psychological distress in patients with autoimmune rheumatic conditions.

Methods

This was a systematic review of effectiveness which included randomised controlled trials of psychological interventions in adults with autoimmune rheumatic conditions. We searched MEDLINE, BNI, CINAHL, EMBASE, EMCARE, PsycINFO, NICE Evidence and The Cochrane Library. Two reviewers screened titles and abstracts and assessed the methodological quality of the included studies using Cochrane Risk of Bias 2 tool. Data were extracted by one reviewer and checked by a second. In each study, the effectiveness data were determined by extracting the reported means (and standard deviation) for each group and calculating the standardised mean differences using RevMan 5.4 software (The Cochrane Collaboration, 2020). The data on primary outcomes (anxiety and depression) are reported here.

Results

The search identified 96 studies. The process of screening and assessing for eligibility resulted in 20 studies for inclusion. Of these, one study had an overall low risk of bias and 19 had ‘some concerns’, mainly due to inadequate blinding and no information on pre-specified analysis plan. Only eight studies reported the effects on anxiety and 10 studies reported on depression. These studies included 919 patients in total. Most interventions were based on cognitive behavioural therapy and the context of delivery ranged from face-to-face individualised treatment to online group therapy. They often included education on topics such as stress and fatigue. Interventions often trained participants to develop skills in relaxation, problem-solving, and thought reframing. Goal setting and action planning were common to help participants make behaviour changes and apply their learning. Table 1 summarises disease group, type of intervention, comparator, outcome measure, and effects.

Conclusion

Conclusion: Most interventions were effective in reducing anxiety and depression in autoimmune rheumatic conditions. However, given the clinical heterogeneity and ‘some concerns’ about the included quality of studies, more work is needed to understand the mechanisms of the intervention effectiveness.

Disclosure

J. Hulley-Mills: None. C. Sliverthorne: None. B. Jones: None. J. Hooper: None. J. Robson: None. E. Dures: None. J. Daniels: None. M. Thompson: None. M. Ndosi: None.
P298 Ocular manifestations in a giant cell arteritis cohort
Authors: Smith Sonja Mansfield , Al-Hashimi Mustafa , Ducker Georgina , Jones Colin , Mukhtyar Chetan
Published: April 2022

Background/Aims

The ophthalmic features of giant cell arteritis involve a spectrum of severity, from transient symptoms to bilateral visual loss due to anterior ischaemic optic neuropathy or, less commonly, central retinal or cilioretinal artery occlusion. Data of reported ocular involvement are varied, with a range of 10-70%. The variation may be related to the specialty of the unit reporting the data. We run an interdisciplinary service in a tertiary academic unit and report a complete picture of the frequency and nature of ocular involvement in GCA.

Methods

The records of 350 consecutive patients, diagnosed objectively on the basis of biopsy or imaging, were reviewed. In our centre, the hospital pathway mandates all individuals with suspected GCA to have a formal ophthalmology assessment including visual acuity, pupil exam and full dilated fundus assessment. We recorded the common signs and symptoms using a structured form which included ophthalmologic symptoms and examination findings, along with symptoms commonly associated with GCA: scalp tenderness, headache, jaw claudication, shoulder girdle stiffness, anorexia, night sweats, weight loss, and fever.

Results

From January 2012 to September 2021, 350 individuals were diagnosed with GCA by biopsy, ultrasonography or positron emission tomography. The mean age was 74 ± 7.7 years. 237 (68%) of patients were females. 99 (28%) presenting with GCA had visual symptoms and or signs. 34 had mono-ocular and two had binocular loss of vision. A summary of the key visual symptoms and signs are shown in Table 1. Only five patients with visual symptoms did not have any symptoms commonly associated with GCA.

Conclusion

We report the frequency of visual involvement in one of the largest cohorts of individuals with GCA. 28% have ocular symptoms. Partial or total monocular field loss occurred in 12% of cases. 1.4% of patients may be presenting with visual symptoms as the only sign of GCA. Rarely, permanent visual loss may occur without any other manifestation of GCA.

Disclosure

S. Mansfield Smith: None. M. Al-Hashimi: None. G. Ducker: None. C. Jones: None. C. Mukhtyar: None.
P300 Comparison of birth rates, gestational diabetes and non-standard maternal deliveries between Takayasu arteritis patients and the England and Wales population
Authors: Groves David J , Rutter Megan , Lanyon Peter C , Odingo Myron , Grainge Matthew J , Bythell Mary , Aston Jeanette , Stevens Sarah , Hannah Jennifer R , Mason Justin , Pearce Fiona A
Published: April 2022

Background/Aims

Takayasu arteritis (TAK) is a chronic, inflammatory vasculitis of the aorta and primary branches, particularly prevalent in women of childbearing age. We studied pregnancy outcomes for women with TAK compared to the general population.

Methods

All people in England with an ICD-10 diagnostic code for TAK 1997/98 to 2020/21 were identified from Hospital Episode Statistics (HES) using National Congenital Abnormality and Rare Disease Registration Service (NCARDRS) data (CAG 10-02(d)/2015). To improve precision of estimated date of diagnosis, we used an English hospital case register of 122 TAK patients. We analysed all HES admissions occurring after their clinician-confirmed diagnosis date and before their first TAK ICD code in HES to identify codes which indicated likely TAK-related activity, and then applied these to the whole NCARDRS cohort. Maternity-related ICD-10 and OPCS-4 code admissions after the estimated diagnosis date were extracted and age-specific values were calculated for number of births, TAK patient years and birth rates. ONS published national data was used for comparison.

Results

We identified 1,437 people with TAK. Between 2001 and 2021, there were 133 babies born (129 singleton, 2 twin births, no stillbirths) to 85 mothers. Mean maternal age at first delivery was 32.2 years (SD 5.3, median 33.2), above the UK mean of 27.8, with age-specific birth rates compared to the 2019 general population (see table). Overall birth rate in women with TAK was 68% of the national England/Wales rate. Gestational diabetes (GDM) was present in 13/131 (9.9%) but large variation in UK prevalence estimates makes national comparison difficult. Mothers with GDM had a median age of 37 and delivery was by C-section in 9/13 cases. For non-GDM, 50% (95% CI: 41%, 59%) were C-section; this is significantly higher than the UK level of 26%. 12% were vacuum and/or forceps assisted deliveries, approximately equal to the general population.

Conclusion

The birth rate in women with TAK is lower than the general population and C-section rates higher. Causes are complex and might include patient choice, reduced fertility related to cytotoxic treatment, or physician recommendation, highlighting the importance of equitable access to specialist pre-conception counselling for people with rare autoimmune diseases.

Disclosure

D.J. Groves: None. M. Rutter: None. P.C. Lanyon: None. M. Odingo: None. M.J. Grainge: None. M. Bythell: None. J. Aston: None. S. Stevens: None. J.R. Hannah: None. J. Mason: None. F.A. Pearce: None.
Tetralogy of Fallot
Authors: Chase Julia R. , Fliehman Maci , LeGal Michael , Spicer Whitney , Rogers Julia L.
Published: April 2022

The Physician Fee Schedule was updated in 2021 by the Centers for Medicare and Medicaid Services. A case study on Tetralogy of Fallot, the most common cyanotic congenital heart defect, is presented with guidance in billing the office visit to reflect the current guidelines.