bluuebunny/crossref_metadata_2025_split huggingface dataset:
E025 A complex case of giant cell arteritis presenting with visual loss, tongue and scalp infarction and multiple cranial neuropathies
Authors: Jade Judith , Barradell Victoria , Coote Annabel 
Published: April 2023

Abstract

Background/Aims
Giant cell arteritis (GCA) is the most common of the vasculitides. Visual loss due to vascular occlusion is a well recognised complication of GCA. It is important to remember that other ischaemic complications can occur, leading to atypical clinical features. We present a case of a patient with GCA who did not complain of headaches, but presented with at least five cranial neuropathies, including a right optic neuropathy, ischaemic ulceration of her tongue and a large area of scalp necrosis.

Methods
We present the results of our patient case review and literature search.

Results
An 82-year-old retired landlady who was an ex-smoker presented with haemoptysis and was diagnosed with non-small-cell lung cancer. She was treated with chemotherapy and radiotherapy in 2020, then had possible disease progression on CT thorax in 2021. She developed thigh pain, swallowing difficulties and decreased oral intake in 2021, and subsequently developed severe oral ulceration, dehydration and delirium and was admitted to the frailty unit. On examination she had a very large scalp lesion, oral lesions, and multiple cranial neuropathies (right optic neuropathy, right partial third nerve, right seventh nerve, recurrent laryngeal, and bilateral twelfth nerve palsies). Her anterior ischemic optic neuropathy meant she could only see hand movements with the right eye. Blood tests revealed anaemia of chronic disease and a CRP of over 200 mg/L. Ultrasound scan of the temporal arteries showed a halo sign confirming the diagnosis of GCA. CT angiography showed vertebral artery wall thickening and stenosis. She was treated with intravenous methylprednisolone followed by high dose oral prednisolone, and started on methotrexate. She improved symptomatically. The cranial neuropathies resolved and the area of scalp necrosis and ischemic tongue ulceration healed, but vision was not regained. Her inflammatory markers never normalised and she was subsequently started on tocilizumab, with the approval of her oncologist. Her ESR and CRP quickly fell to normal. It remains to be seen how biologic treatment may impact her malignancy.

Conclusion
This case highlights multiple atypical presenting features of GCA and the need for a high index of suspicion to treat early and avoid irreversible visual loss. A review of the literature reveals that cranial neuropathies secondary to ischemia can be the presenting feature in GCA and multiple cranial neuropathies can occur. This is the first case in the literature, that we are aware of, which involves this number of cranial neuropathies alongside extensive scalp and tongue infarction. These features have all resolved with treatment, with the exception of unilateral permanent visual loss.
Disclosure
J. Jade: None. V. Barradell: None. A. Coote: None.
E031 A rare case of diffuse osteosclerosis
Authors: Mohamed Sarah , Phillips Abimbola , Thomas Joegi , Makkuni Damodaran 
Published: April 2023

Abstract

Background/Aims
Diffuse skeletal fluorosis is a common condition in countries with high content of fluoride in the ground water like India and China. Skeletal fluorosis in the UK is rare and presents with non-specific findings such as back pain, elevated ALP or bone sclerosis. We describe a case referred from haematology for investigation of osteosclerosis.

Methods
74-year-old female presented to the emergency department on several occasions with recurrent abdominal pain, severe low back pain and swelling of her lower limbs. Her mobility had been reduced due to previous femoral nerve injury. Blood tests on admission showed mild macrocytic anaemia. Alkaline phosphatase, renal, liver function tests and electrolytes were within normal range. Furthermore, calcium, phosphate, vitamin D and parathyroid hormone levels were within normal limits. CT CAP was done and no obvious or sinister cause for abdominal pain was found. However, it showed diffuse bony sclerosis. Bone densitometry DXA scan results as follows: T-score of + 1.6 in the hip and +1.7 in the neck of femur, they were unable to scan the lumbar spine as the patient was unable to tolerate positioning for the scan. This confirmed relatively raised bone density for patient’s age despite having risk factors for osteoporosis like previous wrist fracture, strong family history of osteoporosis, early menopause following total hysterectomy at age 41, coeliac disease and multiple falls due to poor mobility.

Results
A bone marrow aspirate and trephine biopsy and immunophenotyping were carried out and did not demonstrate any evidence of myeloid or lymphoid neoplasm. The next line of investigations included an isotope bone scan and blood tests including serum tryptase, uric acid and fluoride levels. Fluoride level came back as 411 which is 8-times more than the normal value. Patient mentioned that she uses fluoride toothpaste and brushed her teeth at least 10-14 times/day. X-ray of the hands showed no evidence of periosteal reactions or sclerosis. We recommended that she use fluoride free toothpaste and referred her for a dental opinion. While there is no effective therapeutic agent to reduce fluoride levels, patient was encouraged to use fluoride free toothpaste and also consult with her dentist for further evaluation.

Conclusion
This case emphasises the need for a comprehensive history and maintaining a high index of suspicion of skeletal fluorosis when encountered with a patient with a high bone mineral density (BMD) score on the DXA scan or diffuse skeletal sclerosis. The increase in bone mineral density is thought to be related to fluoride’s enhanced osteoblastic activity.
Disclosure
S. Mohamed: None. A. Phillips: None. J. Thomas: None. D. Makkuni: None.
E046 Clinical triage and targeting education to improve quality of referrals
Authors: MacPhie Elizabeth , Fish Sarah M , Madan Ayesha , Rao Chandini , Horton Sarah 
Published: April 2023

Abstract

Background/Aims
Referral quality has been an ongoing challenge over the years. We have delivered education sessions to primary care, trialled a referral proforma and co-produced referral pathways with our clinical commissioning group. The changing workforce of primary care is important to acknowledge with more referrals now coming from nurse practitioners, pharmacists, paramedics, physician associates and first contact practitioners (FCP). This project evaluates the impact of clinical triage to help identify if we can better target education to improve the quality of referrals and the patient pathway.

Methods
Consultant-led triage of every rheumatology referral was introduced in July 2021. Referrals are reviewed on the electronic referral system and accepted, returned or redirected to another service. To ensure consistency standard responses have been developed. All triage outcomes are recorded in a spreadsheet alongside the referring GP practice and professional role of the referring clinician. Referral rates per 1000 patients were calculated, adjusting for population served and high and low referring practices identified.

Results
In January to June 2022 we received 1194 referrals; 1054 were from primary care. Table 1 shows the breakdown of referrals and triage outcome by referring clinician.

Conclusion
Review of triage data has helped to understand how we can target education to improve the quality of referrals. We have identified that nurse practitioners and FCPs would benefit from education focusing on what should be referred to rheumatology and what information is important to include in a referral. Many referrals were returned with a request that the patient be examined and helping clinicians understand the value of examining patients is another important theme we identified. Next steps are to organise a workshop for nurse practitioners and FCPs and to visit the high referring practices who also have higher numbers of returned referrals.
Disclosure
E. MacPhie: None. S.M. Fish: None. A. Madan: None. C. Rao: None. S. Horton: None.
E052 Implementation of a disease modifying anti-rheumatic drug blood monitoring software: 8 years of experience in a single centre
Authors: Paul Anindita , Bromilow Laura , Eckersley Dean 
Published: April 2023

Abstract

Background/Aims
Who is responsible for the blood monitoring of disease modifying anti-rheumatic drugs (DMARDs)? It may be Primary Care, Specialist Rheumatology teams or a combination. In Greater Manchester, it was deemed good practice to initiate ‘amber’ conventional synthetic DMARDs (cs-DMARDs) by Rheumatology and then transfer to Primary Care under shared care after 3 months or when blood monitoring was stable. ‘Red’ drug prescribing and monitoring for targeted synthetic DMARDs (ts-DMARDs) and biologics (b-DMARDs) would remain under Specialist care long-term. In 2013, the process began for the Rheumatology team at Bolton to take on the ‘in house’ initiation of cs-DMARDs and the implementation of formal shared care. It would not be possible to manage this with paper slips of pathology results and shared care booklets, as the workload and safety implications would be un-manageable for our small team. The Rheumatology department purchased blood monitoring software, allowing only abnormal results or concerning trends to filter through. The implementation of this new way of working required collaborative working with the Informatics, Laboratory, Software and Rheumatology teams, as well as Primary care. At the time, the likely numbers of patients monitored and the workload of abnormal results/trends was unknown. Here we share our experience of software-based rheumatology DMARD monitoring in a district general hospital setting, serving a population of nearly 300 000.

Methods
Roles and responsibilities were allocated across the multidisciplinary, administration/clerical and informatics teams. A workflow and weekly timetable of activity and cross cover for leave was implemented. Two ‘super-users’ (AP/LB) undertook the initial testing of the software with informatics support (DE) and still work closely to address any data flow issues, software updates and testing related to emerging DMARDs. The patient cohort included those starting ‘amber’ DMARDs (to the point of shared care), stabilization following a dose increase and those remaining on specialist only ‘red’ DMARDs. Since 2019, a monthly monitoring report has allowed a more detailed documentation of workload.

Results
There were 518 patient licenses in use December 2019, 589 in 2020 and 635 in 2021. In the pre-pandemic year of 2019, 203 patients were newly registered or re-registered. Abnormal results and concerning trends were ‘flagged’ by the software for 719 patients. 263 shared care agreements were generated for monitored DMARDs.

Conclusion
This workload data relates to the use of software-based DMARD monitoring in a Rheumatology department, where patient flows relate to 1 whole time equivalent Rheumatology Consultant per 100 000 population. Centres considering taking on a similar form of DMARD monitoring can extrapolate this data for their population and patient flow to guide regarding staffing needs.
Disclosure
A. Paul: None. L. Bromilow: None. D. Eckersley: None.
E054 Rheumatic autoimmune diseases following COVID-19 infection: an observational study in Iraqi Kurdistan Region
Authors: Jalal Aryan M , Albarzinji Niaz 
Published: April 2023

Abstract

Background/Aims
In December 2019, a new type of novel coronavirus (COVID-19) was identified in Wuhan, China. The likelihood of developing an autoimmune and/or rheumatic diseases in COVID-19 survivors is high and a serious matter. The acute SARS-CoV-2 infection may unmask previously undiagnosed rheumatic conditions. We aimed to study rheumatic autoimmune disease manifestations diseases following COVID-19 infection survival.

Methods
The study was an observational case series study. The data collection was carried out in Iraqi Kurdistan region between the 1st of July 2021 and 20th of March 2022. Seventy-five patients were included: the patients who previously had confirmed COVID-19 infection who developed symptoms of rheumatic autoimmune diseases post COVID-19 cure. The study was conducted via a rigorous evaluation by two rheumatologists. Patients were investigated by (ESR (mm/h) and CRP (mg/L), some autoimmune screen panel for suspecting rheumatological disease patients were sent for ANA, anti-CCP (U/ML) and rheumatoid factor (IU/M) L. Then, patients were diagnosed according to the classification criteria for suspected autoimmune diseases and those with exacerbation were evaluated clinically and by laboratory; rheumatoid arthritis by DAS28, systemic lupus erythematosus by C3, C4.

Results
A total of seventy-five participants post-COVID-19 infection were enrolled in this study. Age of the participants was 47.15 ±16.18 SD, more of the participants were female (69) out of 75. For most of the patients the ESR were high with p value of 0.012, which was statistically significant. ANA was high titre in SLE patients which was (3.05±2.4) and in antiphospholipid syndrome p-value was significant at 0.042, Anti-CCP were positive in RA patients and in those with exacerbation of RA (44±10, 31.7±5.7 respectively), DAS28 was (4.95±0.59) moderate and high disease activity in patients with exacerbations. C3, C4 were low in patients with exacerbation of SLE (0.47±0.22, 0.03±0.01, respectively). Most of the patients developed symptoms post-COVID-19 between 4-10 weeks (37 participants).

Conclusion
Rheumatic autoimmune diseases presenting post-COVID-19 survival most commonly were systemic lupus erythematous followed by rheumatoid arthritis. and previous autoimmune diseases presented with exacerbation.
Disclosure
A.M. Jalal: None. N. Albarzinji: None.
E069 Antagonism of IL-27 bioactivity
Authors: Green Lara 
Published: April 2023

Abstract

Background/Aims
Rheumatoid arthritis (RA) is a chronic autoimmune disease that results in joint damage and bony destruction. The inflammatory process is central in the pathology of RA, with cytokines mediating the inflammatory response. Cytokines, in particular IL-6, have been targeted as treatments for RA. The IL-27 cytokine is part of the IL-6 family, and acts via the same signalling pathway. IL-27 can have the same function as IL-6, but can also antagonise IL-6. The presence of ectopic lymphoid structures (ELSs) is associated with more severe disease and worse joint damage, and is also associated with low IL-27 levels. Hence, targeting IL-27 may aid in severe RA and offer a novel treatment option. Aims: 1) To generate a recombinant IL-27 antagonist, 2) to determine the concentration and bioactivity of IL-27 antagonist, 3) to test the bioactivity of IL-27 antagonist as an inhibitor of IL-27 activity.

Methods
Chinese hamster ovarian (CHO) cells were transduced with adenovirus to express the IL-27 antagonist. The concentration of these IL-27 antagonists was determined using ELISA. To determine the bioactivity of the IL-27 antagonist, both acute leukaemia cell lines (THP1 cells) and cells from C57BL/6J mice were treated with human IL-27 and IL-27 antagonists and STAT3 activation determined using flow cytometry and ELISA.

Results
THP1 cells treated with both 5ng/ml and 15ng/ml of human recombinant IL-27 showed STAT3 activation, with 29.4% and 30.7% of cells being STAT3 positive respectively. Cells that remained untreated only showed STAT3 activation in 2.54% of cells, indicating IL-27 has a role in the activation of the STAT3 pathway. STAT3 activation was not witnessed in CD4 cells from C57BL/6J mice treated with human recombinant IL-27. Furthermore, the recombinant IL-27 antagonist was not seen to be able to antagonise the actions IL-27 and alter STAT3 activation.

Conclusion
In conclusion, this study has demonstrated that IL-27 treatment of cells can cause increased STAT3 activation, however we were unable to demonstrate that the IL-27 antagonist was able to inhibit the actions of IL-27. IL-27 shows great potential for use as a novel therapy, since both its pro and anti-inflammatory functions can be targeted, however there needs to be much more information about the action of IL-27 as this is still largely unknown. Future studies could aim to optimise the protocol in order to understand the bioactivity of the IL-27 antagonist, thus understanding its role in altering STAT3 activation.
Disclosure
L. Green: None.
E076 To investigate the mechanisms of action of novel compounds ex vivo in cells obtained from the inflammatory synovium of patients with rheumatoid arthritis
Authors: Turner Madeleine 
Published: April 2023

Abstract

Background/Aims
To investigate the ex vivo effect of JAK1/2 inhibitor baricitinib on the expression of pro-inflammatory mediators in normal and RA diseased fibroblasts stimulated with oncostatin M (OSM) as well as in cells derived from RA inflamed synovial membrane cells (RA SMC).

Methods
RA SMC and fibroblast like synoviocytes (RA FLS) were isolated from synovial membranes of patients undergoing surgery using enzymatic digestion and cell propagation. Normal fibroblast-like synoviocytes (FLS) and normal human skin fibroblasts (HSF) were purchased from commercial sources. All fibroblasts were cultured in media supplemented foetal bovine serum and antibiotics in incubators using established laboratory procedures, and serum-starved prior to stimulation with OSM with or without baricitinib. RA SMC were cultured in the presence of baricitinib without OSM as they spontaneously release pro-inflammatory cytokines. Levels of mRNA and proteins of the various inflammatory cytokines (IL-6, IL-8, MCP-1, CCL5 and IP-10) were determined by qPCR, ELISA and MSD. Western blot was used to measure levels of phospho-JAK specific components.

Results
qPCR analysis found that baricitinib inhibited OSM induced IP-10 and MCP-1 mRNA expression but not RANTES mRNA expression in normal human skin fibroblasts and RA FLS. In normal FLS however, baricitinib was found to inhibit RANTES and MCP-1 mRNA expression, but not IP-10 mRNA expression. Expression levels of IL-8 were too low for accurate conclusions to be drawn in all cell types apart from the RA SMCs, and in this case baricitinib did not inhibit IL-8 expression. ELISA results showed that baricitinib inhibited OSM induced MCP-1 expression in all cell types investigated. OSM induced IL-6 expression was inhibited in RA FLS but no other cell type.

Conclusion
Baricitinib inhibits OSM stimulated pro-inflammatory cytokine production through JAK1, 2. It has a varying inhibitory effect on the production of different cytokines: it is shown to particularly inhibit MCP-1. Baricitinib was found to have no effect on the production of spontaneously released pro-inflammatory cytokines in RA mixed cell populations.
Disclosure
M. Turner: None.
E080 Catheter-related venous thrombosis in patients with systemic sclerosis: a case series
Authors: Giacomassi Chiara , Eldaw Tahlil , Vigneswaran Shivanee , Debres Anja , Denton Christopher , Ong Voon 
Published: April 2023

Abstract

Background/Aims
Systemic sclerosis (SSc) is characterised by endothelial dysfunction and vasculopathy, which may lead to venous thrombosis. Here, we report four cases of extensive venous thrombosis of the upper limbs and right atrium associated with implantable venous access devices (port-a-cath) in patients with a diagnosis of SSc, who presented to our specialist centre between 2018 and 2022.

Methods
We retrospectively reviewed four patients with SSc and port-a-cath-associated thrombosis who presented to the Department of Rheumatology, Royal Free Hospital NHS Trust between 2018 and 2022. All patients are diagnosed with systemic sclerosis according to the 2013 ACR/EULAR classification criteria.

Results
Three patients were diagnosed with a port-a-cath-associated thrombosis in 2022, and one in 2018. Two patients had limited cutaneous SSc with positive anti-centromere antibodies, and 2 had diffuse subset with anti-U3RNP antibodies. All patients had a right-sided port-a-cath that had been in-situ for at least 3 years. Two patients were diagnosed with right atrium thrombus (measuring 2.2 and 3 cm respectively), one patient with an internal jugular vein and right subclavian thrombosis, and one with a left subclavian thrombosis. None had a history of previous thromboembolic event. A full thrombophilia screen was negative in 2 patients, and is pending in the others. Of note, 2 patients had COVID-19 infection within the 3 months prior the thrombotic event. 1 patient had tocilizumab administered through the line, 1 rituximab and IVIG, the other 2 had prostanoids only.

Conclusion
We described four recent cases of port-a-cath-associated thrombosis of the upper limbs and right atrium in SSc patients with no previous history of thrombosis. This highlights the increased risk of thrombosis related to long term indwelling catheters in SSc and demonstrates the potential interplay between covid microvasculopathy and the associated thrombotic risks reported with both ACA and antiU3RNP antibodies in SSc. We note that from previous reports the relative lower risk of adverse outcomes in SSc patient receiving parenteral nutrition. Further research into frequency of port-a-cath-related thrombosis in SSc patients is warranted, especially with use of prostanoids, and adequate screening and non-invasive follow up might be needed to avoid life-threatening thromboembolic complications.
Disclosure
C. Giacomassi: None. T. Eldaw: None. S. Vigneswaran: None. A. Debres: None. C. Denton: None. V. Ong: None.
E084 Analysis of the Early Inflammatory Back Pain Clinic at the Royal National Hospital for Rheumatic Diseases
Authors: Cowan Matthew , Sengupta Raj 
Published: April 2023

Abstract

Background/Aims
The Royal National Hospital for Rheumatic Diseases (RNHRD) has a long-standing history of excellence in the care and management of axial spondyloarthritis (axSpA). Research has shown that early diagnosis of axSpA is associated with health-related benefits. However, because diagnosis earlier in the disease trajectory can be clinically challenging, there is often a significant delay. New referrals of possible axSpA are reviewed in the early inflammatory back pain clinic, and this audit of the demographics and diagnoses of clinic attendees aims to follows-up the patients post clinical evaluation to assess the frequency of missed diagnoses.

Methods
Electronic medical records of 100 consecutive new referrals attending for back pain between 03/09/2015 and 27/07/2016 were retrospectively reviewed. The information abstracted included sex, age, smoking status, investigations, and diagnosis. Clinic letters and follow-up records at the Royal United Hospitals until July 2022 were reviewed.

Results
The average age of the patients was 37.4 years; 42% were male and 58% female. The diagnostic breakdown of the 100 patients is presented in Table 1.
Of the 21 patients diagnosed with axSpA, 14 (66%) were human leukocyte antigen B27 (HLA-B27) +ve; compared to only 7 (8.8 %) of the 79 patients who did not have an axSpA diagnosis. The electronic records of these 7 HLA-B27+ve patients were reviewed in July 2022, and no indication of a subsequent axSpA diagnosis was found; notably, one patient subsequently developed enteropathic arthritis. Of the 71 HLA-B27-ve patients categorised as having a non-inflammatory cause of their symptoms, three were re-referred to the RNHRD, but none were diagnosed with axSpA.

Conclusion
We found no evidence of a subsequent diagnosis of axSpA in patients who did not have the disease diagnosed at the time of initial referral. This audit identified a small cohort of HLA-B27+ve patients without evidence of axSpA; a more in-depth analysis of this patient group is planned.
Disclosure
M. Cowan: None. R. Sengupta: None.