bluuebunny/crossref_metadata_2025 huggingface dataset:
E004 Inpatient admission pathway for patients with rheumatological disorders
Authors: Hall Andrea , Botros George , Panthakalam Sathianathan
Published: April 2023

Background/Aims

Rheumatology is a complex specialty covering many conditions of varying severity, from muscle pain through inflammatory arthritis such as Rheumatoid arthritis (RA) and connective tissue diseases. Most of the conditions can be managed in an outpatient/day case setting. However, acutely ill patients require safe and prompt inpatient management including specific intravenous infusions. This need to be done urgently and cannot wait to be accommodated through the Infusion unit at our hospital. Historically Medicine Acute Admission Unit has been the route to bring in these patients. However, operational bed pressures faced challenges leading to instances of delayed treatment with complications including fatality. This led to creating a direct inpatient admission pathway to the specialist ward.

Methods

Ward Matron designed the following robust pathway for direct patient admission to our specialist Rheumatology ward, Jevington ward. This was implemented in February 2022 after discussion and agreement with Clinical Lead consultant, pharmacist, clinical site managers and other colleagues.

Rheumatology team and nurses covered the ward during working hours and by the on-call team out of hours. The overall responsibility remained with the rheumatology team. The referrals accepted only after completing appropriate paperwork. Patients carried out Lateral Flow Test (LFT) at home prior to admission. We ensured negative results and followed the Trust COVID 19 screening protocols. Subsequent screenings were done according to the updated guidelines. The planned assessment and treatments were carried out by the ward team complying with BSR/EULAR Guidelines, infusion protocols such as standard and continuous Iloprost Infusion Protocols of the Trust.

Results

We assessed the delay in patient's admission, length of stay, patient outcome and experience after implementing the pathway. The significant change has been in the time to admit; from two weeks in 2018 & 19 to two days this year. This is reflected in the patient feedback. All our acutely ill patients were assessed, treated and discharged promptly on this specialist ward.

Conclusion

This pathway allowed safe and prompt treatment, prognosis and excellent experience for acutely ill patients with rheumatological disorders. This additionally enabled reduced length of stay supporting financial sustainability of the Trust.

Disclosure

S. Panthakalam: None.
E005 An audit of demographics, symptoms & investigations: Part of the survey of GCA in the SEHSCT during the COVID-19 pandemic
Authors: Tan Han Jun , Brennan David , Masih Claire , Joyce Amy , French Blathnaid
Published: April 2023

Background/Aims

Giant cell arteritis (GCA) is the most common vasculitis in adults aged over 50 years old with the highest incidence among persons aged 70-79. It is more commonly seen in female patients. Most cases have been reported in whites of Northern European descent. A broad range of symptoms can be reported including headache, jaw or tongue claudication, visual disturbances, PMR and other systemic features including weight loss, fever and sweats. In recent years new evidence has emerged regarding the investigation and treatment of GCA. This audit is to review the demographics, symptoms and investigations of patients who presented to the Rheumatology Department in SEHSCT with features concerning for possible GCA.

Methods

Retrospective collection of data from January 2020 to July 2021 using the regional Electronic Care Record NI with reference to presentations, investigation results, clinic records and follow-up letters.

Results

70 patients were included (24 males and 46 females). Mean age was 72 years old. Table 1 shows the percentages of clinical symptoms reported. All patients investigated had an ESR (mean 57.8) and CRP (mean 54.1) checked. 43 patients had ANCA checked with 3 positive results. 40 patients underwent CT brain with 2 abnormalities reported unrelated to GCA. TA ultrasound was performed on one occasion with a positive result demonstrating “halo” sign recorded. 6 patients underwent CTPET with 3 diagnoses of LVV and 1 of PMR. 70 TAB performed with 12 positive results and 4 ‘suggestive’ of GCA.

Conclusion

Our cohort of patients demonstrated demographics similar to the current global geographic trends in GCA. There are a broad range of clinical symptoms that can present in GCA, none of which are entirely specific or pathognomonic. Clinical diagnosis is based on clinical symptoms, signs and laboratory tests, each of which are imperfect markers for GCA. Our audit demonstrated that the use of additional confirmatory diagnostic tests including temporal artery ultrasound and CTPET was being under-utilized in the SEHSCT. Use of these tests may improve the diagnostic yield in this challenging condition. As a result of this audit, a quality improvement project to provide a rapid access GCA pathway is being designed.

Disclosure

H. Tan: None. D. Brennan: None. C. Masih: None. A. Joyce: None. B. French: None.
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

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

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

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

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

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

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

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

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

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.
E091 Improving recognition and referrals for axial spondyloarthritis in primary care
Authors: Hamilton Daniel
Published: April 2023

Background/Aims

It is estimated there is an 8.5-year delay from onset of symptoms to diagnosis of axial spondyloarthritis. The condition is poorly recognised in the United Kingdom. An audit of a general practice with 7,500 patients revealed only 7 patients living with the diagnosis, of which only 2 were diagnosed before the age of 45. Estimates suggest this figure should be closer to 35 to 40. In the 12 months prior to the audit, only 2 referrals were sent to rheumatology suspecting axial spondyloarthritis. The short-term aim of this study was to improve the recognition and referrals for axial spondyloarthritis in primary care from 2 per year to 15. The long-term goal is to increase the number of patients living with the diagnosis from 7 to 37.

Methods

EMIS alerts and pop-ups were created for patients, aged 20-45, presenting with “low back pain” or a similar coding to increase clinicians' clinical suspicion index. The alerts and pops included a hyperlink to the SPADE tool to facilitate reasoning. This was consolidated with an education webinar to refresh General Practitioners and Advanced Nurse Practitioners on the diagnostic criteria for axial spondyloarthritis.

Results

Following implementation of alerts and hyperlinks to the SPADE tool, general practitioners' confidence and knowledge of knowing when to suspect, assess and refer axial spondyloarthritis increased from 1.9, 1.7 and 2 to 4.8, 4.4 and 4.2 on a 5 point self-rated Likert scale. In the 12 months following the intervention, number of referrals had increased from 2 per year to 11.

Conclusion

This study has highlighted various findings. It has highlighted an insufficient number of referrals to rheumatology suspecting axial spondyloarthritis in order to maintain the suspected population of a general practice. This is likely linked to poor awareness and screening of the condition. It has also highlighted that clinicians in primary care respond positively to training for knowing when to suspect, assess and refer on for axial spondyloarthritis. However, this only measured a short-term change in knowledge and further testing is required to assess long term change. Furthermore, combined with education and the use of pop up and alerts has increased the awareness and referrals for axial spondyloarthritis from primary to secondary care. Future audits are required in the long term to assess for success in the long term goal.

Disclosure

D. Hamilton: None.
E094 Challenges in the diagnosis and investigation of giant cell arteritis during COVID-19 pandemic restrictions: does the Southend pretest probability score help to guide clinical diagnosis?
Authors: Gew Jessie , Loughrey Sarah , Wray Maria
Published: April 2023

Background/Aims

During 2020-2021 many usual hospital services were affected as focus turned towards managing COVID-19. Elective outpatient surgery ceased and rheumatology staff were redeployed to covid wards. This reduced the availability of temporal artery biopsy (TAB) and temporal artery ultrasound (TAUS) to aid in diagnosing giant cell arteritis (GCA). The rheumatology team making diagnoses of GCA or not-GCA were doing so often based entirely on clinical and laboratory findings. We aimed to determine referral patterns and investigations for suspected GCA during the covid pandemic, compare diagnoses at 6 months after initial assessment and retrospectively apply the Southend Pretest Probability Score (PTPS) and correlate with the diagnosis of GCA or not-GCA.

Methods

We reviewed all electronic referrals for suspected GCA from July 2020 - June 2021. Clinical details and investigations reviewed. PTPS applied giving a result of low, intermediate or high probability of GCA.

Results

84 referrals for suspected GCA over 12 months. 20 diagnosed GCA/large vessel vasculitis (LVV), 64 not-GCA. Peak referral months Nov 2020 and April 2021 with 13 and 16 referrals. Lowest in October 2020 with 1 referral. 57 female, 27 male. Mean age 70.1 years. 19% male referrals diagnosed GCA, 26% female diagnosed GCA. All LVV and PMR diagnoses were female. 27 TAUS, 6 TAB, 7 PET, 13 CT, 3 MRI performed. 30 patients had no additional investigations. Of 20 GCA; 14 had supporting investigations, 6 were clinical diagnoses. All GCA diagnoses were consistent at 6 months. One not-GCA case was subsequently diagnosed with LVV on CTPET. All other not-GCA diagnoses were consistent at 6 months. The PTPS was retrospectively applied based on available clinical information in all except 2 cases, and compared to GCA/not-GCA diagnosis and investigations undertaken.

Conclusion

Referral numbers for suspected GCA were higher than previous years however the number of actual GCA diagnoses was similar. With limitations on diagnostic investigations due to covid, diagnoses of GCA with and without additional tests were accurate at 6 months, and correlated with a high probability score. The PTPS is a therefore valuable clinical tool in the assessment of GCA.

Disclosure

J. Gew: None. S. Loughrey: None. M. Wray: Honoraria; M.W. has received honoraria from Roche- Chugai, Novartis.
Duloxetine, Gabapentin, and the Risk for Acute Myocardial Infarction, Stroke, and Out-of-Hospital Death in Medicare Beneficiaries With Non-Cancer Pain
Authors: Corriere Meghan A. , Dickson Alyson L. , Daniel Laura L. , Nepal Puran , Hall Kathi , Plummer W. Dale , Dupont William D. , Murray Katherine T. , Stein C. Michael , Ray Wayne A. , Chung Cecilia P.
Published: April 2023

Objective:

Duloxetine is a serotonin-norepinephrine reuptake inhibitor prescribed for musculoskeletal and other forms of chronic pain. Its dual pharmacologic properties have the potential to either raise or lower cardiovascular risk: adrenergic activity may increase the risk for acute myocardial infarction (AMI) and stroke, but antiplatelet activity may decrease risk. Gabapentin is another nonopioid medication used to treat pain, which is not thought to have adrenergic/antiplatelet effects. With the current emphasis on the use of nonopioid medications to treat patients with chronic pain, assessing cardiovascular risks associated with these medications among high-risk patients is important.

Materials and Methods:

We conducted a retrospective cohort study among a 20% sample of Medicare enrollees, aged 65 to 89, with chronic pain who were new users between 2015 and 2018 of either duloxetine (n = 34,009) or gabapentin (n = 233,060). We excluded individuals with cancer or other life-threatening conditions at study drug initiation. The primary outcome was a composite of AMI, stroke, and out-of-hospital mortality. We adjusted for comorbidity differences with time-dependent inverse probability of treatment weighting.

Results:

During 115,668 person-years of follow-up, 2361 patients had the composite primary outcome; the rate among new users of duloxetine was 16.7/1000 person-years compared with new users of gabapentin (21.1/1000 person-years), adjusted hazard ratio = 0.98 (95% CI: 0.83, 1.16). Results were similar for the individual components of the composite outcome as well as in analyses stratified by demographic and clinical characteristics.

Discussion:

In summary, cohort Medicare patients with non-cancer pain beginning treatment with duloxetine had rates of AMI, stroke, and out-of-hospital mortality comparable to those who initiated gabapentin.
The Telehealth Tradeoff
Authors: Bento Samantha P. , Stewart Catherine , Conroy Caitlin , Smith Allison , Reece Leah , Jervis Kelsey , Cole-Lewis Yasmin , Logan Deirdre , Randall Edin T.
Published: April 2023

Objectives:

The COVID-19 pandemic required intensive interdisciplinary pain treatment (IIPT) programs to shift to virtual models of care. This study used a multimethod approach to examine outcomes of a pediatric hybrid IIPT program (50% in-person treatment and 50% synchronous video-based telehealth) and assessed staff experiences while treating within this model.

Materials and Methods:

Patients (M=14.73, SD=2.04; 79% female) reported pain intensity, functional disability, and psychological factors (anxiety, depressive symptoms, fear of pain, pain catastrophizing, social functioning) at admission, discharge, and short-term follow-up. Differences in treatment outcomes at discharge and short-term follow-up between patients who participated in the hybrid IIPT model (n=42) during the pandemic and those who participated in the traditional in-person model before the pandemic (n=42) were examined. Quantitative assessments of staff burnout and perceived effort and qualitative assessments exploring staff perspectives about the challenges and advantages of the hybrid IIPT model were gathered.

Results:

Youth in both groups made significant improvements across the majority of treatment outcomes; however, the hybrid group reported higher levels of pain at discharge and anxiety at follow-up. The majority of IIPT staff reported moderate to high levels of overall burnout, and almost half reported high levels of emotional exhaustion. Staff highlighted various challenges and benefits associated with treating within the hybrid model.

Discussion:

When considering telehealth as a treatment tool for youth with complex chronic pain, it is crucial to leverage its benefits while addressing its challenges for patients and providers.
Implementing Pediatric Surviving Sepsis Campaign Guidelines: Improving Compliance With Lactate Measurement in the PICU
Authors: Mazloom Anisha , Sears Stacey M. , Carlton Erin F. , Bates Katherine E. , Flori Heidi R.
Published: April 2023

OBJECTIVES:

The 2020 pediatric Surviving Sepsis Campaign (pSSC) recommends measuring lactate during the first hour of resuscitation for severe sepsis/shock. We aimed to improve compliance with this recommendation for patients who develop severe sepsis/shock while admitted to the PICU.

DESIGN:

Structured, quality improvement initiative.

SETTING:

Single-center, 26-bed, quaternary-care PICU.

PATIENTS:

All patients with PICU-onset severe sepsis/shock from December 2018 to December 2021.

INTERVENTIONS:

Creation of a multidisciplinary local sepsis improvement team, education program targeting frontline providers (nurse practitioners, resident physicians), and peer-to-peer nursing education program with feedback to key stakeholders.

MEASUREMENTS AND MAIN RESULTS:

The primary outcome measure was compliance with obtaining a lactate measurement within 60 minutes of the onset of severe sepsis/shock originating in our PICU using a local Improving Pediatric Sepsis Outcomes database and definitions. The process measure was time to first lactate measurement. Secondary outcomes included number of IV antibiotic days, number of vasoactive days, number of ICU days, and number of ventilator days. A total of 166 unique PICU-onset severe sepsis/shock events and 156 unique patients were included. One year after implementation of our first interventions with subsequent Plan-Do-Study-Act cycles, overall compliance increased from 38% to 47% (24% improvement) and time to first lactate decreased from 175 to 94 minutes (46% improvement). Using a statistical process control I chart, the preshift mean for time to first lactate measurement was noted to be 179 minutes and the postshift mean was noted to be 81 minutes demonstrating a 55% improvement.

CONCLUSIONS:

This multidisciplinary approach led to improvement in time to first lactate measurement, an important step toward attaining our target of lactate measurement within 60 minutes of septic shock identification. Improving compliance is necessary for understanding implications of the 2020 pSSC guidelines on sepsis morbidity and mortality.
The Role of Excision for Treatment of Chromoblastomycosis: A Cutaneous Fungal Infection Frequently Mistaken for Squamous Cell Carcinoma
Authors: Rolon Alyssa M. , Tolaymat Leila M. , Sokumbi Olayemi , Bodiford Katherine
Published: April 2023

BACKGROUND

Chromoblastomycosis is an uncommon fungal infection of the skin caused by a variety of dematiaceous fungal species that is typically contracted through direct inoculation into the skin.

OBJECTIVE

To collect and examine data pertaining to the clinical presentation and management of patients with chromoblastomycosis.

METHODS

Through a retrospective study, a pathology medical record search was performed from January 2004 to December 2020 at a single institution.

RESULTS

A total of 9 patients were identified. Seven of 9 cases occurred in solid organ transplant recipients. All cases were located on the extremities. Six of 9 cases were clinically suspected to be squamous cell carcinoma. Seven of 9 cases were treated with surgical excision. Six of 9 patients were treated with oral antifungal medication. Four of 9 patients had received combination therapy. Eight of 9 patients had no recurrence of the disease after treatment.

CONCLUSION

Chromoblastomycosis presents as verrucous papules or nodules and may clinically and histopathologically mimic squamous cell carcinoma. Immunosuppression is likely a risk factor for the development of chromoblastomycosis. This study highlights the importance of clinical awareness of this disease's clinical presentation and prevalence in immunosuppressed patient populations.
Superficial Peroneal Nerve Schwannoma
Authors: Abebe Metasebia W. , Weldemicheal Hanna A.
Published: April 2023

Schwannomas are benign tumors of the nerve sheath that arise from the proliferation of active peripheral Schwann cells. Although schwannomas are the most common benign peripheral nerve sheath tumors, superficial peroneal nerve schwannomas are rare in published works of literature. We report a 45-year-old woman with a 4-year history of progressively worsening dull aching pain and paresthesia over the right lateral leg. Physical examination revealed a 4 × 3 cm firm palpable mass and a decreased touch and pain sensation over the lateral aspect of the right calf and dorsum of the foot. She also had an electric shock-like pain on palpation and percussion of the mass. Magnetic resonance imaging demonstrated a well-defined, oval, smooth-walled heterogeneous lesion beneath the peroneus muscle with avid postcontrast enhancement and a split fat sign. Fine needle aspiration cytology also suggested the diagnosis of schwannoma. Based on clinical findings of a mass, decreased sensation, and a positive Tinel sign on the dermatome of superficial peroneal nerve, surgical management was decided. Upon surgical exploration, a firm, glistening mass arising from the superficial peroneal nerve was identified, carefully dissected, and shelled out while maintaining continuity of the nerve. At the 5-month follow-up, the patient reported complete resolution of the pain and paresthesia. Physical examination revealed intact sensation in the lower lateral aspect of the right calf and dorsum of the foot. Therefore, surgical excision should be considered a reasonable option in the management of this rare condition, with most patients achieving good to excellent results.
Hallermann–Streiff Syndrome in Concordant Monozygotic Twins With Congenital Cataracts, Exudative Retinal Detachments, and One Case of Corneal Perforation Requiring Keratoplasty
Authors: Sims Deion T. , Mattson Nicole R. , Huang Laura C. , Lee Michele D. , Bly Randall A. , Gallagher Emily R. , Baran Francine M. , Cabrera Michelle T.
Published: April 2023

Purpose:

We describe the management of Hallermann–Streiff syndrome in monozygotic female twins with congenital cataracts, exudative retinal detachments, and 1 case of corneal descemetocele with associated dellen and subsequent perforation.

Methods:

This study was a case report and review of the literature.

Results:

Twins 1 and 2 exhibited all 7 cardinal characteristics of Hallermann–Streiff syndrome, presenting with spontaneous lenticular resorption, anterior uveitis, and glaucoma. They underwent bilateral cataract extraction with near total capsulectomy. Both twins experienced recurrent glaucoma, for which twin 1 underwent successful endocyclophotocoagulation in both eyes and twin 2 in the left eye alone. The fellow eye developed 2 sites of perilimbal corneal descemetoceles with associated dellen at the inferotemporal limbal corneal junction leading to spontaneous perforation of 1 site, requiring a full-thickness corneal graft. Both twins developed recurrent bilateral exudative retinal detachments unresponsive to oral prednisolone. Twin 1's last best-corrected visual acuity with aphakic spectacles was 20/260 in the right eye and 20/130 in the left eye at age 4 years and 8 months. Twin 2's last best-corrected visual acuity was 20/130 in each eye at age 4 years and 11 months, over a year after right eye penetrating keratoplasty.

Conclusions:

We describe 2 rare cases of Hallermann–Streiff syndrome in monozygotic twins complicated by corneal perforation requiring penetrating keratoplasty in 1 eye of 1 twin. Although corneal opacities have been described in this condition, this is the first case of corneal descemetocele in Hallermann–Streiff syndrome. The cornea was stabilized with a relatively favorable visual outcome over 1 year later.
Corneal Guttae After Descemet Membrane Endothelial Keratoplasty
Authors: Vasanthananthan Keamela , Bourgonje Vincent J. A. , Lie Jessica T. , Mulders-Al-Saady Rana , Groeneveld-van Beek Esther A. , de Leeuw Jacky , Miron Alina , Wijdh Robert H. J. , Stoutenbeek Remco , Melles Gerrit R. J. , Oellerich Silke , Kocaba Viridiana
Published: April 2023

Purpose:

The aim of this study was to report on the occurrence of corneal guttae after Descemet membrane endothelial keratoplasty (DMEK).

Methods:

In this retrospective case series, 13 eyes of 13 patients who underwent DMEK at 2 tertiary referral centers between 2007 and 2021 (average available follow-up 73 ± 52 months, range 18–174 months) and showed corneal guttae during postoperative examinations were included. Eye bank images were retrospectively reviewed.

Results:

Occurrence of guttae was observed by specular microscopy in 13 eyes. In 11 cases, presence of guttae was confirmed by confocal microscopy and in 1 case by histology. Five eyes showed an increase in guttae density during the postoperative course. Surgery indications were Fuchs endothelial corneal dystrophy (n = 11), pseudophakic bullous keratopathy (n = 1), and DMEK graft failure after allograft rejection (n = 1); the latter eye had shown no signs of guttae after primary DMEK. Two eyes with guttae required a repeat DMEK due to graft failure. At the last available follow-up, all 11 remaining eyes had clear corneas and 10 eyes had a best-corrected visual acuity of ≥0.9 (decimal). During donor cornea processing in the eye bank, no guttae were observed on the donor tissue.

Conclusions:

Corneal guttae can occur after DMEK including in eyes operated for indications other than Fuchs endothelial corneal dystrophy and most likely guttae were present on the donor graft but were not detectable by routine slit-lamp and light microscopy evaluation in the eye bank. Postoperative guttae density varies among patients and especially small isolated guttae do not seem to affect clinical outcomes.