bluuebunny/crossref_metadata_2025_split huggingface dataset:
O24 Risk of death during the 2020 UK COVID-19 epidemic and annual influenza seasons among people with vasculitis compared to the general population: a whole-population study using data from the NDRS and the RECORDER project
Authors: Rutter Megan , Lanyon Peter C , Grainge Matthew J , Hubbard Richard B , Peach Emily J , Bythell Mary , Aston Jeanette , Stevens Sarah , Pearce Fiona A 
Published: April 2021

Abstract
Background/Aims
To quantify the risk of death among people with vasculitis during the UK 2020 COVID-19 epidemic compared with baseline risk, risk during annual influenza seasons and risk of death in the general population during COVID-19.
Methods
We performed a cohort study using data from the National Congenital Anomaly and Rare Disease Registration Service (NCARDRS) under their legal permissions (CAG 10-02(d)/2015). Coded diagnoses for vasculitis (ANCA-associated vasculitis, Takayasu arteritis, Behçet's disease, and giant cell arteritis) were identified from Hospital Episode Statistics from 2003 onwards. Previous coding validation work demonstrated a positive predictive value &gt;85%. The main outcome measure was age-standardised mortality rates (ASMRs) for all-cause death. ONS published data were used for general population mortality rates.
Results
We identified 55,110 people with vasculitis (median age 74.9 (IQR 64.1-82.7) years, 68.0% female) alive 01 March 2020. During March-April 2020, 892 (1.6%) died of any cause. The crude mortality rate was 9773.0 (95% CI 9152.3-10,435.9) per 100,000 person-years. The ASMR was 2567.5 per 100,000 person-years, compared to 1361.1 (1353.6-1368.7) in the general population (see table).
The ASMR in March-April 2020 was 1.4 times higher than the mean ASMR for March-April 2015-2019 (1965.6). The increase in deaths during March-April 2020 occurred at a younger age than in the general population.
We went on to investige the effect of previous influenza seasons. The 2014/15 season saw the greatest excess all-cause mortality nationally in recent years, and there were 624 deaths in 38,888 people (6472.5 person-years) with vasculitis in our data (crude mortality rate 9640.8 (8913.3-10427.7); The ASMR was 2657.6, which was marginally higher than the ASMR among people with vasculitis recorded during March-April 2020 during the COVID-19 pandemic.
Conclusion
People with vasculitis are at increased risk of death during circulating COVID-19 and influenza epidemics. The ASMR among people with vasculitis was high both during the 2014/15 influenza season and during the first wave of the COVID-19 epidemic. COVID-19 vaccination and annual influenza vaccination for people with vasculitis are both important, regardless of patient age.
Disclosure
M. Rutter: None. P.C. Lanyon: Grants/research support; PCL has received funding for research from Vifor Pharma.. M.J. Grainge: None. R.B. Hubbard: None. E.J. Peach: Grants/research support; EJP has received funding for research from Vifor Pharma. M. Bythell: None. J. Aston: None. S. Stevens: None. F.A. Pearce: Grants/research support; FAP has received funding for research from Vifor Pharma..
P006 The potential of colour Doppler ultrasound in reducing temporal artery biopsies in suspected giant cell arteritis
Authors: Sidhu Arslan A , Nandagudi Anupama , Bharadwaj Anurag 
Published: April 2021

Abstract
Background/Aims
Giant cell arteritis (GCA) is an emergency. The initial treatment with high dose glucocorticoids (GC) is often started on clinical suspicion without waiting for temporal artery biopsy (TAB) results. Colour Doppler ultrasound (CDUS) is a simple, non-invasive test which is readily available. However, like any other ultrasound, it is operator dependent. Non-compressible ‘halo sign’ is the most specific abnormality on CDUS. British society for Rheumatology (BSR) guidelines advises to avoid TAB in patients with low clinical probability and negative CDUS as well as in high clinical probability and positive CDUS.
Methods
We adopted the quality improvement methodology for assessment. Retrospective data of suspected GCA patients was collected over the last two years. CDUS was introduced to investigative plan midway, after eleven months. Two rheumatology consultants were trained in CDUS. Results were compared before and after the introduction of ultrasound as a diagnostic tool. In collecting the data, our main focus for documentation was based on clinical symptoms, CDUS and TAB results. Patient were divided into high, medium and low probability groups based on clinical assessment by a rheumatologist. Final diagnosis was decided on the basis of clinical assessment at 6 months.
Results
It was a retrospective review from January 2018 to November 2019, 101 patients were referred with suspected GCA. Median age was 72 years (50 - 91 years) with male to female ratio of 1:3. Thirty five patients were referred in the first 11 months and 28.6% were diagnosed with GCA. CDUS and TAB was done in 20% and 49% of patients respectively. Sixty six patients were referred in the next 12 months after CDUS was introduced and 21.2% were diagnosed as GCA. CDUS and TAB were done in 82% and 38% of the patients respectively. We reviewed all TABs in the second phase of QIP (38%). As per current BSR guideline, 8 TABs could have been avoided in patients with positive CDUS and high probability of GCA or negative CDUS and low probability of GCA. Even if we deduct these 8 TABs from total of 25, 26% of our suspected GCA referrals would still require TAB for diagnostic workup.
Conclusion
After the routine introduction of CDUS, the percentage of patients requiring TAB has declined. Approximately one fourth patients would still require TAB as per BSR guidelines. To improve the clinical relevance of biopsies further we recommended; the routine use of GCA probability score, improve CDUS skills and arrange availability of urgent slots in clinic for CDUS. We also noticed that the number of patients referred has almost doubled. This might be due to better education and awareness at the primary and secondary care level which was done as part of the project.
Disclosure
A.A. Sidhu: None. A. Nandagudi: None. A. Bharadwaj: None.
P024 The mighty mimic: intravascular large b-cell lymphoma masquerading as myositis: a case series
Authors: Mirza Maria A 
Published: April 2021

Abstract
Background/Aims
Intravascular large b-cell lymphoma (IVLBCL) is a rare and aggressive form of extranodal diffuse lymphoma which due to its heterogeneous manifestations is often diagnosed at post mortem. The disease is typically contained within the small vessels thus sparing the surrounding tissue. Indeed, the presentation of the disease will depend on the organ involved with classical involvement of the central nervous system and skin.
Methods
We herein report two compelling cases of patients presenting with features of myositis, only to find on muscle biopsy evidence of IVBCL. Biopsy of the affected area and indeed random skin biopsies (RSB) may be a fundamental tool in establishing this elusive diagnosis.
Results
Features of the two cases are discussed, along with their various presentations, investigations, treatment and overall trajectory.
Conclusion
IVBCL is a rare disease that can present in various ways and often a delay in reaching a diagnosis leads to patient deterioration and death. A multidisciplinary approach involving the microbiologists, rheumatologists and haematologists led to a timely identification of the disease and subsequent management in these two cases. As the disease is characteristically contained within the blood vessels, the diagnosis is normally not confirmed from peripheral blood tests or even bone marrow biopsy. Biopsy of the affected area is what revealed the diagnosis in our two cases. Random skin biopsies may be very useful in establishing the diagnosis when suspecting IVBCL. Treatment may be very effective in controlling the disease as demonstrated by these two cases.
Disclosure
M.A. Mirza: None.
P038 Is it all in the eyes?
Authors: Gharatya Anil , Wajed Julekha , Cernovschi Natalia , Christidis Dimitrios 
Published: April 2021

Abstract
Background/Aims
The ocular manifestations of giant cell arteritis (GCA) and ocular myasthenia gravis (OMG) have significant overlap, which can lead to clinical uncertainty in the absence of awareness and routine diagnostic testing.
Methods
We present the case of a 63-year-old male patient with a strong family history of autoimmune disease. He presented to the Rheumatology department in 2009 with symptoms of polymyalgia rheumatica (PMR) and was treated successfully with a reducing regime of Prednisolone. In 2013, he presented to the Ophthalmology department with blurred vision and diplopia. Giant cell arteritis was suspected and a high dose of Prednisolone was started. His temporal artery biopsy was normal and inflammatory markers were not raised, but there was a complete resolution of his symptoms since starting steroids. Under the care of the Rheumatology department, reducing regimes of Prednisolone were attempted and a steroid sparing agent, Methotrexate was introduced. Unfortunately, he continued to have recurring symptoms of blurred vision and diplopia, particularly at Prednisolone doses less than 20mg. Over the course of a few years, there was a progression of his ocular symptoms. He developed a horizontal diplopia, monocular ptosis and a 4th nerve palsy. He was referred back to the Ophthalmology department, where alternate diagnoses were investigated. Anti-acetylcholine receptor antibodies (AChR) were strongly positive at 35 mU/L (normal &lt;0.44 mU/L). MRI head was normal, and CT chest showed no thymoma. Single fibre EMG confirmed severe OMG. Our patient was subsequently commenced on Pyridostigmine, with which symptoms improved.
Results
The literature on OMG mimicking GCA is sparse. The prevalence of an exclusively ophthalmic presentation in patients with GCA is 1 in 5. Ocular signs and symptoms in GCA can vary drastically due to the propensity of the disease to affect different structures within the eye. Transient and permanent visual loss, affects 10-30% of GCA patients due to anterior ischemic optic neuropathy. Diplopia affects 5% of GCA patients. In comparison, OMG can be the only presenting feature in 15% of patients with Myasthenia Gravis, and is characterised by unilateral ptosis, oculomotor paresis and binocular diplopia.
Conclusion
This case highlights the clinical similarities of OMG and GCA, and that OMG should be considered as a differential diagnosis when the clinical picture of GCA does not truly fit. In patients presenting with GCA with ocular symptoms, normal inflammatory markers and no systemic features, OMG should be considered and AChR antibody testing be performed. More awareness of OMG is needed. We also propose that OMG should be included as a differential diagnosis, in the British Society of Rheumatology guidelines for GCA.
Disclosure
A. Gharatya: None. J. Wajed: None. N. Cernovschi: None. D. Christidis: None.
P043 Fascinating phosphate: seek and you will find
Authors: Bakshi Jyoti , Batten Clare 
Published: April 2021

Abstract
Background/Aims
A 62-year-old accountant was referred to the metabolic bone clinic with a 2 year history of thoracic back pain and a persistently raised ALP. There were no associated red flags for her back pain. She had a history of a gluteal lump, thought to be benign, for which she had declined excision. She has hypertension and had a previous navicular fracture. Medications included bendroflumethiazide and Adcal D3. She had restriction in neck movements and was tender to percussion in the thoracic spine. There was no proximal weakness or focal neurology. Systems and joint exam were unremarkable.
Methods
The case is discussed below.
Results
Salient abnormal results on presentation were a raised ALP of 207 and corrected calcium of 2.34. PTH was elevated at 8.2 (NR:1.6-6.9), Vitamin D 79 and a low phosphate of 0.34 (NR:0.8-1.50). Alkaline phosphatase isoenzymes showed the raised level came from bone. Protein and urine electrophoresis were normal. A bone density scan was normal, and a recent thoracic MRI showed only degenerative change. An isotope bone scan was requested and was reported to show increased activity in the nasal bone, maxilla and both orbits, raising the possibility of Paget’s disease. However, when reviewed in the Radiology meeting with a skull x-ray, the appearances were not felt to be in keeping with Paget’s. Despite physiotherapy, hydrotherapy, acupuncture and neuropathic medication the patient’s back pain continued. Her phosphate remained low and her calculated tubular reabsorption of phosphate from a 24h urine collection (TmP/GFR) was low at 0.42mmol/l (NR 0.80-1.35). She was started on phosphate replacement and calcitriol, and Adcal D3 was continued. The Fibroblast Growth Factor (FGF) 23 levels were sent and came back significantly elevated at 1380 (NR &lt; 100). A 68Ga DOTA-TATE scan (whole body PET/CT scan), confirmed the right gluteal lump as the source of the FGF 23. The patient went on to have an excision biopsy and histology confirmed a mesenchymal tumour of the right buttock. Her phosphate replacement was gradually weaned, but on reducing the dose phosphate levels dropped and her symptoms returned. The repeat TmP/GFR was again low at 0.61, and FGF 23 levels were still raised at 204. A repeat 68Ga DOTA-TATE scan, 4 years after the first one, showed recurrence of the right gluteal lesion and a possible small lesion in the left gluteal muscle. She has been sent for further excision.
Conclusion
Tumour induced osteomalacia (TIO) is a rare condition and should be considered in cases of hypophosphataemia. Classical symptoms are proximal weakness and muscle and bone pain. They are typically associated with small benign tumours (most commonly mesenchymal tumours) which may be difficult to find. Excision is curative but if small amounts of tumour remain, relapses may occur.
Disclosure
J. Bakshi: None. C. Batten: None.
P063 SystmOne clinical reporting to identify high-risk rheumatology patients for shielding
Authors: Kirby David , Nadesalingam Kavitha 
Published: April 2021

Abstract
Background/Aims
At the start of the COVID-19 pandemic, shielding guidance was issued by Public Health England with a risk stratification guide developed by the BSR to assist with patient identification. At BTHFT we benefited with all GP practices in the region using TPP SystmOne (S1) for medical records. We describe how we used S1 to help identify high risk patients under our care.
Methods
We did not appreciate the full extent we could use S1 to identify patients in our initial shielding identification process. As such, we reviewed notes of patients on biologic prescriptions and SC MTX from Homecare prescription lists. To capture patients on high dose prednisolone, we identified patients with a recent diagnosis of GCA and patients with recent prescriptions of &gt; 20mg prednisolone through pharmacy records. On recognising the ability of S1 to identify patients that had glucocorticoid exposure through primary care and/or on cDMARDS we used S1 reporting mechanisms to identify these patients. Clinical record sharing enabled BTHFT’s rheumatology department to access S1 for all patients under their care. S1’s clinical reporting function was used to search for patients within at least one of the following cohorts before being combined in a single cohort of ‘at risk’ patients (heart, lung or kidney disease, ≥70 years, diabetes or hypertension [including pulmonary artery hypertension]). Reports were created for patients on any DMARD issued within primary care within the previous 12 weeks. High dose steroid use was more challenging to demonstrate, but a report was devised to include all patients with an issued prescription for prednisolone 5mg tablets within the preceding 8 weeks (for any reason). An additional report was created for patients with a rheumatological diagnosis co-existing with an interstitial lung disease. Using S1's report joining function, the different drugs and ‘at risk’ cohorts were combined to provide an accurate list of patients with features increasing their vulnerability to COVID-19. This list was easily modifiable and searches were re-run to update lists once inclusion on the shielding list was updated.
Results
Patients meeting the criteria for shielding were then advised in writing including signposting to BTHFT’s rheumatology website and helpline for further information if required. By searching for all prednisolone prescriptions, this would have included patients given prednisolone for other reasons. 5mg tablet strength ensured patients on low dose were excluded, but we recognise some patients were likely over-recruited into this cohort.
Conclusion
We can update our searching methods to easily include patients on biologics and SC MTX by some additional bulk coding in preparation for future shielding specifications thereby bypassing the need to review individual notes for patients. We were able to achieve an accurate shielding list in a relatively short space of time to reduce patient risk.
Disclosure
D. Kirby: None. K. Nadesalingam: None.
P108 Anti-nerve growth factor (anti-NGF) antibodies as analgesia in the management of osteoarthritis
Authors: Rammanohar Jashmitha , Sutton James , Seah K T Matthew , Khan Wasim S , To Kendrick 
Published: April 2021

Abstract
Background/Aims
Osteoarthritis is a major cause of morbidity and disability. Much of this comes from joint pain, which is exacerbated by movement and exercise. Pharmacological analgesia therefore not only has the obvious benefit of alleviating pain, but in doing so, it also facilitates exercise (a pillar of conservative management). Non-steroidal anti-inflammatory drugs (NSAIDs) and opioids are currently the main analgesics used in this context. However, these agents can cause unwanted side effects and are contraindicated in some patients. We thus conducted a systematic review and meta-analysis using the Cochrane collaboration criteria to evaluate the efficacy of anti-nerve growth factor (anti-NGF) antibodies as potential alternative analgesics in osteoarthritis of the hip and/or knee. Whilst tanezumab has been studied extensively and monoclonal anti-NGF antibodies have been reviewed in other pain states, this is the first systematic review of three key anti-NGF antibodies: tanezumab, fulranumab and fasinumab in symptomatic hip and/or knee osteoarthritis.
Methods
An interdisciplinary work group conducted a literature search across seven electronic databases for the use of anti-NGF antibodies in osteoarthritis. All hip/knee osteoarthritis studies investigating anti-NGF antibodies regardless of dose regimen or phase of trial were included. Studies in which participants received NSAIDs or analgesics other than anti-NGF antibodies, or studies in which the only intervention was the administration of anti-NGF antibodies in combination with NSAIDs or other analgesics were excluded. The Jadad Scale score was used to assess the quality of each study.
Results
Thirteen studies involving 8,145 patients with a diagnosis of hip and/or knee osteoarthritis were analysed. Demographic information including duration of disease and Kellgren-Lawrence grades were also extracted. Anti-NGF antibodies showed significant improvements compared to placebo as rated on the Western Ontario and McMaster Universities Arthritis Index (WOMAC) scales for pain (SMD= -0.50, 95% CI -0.71 to -0.28, P &lt; 0.00001; I2 = 88%), physical function (SMD= -0.82, 95% CI -1.09 to -0.55, P &lt; 0.00001; I2 = 94%) and stiffness (SMD= -0.88, 95% CI -1.22 to -0.54, P &lt; 0.00001; I2 = 95%). These agents were not associated with a significant increase in serious adverse events but were associated with a significant increase in discontinuation due to adverse events, abnormal peripheral sensations and peripheral neuropathy.
Conclusion
Anti-NGF antibodies appear very promising with regard to alleviating osteoarthritic hip/knee pain but more studies are needed to determine the optimal dosage and the overall risk-to benefit ratio, particularly with long-term use.
Disclosure
J. Rammanohar: None. J. Sutton: None. K. Seah: None. W.S. Khan: None. K. To: None.
P167 IgG4 related disease: response to immunosuppressive therapy: a single centre retrospective study in the United Kingdom
Authors: Sangle Shirish , Morton Neil T , Casian Alina , Nel Louise , Hanna Jennifer , Fernando Archana , Bell Rachel , Taylor Peter , Pattison James , O'Brien Tim , D'Cruz David 
Published: April 2021

Abstract
Background/Aims
IgG4 related disease (IgG4-RD) is a rare immune-mediated condition, increasingly being recognised as a multi-organ disorder. It is a relatively new entity and the precise prevalence is not known. It is a chronic fibro-inflammatory condition with raised circulating levels of immunoglobulin G4 and accumulation of plasma cells and fibrosis in the affected tissue. We report a retrospective observational study of patients with IgG4 related disease (IgG4-RD).
Methods
We conducted a retrospective observational study of patients seen at Guy’s and St Thomas’ NHS Foundation Trust Hospitals, London, UK. The data collected was analysed for clinical presentation, laboratory markers of inflammation, immunoglobulin subsets, autoantibody profiles, imaging and histopathology and compared to the 2019 ACR/EULAR criteria to determine a confirmed diagnosis of IgG4-RD. Data was also collected post standard-of-care treatment, including patient clinical outcomes and possible improvement.
Results
The study included a multi-ethnic cohort of 83 patients with multi-organ involvement. Fifty-nine of the eighty-three patients were classified as confirmed IgG4-RD (71%). Seventy one patients had biopsies of which 49 of the 59 (91%) confirmed IgG4-RD patients had biopsies consistent with IgG4-RD. Fifty patients underwent PET-CT scanning of which uptake was seen in 76% of those with confirmed IgG4-RD (26/34). Immunoglobulin IgG subclass analysis showed significantly higher IgG4 levels in the confirmed IgG4-RD group as compared to possible IgG4. Treatments were with corticosteroids and immunosuppressants such as azathioprine, methotrexate, mycophenolate, cyclophosphamide and rituximab. P167 Table 1:IgG4 levels and inflammatory markers for pre and post treatment groupsPre-RxPost-Rxp-valueCRPmean29 mg/l9 mg/l0.0298*Range0-300 mg/l1-70 mg/lESRmean35 mm/hr17 mm/hr0.0002*Range2-131 mm/hr1-74 mm/hrSerum IgG4mean4.9 g/L2.83 g/L0.0001*Range0.1-24.2 g/L0.02-13.3 g/L
Conclusion
IgG4-RD is a fibro-inflammatory disorder involving multiple organs. If not treated adequately may develop severe organ damage. It often responds to corticosteroids but may require other immunosuppressive therapy. Treatment with biologics such as B cell depletion therapy results are encouraging.
Disclosure
S. Sangle: None. N.T. Morton: None. A. Casian: None. L. Nel: None. J. Hanna: None. A. Fernando: None. R. Bell: None. P. Taylor: None. J. Pattison: None. T. O'Brien: None. D. D'Cruz: None.