bluuebunny/crossref_metadata_2025_split huggingface dataset:
P09 Audit of anti-nuclear antibody testing in East Kent and the British Society for Rheumatology Choosing Wisely recommendations
Authors: Rasool Sheraz , Beharry Karen , Afifi Amr , Shahzad Faisal , mahadi riham , Lord denise De 
Published: April 2020

Abstract

Background
The British Society for Rheumatology has recently issued Choosing Wisely guidance on ANA testing, to avoid unnecessary testing and inappropriate use of resources. Currently there are no local guidelines on ANA testing. The aim of this audit was to use the BSR guidance as best practice to assess current local testing and then develop guidelines. The standards assessed were as follows: testing ANA should be reserved for patients suspected to have a diagnosis of a connective tissue disease, e.g. lupus. Testing ANA should be avoided in the investigation of widespread pain or fatigue alone. Repeat testing is not normally indicated unless the clinical picture changes significantly.

Methods
Retrospective data collection of ANA requests at East Kent Hospitals University & Foundation Trust was extracted from the pathology IT system (Apex) from January to March 2019. The clinical indications entered for electronic requesting were recorded. All ANA requests were recorded as ‘appropriate’, ‘inappropriate’ or having ‘insufficient documentation’. A request was considered appropriate if it documented one of the following indications/suspected diagnoses: inflammatory arthritis, connective tissue features - Raynaud’s, vasculitis/photosensitive rash, scleroderma, clinical myositis, autoimmune haemolytic anaemias, cytopenia, Suspected autoimmune liver disease, pleurisy, pulmonary fibrosis, pericarditis, serositis, autoimmune renal disease, autoimmune neurologic disease, BILAG registered patients, patients on/requiring biologics. All other requests which did not meet these standards were considered inappropriate.

Results
There were 1,517 requests made in 3 months; 180 requests (11.86%) were positive and 1,337 (88.14%) were negative. 61% of ANA requests were from outpatients, 25% were inpatient requests and 14% were requests from GPs. Outpatients requests: neurology, rheumatology, gastroenterology, respiratory and renal medicine (in order of frequency). Only 17% of requests met the set standards; 41% were inappropriate requests and 42% were requests with insufficient details.

Conclusion
ANA testing is relatively expensive compared to other routine screening tests. The assay is time consuming and labour-intensive, and due to the volume of requests made, 2 batches are tested daily in East Kent. With only 17% of the 1,517 tests meeting the standards, this suggests the majority were inappropriately requested. There were &gt;15,000 requests from East Kent and nearby trusts from April 2018 to December 2018. At £2.50 per test, adhering to guidelines could save potentially £12,000 per year. Local guidelines have now been devised based on the Choosing Wisely standards, indications & suspected diagnoses documented. ANA testing will be re-audited in 6 months.

Disclosures
S. Rasool None. K. Beharry None. A. Afifi None. F. Shahzad None. R. mahadi None. D. De Lord None.
P35 Vasculitis by any other name would present just the same
Authors: Rasul Sajida , Dockery Morven , Tattersall Rachel , Hawley Dan , Maltby Sarah , McMahon Anne-Marie 
Published: April 2020

Abstract

Background
Vasculitis can present in many ways and large vessel vascultis is reported rarely to co-present with inflammatory bowel disease. We would like to present two adolescent patients who presented in very similar ways via the gastroenterology team with a seemingly clear diagnosis of inflammatory bowel disease but who were found to have large vessel vasculitis later in their disease journey. The presentation is to raise awareness of this rare co-presentation and to discuss treatment challenges in particular those apparent in adolescent patients crossing the transition bridge.

Methods
Patient A is a 17 year old boy who has had a long and rocky road to control of his inflammatory bowel disease which presented when he was 2 years old. Histologically it fitted a Crohn’s classification. His journey included moderate response to oral steroids and little to no response to a range of DMARDs, biologics (including infliximab and adalimumab which both had secondary failure) and elemental nutrition, over a period of 12 years. Vedolizumab was introduced this year with almost immediate improvement of gut symptoms, but with ongoing raised inflammatory markers (CRP 79, ESR 86). Incidental investigations of neck pain following the start of vedolizumab revealed significant abnormality in the external carotids, with 70% stenosis. MR angiography confirmed a typical pattern of stenotic large vessel vasculitis. The second patient, B is also 17 and was diagnosed with histological ulcerative colitis aged 14. He has an older brother with IBD but has recently been found to have small bowel disease and is likely therefore to have Crohn’s disease. He is on infliximab 10mg/kg 4 weekly but presented with a 3-month history of high inflammatory markers, malaise towards the end of the 4 week infliximab cycle and drenching night sweats. CT Chest confirmed vasculitis in the thoracic aorta, subclavians and carotids. On PET CT there is mural thickening and no stenosis.

Results
Patient A presented in paediatric care and B in adult care but because of the seamless rheumatology service and combined MDT with gastroenterology both patients’ care has been widely discussed amongst relevant adult and paediatric teams.

Conclusion
Large vessel vasculitis might be driving the inflammatory bowel disease in both patients as such the life threatening element of the disease ought to be managed immediately, while ensuring safe transition to between paediatric and adult care.

Disclosures
S. Rasul None. M. Dockery None. R. Tattersall None. D. Hawley None. S. Maltby None. A. McMahon None.
P50 Gossypiboma causing SLE and dermatomyositis overlap
Authors: Borukhson Liubov , Wing Charlotte , Stratton Richard J 
Published: April 2020

Abstract

Background
Gossypiboma is a term referring to tumours arising from a retained, non-absorbable cotton matrix left behind during surgery. Serious implications for patients include infections, migration of the retained particles into a systemic circulation, local obstructions, as well as autoimmune reactions. To our knowledge, we describe the first case of SLE-dermatomyositis overlap associated with the gossypiboma.

Methods
A 44 year old lady, who is originally from Poland, presented with a few months history of oral ulcers, photosensitive rash over her chest and neck, myalgia, heliotrope rash and subcutaneous calcified nodules. Her ANA screen was positive 1:100 homogenous pattern, ENA, DsDNA negative, normal complements, CK up to 1636 u/L, nailfold capillaries demonstrated dilated capillaries and capillary dropouts. She was initially managed by a hydroxychloroquine and methotrexate combination for a presumed lupus and dermatomyositis overlap, followed by rituximab for recurrent lupus flare ups. The left knee MRI revealed a well-defined lesion medial to the tibia measuring 4.5 x 2.8 x 2.2 cm. It was heterogenous and contained calcium depositions. The initial diagnosis was in favour of peripheral nerve sheath tumour (schwannoma). However, surgical resection and histological analysis revealed foreign body sponge like material consistent with a retained surgical sponge. On revisiting the past medical history, the patient recalled some form of an operation on her left knee, evidenced by a small scar over posterior aspect of the knee, around twenty-five years ago. A subsequent operation was undertaken to remove the gossypiboma. After this, the patient’s symptoms had gradually improved and ANA screen came back as negative, and she was able to cease steroid therapy.

Results
Immune type of reactions secondary to adjuvants (silicone, pristane, aluminium, vaccines, etc) have been described by Israeli immunologist Shoenfeld and named ASIA syndrome (Autoimmune Syndrome Induced by Adjuvants) in 2011. He described late-onset (sometimes after many years) systemic symptoms associated with the immune reaction following silicone breast implantation. As per his theory, adjuvants influence both adaptive and innate arm of the immune system via different mechanisms, especially in genetically predisposed individuals. The great variety of manifestations have been described, among which Sjögren’s and lupus-like syndromes. We believe that our patient developed autoimmune disease secondary to constant antigenic stimulation caused by a retained cotton wool in the knee. The fact that the symptoms have improved after the removal of the foreign body combined with the negative ANA test is the evidence of the above-mentioned causality.

Conclusion
In summary, we believe that the presence of the foreign body in our patient’s knee has propagated the foreign body reactions and lead to an autoimmune disease.

Disclosures
L. Borukhson None. C. Wing None. R.J. Stratton None.
P53 Gastric antral vascular ectasia in Sjögren's syndrome?
Authors: Tan Yvonne , Gough Andrew 
Published: April 2020

Abstract

Background
Gastric antral vascular ectasia (GAVE) is a rare, but severe cause of non-variceal upper gastrointestinal bleeding (UGIB). It commonly manifests as iron deficiency bleeding and associated with liver cirrhosis, chronic renal failure and connective tissue diseases. Many therapeutic options have been proposed, but the first line of treatment for GAVE remains as endoscopic Argon plasma coagulation (APC). At present, there are no clear satisfactory results to opt for medical therapy as an alternative to invasive approach. We present a case of primary Sjögren's syndrome who has developed GAVE and successfully treated with medical therapy.

Methods
A 68 years old lady was initially referred to Rheumatology with new symptoms of Raynaud’s, affecting her hands and toes. She had dry eyes and mouth, which were managed conservatively. There was no skin change or gastrointestinal symptoms. She had strongly positive ANA with negative ENA and low levels of IgG and IgM. Nifedipine was initiated to control her Raynaud’s symptoms and the diagnosis of Sjogren’s syndrome was made. Eight months following the initial diagnosis, she was found to have iron deficiency anaemia. There was no change in her symptoms or dietary intake. CT colonography revealed no colonic abnormality. Gastroscopy revealed gastric antral vascular ectasia (GAVE) and the patient was treated with endoscopic argon plasma coagulation (APC). This patient underwent second APC after three months as her haemoglobin dropped despite regular iron supplements. We had made a decision to start pulsed cyclophosphamide and methylprednisolone infusion to control her disease activity. Her haemoglobin remained stable for seven months following the last infusion. She then underwent her third APC and another pulsed cyclophosphamide infusion was resumed. Nine months since her last infusion, her haemoglobin dropped once again, and she underwent another APC. In view of her good response to cyclophosphamide infusion, this lady was started on mycophenolate as maintenance. Since the introduction of mycophenolate, we were pleased that this lady’s haemoglobin had normalised, and the iron supplement was no longer required.

Results
The association of GAVE with Sjögren's syndrome is not commonly known. There were two other case reports of patients with primary Sjögren's syndrome and developed a mild form of GAVE. As patients with GAVE in the absence of cirrhosis are more at risk of active upper gastrointestinal bleeding, they require more frequent endoscopic treatments following initial diagnosis. APC is an effective treatment; however, it is uncomfortable for these patients to experience repeatedly.

Conclusion
This case has shown the importance of recognition the possible association of primary Sjögren's and GAVE syndrome as a cause of iron deficiency anaemia. The successful medical therapy of GAVE provides a safe alternative for endoscopic APC, however, more evidences are required to prove the significant of the outcome.

Disclosures
Y. Tan None. A. Gough None.
P55 Carcinoid syndrome on etanercept and subsequent treatment with secukinumab in a patient with psoriatic arthritis
Authors: Sheikh Ali S. F , Srivastva Sagar G , Wood Fiona 
Published: April 2020

Abstract

Background
Psoriatic arthritis requires early recognition and treatment for prevention of disease progression. Conventional disease modifying drugs are first-line agents followed by biologic DMARDs for patients with active disease. TNF-alpha blockers are first line biologic agents in the UK. Th17 inhibitors are used since the elucidation of Th17 pathway. Safety and efficacy profiles of biologic agents inhibiting the Th17 pathway, including secukinumab (IL-17A) and ustekinumab (IL-12/23p40) have been studied.

Methods
We report a case of carcinoid syndrome in a lady on etanercept for psoriatic arthritis, carcinoid as a potential TNF alpha side effect. We also report safety of Th17 (secukinumab) inhibitors in the patient to date (&gt;1 year).

Results
A female with a history of acne rosacea, was diagnosed with psoriatic arthritis in 2000 age 32. Initial sulphasalazine failed, then received methotrexate until 2011, when her arthritis flared. She was commenced on etanercept which proved effective. After 4 years of etanercept and methotrexate, her liver profile became deranged. Investigations off treatment included ultrasound abdomen demonstrating a liver mass, which resembled focal nodular hyperplasia on magnetic resonance imaging. Further screening revealed high urinary 5HIAA (527 umol/24h) and raised chromogranin A & B levels (1574 pmol/L and 373 pmol/L respectively). She had no symptoms suggestive of carcinoid, although facial flushing could have been camouflaged by her rosacea. Octreotide scan was positive, CT enterogram showed a distal ileal neuroendocrine tumour with adjacent lymphadenopathy. She underwent right hemihepatectomy and hemicolectomy. Her liver profile deteriorated again on methotrexate, leflunomide was ineffective. In November 2017 she was started on ustekinumab, which was ineffective and was withdrawn after 8 months. There was no alteration in carcinoid blood markers and no CT changes. In July 2018, she was commenced on secukinumab, which has allowed reduction in steroids. The patient is aware of lack of safety data in her circumstances. Regular surveillance has shown no recurrence of carcinoid with serial negative 5HIAA and chromogranin levels. There are no progressive CT changes at 1 year.

Conclusion
This is a case of carcinoid tumour occurring on TNF-alpha blockers and may represent a rare complication. Screening biomarkers including 5HIAA and chromogranin levels can be useful if disease is suspected. We could not find other similar case reports to guide further management. Within time limited data available - ustekinumab had no effect on the carcinoid. Th17 inhibitors can be safe options for treating psoriatic arthritis and psoriasis with highly sustained efficacy and favourable safety profile seen in large clinical trials. In this case, after &gt; one year of secukinumab treatment - there is no adverse effect on carcinoid syndrome.

Disclosures
A.S.F. Sheikh None. S.G. Srivastva None. F. Wood None.
P60 Daily fluctuations in fatigue severity and C-reactive protein in people with rheumatic diseases
Authors: Druce Katie L , Gibson David S , McEleney Keven , Meleck Stephanie , James Ben , Hellman Bruce , Dixon William G , Mcbeth John 
Published: April 2020

Abstract

Background
Fluctuations in fatigue severity are common in people with inflammatory (e.g. rheumatoid arthritis (RA)) and non-inflammatory (e.g. fibromyalgia (FM) and osteoarthritis (OA)) rheumatic diseases. We tested whether fluctuations in fatigue in RA, OA and FM were explained by fluctuations in inflammation (C-reactive protein (CRP)), pain and mood.

Methods
Participants in the GIRAF (Gaining Insight into RheumAtic Fatigue) study used a patient co-configured app to report fatigue severity, pain severity and mood on a 5-point ordinal scale (increasing scores = worse state) twice daily for 7 days. Daily CRP (mg/L) was measured via dried blood spot sampling. Fluctuations in fatigue were calculated as within-day (morning to evening variability) and between-days (morning to morning; evening to evening variability). Multi-level mixed effects ordered logistic regression models tested the relationship between fluctuations in CRP and fatigue over 7 days. The relationships between fluctuations in fatigue, pain and mood were also examined. Models were adjusted for age, sex and disease diagnosis.

Results
Thirty-eight people (RA:12, OA:13, FM:13) participated and contributed data on 190 (71% of eligible) days. Participants were mostly female (81.6%), with a median age of 56 years. There were no demographic differences between disease groups. Within-day changes in fatigue severity of ≥one point were observed on 97 (51.1%) days and between-days changes were observed on 52% of mornings (92/177 eligible periods) and 49.4% of evenings (85/172 eligible). Fluctuations in pain severity and mood of ≥one point were similarly common (data not shown). Median CRP levels were low (&lt;5mg/L) across all diseases and did not fluctuate substantially between days (change ≥1mg/L:17.5% (33/189) of days). 8 participants (21.1%: RA:4, OA:2, FM:2) had “active inflammation” (CRP&gt;5mg/L) on a total 22 (8.3%) days in the study (range:1-8 days). Fluctuations in CRP were not associated with fluctuations in fatigue (odds-ratio:1.01, 95%CI:0.83-1.22). Fluctuations in pain severity (6.93, 4.68-10.28) and mood (4.58, 3.26-6.45) were associated with fluctuations in fatigue (Table 1).

Conclusion
Fluctuations in fatigue severity were common in people with rheumatic diseases. Inflammation was low and did not predict fatigue variability. Optimal management should target fatigue independently of inflammatory disease management and may benefit from modification of pain and mood.

Disclosures
K.L. Druce None. D.S. Gibson None. K. McEleney None. S. Meleck None. B. James None. B. Hellman None. W.G. Dixon None. J. Mcbeth None.
P63 The association between presenting clinical features and subsequent diagnosis of giant cell arteritis
Authors: Barnett Lauren A , Helliwell Toby , Jordan Kelvin P , Prior James A 
Published: April 2020

Abstract

Background
The diagnosis of giant cell arteritis (GCA) remains challenging due to the varied number and type of clinical features (symptoms and comorbidities) that patients with GCA can present with. Prompt diagnosis is important, as if left undiagnosed and untreated, some patients may experience irreversible sight loss. Given the rarity of GCA and that many of the presenting clinical features may pertain to other, more prevalent illnesses in the typical age range for GCA, diagnostic delay is common. Such delay may increase the risk of GCA related complications. The aim of this study was to quantify the association between a diagnosis of GCA and the clinical features observed prior to diagnosis.

Methods
Patients with a coded record of GCA and aged 40 years or older at the time of diagnosis, were identified from the Clinical Practice Research Datalink (CPRD) between 1990 and 2017. CPRD is a large, national UK database of primary care records. Every GCA case was matched to 5 controls with no GCA diagnosis, by year of birth, gender and practice. Clinical features linked to GCA in previous research studies were identified in the patient’s primary care records at any time prior to GCA diagnosis. Conditional logistic regression was used to determine associations between clinical features and a subsequent diagnosis of GCA, adjusting for Body Mass Index, smoking status, and alcohol consumption.

Results
9,205 patients with GCA were included, the majority of which were female (70.9%). 15 clinical features were examined. For example, 53.3% of GCA cases had a recorded consultation for headache prior to diagnosis (38.4% within six months prior to diagnosis), compared to 9.9% of controls; 3.2% of cases had recorded jaw pain (0.3% within six months prior to diagnosis) compared to 0.5% of controls; 39.4% of cases had a diagnosis for hypertension prior to their diagnosis of GCA. GCA cases were more likely than controls to have recorded consultations for headache (adjusted OR 10.57; 95% CI: 9.93, 11.25), jaw pain (5.37; 4.47, 6.44) and hypertension (1.33; 1.26, 1.40). Other clinical features that were statistically significantly associated with GCA included fever, anxiety/depression, and PMR. Cancer was the only clinical feature not associated with GCA.

Conclusion
Symptoms such as headache, jaw pain and hypertension are highly prevalent in GCA, but are also common symptoms in the usual age group affected by GCA and common features of many conditions. In isolation and considering the rarity of GCA in the UK population, these symptoms may not be immediately attributed to GCA by the diagnosing GP. It is therefore necessary to conduct further research where clinical features are not treated independently, but as groups or clusters, which together may more accurately help clinicians to diagnose GCA early.

Disclosures
L.A. Barnett None. T. Helliwell None. K.P. Jordan None. J.A. Prior None.
P68 Age-related muscle strength decline in mid-late life varies in men and women and is associated with diet and physical activity: observations from the Hertfordshire Cohort Study
Authors: Zhang Jean , Laskou Faidra , Jameson Karen , Cooper Cyrus , Dennison Elaine M 
Published: April 2020

Abstract

Background
In the context of an aging population, age-related decline in muscle strength is well recognised, and is associated with functional limitation and increased mortality. However, scarce epidemiological data are available regarding its prevalence, or lifestyle associations at a time when intervention is still possible to retard or prevent loss. We considered these issues in the Hertfordshire Cohort Study, a cohort of late-middle aged community dwelling adults.

Methods
2,987 participants were seen in 1999-2004, 1,572 men and 1,415 women. A lifestyle questionnaire was administered that asked about social class, physical activity, diet, co-morbidities and cigarette and alcohol consumption. Grip strength was measured with a Jamar dynamometer, three times on each side with the highest value used. Age related muscle strength decline was defined, according to current convention, as a grip strength &lt;30kg in men and &lt;20 kg in women.

Results
The mean age of participants was 65.8 (IQR 63.5-67.8) years for men and 66.5 (IQR 64.5-68.7) years for women. Muscle strength decline was more common in women (10.3%) than men (3%); the odds ratio (OR) for age-related muscle strength decline was 3.73 (95%CI 2.66-5.23) in women relative to men (p &lt; 0.001). Factors that appeared protective included higher physical activity scores in men (OR 0.97, 95%CI 0.96-0.99, p = 0.003) and in women (OR 0.97, 95%CI 0.96-0.98, p &lt; 0.001) and a higher prudent diet score in women (OR: 0.74, 95%CI 0.60-0.90 p = 0.003). Muscle strength decline was strongly associated with quality of life in women (p ≤ 0.001) as assessed by SF36 and EuroQoL EQ-5D for all domains, although EuroQOL anxiety/depression and SF36 role emotional were less strongly associated (p-value=0.042, p-value=0.006, respectively). Table 1 shows self-reported impaired ability to move, self-care, perform usual activities and increased pain were associated with higher risk of age-related muscle strength decline in women.

Conclusion
Age related muscle strength decline was more common in community dwelling women than men, and diet and physical activity were predictors of such decline. Research into effective preventative interventions is now warranted.

Disclosures
J. Zhang None. F. Laskou None. K. Jameson None. C. Cooper None. E.M. Dennison None.