bluuebunny/crossref_metadata_2025_split huggingface dataset:
P01 Hydroxychloroquine retinopathy: is screening necessary?
Authors: Gobbett Anne , Smith Jonathan , Bracewel Claire 
Published: April 2020

Abstract

Background
Hydroxychloroquine (HCQ) has long been associated with a low prevalence (0.5%) of retinal toxicity with possible irreversible sight loss. New retinal imaging techniques have suggested a significantly increased prevalence of HCQ retinopathy (7.5%). In light of this result, the American Academy of Ophthalmology (AAO) in 2016 and subsequently the Royal College of Ophthalmologists (RCO) in 2018 produced new screening guidelines. We report the HCQ retinopathy screening results for all patients referred to our Ophthalmology department from 1/11/2017 to date.

Methods
We collected prospective data from all patients referred for HCQ screening. Patients were screened according to the new RCO guidelines. We recorded each patient's dose and duration of HCQ treatment, their weight and renal function, and concurrent use of tamoxifen to determine their risk factors. Retinal images: colour fundal photos, ocular coherence tomography (OCT) and auto-fluorescence (AF), were performed on all patients (baseline tests), and visual acuity, automated central visual field testing (Humphrey 10:2) and multi focal electroretinography (mfERG) performed as indicated for those at risk, needing annual testing.

Results
We have diagnosed two definite cases of hydroxychloroquine retinopathy, giving us a prevalence of 2/639 (0.31%) of all screened, and 2/308 (0.64%) of those considered to be at risk according to RCO guidelines. 711 patients have been referred to our screening service so far. 639 of these have been screened with 72 failing to attend. 308 of all those screened have been categorised to be at increased risk of retinopathy: duration of treatment &gt;5 years (224), dose &gt; 5mg/kg/day (40), eGFR&lt; 60ml/min/1.73m2 (111) and use of tamoxifen (0). 64 have multiple risk factors. 119 of those screened have completed their second cycle of screening, with 48 patients failing to attend for this repeat screening.

Conclusion
Our results for HCQ retinopathy screening suggest a prevalence more in keeping with the originally proposed figure than that in the recent literature; this may be multifactorial. We have a smaller number of patients, with fewer of those patients in the higher risk categories than in the study that prompted the change in screening guidelines. We only have baseline or two years of serial images for each patient at present, the comparison of multiple serial images may highlight more cases of retinopathy. More data is needed before we can suggest our figures are statistically different.

Disclosures
A. Gobbett None. J. Smith None. C. Bracewell None.
P08 The impact of patient’s compliance and delivery delays: a cost-saving audit on biologics
Authors: Pookarnjanamorakot Patra , Suresh Resmy , Webb Paul 
Published: April 2020

Abstract

Background
Inflammatory arthritis or ankylosing spondylitis patients who are qualified for biologics treatment will have their medications delivered to their homes by Healthcare at Home (HaH). HaH sends rheumatology department the requests for repeat prescriptions in which the drug delivery due date should be within two weeks of the faxed date on the prescription. Anti-TNF is costly and if the patient already has excess stocks of medications at home then future delivery should be postponed in order to reduce the economic impact on the Trust. This audit aims to investigate the amount of money that could be saved by checking patient’s available medications at home and delay future deliveries.

Methods
168 patients were contacted at Croydon University Hospital between 1 - 19 November 2018 by telephone before signing the biologics repeat prescriptions. Patients were asked regarding the number of injections/tablets they have left and whether they had missed any and their reasons for non-adherence. Number of weeks the next prescriptions could be delayed due to the excess amount of injections/medications patients have was calculated and the amount of money saved by delaying the prescription was calculated.

Results
133 out of 168 patients answered the phone. Among patients who answered the phone, 59 of them were on etanercept, 6 were on secukinumab, 13 were on etanercept, 24 were on adalimumab, 9 were on methotrexate, 17 were on baricitinib and 5 were on golimumab. Their drug stock at home varied between 0 to 12 weeks. There are various reasons for their excess drug stock in the last 2 months including previous delivery delays or double-delivery (3 patients), infection (20 patients), simply forgotten (7 patients), high liver function tests (1 patient), holiday (6 patients), underwent surgery (1 patient), and underwent a dental procedure (1 patient). By analysing the number of injections/tablets patients have left, we were able to delay repeat prescriptions in 62 patients by 1 to 8 weeks. Theoretically, by delaying sending prescription out to Healthcare at Home for all 62 patients, we would be able to save £13,546.585 for the Trust during the month of November. This made up 6.5% of the monthly cost of anti-TNF inflicted on the Trust in November 2018 (£208,099.94).

Conclusion
This is a cost-saving audit concerning patient’s adherence to biologics. By verifying the number of excess biologics at patients’ home prior to requesting the repeat prescription, we would be able to reduce the cost inflicted on the Trust greatly. This calls for an improvement in patient education and compliance monitoring. We have launched a biologic patient education programme at Croydon University Hospital which enhances patient’s understanding of biologic treatment. Patients are also encouraged to contact our biologic coordinator if they are non-compliant for any reasons.

Disclosures
P. Pookarnjanamorakot None. R. Suresh None. P. Webb None.
P29 Use of novel biologic therapies for arthropathy affecting common variable immunodeficiency patients
Authors: Henneberry Robert , Burns Siobhan , Stratton Richard , Lowe David M 
Published: April 2020

Abstract

Background
CVID is the most common adult immunodeficiency with an incidence of 1:25000. It is characterised by hypogammaglobulinaemia and recurrent bacterial infections. Patients also develop granulomatous interstitial lung disease and are at increased risk of lymphoma. Autoimmune disease, most commonly AIHA, ITP and thyroiditis, occurs at a rate of 25%. Previous studies suggested that 3% of patients have inflammatory arthritis, but from our experience a higher proportion complain of chronic arthralgias. Treating arthropathy in CVID is challenging; examination and inflammatory markers may be normal and there is concern about the risk of infections if using methotrexate or biologics.

Methods
We present a series of 7 patients with arthropathy in CVID. We chose DMARDs based on the individual case, tailor-made to the patients clinical and immunologic status.

Results
Patients had longstanding symptoms, with a median duration of 11 years. All patients had disabling joint pain, in three it was the most prominent symptom of their illness. All patients had pain/swelling in a typical symmetrical small joint polyarthritis distribution. Fatigue was also prominent. On examination three patients had clear evidence of synovitis, one had tenderness but no swelling, and three had no evidence of synovitis; there was no deformity. No patient had positive autoantibodies. Three had chronically raised CRP/ESR, one had raised inflammatory markers during flares, but the other three had normal markers. Prednisolone (10-15 mg) controlled arthritic symptoms. There was little response to conventional DMARDS or rituximab. Two patients treated with abatacept showed improvement in symptoms, but one died from PJP pneumonia. Two patients treated with baricitinib showed improvement in symptoms but one relapsed after 5 months (Table 1).

Conclusion
Arthopathy is a common, and probably under-diagnosed, feature of CVID. It is difficult to treat, with a poor response seen to most DMARDs. We have seen promising results with Baricitinib and propose using it first line in patients with disabling rheumatic symptoms. There may be a role for abatacept in co-existent severe joint and lung disease. A striking proportion, 4/7 (versus 10% in general CVID population), of patients had a causal genetic mutation identified. Arthropathy may, therefore, push immunologists towards genetic testing.

Disclosures
R. Henneberry None. S. Burns None. R. Stratton None. D.M. Lowe None.
P38 A rare case of rheumatoid arthritis with perforating collagenosis
Authors: Deb Subhasish , Srirangan Srinivasan 
Published: April 2020

Abstract

Background
Rheumatoid arthritis has multiple cutaneous manifestations with rheumatoid nodule being the commonest one. A maculopapular rash in setting of rheumatoid arthritis has a varied list of differentials including rheumatoid vasculitis, pyoderma gangrenosum, rheumatoid neutrophilic dermatosis, interstitial granulomatous dermatitis and palisaded neutrophilic granulomatous dermatitis. We present a rare case of patient with rheumatoid arthritis with perforating collagenosis.

Methods
A case report is presented in the results section.

Results
A 64 year old lady known to have seropositive rheumatoid arthritis for the past 6 years with poor compliance to treatment presented with maculopapular lesions on both her legs and abdomen. The lesions were itchy in nature and later became ulcerative. The lesions spread to various parts of the body over the next 4 weeks and patient was seen by a dermatologist who initially prescribed topical therapy and the patient came off her disease modifying anti rheumatic agents. She underwent a skin biopsy as initial investigations were otherwise unremarkable. Skin biopsy revealed a diagnosis of perforating collagenosis. She was given topical steroids and counselled regarding the need to take her disease modifying anti rheumatic drugs regularly. There was 95% resolution of skin lesions within 6 months.

Conclusion
Perforating collagenosis is a skin disorder characterised by transepithelial expulsion of dermal tissue which presents as a papulonodular rash with central keratin plugs. It is usually associated with diabetes mellitus and chronic kidney disease but rarely been reported in association with rheumatoid arthritis. Literature review revealed that there have been only 2 cases reported previously. The first case has been described in 2010 in a man from Singapore on treatment for RA for 2 years. Second case has been described by Ikeda et al in 2019 in a case of rheumatoid vasculitis. Clinically it presents as umbilicated, hyperkeratotic papules with a central white keratotic crust. Koebnerization may be seen and itching of surrounding regions may lead to papules to coalesce and form plaques. A biopsy will reveal features similar to those seen in the primary disease where perforating collagenosis shows cup shaped invagination of the epidermis filled with a plug consisting of keratin and cellular debris. There have been no trials and treatment is based on case reports. We believe that better compliance to her disease modifying anti rheumatic agent therapy, leading to improved treatment of the systemic cause had a significant role in resolving her skin lesions.

Disclosures
S. Deb None. S. Srirangan None.
P40 Spontaneous pneumomediastinum in mixed connective tissue disease
Authors: Hasan Fuad , Kumar Vinod , Silburn Suzy , Thomson Rosanna 
Published: April 2020

Abstract

Background
Spontaneous pneumomediastinum refers to dissection of free air from the airways on lung parenchyma into the mediastinal structures without any antecedent thoracic trauma or surgical intervention. Pneumomediastinum has been described as a rare complication of connective tissue disease, most commonly in dermatomyositis and polymyositis. Here we report 2 cases of mixed connective tissue disease (MCTD) who developed pneumomediastinum.

Methods
The first case was a 76 year old male undergoing assessment for MCTD. During assessment, he was found to have bi-basal crepitations without any obvious respiratory symptom. He was on steroids for last 4 weeks for proteinuria. His High- resolution CT scan (HRCT) showed pneumomediastinum with evidence of Interstitial lung disease (ILD) without any complication. He was monitored closely and his pneumomediastinum resolved without any intervention. The second case was a 49 year old female with known MCTD & was on steroids for 5 months. She was admitted to the hospital with weight loss and dysphagia. This was a long hospital stay during which she developed pneumomediastinum with pneumothorax and surgical emphysema around the neck. She required ICU admission and was transferred to cardiothoracic centre where she underwent bronchoscopy, VATS inspection and required chest drains.

Results
In both cases there were similarities where both patients with MCTD were on variable duration of steroids & had ILD. They then developed pneumomediastinum with surgical emphysema, however their course was quite contrasting where the first case was relatively benign & resolved without any intervention while the second one required long ITU stay with multiple complications and interventions.

Conclusion
Spontaneous pneumomediastinum is an uncommon manifestation of CTD and only few cases have been reported with MCTD. Although spontaneous mediastinum seems to be associated with a relatively benign short-term course, one should be wary of complications and have low threshold for investigating for any new lung symptoms in context of CTD. It is uncertain whether severity of MCTD or duration of steroids use impacts the progress of spontaneous pneumomediastinum as there are very limited number of cases.

Disclosures
F. Hasan None. V. Kumar None. S. Silburn None. R. Thomson None.
P51 Serious pathology mimicking GCA: two cases of carcinoma tongue
Authors: kayani Abdul , Sebastian Alwin , Borukhson Liubov , Whitlock Madeline , Dasgupta Bhaskar 
Published: April 2020

Abstract

Background
Jaw and tongue pain with constitutional symptoms and raised inflammatory markers are considered pathognomonic ischemic features of giant cell arteritis (GCA). Temporal artery ultrasound (US) (or biopsy) available in GCA fast-track clinics (FTC) for a rapid assessment of patients with suspected GCA. Atypical presentation and negative US or biopsy require further workup to look for an alternative diagnosis. ENT pathology can occur as a mimic of GCA. Herein we discuss two clinical cases of squamous cell cancer tongue presenting with signs and symptoms that resemble GCA.

Methods
We have put together a case report.

Results
Case-1: A 58-year-old male with a recent presumed diagnosis of relapsing GCA was referred with worsening visual symptoms and right eye pain despite ongoing steroid treatment (60mg), for consideration of Tocilizumab (TCZ). His initial presentation was 5 months ago with a right parietal and retro-orbital headache and blurred vision. He had a normal eye examination. His C-reactive protein (CRP) was raised (24). He was started on prednisolone 40 mg for GCA with complete resolution of symptoms with normalised CRP within weeks. Several weeks later, symptoms reoccurred. CT brain, abdomen and pelvis was normal and temporal artery biopsy negative. His prednisolone was increased to 60 mg. 6 months later, his jaw and tongue pain worsened, and he was treated with pulsed methylprednisolone. Due to partial response to steroids, he was referred to consider TCZ. He had tender left TMJ with normal temporal artery US. Urgent MRI head and neck revealed a left posterior tongue mass with the histology confirmed poorly differentiated squamous cell carcinoma. He was managed with chemo and radiotherapy.
Case-2: A 75 years old female, presented with right scalp pain, tongue pain, painful swallowing and chewing. Her blood investigations were normal except a raised ESR (48) and presumed GCA she was started on steroids (60 mg). she had initially good response but, within a few weeks, her symptoms returned. She was then referred to our FTC. Temporal artery US and biopsy were normal. MRI of the head and neck showed a large mass seen in the right half of the posterior tongue extending into the deep aspect of the anterior tongue. Histology confirmed poorly differentiated Squamous cell carcinoma. She was treated with a combination of chemotherapy and radiotherapy.

Conclusion
GCA mimics represent a major diagnostic dilemma. FTC helps to stratify the GCA from mimics. Careful evaluation of the history, examination as well as a temporal artery US helps to exclude GCA and aids prompt requesting of appropriate tests to find an alternative diagnosis such as tongue cancers as in our cases. We have now introduced negative weightage for consideration of alternative diagnoses in our GCA probability score.

Disclosures
A. Kayani None. A. Sebastian None. L. Borukhson None. M. Whitlock None. B. Dasgupta Consultancies; Roche, Sanofi. Grants/research support; Roche.
P64 Does pain peak at night? The daily rhythm of musculoskeletal searches on the NHS website
Authors: Fuggle Nicholas R , Masefield Nicola , Dennison Elaine M , Cooper Cyrus 
Published: April 2020

Abstract

Background
With the advent of smartphone technology and the constant availability of information on the internet, it is suspected that searches for clinical symptoms or medical conditions could be a real time indication of the timing of these symptoms. Therefore, we aimed to investigate the daily timing of visits to musculoskeletal pages compared to mental health pages on the NHS website (a major source of online health information in the UK).

Methods
In collaboration with NHS Digital we acquired 6 months of data on visits to the NHS website and visits to individual webpages relevant to pain management (“Managing chronic pain”, “10 ways to ease pain”) musculoskeletal conditions (Rheumatoid arthritis, Osteoarthritis, Arthritis”), anatomical pain (Neck, Back, Shoulder, Elbow or Arm, Foot, Hip, Knee, and Hand pain) and an assortment of mental health pages, (Clinical depression, Generalised Anxiety Disorder). In order to adjust for the overall traffic to the NHS website (which peaks strongly at 9-10pm) the mean numbers of webpage visits per hour of the day over the 6 month period were calculated and divided by the mean number of NHS website visits per hour of the day over the 6 month period. Fourier analysis was used to shape appropriate models and assess daily rhythm. Analysis were performed using STATA version 15.

Results
There were a total of 400 million visits to the NHS website over the 6-month period of data collection. The overall trends for traffic to the website included a marked peak in visits in the evening at 9-10pm and a distinct trough overnight. Visits to ‘pain management’ pages peaked at 11-4am, whereas visits to ‘musculoskeletal conditions’ pages had a bimodal distribution with the largest peak at 6-7am (and a lesser peak at 9-10pm). This bimodal distribution was echoed on the ‘anatomical pain’ pages with a greater peak at 6-7am and lesser peak at 9-10pm, except for ‘neck pain’ and ‘back pain’ which had higher peaks at 9-10pm and lesser peak at 6-7am. In comparison, visits to ‘mental health’ pages peaked clearly at 12-1am.

Conclusion
Through this investigation of visits to the NHS website we saw clear differences in the distribution of visits relating to ‘musculoskeletal conditions’, ‘anatomical pain’ and ‘pain management’. These profiles differed substantially to the distribution of ‘mental health’ page visits. It is interesting that searches for pain management (including the page “10 ways to ease pain” which is the top NHS webpage listed on Google when searching the word “pain”) peaked overnight between 11-4am, as this replicates our previous finding from Google search data. These findings provide an insight into the circadian rhythm of interactions with online health data and may even hint at patterns of symptom experience over a 24-hour period.

Disclosures
N.R. Fuggle None. N. Masefield None. E.M. Dennison None. C. Cooper None.
P72 Muscle weakness affects function differently in males and females in inclusion body myositis: providing results for focused clinical care and clinical trial design
Authors: Williams Jacob , Oldroyd Alexander , Lilleker James , Chinoy Hector , Miller James 
Published: April 2020

Abstract

Background
Sporadic inclusion body myositis (IBM) is a muscle wasting disorder usually affecting those aged over 50 years, characterised by slowly progressive weakness of the distal upper limbs and proximal lower limbs. Particular weakness of pinch, knee extension and ankle dorsiflexion result in functional decline and disability. Weakness-associated functional decline differences between genders has not previously been investigated. This study aimed to investigate for differences of functional decline in men and women with IBM.

Methods
A cohort of verified adult IBM patients were followed at a single specialist neuromuscular clinic (Royal Victoria Infirmary, Newcastle-upon-Tyne, UK) between 2004-15, with data collected at each clinic visit. Strength of 11 movements was measured via dynamometry at each clinic visit. Patient-reported function was measured via the Neuromuscular Symptom Score (NSS). Generalised linear modelling, adjusted for disease duration, was carried out to identify associations between strength and function (NSS). Pinch, knee extension and ankle dorsiflexion strength measurements were analysed. Modelling was carried out separately for male and female cohorts.

Results
Data of 75 participants (47% female) was analysed. A total of 591 measurements were taken. Median age at time of IBM diagnosis was 68.8 years. Median baseline NSS score was 45 (IQR 37, 54) for the male cohort and 40 (IQR 29, 45) for the female cohort. The median disease duration of the study cohort was 2.6 years. Reduced function (NSS) was significantly associated with reduced pinch, knee extension and ankle dorsiflexion strength in the male cohort only (Table 1). No significant associations were observed in the female cohort.

Conclusion
This is the first study to demonstrate that the progression of weakness, in the characteristic muscle groups affected in IBM, impacts on function differently in men and women. These results illustrate the importance of functional assessment in patients with IBM and the importance of focused physiotherapy upon function-dependent muscle groups. Further, potential gender-based differences of weakness-related function in IBM should be considered when designing clinical trials.

Disclosures
J. Williams None. A. Oldroyd None. J. Lilleker None. H. Chinoy None. J. Miller None.