bluuebunny/crossref_metadata_2025_split huggingface dataset:
P018 Multi-disciplinary team management of haemophagocytic lymphohistiocytosis: improving patient outcomes in a central London teaching hospital
Authors: Ludwig Dalia R , Manson Jessica , Jones Alexis , Mackenzie Strachan , Mccann Naina 
Published: April 2021

Abstract
Background/Aims
Haemophagocytic lymphohistiocytosis (HLH) is a syndrome characterised by the presence of severe uncontrolled systemic inflammation due the overproduction of inflammatory cytokines and macrophage activation. The majority of adult patients have secondary HLH, triggered by either infection, haematological malignancy or rheumatic disease. Early recognition and initiation of definitive treatment is of vital importance as HLH leads to multi-organ failure and death if left untreated, with mortality of up to 80% in some cohorts.
Methods
Under-recognition of the disease and vast differences in the management of patients with HLH between specialities have been common problems. To address this, a Rheumatology-led HLH multi-disciplinary team (MDT) meeting was established at University College London Hospital (UCLH) in 2018. The MDT includes physicians from Rheumatology, Infectious Disease, Haematology, Intensive Care, Nephrology and Neurology and feeds into the national HLH group HASC - HLH Across Specialty Collaboration. The MDT is now held bimonthly, discussing between 2 and 4 cases each meeting. The MDT functions as a decision-making tool, while providing consensus on patient management, including collaboration on how to access urgent HLH-directed treatments such as Anakinra for secondary care centres without access. It also serves as a unique learning opportunity for trainees from different disciplines.
Results
Since the introduction of the MDT the number of patients diagnosed with HLH at ULCH has increased from an average of 6 per year in 2014-2016 to 12 per year in 2018-2020. In the last two years we have seen improved survival from years 2017/18 to 2019/20 (Table 1); survival was approximately 30% in 2017 and 75% in 2019. P018 Table 1:Outcomes of patients diagnosed with HLH (using ICD codes D761 and D762) at University College London Hospital since 2005.YEARALIVEDECEASED200511200601201021201111201220201334201442201525201641201737201839201993202095
Conclusion
We believe that the HLH MDT has directly contributed to these improvements by encouraging earlier recognition and subspeciality collaboration. We encourage other sites to adopt a multi-disciplinary approach to managing patients with HLH to promote awareness of the condition and improve patient outcomes.
Disclosure
D.R. Ludwig: None. J. Manson: None. A. Jones: None. S. Mackenzie: None. N. Mccann: None.
P020 A new complex musculoskeletal clinic combining rheumatology and sport and exercise medicine services
Authors: Navarajasegaran Joshua A , Betts Jill , Baldock James , Soni Anushka , Smith Ralph 
Published: April 2021

Abstract
Background/Aims
Sport and Exercise Medicine (SEM) can play a key role to help manage the increasing demands on NHS Rheumatology and Musculoskeletal services. The ‘Complex MSK clinic’ has been developed as a combined consultant-led service staffed by Rheumatologists and Sport and Exercise Medicine (SEM) physicians at the Oxford University Hospitals. Patients referred from primary care to the rheumatology services were allocated to this clinic via an internal triage process. The aim of this project was to identify the spectrum of diagnoses and the epidemiological characteristics of patients seen in the clinic to help develop patient pathways, optimise patient care, and to showcase this collaborative approach
Methods
A retrospective analysis of the electronic patient record (EPR) of 194 consecutive patients attending the Complex MSK Clinic between 1 October and 30 November 2019 was performed. Patient age, reason for referral, length of time to appointment, prior investigations and treatment were all recorded, as were the primary/secondary diagnoses made, investigations requested, treatment provided and follow up arrangements. 16 patients had missing data or did not attend their appointment, leaving 178 patients that were included in the analysis.
Results
Mean age at appointment was 49 (range 17-90). 86.5% of all referrals were made by Oxfordshire GPs , with the main reason for referral being diagnostic uncertainty (78%). Almost 1 in 4 patients were given a primary diagnosis of osteoarthritis (23.6%), of which 64.3% involved the hands. Other prominent diagnoses included chronic pain syndrome/fibromyalgia (20.2%), mechanical pain (15.7%), and inflammatory arthritis or spondyloarthritis (14.0%). The most common further management was referral to physiotherapy services (46.1%), with 23.6% of patients offered additional medication and 11.2% a therapeutic injection. 104 (58%) patients were discharged after their first appointment, with 52 (29%) followed up in the complex MSK service and only 22 (12%) referred to another specialist clinic.
Conclusion
This report illustrates the spectrum of diagnoses seen within this unique service, highlighting a role for both Rheumatology and SEM within the same clinic. The low onward referral rate demonstrates appropriate triage and an efficient approach that is appropriate for this cohort of patients. It could reduce overall costs and patient journeys. Further work is required to streamline patient pathways, evaluate patient satisfaction and to determine whether the clinic is cost-effective and improves patient pathways.
Disclosure
J.A. Navarajasegaran: None. J. Betts: None. J. Baldock: None. A. Soni: None. R. Smith: None.
P101 Identifying factors that predict fracture risk in polymyalgia rheumatica
Authors: Ark Rajiv , Bukhari Marwan 
Published: April 2021

Abstract
Background/Aims
Polymyalgia rheumatica (PMR) is a chronic inflammatory condition that commonly affects the elderly and may be treated with long-term steroids. In these patients, fracture risk is higher than the general population and can lead to increased morbidity and mortality. This retrospective observational cohort study aimed to identify factors which help predict fracture risk in PMR patients.
Methods
Data were collected from June 2004 to October 2010 on patients with PMR at a District General Hospital. This included dexa scan data: bone mineral density (BMD), Z score, T score, fat mass, lean mass, percentage body fat, BMI and average tissue thickness. Demographic data, steroid use, alcohol consumption, smoking, secondary osteoporosis and presence of fracture was also recorded for each patient. Fracture risk was predicted by a series of binomial logistic regression models, which were adjusted for age and sex. Odds ratios with 95% confidence intervals and area under ROC curve (AUC) were calculated.
Results
714 patients with PMR were studied of whom 532 were female, the mean age was 70.5. Steroid use, secondary osteoporosis, lean mass, fat mass, BMI, average tissue thickness, average percentage fat and alcohol consumption were not significant predictors of fracture in regression models. BMD, T score and Z score predicted fracture risk. AUC of BMD was lower than that of T and Z score for each level. The AUC for L2 models were higher than other levels in BMD, T and Z score. Odds ratios, 95% confidence intervals and AUC of the significant predictors of fracture are shown in the table. P101 Table 1:Odds ratios, 95% confidence intervals and AUC valuesLevelBone Mineral DensityBMD AUCT ScoreT score AUCZ ScoreZ Score AUCLeft Hip0.098 (0.023,0.412)0.68240.728 (0.607,0.873)0.69380.677 (0.552,0.831)0.6938Right Hip0.062 (0.014,0.285)0.69170.713 (0.593,0.858)0.69180.662 (0.538,0.815)0.6934Left Femoral Neck0.104 (0.022,0.492)0.67270.738 (0.600,0.908)0.68330.703 (0.560,0.881)0.6848Right Femoral Neck0.087 (0.014,0.430)0.68370.734 (0.597,0.902)0.68360.694 (0.553,0.871)0.6836L10.192 (0.066,0.560)0.67890.820 (0.716,0.940)0.69160.798 (0.688,0.924)0.6907L20.138 (0.053,0.358)0.69770.787 (0.697,0.888)0.70950.763 (0.669,0.871)0.7108L30.192 (0.079,0.463)0.68810.823 (0.735,0.921)0.69600.805 (0.713,0.908)0.6963L40.243 (0.108,0.544)0.68370.852 (0.768,0.945)0.69110.837 (0.749,0.934)0.6914
Conclusion
These data suggest that BMD, T and Z score help predict fracture in PMR patients. Lifestyle factors and other body composition data from dexa scans do not predict fracture risk. Strongest predictor models were at the level of L2. FRAX could therefore underestimate the fracture risk as it uses femoral measurings. Limitations of the study are that it was retrospective and only studied patients who underwent DEXA scans. Steroid data were binary, not reflecting dose and duration of use. The study may have been underpowered to detect the impact of some factors predicting fracture risk.
Disclosure
R. Ark: None. M. Bukhari: None.
P114 Effect of vitamin D on parathyroid hormone, serum calcium and bone mineral density in patients with primary hyperparathyroidism being managed conservatively
Authors: Khalid Mahrukh , Deshani Vismay , Jadoon Khalid 
Published: April 2021

Abstract
Background/Aims
Vitamin D deficiency is associated with more severe presentation of primary hyperparathyroidism (PTHP) with high parathyroid hormone (PTH) levels and reduced bone mineral density (BMD). We analyzed data to determine if vitamin D levels had any impact on PTH, serum calcium and BMD at diagnosis and 3 years, in patients being managed conservatively.
Methods
Retrospective analysis of patients presenting with PHPT. Based on vitamin D level at diagnosis, patients were divided into two groups; vitamin D sufficient (≥ 50 nmol/L) and vitamin D insufficient (≤ 50 nmol/L). The two groups were compared for age, serum calcium and PTH levels at diagnosis and after mean follow up of 3 years. BMD at forearm and neck of femur (NOF) was only analyzed in the two groups at diagnosis, due to lack of 3 year’s data.
Results
There were a total of 93 patients, 17 males, mean age 70; range 38-90. Mean vitamin D level was 73.39 nmol/L in sufficient group (n = 42) and 34.48 nmol/L in insufficient group (n = 40), (difference between means -38.91, 95% confidence interval -45.49 to -32.33, p &lt; 0.0001). There was no significant difference in age, serum calcium and PTH at the time of diagnosis. After three years, there was no significant difference in vitamin D levels between the two groups (mean vitamin D 72.17 nmol/L in sufficient group and 61.48 nmol/L in insufficient group). Despite rise in vitamin D level in insufficient group, no significant change was observed in this group in PTH and serum calcium levels. BMD was lower at both sites in vitamin D sufficient group and difference was statistically significant at NOF. Data were analyzed using unpaired t test and presented as mean ± SEM.
Conclusion
50% of patients presenting with PHPT were vitamin D insufficient at diagnosis. Vitamin D was adequately replaced so that at 3 years there was no significant difference in vitamin D status in the two groups. Serum calcium and PTH were no different in the two groups at diagnosis and at three years, despite rise in vitamin D levels in the insufficient group. Interestingly, BMD was lower at forearm and neck of femur in those with sufficient vitamin D levels and the difference was statistically significant at neck of femur. Our data show that vitamin D insufficiency does not have any significant impact on PTH and calcium levels and that vitamin D replacement is safe in PHPT and does not impact serum calcium and PTH levels in the short term. Lower BMD in those with adequate vitamin D levels is difficult to explain and needs further research.
Disclosure
M. Khalid: None. V. Deshani: None. K. Jadoon: None.
P139 Disease modifying treatment: the end of a golden age
Authors: Stanway James , Walker David 
Published: April 2021

Abstract
Background/Aims
The role of gold changed from first line DMARD to use in cases where other options were limited, either due to contraindication or ineligibility for biologics. In Autumn 2019, IM gold was withdrawn by the manufacturer due to supply issues, without consultation of the Rheumatology community. As such, a group of patients with difficult to manage disease have lost a key DMARD. We aimed to assess the impact of the withdrawal of gold on the management of IA within Northumbria Healthcare Trust.
Methods
Retrospective case note review.
Results
We identified 37 patients on gold as of Autumn 2019; 16 for seropositive RA; 4 for PsA; 16 for seronegative RA and 1 for axial spondyloarthritis. 28 were taking gold monotherapy, with 3 receiving regular glucocorticoid in addition. Eight were taking gold in combination with a csDMARD and one in combination with rituximab. Data are currently available for 30 patients regarding the circumstances of gold initiation. Of these, all had received at least one alternative csDMARD which was either inefficacious or intolerable. Reasons for avoiding biologics were not documented in all cases; concerns regarding frequent chest infections were common and documented as a clear contraindication in 5. Follow up has been impacted by COVID-19; only 19 patients have had follow up within the last 6 months, 7 have not been followed up since gold cessation. Fourteen (37.8%) have commenced an alternative DMARD. Six of these have commenced a high cost drug; rituximab (3), adalimumab (2) or tofacitinib (1). Five have commenced hydroxychloroquine (with one subsequently switching to leflunomide),2 methotrexate and 1 minocycline. Seropositive RA and PsA patients are more likely to have commenced an alternative DMARD, 8/16 and 2/4 respectively; compared to 4/16 with seronegative RA. Of 23 patients who have not commenced alternative DMARDs, 17 have been reviewed following gold cessation; 3 were felt to need additional treatment; 1 has been deferred due to COVID-19 and 2 did not meet criteria for biologic treatment when assessed in early 2020 and subsequent follow up has been delayed. Fourteen patients were felt not to need an additional DMARD, although 1 required an increase in methotrexate and 6 continued co-prescribed DMARDs at the usual dose.
Conclusion
The majority of patients have not required additional treatment although for some patients this may reflect the fact that the COVID-19 pandemic has significantly restricted routine follow up. This unusual case of forced DMARD cessation highlights the fact that drug free remission is possible and the ongoing need for immunosuppression should be reviewed. This also raised the ethical question of weather it is right to cease the production of medication which for some patients may be the only effective treatment option.
Disclosure
J. Stanway: None. D. Walker: None.
P161 A comparative study of childhood-onset SLE with adult-onset patients: a single centre study
Authors: Morton Neil T , Rutter-Locher Zoe , Durrani Maumar , Sangle Shirish , Wilkinson Nick , D'Cruz David 
Published: April 2021

Abstract
Background/Aims
Childhood-onset SLE (cSLE) was once associated with significant mortality but 5-year survival rates have now improved significantly. We retrospectively compared the clinical features and outcomes of cSLE patients with an adult-onset SLE (aSLE) group.
Methods
We retrospectively studied data from 30 patients from our cSLE patients (onset before age 16) compared with 65 aSLE randomly selected in a 2:1 ratio, from our Lupus clinic patients fulfilling the 1997 ACR SLE criteria. Data regarding ethnicity, age of onset, family history, serology, complications, medications and outcomes were recorded.
Results
Of cSLE patients 24 were female and 6 male (F:M ratio 4:1), with 16 patients (53%) Afro-Caribbean, 8 Caucasian, 5 Asian and 1 of mixed ethnicity. In the aSLE group, 60 patients were female and 5 male (ratio 9:1), with 29 (44%) Afro-Caribbean, 13 Asian, 21 Caucasian and 2 of mixed ethnicity. Median age at disease onset was 14 years (10-16) in cSLE and 29 years (17-50) in aSLE. A positive family history of SLE was seen in 37% (11/30) of cSLE patients compared to 11% (7/65) of aSLE [p = 0.003]. There was no difference in family history of other autoimmune rheumatic diseases (10% vs 14% [p = 0.57]).
Table 1 shows frequency of different autoantibodies, with anti-dsDNA and anti-cardiolipin antibodies significantly more frequent in the cSLE group [p = 0.017, p = 0.016 respectively]. Lupus nephritis appears to be more common in cSLE but not significantly (80% renal involvement vs 69% aSLE).There was no difference between involvement of chest, haematological, VTE or APS. Standard of care treatment was used in both groups, including oral prednisolone (&gt;80% patients), anti-malarial and immunosuppressive medications. Renal flares were significantly more frequent in cSLE (23% vs 8%, [p = 0.027]). Of those deceased, only 1 was directly due to SLE. P161 Table 1:Serological markercSLE (n = 30)aSLE (n = 65)Number of patients%Number of patients%ANA2790%6498%dsDNA23*77%33*51%ENA1447%3655%RNP827%2335%Anti-Ro1033%2132%Anti-Sm827%1218%C1Q14**64%19**63%ACL17*57%20*31%LAC930%1828%RF517%69%Comparison of serological markers.*p &lt; 0.05 (Chi-square used).**10 patients’ data missing.
Conclusion
More cSLE patients had a positive SLE family history and had higher anti-dsDNA antibody positivity compared to aSLE. Renal involvement was observed more commonly in cSLE than aSLE patients but was not statistically significant. Our results suggest that the cSLE cohort have a higher prevalence of specific lupus serology and a positive family history of SLE which may point towards more aggressive disease.
Disclosure
N.T. Morton: None. Z. Rutter-Locher: None. M. Durrani: None. S. Sangle: None. N. Wilkinson: None. D. D'Cruz: None.
Cannabis Legalization and Adolescent Use of Electronic Vapor Products, Cannabis, and Cigarettes
Authors: Bhatia Devika , Hinckley Jesse , Mikulich Susan , Sakai Joseph 
Published: April 2021

Objectives:
Cannabis legalization may have unintended consequences, such as effects on prevalence of adolescent use of other substances. We hypothesize that in states that have legalized recreational cannabis (RCL states), electronic vapor product use (“vaping”), cannabis, and cigarette use among adolescents is more prevalent than in states that have only legalized medical cannabis (MCL states) and states that have neither legalized recreational nor medical cannabis (NL states).

Methods:
Data are from the Center for Disease Control's 2017 Youth Risk Behavior Surveillance Survey (n = 107,665), a nationally representative survey of US high school students. We categorized student responses by cannabis legalization status of the state in which they lived at time of survey, then tested associations between cannabis legalization status and adolescent vaping, cannabis, and cigarette use, using logistic regressions.

Results:
Students in RCL states were significantly more likely to report current vaping behaviors compared to NL students [odds ratios (OR's) 2.07–2.21]. Students in cannabis-legal states were significantly more likely to report ever or currently using cannabis compared to NL students (OR's 1.27–1.40). MCL students were significantly less likely to report current cigarette smoking (OR = 0.86) compared to NL students.

Conclusions:
Adolescents in cannabis-legal states were more likely to report vaping and cannabis use compared to adolescents in NL states. Public health officials, policymakers, and clinicians should consider the associations between cannabis legalization and adolescent vaping, cigarette, and cannabis use patterns.
Clinical Significance of Middle Cerebellar Peduncle Ischemia After Translabyrinthine Vestibular Schwannoma Resection
Authors: Maxwell Anne K. , Muelleman Thomas , Barnard Zachary , Slattery William H. , Mehta Gautam U. , Lekovic Gregory P. 
Published: April 2021

Objective:
To assess clinical symptoms, signs, and radiographic evolution of middle cerebellar peduncle (MCP) diffusion restriction (DR) abnormalities following vestibular schwannoma (VS) resection.

Study Design:
Retrospective chart and imaging review.

Setting:
Tertiary-referral neurotology and neurosurgery practice.

Patients:
All consecutive patients who underwent translabyrinthine VS resection over a 2-year period (August 2017–May 2019).

Intervention:
Translabyrinthine craniotomy for VS resection.

Main Outcome Measures:
Magnetic resonance imaging (MRI) obtained on postoperative day 1 were reviewed for DR within the pons and cerebellum, with 3 months follow-up MRI to assess for evolution of these vascular changes.

Results:
Of the 31 patients who met inclusion criteria, MRI demonstrated MCP DR consistent with acute ischemia in 29% (9/31). Of those, two showed corresponding T2 signal abnormalities on follow up MRI consistent with cerebrovascular accident (CVA) within the MCP. Both had severe gait ataxia and dysmetria requiring acute rehabilitation admission and significantly larger tumors (p = 0.02). The remaining seven were asymptomatic, and DR abnormality resolved without lasting radiographic changes. Brainstem compression was present in 100% of patients with postoperative MCP DR (mean MCP ipsilateral:contralateral ratio 0.59 ± 0.19), and 68.1% of those without (mean MCP ratio 0.71 ± 0.25), a difference that was not statistically significant (p = 0.14). In the two patients with CVA, MCP asymmetry persisted, whereas the asymmetry resolved in all others.

Conclusions:
Asymptomatic acute MCP ischemia discovered incidentally does not require intervention. However, when the ischemic area is large and patients are symptomatic, especially if an acute rehabilitation admission is required, surgeons should suspect true CVA.
Evidence of Cochlear Synaptopathy and the Effect of Systemic Steroid in Acute Idiopathic Tinnitus With Normal Hearing
Authors: Park Euyhyun , Song Insik , Jeong Yong Jun , Im Gi Jung , Jung Hak Hyun , Choi June , Rah Yoon Chan 
Published: April 2021

Objective:
To evaluate the electrophysiologic evidence of cochlear synaptopathy and the effects of systemic steroids in acute idiopathic tinnitus with normal hearing.

Study Design:
Retrospective review of medical data.

Setting:
Tertiary referral center.

Patients:
Fifty-nine patients who experienced acute-onset idiopathic tinnitus (within 12 weeks) with normal hearing and the same number of age- and pure-tone threshold-matched control groups.

Intervention:
Electrophysiologic studies of the auditory pathway, oral steroids, and ginkgo biloba.

Main Outcome Measures:
Pure-tone thresholds, wave I and wave V amplitudes of the auditory brainstem response (ABR), tinnitus handicap inventory (THI), and visual analog scale (VAS).

Results:
Significantly reduced ABR wave I amplitude and wave I/wave V ratio were found in the tinnitus group compared with the no tinnitus group. Age and pure-tone threshold were significantly correlated with reduced wave I amplitude and small wave I/wave V ratio. The THI and VAS scores were decreased at 3 and 12 weeks after steroid administration; however, overall changes in THI and VAS scores were not significantly different between the steroid and ginkgo biloba groups.

Conclusion:
Potential cochlear synaptopathy was suspected in the early stage of acute idiopathic tinnitus, even in patients with normal hearing. Age and hearing threshold were potentially associated with the development of cochlear synaptopathy. Low-dose oral steroids and ginkgo biloba induced early subjective relief of tinnitus, which maintained up to 12 weeks, however, those changes did not differ between groups.