bluuebunny/crossref_metadata_2025_split huggingface dataset:
P166 Parotid benign lymphoepithelial cyst in connective tissue disease: a rare case series and literature review
Authors: Palaniappan Nachammai , Nallasivan Subramanian 
Published: April 2023

Abstract

Background/Aims
Benign lymphoepithelial lesions are swellings of salivary glands caused by a reactive lymphoid infiltrate with follicular hyperplasia, obliterating the acinar glands and causing proliferation and disruption of the ductal epithelium. These have been described in HIV, lymphoma and Sjögren's syndrome. We report a case series with mixed connective tissue disease/Sjögren's syndrome and reviewed the literature with similar presentations in order to highlight this unusual manifestation.

Methods
Index patient: A 25-year-old lady presented with a history of arthralgia, fatigue and Raynaud's 3 years ago and was diagnosed by rheumatologist to have MCTD with ANA showing U1RNP, Ro positive and histones antibody positive. She was managed with mycophenolate and improved. A year ago she developed parotitis which improved with antibiotics. She recently came to the outpatient department with painful swelling in the parotid region. Blood counts remain normal, ESR 36 mm/hr , CRP 6 mg/dl, ACE level, HIV screening normal. MRI parotid showed bilateral multiple lymphoepitheial cysts in the parotid glands. Opinion was obtained from oncology and the ENT surgeon. No evidence of malignancy was found. In view of this, consensus opinion among rheumatologists was to stop mycophenolate and give 2gm rituximab. We also reviewed 10 cases of benign lymphoepithelial cyst arising in the parotid region in acquired immunodeficiency syndrome negative patients. Cases were from 8 published articles plus the three cases of benign lymphoepithelial cyst in a mixed connective tissue disease/Sjögren's syndrome in AIDS negative patients reported in Table 1. Most of the patients had been treated surgically, however, immune therapy is sufficient.
Results:

Conclusion
Benign lymphoepitheial cysts have been described in patients with sarcoidosis, HIV, cancer and CTD. Our patients had features of MCTD with Sjögren's syndrome and manifested with benign lymphoepithelial cysts in the parotids, which is very unusual. Parotid swelling in connective tissue disease requires evaluation and multidisciplinary input. Benign lymphoepithelial cysts will not require any surgery; once malignancy is excluded, they will require modification of immune therapy.
Disclosure
N. Palaniappan: None. S. Nallasivan: None.
P183 Disease activity, comorbidities and functional status predict treatment response to biologic and targeted synthetic disease-modifying anti-rheumatic drugs in psoriatic arthritis
Authors: Addae-Kyereme Christine , Lembke Stephanie , Macfarlane Gary J , Jones Gareth T 
Published: April 2023

Abstract

Background/Aims
Much discussion surrounds the optimal therapeutic treatment plan for biologic and targeted synthetic disease modifying anti-rheumatic drugs (bDMARDs/tsDMARDs) when treating psoriatic arthritis (PsA). The current study aims to identify predictors of treatment response among PsA patients initiating bDMARD/tsDMARD treatment, focusing on disease activity, functional status (Health Assessment Questionnaire, HAQ), and comorbidities.

Methods
The systematic review of clinical trials and observational studies used Medline, Embase and Cochrane databases. Eligible studies included full text papers with quantitative estimates of effect (or data to allow these to be computed). Papers also required a validated assessment of treatment response i.e., Minimal Disease Activity (MDA), or some other marker of treatment response such as treatment continuation. Case reports and conference abstracts were excluded.

Results
From 2824 articles identified, 27 were included. Across these, &gt;15 different outcomes were used, and measured at &gt; 10 different timepoints. There was consistent evidence to suggest that higher baseline disease activity was associated with a reduction in the likelihood of treatment response, see Table 1. Some studies reported up to 20% reduction in odds of LDA at 6 months associated with each 1 unit increase in DAPSA. Others demonstrated that patients with a high DAS28 (&gt;3.2) experienced over a 90% reduction in the odds of MDA at 12 weeks. Eleven studies (from 15) demonstrated a significant association between baseline function and odds of treatment response. Each 1 unit increase in HAQ-DI decreased the odds of a good EULAR response at 3 months by up to 66%. The presence of comorbidities was also consistently associated with a decrease in treatment response. Studies showed that the presence of comorbidities at baseline lessened the odds of MDA at 6 months by up to 80%. Others reported that comorbidities were associated with a reduction in the odds of 5 year treatment persistence by up to 40%.

Conclusion
This review shows clear evidence of reduced bDMARD/tsDMARD response in PsA among patients with disease activity, poor function, and comorbidities. Clinicians should be aware of the reduced chances of treatment success and should consider early review of therapy, to consider whether treatment escalation or switching is required.
Disclosure
C. Addae-Kyereme: None. S. Lembke: None. G.J. Macfarlane: None. G.T. Jones: None.
P195 Use of the Giant Cell Arteritis Score (GCAPS) in a district general hospital
Authors: Waller Rosemary , Carty Sara , Schmitt Tobias 
Published: April 2023

Abstract

Background/Aims
The Giant Cell Arteritis Score (GCAPS) was developed by the specialist team in Southend, Essex, UK in 2018. It is a clinically focused tool to aid diagnosis of GCA by providing a pre-test probability, with a threshold of greater than 9 denoting higher risk of GCA requiring further diagnostic testing. So far, it has been validated externally in patients in 2 centres. In all cases, scoring was completed by a rheumatology consultant. We are keen to validate it in our setting, a district general hospital without rapid access to vascular ultrasound, to determine whether it was a valid and reliable tool for assessing which patients required temporal artery biopsy and which patients could rapidly taper steroids following a negative result. We also aimed to see whether scoring could be completed by clinicians other than rheumatology consultants.

Methods
We retrospectively applied the GCAPS score to all patients referred as possible GCA to our service between November 2021 - April 2022 by reviewing clinical notes. A medical student (TS) with no prior experience was trained to use the score, with scoring independently repeated by a rheumatology consultant (RW).

Results
34 patient records were reviewed. 14 (41%) were male, mean age 70.5 (range 49 - 89). 6 had biopsy confirmed GCA, 7 had a clinical diagnosis of GCA and 21 had a low probability of GCA. All 13 patients with biopsy confirmed or clinical GCA were scored &gt; 9 by both assessors. There was GCAPS agreement in 7/34 patients (21%). Of 27 patients with differing scores, TS gave a higher score in 17 (50%). In 4 patients, there was a difference in probability threshold. In all cases, the patient was scored more highly by TS. None of the patients had a final diagnosis of GCA. 5 patients had known GCA and a relapse was being questioned. 2/5 had a flare - one clinical and one biopsy proven. The GCAPS was &gt;9 in both patients. There were no missing data.

Conclusion
We have demonstrated that it is possible to train someone without prior experience to use GCAPS. There were differences in probability threshold in 4 patients, with the less experienced assessor tending to overscore. The GCAPS is applicable in our non-specialised centre and is feasible to use as evidenced by the lack of missing data. No patients with a GCAPS under 9 had a final diagnosis of GCA. This good negative predictive value is in keeping with previously published data. In a small patient number, the GCAPS threshold remained valid in distinguishing relapse. We feel GCAPS has two roles. It can be used to triage who needs review in rheumatology clinic. It can also be used in rheumatology clinic to determine who requires temporal artery biopsy.
Disclosure
R. Waller: None. S. Carty: None. T. Schmitt: None.
E010 Checkpoint inhibitor-induced rheumatological adverse events: real-world experience from Northeast England
Authors: Gault Abigail , Williams Kristian , Stewart Christopher , Anderson Amy E , Plummer Ruth , Pratt Arthur G 
Published: April 2023

Abstract

Background/Aims
Immune checkpoint inhibitors (CPIs), in the form of either single agent anti-PD1 or combination therapy with anti-PD1/anti-CTLA4 monoclonal antibodies, are widely used in clinical practice. Whilst CPI-induced rheumatological immune-related adverse events (RirAEs) are relatively infrequent compared to other toxicities (8-12%), increasing CPI use in oncology settings means a growing number of patients are affected. As part of an audit of inter-specialty referrals, we evaluated patients developing new musculoskeletal symptoms on CPI treatment and the breadth of specialist-confirmed RirAEs.

Methods
Two sources of data were reviewed. First, consecutive consultant-confirmed RirAE cases in our rheumatological service between 2017 and 2022 (referred to as the ‘RirAE cohort’) were evaluated. Second, an observational cohort of 50 consecutive oncology patients treated with CPIs from 2019-2022 in whom symptom questionnaires had been completed (“Oncology cohort”) was reviewed. Local audit registration/safeguarding approvals were obtained. Medical records were reviewed by two medical professionals, and anonymised data analysed in a spreadsheet.

Results
24 patients were identified in the RirAE cohort, of whom most presented with arthritis (Table 1). Amongst 50 patients in the Oncology cohort, 20 (40%) reported new-onset/worsening musculoskeletal symptoms on a questionnaire (“MSK-symptomatic Oncology Cohort”). Three of these were referred, diagnosed and counted amongst the RirAE cohort. Of the remaining 17, 6 were identified as having developed musculoskeletal pain by consulting oncologists; the remaining 11 were not objectively identified as having experienced potential musculoskeletal toxicity (Table 1).

Conclusion
Confirmed RirAEs comprised predominately seronegative, oligoarticular presentations with notable PMR overlap. In the oncology setting, the self-reported joint symptoms of 20/50 (40%) patients were identified by a physician and 3/9 of these (33%) were referred for a specialist opinion. RirAEs may be more common than appreciated amongst CPI recipients, and the extent of low-grade inflammatory arthritis unreported or “hidden” amongst other irAEs is not clear. Early evaluation of new-onset musculoskeletal symptoms in CPI recipients, and close cross-specialty collaboration, could reduce morbidity for patients who already face significant health challenges.
Disclosure
A. Gault: None. K. Williams: None. C. Stewart: None. A.E. Anderson: None. R. Plummer: None. A.G. Pratt: None.
E014 Severe myocarditis with cardiogenic shock in adult-onset Still’s disease after mRNA COVID-19 vaccination: a case of atypical presentation
Authors: Kan Andy Ka Chun , Yeung Winnie Wan Yin , Lau Chak Sing , Li Philip H 
Published: April 2023

Abstract

Background/Aims
Adult-onset Still’s disease is a systemic inflammatory disease of unknown aetiology. Post-COVID-19 vaccine adult-onset Still’s disease has been reported and was associated with only mild myocarditis. Here we report the first case of adult-onset Still’s disease after mRNA COVID-19 vaccination presenting with severe myocarditis with acute heart failure and cardiogenic shock.

Methods
We described the case history of the patient.

Results
A 72-year-old Chinese woman developed gradual onset of fever, shortness of breath, sore throat, generalised arthralgia, malaise and poor appetite 15 days after receiving the first dose of BNT162b2 mRNA COVID-19 vaccine. Physical examination revealed fever, bilateral ankle oedema and elevated jugular venous pressure. Significant investigation results are shown in Table 1. Extensive viral panel tests (including enterovirus, influenza and cytomegalovirus) were all negative. Echocardiography showed severely reduced left ventricular ejection fraction of 20%. The acute heart failure was complicated by cardiogenic shock requiring intensive care unit admission. Myocarditis was later diagnosed. Although the heart condition subsequently improved, there were persistent fever and arthralgia, as well as the development of generalised maculopapular skin rash. In view of that, series of investigations were performed, which revealed persistent neutrophilic leucocytosis, hyper-ferritinaemia and liver function derangement, while autoimmune panel was grossly unremarkable and septic/viral workup was negative (Table 1). Contrast PET-CT scan showed no features of malignancy. Adult-onset Still’s disease was diagnosed, and the patient was treated with oral prednisolone 40mg daily. The patient’s condition responded to the treatment; the fever subsided and the leucocyte count and inflammatory markers were normalised, and she was subsequently discharged. Three months after discharge, the patient was clinically well with prednisolone tapered down to 5mg daily. Reassessment echocardiogram showed full recovery with LVEF 60%.

Conclusion
Severe myocarditis with acute heart failure and cardiogenic shock is a possible initial presentation of adult-onset Still’s disease after mRNA COVID-19 vaccination. After exclusion of more common aetiologies, it is important to consider adult-onset Still’s disease as one of the differential diagnoses in the presence of compatible features following COVID-19 vaccination, such that appropriate and timely workup and treatment can be offered.
Disclosure
A. Kan: None. W. Yeung: None. C. Lau: None. P.H. Li: None.
E040 Disease flare-ups after SARS-CoV-2 vaccination among patients with rheumatic and musculoskeletal diseases in a Rheumatology Specialized Care Center: Sri Lanka
Authors: Ubhayasiri Sasheela K , Abeysinghe Duminda 
Published: April 2023

Abstract

Background/Aims
In the recent past, there has been growing research interest in COVID-19 vaccines and their impact on the disease dynamics of rheumatic and musculoskeletal diseases (RMDs). The intersection of COVID-19 and autoimmunity has led to the theoretical possibility of flare-ups of autoimmune diseases with COVID vaccines. This study was conducted to evaluate the occurrence and the nature of flare-ups following the COVID vaccination on patients with RMDs.

Methods
This cross-sectional analytical study was conducted at Ragama Rheumatology and Rehabilitation Hospital, Sri Lanka involving 248 clinic patients using a structured questionnaire.

Results
The mean age of the study population was 52.69 and 75.4% were females. 12 patients (4.7%) reported flare symptoms following 1st dose of vaccination. Of them, 67% were females and 41.7% were within the 40-45 years age group. 75% of patients have experienced the onset of the flare symptoms following 1 week of the vaccination and 41.7% of symptoms have lasted more than 8 weeks. 66.7% who had this flare have received Sinopharm, while 25% received Covishield. In this flare 58.3% got polyarthritis,16.7% monoarthritis, 8.3% oligoarthritis and 16.7% generalized rash. By contrast, 42 (16.9%) patients who received the 2nd dose of the vaccine had flare symptoms. 90.5% of this population were females and 38.1% were within the 50-59 years age group. 42% got flare following 1st week of the vaccination and 57% of symptoms have lasted more than 8 weeks. 5 patients who had flare symptoms following the first dose reported having flare after the 2nd dose too. None of the flare symptoms following 1st or 2nd dose of the vaccinations needed hospitalization. Interestingly gender (p = 0.012), use of methotrexate (p = 0.043), and the presence of flare to the first dose (p = 0.02) were found to be significantly correlated with the occurrence of flare symptoms following 2nd dose of vaccination.

Conclusion
This study reveals a considerable incidence of non-severe RMD flare-ups following COVID vaccination, mainly with the 2nd dose. Further studying on the effects of repeated and periodic COVID vaccination among patients with RMDs is timely to reassure and to improve vaccine acceptance in this group of patients.
Disclosure
S.K. Ubhayasiri: None. D. Abeysinghe: None.
E049 The Initial Impacts of the UK-Wide Rheumatology Physiotherapy Capabilities Framework
Authors: Gregory William J , Chambers Hannah , McCrum Carol 
Published: April 2023

Abstract

Background/Aims
The Rheumatology Physiotherapy Capabilities Framework, first published September 2021, was developed to describe the physiotherapy-specific skills and knowledge unique to the speciality and to act as a profession standard. Dissemination strategies for the framework included profiling through professional organisations, specialist interest groups and online via Twitter and ResearchGate. This project aims to describe the initial dissemination of the Framework and to provide an evaluation of the areas of impact within rheumatology networks.

Methods
An appraisal was undertaken in October 2022 on how widely the Rheumatology Physiotherapy Capabilities Framework has been used and embedded in UK clinical practice and service delivery. Evaluation strategies included data on downloads since publication and a brief online survey of a convenience sample of experienced, specialist rheumatology physiotherapists. The open-ended survey question asked for “implementation feedback on your use of the framework” and allowed undirected free-text comments. Responses were analysed using qualitative content and thematic analysis.

Results
The framework has been downloaded from the British Society for Rheumatology website 154 times and the launch news story on the BSR website has been viewed 4145 times; the Framework has been accessed 354 times from ResearchGate; analysis of impact on the Twitter shows 33 tweets, 238 likes and 95 “re-tweets”.
Thematic analysis identified four key areas of use of the framework:(i) Increasing awareness of the physiotherapy role in a Rheumatology service(ii) Personal and team professional development(iii) Recruitment, job matching, re-banding and promotion(iv) Referral pathways and service improvement. Quotes from the survey responses are collated according to these identified themes in Table 1.

Conclusion
In its first year of publication, this brief survey indicates that the Rheumatology Physiotherapy Capabilities Framework has already started to accomplish many of the aims of its development. The ability for individuals and services to compare their capabilities to an agreed national standard is supporting staff and services in valuable ways. Service improvement projects have been an additional benefit of the framework. It has already become fairly well embedded in UK rheumatology practice; however, further dissemination, awareness-raising strategies and utilisation support are required to fully accomplish its aims.
Disclosure
W.J. Gregory: Honoraria; W.G. has received honoraria for speaker and advisory board from Abbvie, Novartis and Pfizer. H. Chambers: None. C. McCrum: Honoraria; C.McC. has received speaker honoraria from Novartis. Grants/research support; C.McC. has received a funding grant from Janssen.
E053 Effectiveness of colchicine among patients with COVID-19 infection: a randomised, open labelled, clinical trial
Authors: Jalal Aryan M , Aref Sheelan , Albustany Dashty 
Published: April 2023

Abstract

Background/Aims
In November 2019, there were abundant cases of COVID-19 for which the first case was reported in Wuhan, China. Cytokine storm syndrome is the severe immune reaction that may cause a severe tissue response in COVID-19 patients. Colchicine has an important role in inhibiting activation of NLRP3 inflammasome that predispose to decrease cytokine production. This study aimed to evaluate whether colchicine is effective in treatment of COVID-19 patients or not.

Methods
A randomized, open labelled, clinical trial of colchicine for the treatment of COVID-19, allocated between 8th May to 18th June 2021. Patients with mild, moderate, or severe COVID-19 infection; confirmed by real time PCR (RT-PCR) and/or lung involvement confirmed by computed tomography scan compatible with COVID-19. The colchicine tablet dosage was 0.5mg twice daily for 14 days added to the standard treatment versus control group who received standard treatment without colchicine, with the trial registration ID: NCT04867226. The study was conducted in Erbil City, Iraq with the endpoints being clinical, laboratory parameters duration of hospitalization and side effects.

Results
80 patients participated in the study. Fewer patients in the colchicine group had musculoskeletal symptoms (17.5%, p: 0.001) in comparison to the patients, who received control treatment. The serum ferritin level in most of patients who treated with colchicine returned to normal in contrast to the control group, whose serum ferritin level was still high (p: 0.041). Similarly, the average of CRP and D-dimer after treatment among the colchicine group participants was significantly lower than the control group, the P-values were 0.011 and 0.043, respectively. The colchicine group patients stayed for a shorter duration at the hospital (18.4 days) compared to the control group (24.24 days). P-value was 0.009. In addition to that the response and cure rate were higher in the colchicine group (56%) in the comparison to control group (43.1%) Table 1: Laboratory Parameters with musculoskeletal symptoms and duration of hospitalization of both Treatment Regimens..

Conclusion
The colchicine drug can be effective in treating patients with COVID-19 infection by improving musculoskeletal symptoms and inhibiting inflammatory biomarkers; it is also effective in reducing duration of hospitalization.
Disclosure
A.M. jalal: None. S. Aref: None. D. Albustany: None.