bluuebunny/crossref_metadata_2025_split huggingface dataset:
OA12 Autoantibodies are common in patients labelled as “idiopathic” interstitial lung disease suggesting a high prevalence of undiagnosed autoimmune connective tissue disease
Authors: Tansley Sarah L , Cotton Caroline V , McMorrow Fionnuala K , Lu Hui , Betteridge Zoe E , New Robert P , Spencer Lisa G , McHugh Neil J , Cooper Robert G ,   the UK-BILD Collaboration
Published: April 2022

Abstract

Background/Aims
In some patients, interstitial lung disease (ILD) may be the dominant or even sole clinically overt manifestation of an otherwise unrecognised autoimmune connective tissue disease (CTD). Accurate diagnosis can be challenging given considerable overlap of the clinical, radiological and histological disease features. Distinguishing CTD-related ILD (CTD-ILD) from idiopathic ILD can avoid a lung biopsy, enables appropriate immunosuppressive treatment, informs prognosis and facilitates monitoring for other CTD-associated complications. Autoantibodies are a hallmark feature of CTD, are highly disease-specific and their presence is strongly suggestive of covert CTD-ILD. We investigated patients who had been labelled with “idiopathic” ILD by their local hospital teams for the presence of autoantibodies that may suggest misdiagnosis.

Methods
Serum from two groups of patients recruited to UK Biomarkers of Interstitial Lung Disease (BILD) were analysed: 183 with a local diagnosis of idiopathic non-specific interstitial pneumonia (iNSIP) and 34 with a local diagnosis of idiopathic or Cryptogenic Organising Pneumonia (COP). Autoantibody status was determined by radio-immunoprecipitation.

Results
Overall, CTD-autoantibodies were identified in 15% of patients with a prior label of “idiopathic” ILD. CTD autoantibodies were more common in those with a prior diagnosis of COP, where more than 1 in 5 patient samples contained a potentially relevant autoantibody, see table. The autoantibodies identified included those readily detectable e.g. anti-Jo1, in addition to rarer antibodies not included in standard assays e.g. anti-EIF2B. Nearly half of all autoantibodies detected were anti-synthetase autoantibodies.

Conclusion
Covert CTD-ILD is potentially being missed as a diagnosis in patients who have been labelled by local teams as having idiopathic forms of inflammatory ILD (COP or iNSIP). More specific guidance on autoantibody testing and the interpretation of these test results could improve diagnosis and ensure patients receive more appropriate management.

Disclosure
S.L. Tansley: None. C.V. Cotton: None. F.K. McMorrow: None. H. Lu: None. Z.E. Betteridge: None. R.P. New: None. L.G. Spencer: None. N.J. McHugh: None. R.G. Cooper: None.
OA41 Audit of the rheumatology PIFU pathway at the UHMB Trust
Authors: Achieng Sheilla , Gray Leanne , Ottewell Lesley 
Published: April 2022

Abstract

Background/Aims
Patient initiated follow up (PIFU) is an initiative that allows patients to access follow-up when required by initiating their own appointments. The disruptive impact of the COVID-19 pandemic has created significant capacity constraints on many services that were already experiencing pressures prior to the pandemic, piquing interest in PIFU as a sustainable model of care. The BSR has produced draft PIFU guidance to support rheumatology units to develop and deliver safe, robust and sustainable PIFU care models. We audited our PIFU pathway in line with the BSR’s recommended standards to establish its safety and efficacy since its implementation in 2018.

Methods
This was a retrospective analysis of patients transferred to an active PIFU access plan within UHMBT between February 2018 and November 2019. Patients were identified by the informatics team from the active PIFU access plans. We captured data to include treatments used. The electronic case notes were reviewed to establish reasons for appointment triggers in those who contacted the service, analyse waiting times for clinical review further to making contact and assess subsequent outcomes. Data were entered and analysed in Microsoft Excel 2016.

Results
We had a total of 420 patients on PIFU. We audited 100 of these. 56 were female. Mean age was 63.4 years (24-96). The most common diagnosis was inflammatory arthritis (n = 78). The majority of patients (n = 53) were on a single disease-modifying anti-rheumatic drug (DMARD). 17 patients were on &gt; 1 DMARD and 5 patients were on prednisolone monotherapy, mean steroid dose 4.9 mg daily. 25 patients were not on any treatment. Of those on DMARDS, 63% (44) were on methotrexate, either as monotherapy or in combination with other DMARDs. 68% (30) of those on methotrexate were on a dose of ≤ 15mg weekly. Of the 100 patients, 9 triggered a review within the follow up period. This was usually via the nurse helpline (n = 7). Occasionally the GP triggered a review on behalf of the patient via the advice and guidance line (n = 2). The most common reason for a trigger was a flare of inflammatory arthritis (n = 7) and the remaining two appointments were due to side effects of DMARDs. Patients were contacted via nurse telephone call back within 48 hours of contacting the helpline (n = 8). One patient required an urgent face-to-face consultant review and was seen within 7 days. After contacting the service, all nine patients were reverted to regular follow-up.

Conclusion
Our results confirmed a robust PIFU pathway with appropriate safety nets ensuring prompt access to clinician input when needed. The use of PIFU pathway did not compromise care or result in any worsening clinical outcomes. After validation at 2 years, the majority of our patients on this pathway were onwardly managed through the PIFU model.

Disclosure
S. Achieng: None. L. Gray: None. L. Ottewell: None.
OA43 Development of a national across specialty multi-disciplinary team for management of hyper-inflammatory conditions during the SARS-CoV-2 pandemic
Authors: McLellan Kirsty , Reilly Liam , Pollock Louisa , Swann Olivia , Anderson Catriona , Martin Neil , Brennan Mary , Hague Rosie , Walsh Jo 
Published: April 2022

Abstract

Background/Aims
Effective multi-specialty team working is extremely beneficial in management of children with hyper-inflammatory conditions. With the recognition of paediatric inflammatory multisystem syndrome (PIMS) it became apparent a standardised process for discussion of patients would be beneficial. This includes urgent discussion, with access to multiple specialties, and sharing knowledge and experience in a novel condition. Delivering equitable healthcare including access to expertise, specific treatments and research is challenging in Scotland due to its geography. This is facilitated by successful clinical networks (Scottish Paediatric & Adolescent Rheumatology Network [SPARN] and Scottish Paediatric& Adolescent Infection & Immunology National Managed Clinical Network [SPAIIN]) and a well-established paediatric transport service (ScotSTAR). Our aim was to develop a multi-specialty multi-disciplinary team (MDT) for management of patients with hyper-inflammatory conditions.

Methods
Narrative account of MDT and service development

Results
We identified a core group of individuals with an interest in inflammatory disorders from different centres and specialties across Scotland including Rheumatology, Infectious Diseases, Cardiology, Intensive Care, Academic Paediatrics, Pharmacists and Clinical Nurse Specialists. Three priorities emerged from initial planning stages: urgent discussions around patient management, peer-to-peer learning and information sharing, and effective prioritisation of research. We designed a written framework and referral pathway, including criteria for acute cardiology involvement, ScotSTAR transfer and intensive care. An electronic proforma was designed to guide MDT discussion, medico-legal documentation and audit purposes. Table 1 describes characteristics of the first nine patients discussed. A particular strength was in diagnostics and consideration of differentials; among patients referred we identified those with haematological malignancy, systemic lupus erythematosus and non-accidental injury. Regular peer-review sessions were held, for reflection on cases and their management in both secondary and tertiary care settings. Clinicians throughout Scotland were encouraged to join via SPARN and SPAIIN networks.

Conclusion
This multi-specialty MDT has been and continues to be beneficial for management of hyper-inflammatory patients. We will review the process but hope the MDT will prove to continue to be beneficial for future patients. The authors would like to acknowledge all members of the MDT.

Disclosure
K. McLellan: None. L. Reilly: None. L. Pollock: None. O. Swann: None. C. Anderson: None. N. Martin: None. M. Brennan: None. R. Hague: None. J. Walsh: None.
P005 Performance of giant cell arteritis pre-test probability score in a tertiary care setting
Authors: Kadamban Premila , Pattapola Shyanthi , Alkoky Hoda , Jordan Natasha , Gupta Bhaskardas 
Published: April 2022

Abstract

Background/Aims
Giant cell arteritis (GCA) is a chronic, idiopathic, granulomatous vasculitis of medium and large arteries comprising overlapping phenotypes of cranial arteritis and extracranial GCA. Vascular complications are generally due to delay in diagnosis and initiation of effective treatment. Due to the imminent risk of visual loss and other ischaemic complications it is vital to secure the diagnosis and it is equally important to exclude the mimics as the treatment of GCA has it's own complications. The objective of this quality improvement project was to assess the performance of Southend GCA Pre-test Probability Score (GCA PS) in a cohort of patients who were referred urgently with suspected GCA to the Rheumatology Department of Addenbrooke’s Hospital.

Methods
We analysed the data of 50 new patients seen between Aug 2019 and Feb 2020. GCA PS were calculated based on the information from GPs (clinical and biochemical data). The likely hood of GCA was determined on review after 1 month, based on the clinical, biochemical, histopathological data and response to steroids.

Results
A total of 50 cases were included in the study. Thirty-eight (76%) of them were females while 12(24%) were males. The median age was 68.5. The median probability score was 12, it ranged between 0 to 24. Finally, 25 patients were diagnosed as likely to have GCA based on clinical, biochemical, histopathological findings and response to steroids. The lowest probability score among those who had a positive biopsy was 10. The p value for the likelihood of GCA when the probability score was 10 or higher was 0.0001 which was very significant.

Conclusion
GCA pre-test probability score is a very promising and utilitarian tool for risk stratification of patients with suspected GCA and prediction of the likelihood of GCA. It enables exclusion of GCA in cases with low probability score and to have a higher degree of scrutiny in those with intermediate and high probability scores. Clinical diagnostic algorithm based on the BSR guidelines could further be utilised for confirmation.

Disclosure
P. Kadamban: None. S. Pattapola: None. H. Alkoky: None. N. Jordan: None. B. Gupta: None.
P009 Biologics prescribing trends over 10 years: a retrospective analysis of 799 patients in routine clinical practice
Authors: Zubairi Rumaisa , Suhaimi Ak Muhd Adiib Pg , Olsson Lewis , Smyth Rachel , Baxter Derek , Laws Arrianne , Perry Martin E 
Published: April 2022

Abstract

Background/Aims
To investigate the biologic prescribing trends for a secondary care patient cohort over a 10-year period for rheumatoid arthritis (RA), psoriatic arthritis (PsA) and spondyloarthropathy (SpA), and compare against current local guidelines.

Methods
Departmental database holding patient data was interrogated. Labelled data were consolidated into distinct diagnostic categories of rheumatoid arthritis, psoriatic arthritis and spondyloarthropathy. For ease of analysis, biologic drugs were classified based on their mechanism of action into TNF-alpha blockers (TNFi) and non-TNF-alpha blockers (non-TNFi). Data was collated identifying prescribing trends of first, second and third-line therapies for RA, PsA and SpA. This was compared against local NHS guidelines. Overall cost of biologic prescription for first line therapies was calculated

Results
There were 799 patients with a diagnosis of RA, PsA or SpA. Both RA and PsA had the same general prescribing pattern of TNFi as first-line, another TNFi as second-line and non-TNFi as third-line. In SpA, TNFi were mostly used as first, second and third-line. An adalimumab biosimilar was used first-line in 90 (30.6%) RA patients, 42 (35.1%) PsA patients and 32 (32.3%) SpA patients. Methotrexate was co-prescribed with a TNF-alpha blocker in 128 (58.7%) RA patients, 51 (36.4%) PsA patients and 7 (7.7%) SpA patients. TNFi biosimilar drug prescribing has increased from 33% in 2016 to being the most prescribed drug in 2020 (62%). In the entire database cohort of 926 patients, the vast majority are prescribed biosimilars (Table 1). The overall cost of biologic prescriptions has reduced by 49% from 2019 to 2020 as the proportion of biosimilars prescribed has almost doubled to 69%.

Conclusion
Biologic prescribing patterns are largely in-keeping with current national and local guidelines. The vast majority of patients are on a biosimilar, which has resulted in significant cost-savings for the department.

Disclosure
R. Zubairi: None. A. Muhd Adiib Pg Suhaimi: None. L. Olsson: None. R. Smyth: None. D. Baxter: None. A. Laws: None. M.E. Perry: Honoraria; AbbVie, Celltrion Healthcare, Gilead, Lilly, Biogen, Jaansen, Fresenius Kabi.
P023 Comparative Utility of Ultrasound in Known Vs Query New inflammatory Arthritis - a Retrospective Case-Note Audit
Authors: Adikari Madura , Koutsianas Christos , John Holly , Klocke Rainer 
Published: April 2022

Abstract

Background/Aims
Ultrasound (US) imaging has become established in clinical Rheumatology practice to aid diagnosis and monitoring of inflammatory arthritis (IA). We firstly wanted to explore the utility of US in the assessment for active synovitis in subjects with known IA, but clinically uncertain disease control. Secondly we set out to compare this with the diagnostic contribution of US in patients presenting with new inflammatory joint symptoms and uncertain clinical synovitis.

Methods
Two contemporaneous samples of subjects were selected from consecutive, pre-Covid, Rheumatology Consultant-delivered Ultrasound Clinic lists in our department: (i) subjects with known IA where clinical disease activity was uncertain, and (ii) subjects without know IA who had symptoms with inconclusive signs of suspected IA. Both grey scale and power Doppler US imaging was used to determine a sonographic conclusion of active IA being present, absent or equivocal. Treatment changes/ decisions were ascertained from the next available clinic letter to explore diagnostic consequences following US.

Results
(i) In the sample of subjects with known IA (n = 30, mean age 53.9 years, 63% female, RA 76.7%, all on DMARDs [20% biologics]), 43.3%(n = 13) had US evidence of active IA, and 50% of inactive IA (and the remainder reported as equivocal). At clinic review (n = 30) only 6 of 13 subjects with active US IA had their DMARD treatment escalated (all within same DMARD) and 5 out of 15 subjects with inactive US IA their DMARD de-escalated. (ii) In the sample of subjects with suspected IA (n = 30, mean age 51.5 years, 73% female): 16.7% (n = 5) had US evidence of IA and 77.7% not (and remainder reported as equivocal). At the next clinic review seven patients were commenced on DMARDs: three patients with IA on US, and 4 without.

Conclusion
In our department, Rheumatology clinicians appear to be referring for diagnostic US in known IA from a situation approaching clinical equipoise which would suggest optimum referral practice. If we assume that this clinical equipoise also applies to the assessment and referral practice of new patients with arthralgia but uncertain clinical synovitis, our diagnostic sensitivity of US is approximately 16.7%, meaning: six patients with suspected IA need referral for US to identify one with sonographic IA. Irrespective of this, we observed that the sonographic diagnosis in known or suspected IA is commonly not followed by a concordant treatment decision, suggesting that the US diagnosis forms only part of the diagnostic ‘jigsaw’ decision process.

Disclosure
M. Adikari: None. C. Koutsianas: None. H. John: None. R. Klocke: None.
P028 An exploration of rheumatology patients’ perceptions and knowledge about sick day rules while using steroids for immunosuppression
Authors: Umair Hafiz M , Clark William , O'sullivan Rory , Fawthrop Fiona 
Published: April 2022

Abstract

Background/Aims
Steroids are commonly used for immunosuppression in the majority of rheumatology conditions. Patients on long-term steroids are vulnerable to adrenal insufficiency during acute illness or if steroids are stopped or weaned down abruptly. During the COVID-19 pandemic, this risk has further multiplied due to change in practice with inclusion of telephone consultations and less frequent hospital visits. Although our patients were routinely provided with steroid alert cards by pharmacy, we aim to ascertain the knowledge and understanding of sick day rules in patient who are on steroids for immunosuppression.

Methods
We designed a novel questionnaire consisting of 10 questions, designed to determine patients’ knowledge about steroid sick day rules. All questions were close-ended, either single best answer or yes/no except question about primary rheumatological condition and type of steroids. A total of 100 patients (including new and follow-ups) attending rheumatology clinics between January and June 2020 were screened and selected randomly. Questionnaires and consent forms were sent by post as agreed with clinical effectiveness team with return envelopes.

Results
29 completely filled questionnaires were received back. The majority of patients (96.5%; n = 28) were on prednisolone and only 3.44% (n = 1) were on hydrocortisone. The majority of the patients (95.6.6%; n = 29) were on steroids for duration between 1-6 months. Answering the question on steroid dose during an acute illness,10.3% (n = 3) patients had knowledge to take double dose of steroids, 55.1% (n = 16) responded to take regular dose, 3.44 (n = 1) answered by lowering dose to half, and strikingly 10.3% (n = 3) answered to stop steroids. For further question on when to seek medical advice during an acute illness while on steroids, 31.0% (n = 9) responded by answering correctly, 37.9% (n = 11) were unsure when to take medical attention, and 3.44% (n = 1) responded to wrong option. Despite providing steroids alert cards to everyone, only 65.5% (n = 19) answered yes on asking about carrying alert card with them.

Conclusion
Despite providing steroid alert card and verbal information, these data highlight a significant dearth of knowledge and understanding about sick day rules among rheumatology patients on steroids for immunosuppression. This knowledge gap increases the risk of potentially life-threatening emergency of adrenal crisis among this patient group. To bridge this knowledge gap, we started to provide steroid sick day rules written information leaflet to all new and old rheumatology patients on steroids to reduce risk of future adrenal crisis. We are aiming to re audit this after 1 year to check improvement in patient knowledge and understanding of sick day rules with this change in practice.

Disclosure
H.M. Umair: None. W. Clark: None. R. O'sullivan: None. F. Fawthrop: None.
P051 A rare mimic of inflammatory arthritis
Authors: Cox Miriam , Kazmi Muhammad F 
Published: April 2022

Abstract

Background/Aims
Rosai-Dorfman syndrome is a rare disorder, primarily affecting young males, characterised by overproliferation and accumulation of histiocytes. It commonly affects lymph nodes but can affect the nervous system, kidneys and skin. Bone involvement is rare (around 5%) and presents as lytic lesions. Diagnosis can be challenging as many cases spontaneously resolve. In severe cases steroids, radiotherapy or chemotherapy may be used.

Methods
We present a case of a 34-year-old gentleman with a 4-month history of fatigue, widespread arthralgia and unintentional weight loss. Pain was severe, nocturnal and involved joints and long bones. There was no joint swelling. He had recently had a self-limiting episode of headaches and night sweats that was presumed secondary to a viral infection. He had no rash and no features suggestive of connective tissue disease. He did not smoke or drink alcohol and had no recent travel history. His symptoms responded to NSAIDS but recurred quickly.

Results
On examination, he had a tender right elbow and tender bilateral tibia. There was no evidence of synovitis. He had no lymphadenopathy. X-rays of elbows and knees showed no effusion or erosions. Viral and autoimmune screens were negative. He had a normocytic anaemia with Hb 108. His CRP was rising at 57, having been 27 when initially referred; this was felt inconsistent with a reactive arthritis. LDH was normal along with normal WBC count. A PET-CT was requested to investigate for malignancy, this showed multiple foci of tracer uptake throughout the skeleton but no lymph node involvement. He was referred to haematology for tissue diagnosis and had two bone biopsies, one of which was suspicious for histiocytosis. A bone marrow biopsy showed non-necrotising histiocytic granulomas. Langerhan's Histocytosis was considered and a repeat PET arranged. This showed progressive increased uptake throughout the skeleton and bone marrow with new lytic lesions. There was tracer uptake in the skin, lung and kidneys, reported as possible Rosai-Dorfman disease. No evidence of primary malignancy was found after extensive investigations. He then developed frontal and occipital skin lesions. MRI head showed dural lesions, suspicious for meningioma. He was referred to dermatology for biopsy of skin lesions. However, the skin and dural lesions spontaneously resolved. This was associated with resolution of fatigue and arthralgia. His CRP settled to 2.8 and anaemia resolved 6 months after presenting. A diagnosis of spontaneously remitting Rosai-Dorfman disease was made in conjunction with the national histiocytosis advisory panel. He remains under surveillance.

Conclusion
This case highlights a rare presentation of a rare condition. As rheumatologists we often see cases which present a diagnostic challenge. Rosai-Dorfman disease has characteristic appearances on PET and should be considered as a diagnosis in patients with atypical joint pain and abnormal PET-CTs.

Disclosure
M. Cox: None. M.F. Kazmi: None.