Survival differences in rheumatoid arthritis interstitial lung disease and idiopathic pulmonary fibrosis may be explained by delays in presentation: results from multivariate analysis in a monocentric UK study.

Rheumatoid arthritis-associated interstitial lung disease (RA-ILD) has a better prognosis compared to idiopathic pulmonary fibrosis (IPF). Recent data suggest that antifibrotics are effective in slowing progression across both groups. Hence, we designed this study to investigate the similarities and differences between these groups of patients. This is a retrospective cohort study examining baseline data, progression and outcomes in patients with RA-ILD and IPF prior to antifibrotic use in the Coventry ILD database. Ethics approval was obtained from the University Hospital Coventry and Warwickshire NHS Trust. Statistical analysis was performed using R software and Cox's proportional hazards technique was used for survival analysis. We identified 131 cases, including 49 patients with IPF, 34 patients with RA-ILD and 48 patients with other forms of idiopathic interstitial pneumonia. At baseline, there were significant differences in the groups with RA-ILD patients being significantly younger (65.7 vs 72.4 years), had preserved lung volumes (FVC 95% vs 84.7%) and higher gas transfer (61.5% vs 48.2%) compared to IPF patients. 5-year survival was better for RA-ILD compared to IPF (87.5% vs 40.4%, p = 0.0042). Univariate analysis revealed gas transfer, FVC, age, sex and phenotype (IPF or RA-ILD) were all significant predictors, but multivariate analysis revealed that gas transfer and age were both significantly associated with prognosis, whereas sex, FVC or phenotype were not significant. This study suggests that the difference between RA-ILD and IPF prognosis may be due to demographics and early diagnosis rather than the diseases behaving differently. This has important management implications.

Relapse after cessation of weekly tocilizumab for giant cell arteritis: a multicentre service evaluation in England.

OBJECTIVES: The National Health Service in England funds 12 months of weekly subcutaneous tocilizumab (qwTCZ) for patients with relapsing or refractory giant cell arteritis (GCA). During the COVID-19 pandemic, some patients were allowed longer treatment. We sought to describe what happened to patients after cessation of qwTCZ. METHODS: Multicentre service evaluation of relapse after stopping qwTCZ for GCA. The log-rank test was used to identify significant differences in time to relapse. RESULTS: 336 GCA patients were analysed from 40 centres, treated with qwTCZ for a median (interquartile range, IQR) of 12 (12-17) months. At time of stopping qwTCZ, median (IQR) prednisolone dose was 2 (0-5) mg/day. By 6, 12 and 24 months after stopping qwTCZ, 21.4%, 35.4% and 48.6% respectively had relapsed, requiring an increase in prednisolone dose to a median (IQR) of 20 (10-40) mg/day. 33.6% of relapsers had a major relapse as defined by EULAR. Time to relapse was shorter in those that had previously also relapsed during qwTCZ treatment (P = 0.0017); in those not in remission at qwTCZ cessation (P = 0.0036); and in those with large vessel involvement on imaging (P = 0.0296). Age ≥65, gender, GCA-related sight loss, qwTCZ treatment duration, TCZ taper, prednisolone dosing, and conventional synthetic DMARD use were not associated with time to relapse. CONCLUSION: Up to half our patients with GCA relapsed after stopping qwTCZ, often requiring a substantial increase in prednisolone dose. One third of relapsers had a major relapse. Extended use of TCZ or repeat treatment for relapse should be considered for these patients.

Development and testing of a bespoke cultural intervention to support healthcare professionals with patients from a diverse background.

OBJECTIVE: Development and test of a culturally sensitive intervention for rheumatology healthcare professionals (HCPs). METHODS: Using a before and after study design, fifteen HCPs were recruited to undertake the bespoke intervention from four NHS sites across England, in areas serving a diverse population. The intervention was evaluated using the validated outcomes: [1] Patient Reported Physician Cultural Competency (PRPCC); and [2] Patient Enablement Instrument (PEI), measuring patients' perceptions of their overall healthcare delivery. Additionally, HCPs completed the Capability COM-B questionnaire (C), Opportunity (O) and Motivation (M) to perform Behaviour (B), measuring behaviour change. RESULTS: 200 patients were recruited before HCPs undertook the intervention (cohort 1), and 200 were recruited after (cohort 2) from fifteen HCPs, after exclusions 178 patients remained in cohort 1 and 186 in cohort 2. Patients identifying as White in both recruited cohorts were 60% compared with 29% and 33% of patients (cohorts 1 and 2 respectively) who identified as of South Asian origin. After the intervention, the COM-B scores indicated HCPs felt more skilled and equipped for consultations. No significant differences were noted in the average overall cultural competency score between the two cohorts in White patients (57.3 vs 56.8, p= 0.8), however, in the South Asian cohort, there was a statistically significant improvement in mean scores (64.1 vs 56.7, p= 0.014). Overall, the enablement score also showed a statistically significant improvement following intervention (7.3 vs 4.3, p< 0.001) in the White patients; and in the South Asian patients (8.0 vs 2.2, p< 0.001). CONCLUSION: This novel study provides evidence for improving cultural competency and patient enablement in rheumatology settings.

Accessing care during the pandemic: A UK wide survey of people with rheumatoid arthritis and adult juvenile inflammatory arthritis during the COVID-19 pandemic.

COVID-19 drastically changed healthcare delivery models for rheumatology services. We sought to understand the impact of these changes for patients with Rheumatoid Arthritis (RA) and adult Juvenile Inflammatory Arthritis (AJIA) in established patients and those newly diagnosed during the pandemic. RESULTS: Of the 316 participants, a significant proportion regularly used analgesics (45.4%, n = 119), corticosteroids (17.9%, n = 47) and Non-Steroidal Anti-Inflammatory Drugs [(NSAIDs) (36.6%, n = 96)]. Two thirds of participants (66.5%, n = 210) did not know their Disease Activity Score-28 (DAS28). Of the remaining third, moderate disease activity (12%, n = 38) was most reported. We found that 16.8% (n = 53) felt their condition was managed well during the pandemic. The remainder felt more negatively. For the newly diagnosed cohort, 34.5% (n = 10) delayed seeking GP help because of COVID-19 concerns. Once assessed, a quarter (24.1%, n = 7) were referred to rheumatology after 4 or more consultations. We found 47% (n = 77) expressed positive opinions on remote consultations, whereas 36% (n = 59) had concerns. The lack of clinical examination (42.5%, n = 25) was flagged. Changing the dynamic from health worker to a patient centred approach was the most wished for improvement (20.3%, n = 64). CONCLUSIONS: Most participants did not know their disease activity status, which is of concern. With a push towards patient-centred and patient-led care, education and supported self-management is critically important. There is high use of NSAIDs and corticosteroids. Pathways of care underwent change with subsequent delays in specialist assessment. The introduction of patient-initiated follow-up (PIFU) and virtual consultations further distances healthcare professionals from patients and could affect outcomes.

Four-factor risk score for the prediction of interstitial lung disease in rheumatoid arthritis.

OBJECTIVE: Interstitial lung disease (ILD) is one of the commonest systemic complications in patients with rheumatoid arthritis (RA) and carries a significant morbidity and mortality burden. We aimed to identify key variables to risk-stratify RA patients in order to identify those at increased risk of developing ILD. We propose a probability score based on the identification of these variables. METHODS: A retrospective, multicentre study using clinical data collected between 2010 and 2020, across 20 centres. RESULTS: A total of 430 RA (210 with ILD confirmed on high-resolution computed tomography (HRCT)) patients were evaluated. We explored several independent variables for the risk of developing ILD in RA and found that the key significant variables were smoking (past or present), older age and positive rheumatoid factor/anti-cyclic citrullinated peptide. Multivariate logistic regression models were used to form a scoring system for categorising patients into high and low risk on a scale of 0-9 points and a cut-off score of 5, based on the area under the receiver operating characteristic curve of 0.76 (CI 95% 0.71-0.82). This yielded a sensitivity of 86% and a specificity of 58%. High-risk patients should be considered for investigation with HRCT and monitored closely. CONCLUSION: We have proposed a new model for identifying RA patients at risk of developing ILD. This approach identified four simple clinical variables: age, anti-cyclic citrullinated peptide antibodies, Rheumatoid factor and smoking, which allowed development of a predictive scoring system for the presence of ILD in patients with RA.

Role of Temporal artery biopsy in a sequential Giant Cell Arteritis fast-track pathway: a 5-year prospective study.

BACKGROUND: Increasing number of centres are establishing sequential fast track pathways (FTP) for management of giant cell arteritis (GCA), with temporal artery ultrasound (US) replacing temporal artery biopsy (TAB) as the first investigational method. Biopsy is performed as second investigation, when US is negative/inconclusive. This study investigates the role of TAB in a sequential GCA-FTP and its utility in those with negative/inconclusive US. METHODS: Prospective study of patients referred for TAB as part of Coventry sequential GCA-FTP May 2014-June 2019. Analysis included sensitivity and specificity of TAB, impact of arterial specimen length and duration of treatment with corticosteroids on sensitivity of TAB and the clinical predictors for a positive biopsy. RESULTS: A total of 1149 patients with suspected GCA were referred to this GCA-FTP, with 109 (9.5%) referred for TAB. Overall sensitivity of TAB was 47% (specificity: 100%) and in patients with negative/inconclusive US sensitivity was 39% (specificity:100%). Post-fixation arterial specimen length <15 mm showed lower sensitivity (14%), which increased to 52% when specimen length was ≥15 mm. Sensitivity of TAB was highest in first 7 (60%) to 10 days (59%) from starting corticosteroids. Predictors of positive biopsy using univariate logistic regression analysis were jaw claudication (OR = 5.40; p = 0.0057), elevated erythrocyte sedimentation rate (OR = 5.50; p = 0.013) and elevated C-reactive protein (OR = 23.7; p = 0.0043). CONCLUSION: This is the first study to look at the role of TAB in a sequential GCA-FTP. Biopsy plays an important role in GCA-FTP, when US is negative/inconclusive. Sensitivity of TAB improved when specimen length was ≥15 mm and performed within 10 days of commencing corticosteroids.

Adherence with mycophenolate mofetil in patients with autoimmune inflammatory rheumatic diseases in coventry: Signs of progress but challenges remain.

OBJECTIVE: The study investigated adherence with MMF treatment among patients attending rheumatology clinics at University Hospitals Coventry and Warwickshire NHS Trust (UHCW) with Autoimmune inflammatory rheumatic diseases (AIIRDs). METHODS: This retrospective study collated hospital pharmacy data in patients who requested the prescription for MMF between January 2015 and December 2018. Clinical data were obtained from paper and electronic notes. Data were analysed using Microsoft Excel. Ethical approval was obtained through Coventry University. RESULTS: We recruited 144 patients into this study with age range from 18 to 91 years, including 100 females and 44 males. There were 112 White patients, 22 of South Asian origin, 3 East Asian and 4 black patients. SLE (56), scleroderma (18), mixed connective tissue disease (15), myositis (13), vasculitis (13) were the commonest diagnoses. Overall adherence with Mycophenolate mofetil was 62%. The adherence rates were below 80% for all age groups with ∼60% of patients having adherence levels of >60%. Poor adherence with MMF correlated with 3-fold increase in risk of flares compared to good adherence (p = 0.002). We also found a significant difference between Asian patients (mean adherence 47%) and White patients (mean adherence 65%, p < 0.001). CONCLUSION: Adherence with MMF has improved considerably compared to historical studies, although these remain suboptimal. Certain population groups such as young adults, elderly and Asian patients continue to have lower adherence and higher risk of flares. Strategies are needed to improve adherence levels overall and specifically in the high-risk groups to reduce risk of flares and organ damage.

Worse outcomes linked to ethnicity for early inflammatory arthritis in England and Wales: a national cohort study.

OBJECTIVE: To assess variability in care quality and treatment outcomes across ethnicities in early inflammatory arthritis (EIA). METHODS: We conducted an observational cohort study in England and Wales from May 2018 to March 2020, including patients with a suspected/confirmed EIA diagnosis. Care quality was assessed against six metrics defined by national guidelines. Clinical outcomes were measured using DAS28. Outcomes between ethnic groups ('White', 'Black', 'Asian', 'Mixed', 'Other') were compared, and adjusted for confounders. RESULTS: A total of 35 807 eligible patients were analysed. Of those, 30 643 (85.6%) were White and 5164 (14.6%) were from ethnic minorities: 1035 (2.8%) Black; 2617 (7.3%) Asian; 238 (0.6%) Mixed; 1274 (3.5%) Other. In total, 12 955 patients had confirmed EIA, of whom 11 315 were White and 1640 were from ethnic minorities: 314 (2.4%) Black; 927 (7.1%) Asian; 70 (0.5%) Mixed; 329 (2.5%) Other. A total of 14 803 patients were assessed by rheumatology within three weeks, and 5642 started treatment within six weeks of referral. There were no significant differences by ethnicity. Ethnic minority patients had lower odds of disease remission at three months [adjusted odds ratio 0.79 (95% CI: 0.65, 0.96)] relative to White patients. Ethnic minorities were significantly less likely to receive initial treatment withMTX[0.68 (0.52, 0.90)] or with glucocorticoids [0.63 (0.49, 0.80)]. CONCLUSION: We demonstrate that some ethnic minorities are less likely to achieve disease remission in three months following EIA diagnosis. This is not explained by delays in referral or time to treatment. Our data highlight the need for investigation into the possible drivers of these inequitable outcomes and reappraisal of EIA management pathways.

Chilblains in immune-mediated inflammatory diseases: a review.

Chilblains were first described over a hundred years ago as cutaneous inflammatory lesions, typically on the digits, occurring on cold exposure. Chilblains can be primary, or secondary to a number of conditions such as infections, including COVID-19, and immune-mediated inflammatory disorders (IMIDs) with SLE being the commonest. Chilblain lupus erythematosus (CHLE) was first described in 1888 as cold-induced erythematous lesions before the terms 'chilblains' or 'perniosis' were coined. Diagnostic criteria exist for both chilblains and CHLE. Histopathologically, CHLE lesions show interface dermatitis with perivascular lymphocytic infiltrate. Immunofluorescence demonstrates linear deposits of immunoglobulins and complement in the dermo-epidermal junction. This narrative review focuses on chilblains secondary to immune-mediated inflammatory disorders, primarily the epidemiology, pathogenesis and treatment of CHLE.

Patient Empowerment: Apni Jung (Our Fight) against Rheumatoid Arthritis for South Asian Population.

Covid-19 has affected many populations in the UK, and ethnic minority communities in particular. People from ethnic minority communities living with long-term chronic diseases have shown to be less engaging with self-management and report having poor medication adherence. The main reason to this problem is the way information is delivered to non-English speaking patients. This editorial discusses an innovation to over this barriers in rheumatology practice.

Rheumatological complications of Covid 19.

INTRODUCTION: COVID-19 has caused unprecedented hardships in the 21st century with more than 150 million infections. Various immunological phenomena have been described during the course of the infection, and this infection has also triggered autoimmunity. Rheumatological illnesses have been described following resolution of the acute infection; hence we sought to conduct a review of the rheumatological complications of COVID-19. METHODS: We conducted a literature search for articles relating to sequelae of COVID-19 from Jan 2020 to 30th April 2021. RESULTS: We found a number of reports of inflammatory arthritis after SARS-CoV-2 infection. SLE and renal disease have been described, and vasculitis also appears to be a common complication. Rhabdomyolysis and myositis has also been reported in a number of patients. We also found some evidence of large vessel vasculitis in 'long COVID' patients. CONCLUSIONS: This review highlights a number of important complications such as inflammatory arthritis, lupus-like disease, myostis and vasculitis following SARS-CoV-2 infection.

Understanding the impact of systemic lupus erythematosus on work amongst South Asian people in the UK: An explorative qualitative study.

SLE has a range of fluctuating symptoms affecting individuals and their ability to work. Although South Asian (SA) patients are at increased risk of developing SLE there is limited knowledge of the impact on employment for these patients in the UK. Understanding ethnicity and disease-specific issues are important to ensure patients are adequately supported at work. Semi-structured interviews were conducted with patients of SA origin to explore how SLE impacted on their employment. Thematic analysis was used to analyse the data which are reported following COREQ guidelines. Ten patients (8 female; 2 male) were recruited from three rheumatology centres in the UK and interviewed between November 2019 and March 2020. Patients were from Indian (n = 8) or Pakistani (n = 2) origin and worked in a range of employment sectors. Four themes emerged from the data: (1) Disease related factors; (2) Employment related factors; (3) Cultural and interpersonal factors impacting on work ability; (4) Recommendations for improvement. Patients' ability to work was affected by variable work-related support from their hospital clinicians, low awareness of SLE and variable support from their employers, and cultural barriers in their communities that could affect levels of family support received. These findings highlight the need for additional support for SA patients with SLE in the workplace.

Respiratory subtype of relapsing polychondritis frequently presents as difficult asthma: a descriptive study of respiratory involvement in relapsing polychondritis with 13 patients from a single UK centre.

INTRODUCTION: Relapsing polychondritis is a rare multisystem vasculitis characterised by recurrent cartilage inflammation. Respiratory involvement, of which tracheobronchomalacia (TBM) is the commonest form, is difficult to treat and is linked to increased mortality. We describe 13 patients with respiratory involvement. METHODS: This is a retrospective study of all the patients with relapsing polychondritis at University Hospitals Coventry and Warwickshire NHS Trust (UHCW), a secondary care provider for ∼500 000. Only patients with respiratory involvement were included in this study. RESULTS: We identified 13 patients who fulfilled the inclusion criteria. Most patients were identified from the "difficult asthma" clinic. TBM was seen in 11 patients, whilst two patients had pleural effusions which resolved with immunosuppression and one patient had small airways disease. Computed tomography scans (inspiratory and expiratory) and bronchoscopy findings were useful in diagnosing TBM. Pulmonary function testing revealed significant expiratory flow abnormalities. All patients were treated with corticosteroids/disease-modifying anti-rheumatic drugs (DMARDs) and some were given cyclophosphamide or biological agents, although the response to cyclophosphamide (1 out of 4) or biologicals (2 out of 4) was modest in this cohort. Ambulatory continuous positive airway pressure ventilation was successful in four patients. CONCLUSIONS: Relapsing polychondritis may be overlooked in "difficult asthma" clinics with patients having TBM (not asthma) and other features of relapsing polychondritis. Awareness of this condition is crucial to enable early diagnosis and interventions to reduce the risk of life-threatening airway collapse. A number of patients respond well to DMARDs and are able to minimise corticosteroid use.

Patient perceptions of co-morbidities in inflammatory arthritis.

OBJECTIVE: Longer life expectancy has resulted in people living with an increasing number of co-morbidities. The average individual with inflammatory arthritis has two co-morbidities, which contribute to higher mortality, poorer functional outcomes and increased health-care utilization and cost. A number of studies have investigated the prevalence of co-morbidities, whereas this study was designed to look at patient perspectives. METHODS: The study comprised two parts: a patient questionnaire and an interview. Individuals with physician-verified inflammatory arthritis along with one or more Charlson co-morbidities were invited to participate. In-depth data were obtained by interviews with 12 willing participants. RESULTS: One hundred and forty-six individuals were recruited; 50 (35%) had one co-morbidity, 69 (48%) had two and 25 (17%) had more than four co-morbidities. Seventy-seven individuals (53%) reported that co-morbidities affected their health as much as their arthritis, and 82 (56%) reported dependence on others for activities of daily living. Lack of education was highlighted by 106 (73%) participants. Qualitative data provided further support for the challenges, with participants highlighting the lack of time to discuss complex or multiple problems, with no-one coordinating their care. This, in turn, led to polypharmacy and insufficient discussion around drug and disease interactions, complications and self-help measures. CONCLUSION: This study highlights the challenges for individuals with inflammatory arthritis who suffer with multiple co-morbidities. The challenges result from limited resources or support within the current health-care environments. Individuals highlighted the poor quality of life, which is multifactorial, and the need for better educational strategies and coordination of care to improve outcomes.

Anti-tumour necrosis factor treatment for the prevention of ischaemic events in patients with deficiency of adenosine deaminase 2 (DADA2).

OBJECTIVE: To evaluate the impact of anti-Tumour Necrosis Factor-α (anti-TNF) treatment on the occurrence of vasculitic ischaemic events in patients with deficiency of adenosine deaminase 2 (DADA2). METHODS: A retrospective analysis of DADA2 patients referred from six centres to Great Ormond Street Hospital for Children was conducted. Ischaemic events, vasculitic disease activity, biochemical, immunological, and radiological features were compared, before and after anti-TNF treatment. RESULTS: A total of 31 patients with genetically confirmed DADA2 were included in the study. The median duration of active disease activity prior to anti-TNF treatment was 73 months (inter-quartile range [IQR] 27.5-133.5 months). Twenty seven/31 patients received anti-TNF treatment for a median of 32 months (IQR 12.0-71.5 months). The median event rate of central nervous system (CNS) and non-CNS ischemic events before anti-TNF treatment was 2.37 per 100 patient-months (IQR 1.25-3.63); compared with 0.00 per 100 patient-months (IQR 0.0-0.0) post-treatment (p< 0.0001). Paediatric vasculitis activity score (PVAS) was also significantly reduced: median score of 20/63 (IQR 13.0-25.8/63) pre-treatment vs. 2/63 (IQR 0.0-3.8/63) following anti-TNF treatment (p< 0.0001), with mild livedoid rash being the main persisting feature. Anti-TNF treatment was not effective for severe immunodeficiency or bone marrow failure, which required haematopoietic stem cell transplantation (HSCT). CONCLUSION: Anti-TNF treatment significantly reduced the incidence of ischaemic events and other vasculitic manifestations of DADA2, but was not effective for immunodeficiency or bone marrow failure.

Delay in seeking medical help in patients with rheumatoid arthritis in India: A qualitative study.

BACKGROUND: Rheumatoid arthritis (RA) is an autoimmune disease with varied articular and extra-articular manifestations. In developing countries such as India patients with RA often delay seeking medical advice which may impact prognosis and disease burden. AIM: To explore perceptions and experiences of patients living in India in seeking medical help for their RA symptoms. METHODS: Clinician-diagnosed RA participants from different socioeconomic backgrounds were interviewed using a semi-structured topic guide. Participants were purposively selected and interviewed following an iterative approach. All interviews were recorded and transcribed by an independent company and analyzed using a thematic framework. Findings were reported in accordance with consolidated criteria for reporting qualitative research guidelines. RESULTS: Twenty participants (13 male, 7 female) with median age 40 years (35.7-46.5) were recruited. Three overarching themes demonstrating participants' experiences and reasons for delay in seeking medical help were identified. (1) "Symptoms perspectives and delay in participants' journey" narrated participants' experiences of having RA symptoms, how these were perceived, rationalized and led to delay. (2) "Participants' experience of the healthcare system" illustrated delay in referral, reaching diagnosis and treatment initiation highlighting their experiences with the health system. (3) "Recommendations for improving care" where participants made recommendations for reducing the delay at local and national levels. CONCLUSION: This is the first qualitative study which explored perceptions and experiences of RA patients in India resulting in delay. Improved provision of rheumatology care, effective referral system and greater involvement of government at local and national levels are needed to improve the delay in seeking medical help for Indian patients.