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The factors determining disease course and survival in fibrotic hypersensitivity pneumonitis (fHP) have not been fully elucidated.The aim of this study was to describe the characteristics of patients with fHP in a real-world cohort and investigate factors associated with worse outcomes. We aimed to explore the use of neutrophil to lymphocyte ratio (NLR) and peripheral blood monocyte levels in predicting mortality.MethodsA retrospective, multicentre, observational UK cohort study.ResultsPatients with fHP were significantly younger than those with idiopathic pulmonary fibrosis (IPF) (median age fHP 73 vs IPF 75 years) and were much more likely to be woman (fHP 61% vs IPF 26%). In almost half of all fHP cases (49%, n=104/211), no causative antigen was identified from either the history or specific antigen testing. Overall, fHP was associated with a better survival than IPF, although median survival of both groups was poor (fHP 62 months vs IPF 52 months).IPF survival in patients with a high NLR was significantly lower than those with a low NLR (44 vs 83 months). A monocyte count ≥0.95 K/uL also predicted significantly poorer outcomes for patients with IPF compared with ConclusionsAlthough fHP has a statistically lower mortality than IPF, absolute survival time of both conditions is poor. High baseline NLR and absolute monocyte counts predict worse survival in IPF but not in fHP, highlighting the potential for divergence in their pathogenic mechanisms.

Rheumatoid arthritis (RA) is the most common of the connective tissue diseases (CTD), affecting up to 0.75% of the United States (U.S.) population with an increasing prevalence. Interstitial lung disease is prevalent and morbid condition in RA (RA-ILD), affecting up to 60% of patients with RA, leading to premature death in 10% and accruing an average of US$170,000 in healthcare costs per patient over a 5-year period. Although there have been significant advances in the management of this joint disease, there are no ongoing randomized clinical trials looking at pharmacologic treatments for RA-ILD, and there currently are no U.S. Food and Drug Administration-approved drugs for RA-ILD. We describe the Treatment for Rheumatoid Arthritis and Interstitial Lung Disease 1 (TRAIL1) trial, a multicenter randomized, double-blind, placebo-controlled, phase 2 study of the safety, tolerability and efficacy of pirfenidone in patients with RA-ILD. The study will enroll approximately 270 subjects across a network of sites who have RA and ILD as defined by a fibrotic abnormality involving greater than 10% of the lung parenchyma. The primary endpoint of the study is the incidence of the composite endpoint of decline in percent predicted forced vital capacity of 10 or greater or death during the 52-week study period. A number of secondary and exploratory endpoints have been chosen to evaluate the safety and efficacy in different domains. The TRAIL1 trial is designed to evaluate the safety and efficacy of pirfenidone in RA-ILD, a disease with significant impact on patients’ quality of life and outcome. In addition to investigating the safety and efficacy of pirfenidone, this trial looks at a number of exploratory endpoints in an effort to better understand the impact of therapy on areas such as changes in quantitative high-resolution computed tomography scores and a patient’s quality of life. Biospecimens will be collected in order to investigate biomarkers that could potentially predict the subtype of disease, its behavior over time, and its response to therapy. Finally, by creating a network of institutions and clinician investigators with an interest in RA-ILD, this trial will pave the way for future studies of investigational agents in an effort to reduce or eliminate the burden of disease for those suffering from RA-ILD. Genentech, a member of the Roche Group. Clinicaltrials.gov, identifier NCT02808871.

Rationale: The impact of coronavirus disease (COVID-19) on patients with interstitial lung disease (ILD) has not been established. Objectives: To assess outcomes in patients with ILD hospitalized for COVID-19 versus those without ILD in a contemporaneous age-, sex-, and comorbidity-matched population. Methods: An international multicenter audit of patients with a prior diagnosis of ILD admitted to the hospital with COVID-19 between March 1 and May 1, 2020, was undertaken and compared with patients without ILD, obtained from the ISARIC4C (International Severe Acute Respiratory and Emerging Infection Consortium Coronavirus Clinical Characterisation Consortium) cohort, admitted with COVID-19 over the same period. The primary outcome was survival. Secondary analysis distinguished idiopathic pulmonary fibrosis from non–idiopathic pulmonary fibrosis ILD and used lung function to determine the greatest risks of death. Measurements and Main Results: Data from 349 patients with ILD across Europe were included, of whom 161 were admitted to the hospital with laboratory or clinical evidence of COVID-19 and eligible for propensity score matching. Overall mortality was 49% (79/161) in patients with ILD with COVID-19. After matching, patients with ILD with COVID-19 had significantly poorer survival (hazard ratio [HR], 1.60; confidence interval, 1.17–2.18; P = 0.003) than age-, sex-, and comorbidity-matched controls without ILD. Patients with an FVC of Conclusions: Patients with ILD are at increased risk of death from COVID-19, particularly those with poor lung function and obesity. Stringent precautions should be taken to avoid COVID-19 in patients with ILD.

RationaleThe impact of COVID-19 on patients with Interstitial Lung Disease (ILD) has not been established.ObjectivesTo assess outcomes following COVID-19 in patients with ILD versus those without in a contemporaneous age, sex and comorbidity matched population.MethodsAn international multicentre audit of patients with a prior diagnosis of ILD admitted to hospital with COVID-19 between 1 March and 1 May 2020 was undertaken and compared with patients, without ILD obtained from the ISARIC 4C cohort, admitted with COVID-19 over the same period. The primary outcome was survival. Secondary analysis distinguished IPF from non-IPF ILD and used lung function to determine the greatest risks of death.Measurements and Main ResultsData from 349 patients with ILD across Europe were included, of whom 161 were admitted to hospital with laboratory or clinical evidence of COVID-19 and eligible for propensity-score matching. Overall mortality was 49% (79/161) in patients with ILD with COVID-19. After matching ILD patients with COVID-19 had higher mortality (HR 1.60, Confidence Intervals 1.17-2.18 p=0.003) compared with age, sex and comorbidity matched controls without ILD. Patients with a Forced Vital Capacity (FVC) of ConclusionsPatients with ILD are at increased risk of death from COVID-19, particularly those with poor lung function and obesity. Stringent precautions should be taken to avoid COVID-19 in patients with ILD.

Objective This study explores whether the prognosis of interstitial lung disease in rheumatoid arthritis (RA-ILD) has improved over time and assesses the potential influence of drug therapy in a large multicentre UK network. Methods We analysed data from 18 UK centres on patients meeting criteria for both RA and ILD diagnosed over a 25-year period. Data included age, disease duration, outcome and cause of death. We compared all cause and respiratory mortality between RA controls and RA-ILD patients, assessing the influence of specific drugs on mortality in four quartiles based on year of diagnosis. Results A total of 290 RA-ILD patients were identified. All cause (respiratory) mortality was increased at 30% (18%) compared with controls 21% (7%) (P =0.02). Overall, prognosis improved over quartiles with median age at death rising from 63 years to 78 years (P =0.01). No effect on mortality was detected as a result of DMARD use in RA-ILD. Relative risk (RR) of death from any cause was increased among patients who had received anti-TNF therapy [2.09 (1.1–4.0)] P =0.03, while RR was lower in those treated with rituximab [0.52(0.1–2.1)] or mycophenolate [0.65 (0.2–2.0)]. Patients receiving rituximab as their first biologic had longer three (92%), five (82%) and seven year (80%) survival than those whose first biologic was an anti-TNF agent (82%, 76% and 64%, respectively) (P =0.037). Discussion This large retrospective multicentre study demonstrates survival of patients with RA-ILD has improved. This may relate to the increasing use of specific immunosuppressive and biologic agents.

We are very grateful to the University Hospitals of Leicester NHS Trust Charitable Funds for supporting the funding of the recumbent cycle ergometer. Dr. Rachael Evans is funded by a National Institute for Health Research (NIHR) clinician scientist fellowship CS-2016-16-020. Dr Neil Greening is funded by a NIHR post-doctoral fellowship PDF-2017-10-052. This research was supported by the NIHR Leicester Biomedical Research Centre. The views expressed in this article are those of the author(s) and not necessarily those of the NHS, the NIHR, or the Department of Health and Social Care.

Objective Anti-nuclear autoantibodies are known to occur in 85-99% of Systemic Sclerosis (SSc) patients, with each SSc autoantibody correlating with a distinct clinical subset of patients. The objective of this study was to investigate novel SSc autoantibodies in the remaining autoantibody negative patients and establish clinical associations. Methods Serum samples and clinical data were collected from 548 SSc patients. Sera were tested for known SSc autoantibodies by routine serological techniques, with negative samples being further investigated by radiolabelled protein immunoprecipitation (IPP). Sera that immunoprecipitated a novel 30 kDa band were analysed by indirect immunofluorescence and IPP using depleted cell extracts to establish a common reactivity. Mass spectrometry (MS) was used to identify the novel autoantigen and findings were confirmed using commercial antibodies. Sera from 426 patients with other forms of connective tissue disease, 103 patients with rheumatoid arthritis, 114 patients with idiopathic ILD and 150 healthy controls were serotyped as controls. Results A novel autoantigen with a molecular weight of ∼30 kDa was recognised by seven sera with SSc, six of whom had interstitial lung disease (ILD) and by no controls. Six of the patients had diffuse cutaneous involvement and four had overlap features with other autoimmune diseases. Immunodepletion experiments indicated that all samples targeted the same autoantigen and MS identified the novel autoantigen as eIF2B (Eukaryotic Initiation Factor 2B). Conclusion We report a novel autoantibody (anti-eIF2B) in a small number of patients with SSc (approximately 1%) that is closely associated with diffuse cutaneous manifestations and the presence of ILD. This article is protected by copyright. All rights reserved.

Objectives. The prevalence of interstitial lung disease (ILD) in RA is 5%. Previous work identified increasing age, active articular disease and articular damage as risk factors for RA-associated ILD (RA-ILD). The roles of high-resolution CT (HRCT) and lung function testing in defining the nature and extent of pulmonary involvement have recently been explored. This study is the first to examine predictive and prognostic factors for the development of RA-ILD and to report on the physiological and radiological characteristics of the condition from a large multicentre UK network. Methods. We collected data from centres across the UK on patients with both RA and ILD (proved on HRCT) diagnosed over a 25-year period from 1987 to 2012 using a standard pro forma. Potential predictors of RA-ILD were analysed. Baseline lung function data were recorded and related to HRCT findings. We analysed HRCT for subtype and extent of lung involved and examined the relationship between these and both all-cause and pulmonary mortality. We compared our results with case controls matched for age and gender using computer-generated selection from the RA population from one contributing centre. Results. A total of 230 patients were identified from across the UK with proven RA-ILD diagnosed over 25 years. Median age at diagnosis was 64 years and the male:female ratio was 1:1.09. Univariate analysis showed anti-CCP antibody titres to be the single most strongly associated predictor of RA-ILD. Male gender, age at onset, smoking and RF were all independently associated with RA-ILD on multivariate analysis. Vital capacity (VC) was preserved in limited disease but reduced in extensive disease, while gas transfer was reduced in both. Usual interstitial pneumonia (UIP) was the most common subtype on HRCT and both this and extensive disease were associated with increased all-cause mortality. Conclusion. This is the largest study of RA-ILD in the UK. Anti-CCP antibodies were strongly associated with RA-ILD in both sexes. Smoking was strongly associated with ILD in males, which may explain the higher frequency of RA-ILD in men. The predominant HRCT pattern was UIP and most patients had limited disease at presentation. The presence of UIP and extensive disease are associated with increased mortality. Baseline gas transfer is a useful screening tool for ILD, while the preservation of VC at baseline might predict limited disease on HRCT.

A factor, however, that was not directly addressed by Holland et al. [1] was the presentation of ILD in rheumatological patients. Because of locomotor difficulties and muscle weakness, in part associated with corticosteroid use, our experience is that patients often have a lower usual level of exercise than patients without joint disease. By the time they notice exertional breathlessness from ILD, disease extent is often greater than that of patients with idiopathic ILD at presentation, who are not limited by extrapulmonary disease. In addition, whilst rheumatologists are well aware of the importance of musculoskeletal physiotherapy, they may be less familiar with the role of PR. Addressing the holistic needs of those patients with sarcoidosis and rheumatoid disease is a crucial part of exercise prescription to enhance the potential for the patients to progress during their course of PR. We hope to see future work that directly addresses the specific PR challenges of this group of patients. We read with great interest the paper by Holland et al. [1] addressing the specific needs of patients attending pulmonary rehabilitation (PR) with an interstitial lung disease (ILD) associated with rheumatological disease. There is accumulating evidence to indicate that PR is safe and effective for patients with ILD [2] . In particular, there has been a recent focus on the importance of patients with idiopathic pulmonary fibrosis (IPF) attending classes. This may be due to idiopathic pulmonary fibrosis being the most common and progressive ILD, alongside the recent publication of quality standards [3] . Holland et al. [1] highlight the challenges of undertaking exercise regimens in patients with rheumatological ILD, not solely because of breathlessness, but also because of the additional difficulties related to joint pain, stiffness and inflammation. These extrapulmonary manifestations mean that, even with normal resting pulmonary function, subjects with rheumatoid arthritis have a lower cardiopulmonary exercise capacity than controls [4] . Published online: June 3, 2015

To investigate novel systemic sclerosis (SSc) autoantibodies in autoantibody-negative patients and establish clinical associations.Serum samples and clinical data for 548 patients with SSc were collected. Routine serologic techniques were used to test the serum samples for known SSc autoantibodies, and samples with negative results were further investigated by radiolabeled-protein immunoprecipitation assay. Sera that immunoprecipitated a novel 30-kd band were analyzed by indirect immunofluorescence and immunoprecipitation, using depleted cell extracts to establish a common reactivity. Mass spectrometry was performed to identify the novel autoantigen, and the results were confirmed using commercial antibodies. Sera from 426 patients with other forms of connective tissue disease, 103 with rheumatoid arthritis, 114 with idiopathic interstitial lung disease (ILD), and 150 healthy subjects were serotyped as controls.A novel autoantigen with a molecular weight of ∼30 kd was recognized by 7 sera from patients with SSc, 6 of whom had ILD, and by no controls. Six of the patients had diffuse cutaneous involvement, and 4 had overlap features with other autoimmune diseases. Immunodepletion experiments indicated that all samples targeted the same autoantigen, and mass spectrometry identified the novel autoantigen as eukaryotic initiation factor 2B (eIF2B).We report the identification of a novel autoantibody (anti-eIF2B) in a small number of patients with SSc (∼1%); this autoantibody is closely associated with diffuse cutaneous manifestations and the presence of ILD.

Introduction/objectives Idiopathic Pulmonary Fibrosis is progressive with poor outcomes. Evidence from the CAPACITY and ASCEND trials suggests Pirfenidone slows disease progression (Noble et al , 2011; King et al , 2014). We reviewed all patients with more than a year since initiation to assess the proportion that discontinued Pirfenidone for adverse drug reactions (ADRs) or due to unsuccessful treatment (>10%/year%FVC decline or died on therapy), to assess temporal patterns of these stoppages and assess any demographic or disease extent predictors of success (age, sex, BMI, smoking history, %FVC prediction and FEV1). Methods 155 patients have been referred to our consultant pharmacist for Pirfenidone initiation since August 2013, of which 65 started and have more than 1 year of data (i.e., we excluded those starting post July 2015). We reviewed hospital databases and medical notes with subsequent data analysis using appropriate parametric statistical methods. Results 42/65 (64.6%) stopped therapy overall divided between ADRs (24/42, 57.1%), FVC decline >10%/year (11/42, 26.2%), dying on treatment (5/42, 11.9%) and unclear (2/42, 4.8%). Table 1 demonstrates association between demographics and those that discontinued, suggesting there is one subgroup associated with treatment continuation (patient previously smoked P ≤ 0.001). The temporal pattern of stopping treatment for ADRs vs those failing for clinical reasons (death on treatment or FVC decline) demonstrated a median cessation time of 40 days vs 226 respectively. Conclusions Our data suggests two important findings. Firstly, patients’ chances of continuing therapy is intriguingly affected positively by smoking history, causation for which remains unclear. Sex, age, BMI or disease extent does not seem to be associated with outcome success. Secondly, those that discontinued for ADRs did so much earlier than those that discontinued due to FVC decline or dying on treatment. Potential implications for when prescribing Pirfenidone are that we cannot predict those at risk of discontinuation from their demographics. However, a smoking history intriguingly seems to be positively associated with continuation. Additionally, most patients affected by ADRs ceased treatment early, suggesting that if side effects are not noted post early follow up, they are unlikely to occur later. This information improves understanding of prescribing practice for Pirfenidone.

The HLA class II (DRB1 and DQB1) associations with sarcoidosis have been studied by several groups but often without consistent results. In this paper, we consider the hypothesis that observed inconsistencies relate to distinct, genetically encoded disease phenotypes which differ in prevalence between centres. We therefore typed HLA-DRB1 and DQB1 in 340 UK, 139 Dutch and 163 Japanese sarcoidosis patients and, respectively, 354, 218 and 168 healthy controls from these populations. We applied consistent phenotyping and genotyping and investigated associations between HLA class II alleles and distinct disease phenotypes within and between ethnic groups. DRB1*01 and DQB1*0501 are protective against all manifestations of sarcoidosis. Lung-predominant sarcoidosis is associated with DRB1*12 and *14. Löfgren's syndrome is a common sarcoidosis phenotype in the Dutch and is strongly associated with the DRB1*0301 allele. This phenotype is not seen among the Japanese in whom DRB1*0301 is absent. The same allele is protective for UK uveitis. Sarcoid uveitis is common in Japan. The DRB1*04–DQB1*0301 haplotype is a risk factor for this disease manifestation in Japanese and UK subjects but protective for sarcoidosis overall. We show that distinct sarcoidosis phenotypes have similar genetic associations across ethnic groups. The disease case mix differs between centres and may be explained by different ethnic allelic frequencies.

Lung involvement is common in patients who have complications of connective tissue disease (CTDs) and causes considerable morbidity and mortality. High resolution CT (HRCT) has a pivotal role in the detection of lung fibrosis. In patients who have coexistent pathologic processes, HRCT often allows the predominant process to be identified. HRCT has an important role in detecting possible complications such as opportunistic infection or the development of malignancy. However, the limitations of HRCT should not be overlooked. In many cases, HRCT appearances are nonspecific and may or may not be related to an underlying CTD. Thus, radiologic findings should never be interpreted without knowledge of the clinical picture.

Introduction Pulmonary Rehabilitation is recommended for patients with Idiopathic Pulmonary Fibrosis (IPF) although the magnitude of benefit appears less compared to those with other chronic lung diseases. Patients with IPF may not be able to sustain high-intensity training to induce physiological change due to a ventilatory limitation to exercise. One strategy to circumvent this in COPD has been to reduce the exercising muscle mass by cycling one leg at a time during the same exercise session. Randomised controlled trials have shown greater improvements in exercise capacity after training using one-legged cycling (OLC) compared to two-legged cycling (TLC).1,2 We, therefore, compared OLC to TLC responses during incremental and constant work rate (CWR) exercise in patients with IPF. Methods Patients were recruited from a tertiary referral centre if they met the current NICE diagnostic criteria for IPF with a MRC dyspnoea grade ≥2. Exclusion criteria included a requirement for long-term oxygen therapy. Participants completed four Cardiopulmonary Exercise Tests (CPETs) to intolerance on a cycle ergometer with expired gas analysis. The tests were completed on separate days: 1) two-legged maximal incremental test (TLC-ICE); 2) one-legged maximal incremental test (OLC-ICE); 3) two-legged CWR (TLC-CWR) test at 70% peak power achieved on the TLC-ICE; 4) one-legged CWR (OLC-CWR) test at 35% TLC-ICE peak power. Results Twelve participants (11 male, mean [SD] 73 [8] yrs, BMI 30.6 [4.8] kg/m2, FVC% predicted 71.8 [20.3]%, resting SpO2 98 [1]%) completed all four CPETs demonstrating a ventilatory limitation to exercise (92 [14]% maximum voluntary ventilation [MVV]). Although the OLC-ICE peak oxygen uptake (peak VO­2) was significantly lower than the peak VO2 TLC-ICE (p < 0.001) the OLC: TLC was high at 0.85. The OLC-CWR was endured for more than twice the TLC-CWR (p < 0.001) at the same muscle-specific power leading to almost double the work being performed ([Table 1][1]). View this table: Abstract P134 Table 1 A comparison between Two-Legged (TLC) vs. One Legged (OLC) Constant Work Rate (CWR) exercise tests Conclusion OLC at the same muscle-specific power compared to TLC enabled patients with IPF to achieve almost double the work in a simulated exercise training session. Future research should investigate OLC as a potentially efficacious aerobic training strategy for patients with IPF. References 1 Bjorgen SJ. Eur J ApplPhysiol 2009; 106 :501–507 2 Dolmage TE. Chest 2008; 133 :370–376 [1]: #T1

Background There is little evidence base to guide clinicians in the management of patients with rheumatoid arthritis (RA) related interstitial lung disease (ILD). In the subgroup who have rapidly deteriorating respiratory function, pulsed cyclophosphamide has been advocated but no assessment of its efficacy has been published. The British Rheumatoid InterstitiaL Lung (BRILL) network has collected data on the treatment and outcomes of 260 patients with RA-ILD, which allow assessment of this therapeutic strategy. Objectives To assess the effect of pulsed IV cyclophosphamide on the outcome of patients with RA-ILD Methods We identified 260 patients across 16 UK centres who met the 2010 EULAR criteria for RA and had confirmation of ILD on high resolution computed tomography (HRCT) of the lungs. We analysed demography, all drug therapy, duration of disease, subtype and extent of ILD on HRCT, smoking history, serology and baseline pulmonary function. For each patient who had received pulsed intravenous cyclophosphamide, we identified two case controls with RA-ILD who had never received this drug and who were matched for age, gender, disease subtype and extent. We compared duration of both RA and of ILD between the two groups, as well as vital capacity (VC) and gas transfer (TLco). Mortality and mean survival times in each group were compared. Results We identified 21 patients who had received pulsed cyclophosphamide for progressive RA-ILD. Ten were male and the group median age was 71 (58-86) years. Most patients were smokers (13/21) and all were seropositive. Median duration of ILD and RA were 2 and 11 years respectively in both the cyclophosphamide treated group and in the case controls. In both groups, 14 patients had UIP (with 7 NSIP), and 14 patients had extensive disease (with 7 limited). Median baseline % predicted VC (range) was lower in the cyclophosphamide patients at 71% (40-117%) than in case controls 84% (55-134%) [p=0.015], as was TLco at 45% (25-109%) vs 57% (27-82%) [p=0.04]. Mortality was identical between the groups at 24%, with mean survival better in those treated with cyclophosphamide than in case controls (72 vs 43 months)[p=0.13]. Conclusions This data from the BRILL network shows that the use of pulsed cyclophosphamide for patients with progressive RA-ILD is associated with mortality and survival at least as good as in RA-ILD patients with lesser impairment of baseline pulmonary function. This retrospective analysis supports the early use of such therapy in patients with deteriorating RA-ILD. A prospective study similar to that conducted in scleroderma lung disease would now seem appropriate to confirm these results and define an optimal treatment regime. Disclosure of Interest None declared DOI 10.1136/annrheumdis-2014-eular.2342

Background Rheumatoid arthritis (RA) is associated with interstitial lung disease (ILD). This is linked with an increased risk of early death from respiratory failure. There is debate on the relative safety of different disease modifying anti rheumatic drugs (DMARDs) in RA-ILD. Objectives We have examined the effect of a range of DMARDs on all cause and respiratory mortality in a large multi centre UK wide retrospective study to address this issue. Methods The British Rheumatoid InterstitiaL Lung (BRILL) network comprises clinicians from seven centres across the UK. We collected data on DMARD use in patients with both RA and ILD (proven on HRCT) identified over a 25 year period from 1987 to 2012 using a standard proforma. We also analysed the age, duration of both RA and ILD, outcome and, where appropriate, cause of death. Equivalent data were obtained from a control group of RA patients without lung disease, matched for age, sex and disease duration. We recorded the number of patients with RA-ILD on each of six commonly prescribed DMARDs, including methotrexate. We used Chi squared tests to assess the significance of any differences in disease characteristics between patients on different DMARDs. We calculated relative risk (RR) for all cause and respiratory mortality in patients with RA-ILD treated with each of these agents. Results A total of 260 patients were identified from across the UK with proven RA-ILD. DMARDs had been prescribed in 218 (84%) patients, and comprised methotrexate (52%), sulphasalazine (50%), leflunomide (21%), hydroxychloroquine (19%), gold salts (14%) and penicillamine (5%). Over half of patients had received at least two different DMARDs. There were no differences between the patients on different DMARDs with regard to age, gender, disease duration, subtype and extent, smoking, serology and lung function. The RR of death from any cause and from respiratory disease were not significantly increased by any commonly used DMARD. Specifically, methotrexate was not associated with any excess increase in mortality. However, gold salts were associated with a RR of 5.3 for death from respiratory disease, although numbers were small. Relative risk (RR) of death in RA patients on DMARDs Conclusions Patients with RA-ILD have increased mortality compared to controls. Most are treated with DMARDs for their articular disease. There is no evidence to relate the more commonly used agents to the increase in mortality. This large retrospective BRILL network study suggests that it is safe to prescribe most DMARDs, including methotrexate, to patients with RA-ILD, although caution should be shown in the use of gold salts. Acknowledgements Kieran Kelly for preparing the database Disclosure of Interest None declared DOI 10.1136/annrheumdis-2014-eular.1246

Background Clinically relevant interstitial lung disease (ILD) is present in approximately 5% of patients with rheumatoid arthritis (RA). Traditionally, oral steroids have been used to treat this manifestation of RA, but no evidence base exists to support this. Indeed, concern has been raised that such an approach might be associated with increased mortality in ILD [1]. Objectives We have examined the effect of oral steroid therapy on mortality in a large multi centre group of patients retrospectively to assess the effect of these agents on survival. Methods For the purposes of this study, we collected data from multiple centres across the UK on patients with both RA and ILD (proven on HRCT) identified over a 25 year period from 1987 to 2012 using a standard proforma. We analysed the age, duration of both RA and ILD, outcome and, where appropriate, cause of death. Equivalent data were obtained from a control group of RA patients from one centre without lung disease, matched for age, sex and disease duration. We recorded the number of patients with RA-ILD who had received oral prednisone therapy for 3 months or more. We used Chi squared tests to assess the significance of any differences in disease characteristics between those who had steroids and those who had not. We calculated relative risk (RR) of all cause and respiratory mortality in all patients with RA-ILD, and in those treated with steroids. Results A total of 260 patients were identified from across the UK with proven RA-ILD. Steroids had been used in 154 (59%) RA-ILD patients, but only 36 (14%) controls, p=0.01. There were no differences between RA-ILD patients on steroids and those not on steroids with regard to age, gender, disease duration and extent of ILD, smoking, serology and lung function. However, steroid treated patients were significantly more likely to have usual interstitial pneumonia (UIP), p=0.02. The RR of death from any cause was significantly increased in steroid treated RA-ILD patients [1.65 (1.2-2.3)], p=0.002, but was normal in those who had not received steroids [1.07 (0.7-1.6)]. The RR of respiratory death was more significantly increased in those who had been on steroids [2.75 (1.6-4.7)], p=0.0002, than in those who had not received steroids [2.06 (1.1-3.8)], p=0.02. Conclusions Patients with RA-ILD have increased mortality over controls, and over half have been treated with oral steroids. These patients are at increased relative risk of death, especially from respiratory causes. The excess of UIP in the steroid treated group is likely to account for some of the excess mortality. This large retrospective multi centre study suggests long term oral steroids should be avoided where possible in patients with RA-ILD, and reflects earlier findings in patients with ILD alone. Disclosure of Interest None declared DOI 10.1136/annrheumdis-2014-eular.1247