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.

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Background: People dying from interstitial lung disease experience considerable symptoms and commonly die in an acute healthcare environment. However, there is limited understanding about the quality of their end-of-life care. Aim: To synthesise evidence about end-of-life care in interstitial lung disease and identify factors that influence quality of care. Design: Systematic literature review and narrative synthesis. The review protocol was prospectively registered with PROSPERO (CRD42020203197). Data sources: Five electronic healthcare databases were searched (Medline, Embase, PubMed, Scopus and Web of Science) from January 1996 to February 2021. Studies were included if they focussed on the end-of-life care or death of patients with interstitial lung disease. Quality was assessed using the Critical Appraisal Skills Programme checklist for the relevant study design. Results: A total of 4088 articles were identified by initial searches. Twenty-four met the inclusion criteria, providing evidence from 300,736 individuals across eight countries. Most patients with interstitial lung disease died in hospital, with some subjected to a high burden of investigations or life-prolonging treatments. Low levels of involvement with palliative care services and advance care planning contributed to the trend of patients dying in acute environments. This review identified a paucity of research that addressed symptom management in the last few days or weeks of life. Conclusions: There is inadequate knowledge regarding the most appropriate location for end-of-life care for people with interstitial lung disease. Early palliative care involvement can improve accordance with end-of-life care wishes. Future research should consider symptom management at the end-of-life and association with location of death.

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

Idiopathic pulmonary fibrosis (IPF) is a chronic, progressive fibrosing interstitial lung disease of unknown aetiology. Patients typically present with symptoms of chronic dyspnoea and cough over a period of months to years. IPF has a poor prognosis, with an average life expectancy of 3–5 years from diagnosis if left untreated. Two anti-fibrotic medications (nintedanib and pirfenidone) have been approved for the treatment of IPF. These drugs slow disease progression by reducing decline in lung function. Early diagnosis is crucial to ensure timely treatment selection and improve outcomes. High-resolution computed tomography (HRCT) plays a major role in the diagnosis of IPF. In this narrative review, we discuss the importance of early diagnosis, awareness among primary care physicians, lung cancer screening programmes and early IPF detection, and barriers to accessing anti-fibrotic medications.

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