Objectives To quantify the risk of death among people with rare autoimmune rheumatic diseases (RAIRD) during the UK 2020 COVID-19 pandemic compared with the general population, and compared with their pre-COVID risk. Methods We conducted a cohort study in Hospital Episode Statistics for England 2003 onwards, and linked data from the NHS Personal Demographics Service. We used ONS published data for general population mortality rates. Results We included 168 691 people with a recorded diagnosis of RAIRD alive on 01/03/2020. Their median age was 61.7 (IQR 41.5–75.4) years, and 118 379 (70.2%) were female. Our case ascertainment methods had a positive predictive value of 85%. 1,815 (1.1%) participants died during March and April 2020. The age-standardised mortality rate (ASMR) among people with RAIRD (3669.3, 95% CI 3500.4–3838.1 per 100 000 person-years) was 1.44 (95% CI 1.42–1.45) times higher than the average ASMR during the same months of the previous 5 years, whereas in the general population of England it was 1.38 times higher. Age-specific mortality rates in people with RAIRD compared with the pre-COVID rates were higher from the age of 35 upwards, whereas in the general population the increased risk began from age 55 upwards. Women had a greater increase in mortality rates during COVID-19 compared with men. Conclusion The risk of all-cause death is more prominently raised during COVID-19 among people with RAIRD than among the general population. We urgently need to quantify how much risk is due to COVID-19 infection and how much is due to disruption to healthcare services.

Introduction: Optimal management of people with advanced NSCLC depends on accurate identification of predictive markers. Yet, real-world data in this setting are limited. We describe the impact, timeliness, and outcomes of molecular testing for patients with advanced NSCLC and good performance status in England. Methods:In collaboration with Public Health England, patients with stages IIIB to IV NSCLC, with an Eastern Cooperative Oncology Group performance status of 0 to 2, in England, between June 2017 and December 2017, were identified. All English hospitals were invited to record information.Results: A total of 60 of 142 invited hospitals in England participated in this study and submitted data on 1157 patients. During the study period, 83% of patients with advanced adenocarcinoma underwent molecular testing for three recommended predictive biomarkers (EGFR, ALK, and programmed death-ligand 1). A total of 80% of patients with nonsquamous carcinomas on whom biomarker testing was performed had adequate tissue for analysis on initial sampling. First-line treatment with a tyrosine kinase inhibitor was received by 71% of patients with adenocarcinoma and a sensitizing EGFR mutation and by 59% of those with an ALK translocation. Of patients with no driver mutation and a programmed death-ligand 1 expression of greater than or equal to 50%, 47% received immunotherapy. Conclusions:We present a comprehensive data set for molecular testing in England. Although molecular testing is well established in England, timeliness and uptake of targeted therapies should be improved.

Background and Aims Psychological morbidity is increased in young people with inflammatory bowel disease [IBD]. Illness perceptions may be an important factor. This study aimed to describe the prevalence and severity of psychological morbidity and to examine relationships between baseline illness perceptions and anxiety, depression, and health-related quality of life [HRQoL], at baseline and 12 months later, in 16–21 year olds with IBD. Methods IBD patients [n = 121] completed measures of anxiety, depression, HRQoL, and illness perceptions [IPQ-R] at baseline and follow-up [n = 100, 83%]. Results Among the 121 patients at baseline [median age 19.3 years, 40% female, 62% Crohn’s disease, 73% in clinical remission], 55% reported elevated symptoms of anxiety/depression and 83% reported low HRQoL. Negative illness perceptions at baseline were significantly correlated with greater anxiety, depression, and lower HRQoL at baseline and follow-up. In regression analysis at baseline, the IPQ-R domain of greater perception of a cyclical nature of IBD was an independent predictor of anxiety, and a greater perceived emotional impact of IBD was an independent predictor of anxiety, depression, and HRQoL. Female gender and clinical relapse were also independent predictors of lower HRQoL. After controlling for baseline measures, clinical risk factors and illness perceptions did not explain additional variance in psychological morbidity at follow-up. Conclusions A high prevalence of psychological morbidity, stable over 1 year, was demonstrated in young people with IBD. Having negative illness perceptions, being female, and having active disease predicted those at greatest risk of psychological morbidity. Illness perceptions may be an appropriate target for psychological interventions.

Objectives: To quantify the risk of death among people with rare autoimmune rheumatic diseases (RAIRD) during the UK 2020 COVID-19 epidemic compared with baseline risk and the risk of death in the general population during COVID-19. Design A cohort study using data from the National Congenital Anomaly and Rare Disease Registration Service (NCARDRS). We used ONS published data for general population mortality rates. Setting: Hospital Episode Statistics for England 2003 onwards, and linked data from the NHS Personal Demographics Service. Participants: 168,691 people with RAIRD who were alive on 1 March 2020. Their median age was 61.7 (IQR 41.5-75.4) years, and 118,379 (70.2%) were female. Our case ascertainment methods had a positive predictive value >85%. Main outcome measure: Age-standardised mortality rates for all-cause death. Secondary outcome measures were age-sex standardised mortality rates, and age-stratified mortality rates. Results: 1,815 (1.1%) participants died during March and April 2020. The age-standardised mortality rate (ASMR) among people with RAIRD (3669.3, 95% CI 3500.4-3838.1 per 100,000 person-years) was 1.44 (95% CI 1.42-1.45) times higher than the average ASMR during the same months of the previous 5 years, whereas in the general population of England it was 1.38 times higher. Compared to the general population, the age-specific mortality rates in people with RAIRD compared to the pre-COVID rates were higher from the age of 35 upwards, whereas in the general population the increased risk began from age 55 upwards. Sex-specific rates were similar in males and females, whereas in the general population females had a lower rate than males. Conclusions: The risk of all-cause death is more prominently raised during COVID-19 among people with RAIRD than among the general population. We urgently need to quantify how much risk is due to COVID-19 infection and how much is due to disruption to healthcare services, in order to inform better guidance about shielding, access to healthcare and vaccine priorities for people with rare diseases.