scholarly journals P054 Risk of death during the 2020 UK COVID-19 epidemic among people with rare diseases compared to the general population

Rheumatology ◽  
2021 ◽  
Vol 60 (Supplement_1) ◽  
Author(s):  
Megan Rutter ◽  
Peter C Lanyon ◽  
Emily Peach ◽  
Matthew J Grainge ◽  
Richard B Hubbard ◽  
...  

Abstract Background/Aims  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. Methods  A cohort study was performed using data from the National Congenital Anomaly and Rare Disease Registration Service (NCARDRS). Coded diagnoses for RAIRD were identified from Hospital Episode Statistics from 2003 onwards. Previous coding validation work demonstrated our case ascertainment methods had a positive predictive value >85%. ONS published data were used for general population mortality rates. The main outcome measure was age-standardised mortality rates (ASMRs) for all-cause death. Secondary outcome measures were age-sex standardised mortality rates, and age-stratified mortality rates. Results  168,691 people with RAIRD 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. 1,815 (1.1%) people with RAIRD died during March and April 2020. The ASMR among people with RAIRD was 3669.3 (95% CI 3500.4-3838.1) per 100,000 person-years, which was 1.44 (95% CI 1.42-1.45) times higher than the average ASMR during the same months of the previous 5 years. In the whole population of England, the ASMR during March and April 2020 was 1361.1 (1353.6- 1368.7) per 100,000 people, which was 1.38 times higher than the average ASMR during the same months of the previous 5 years (see related abstract about influenza seasons). Unlike in the general population, sex-specific rates in RAIRD were similar in males and females. When comparing risk of death during COVID-19 to pre-COVID-19, people with RAIRD had an increased risk of death from age 35 upwards, compared to around age 55 upwards in the general population. As the protective effect of being female was not seen in RAIRD, the group at the largest increased risk compared to their pre-COVID-19 risk were women aged 35 upwards. The absolute risk of all-cause death for someone aged 20-29 with RAIRD was similar to someone in the general population aged >20 years older, someone aged 40-49 years with RAIRD similar to someone in the general population 20 years older, and someone aged 60-69 with RAIRD similar to someone in the general population aged >10 years older. Conclusion  The excess risk of all-cause death during COVID-19 occurs at a younger age among people with RAIRD than among the general population, and particularly affects females. . We urgently need to quantify how much risk is due to COVID-19 infection and how much due to disruption to healthcare services to inform better guidance about shielding, access to healthcare and vaccine priorities for people with rare diseases. Disclosure  M. Rutter: None. P.C. Lanyon: None. E. Peach: None. M.J. Grainge: None. R.B. Hubbard: None. J. Aston: None. M. Bythell: None. S. Stevens: None. F.A. Pearce: None.

Rheumatology ◽  
2020 ◽  
Author(s):  
Emily Peach ◽  
Megan Rutter ◽  
Peter Lanyon ◽  
Matthew J Grainge ◽  
Richard Hubbard ◽  
...  

Abstract 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.


Author(s):  
Emily Peach ◽  
Megan Rutter ◽  
Peter Lanyon ◽  
Matthew J Grainge ◽  
Richard Hubbard ◽  
...  

AbstractObjectivesTo quantify the risk of death among people with rare autoimmune rheumatic diseases (RAIRD) during the UK 2020 COVID-19 pandemic compared to the general population, and compared to their pre-COVID risk.MethodsWe 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.ResultsWe 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 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. Women had a greater increase in mortality rates during COVID-19 compared to men.ConclusionThe 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.Key messagesPeople with RAIRD had an increased risk of dying during COVID-19 from age 35 years onwards, whereas in the general population it increased from the age of 55 onwards.Women had a greater increase in their risk of death during COVID-19 compared to men.The risk of working age people with RAIRD dying during COVID-19 was similar to that of someone 20 years older in the general population.


Rheumatology ◽  
2021 ◽  
Vol 60 (Supplement_1) ◽  
Author(s):  
Megan Rutter ◽  
Peter C Lanyon ◽  
Matthew J Grainge ◽  
Richard B Hubbard ◽  
Emily J Peach ◽  
...  

Abstract Background/Aims  To quantify the risk of death among people with vasculitis during the UK 2020 COVID-19 epidemic compared with baseline risk, risk during annual influenza seasons and risk of death in the general population during COVID-19. Methods  We performed a cohort study using data from the National Congenital Anomaly and Rare Disease Registration Service (NCARDRS) under their legal permissions (CAG 10-02(d)/2015). Coded diagnoses for vasculitis (ANCA-associated vasculitis, Takayasu arteritis, Behçet's disease, and giant cell arteritis) were identified from Hospital Episode Statistics from 2003 onwards. Previous coding validation work demonstrated a positive predictive value >85%. The main outcome measure was age-standardised mortality rates (ASMRs) for all-cause death. ONS published data were used for general population mortality rates. Results  We identified 55,110 people with vasculitis (median age 74.9 (IQR 64.1-82.7) years, 68.0% female) alive 01 March 2020. During March-April 2020, 892 (1.6%) died of any cause. The crude mortality rate was 9773.0 (95% CI 9152.3-10,435.9) per 100,000 person-years. The ASMR was 2567.5 per 100,000 person-years, compared to 1361.1 (1353.6-1368.7) in the general population (see table). The ASMR in March-April 2020 was 1.4 times higher than the mean ASMR for March-April 2015-2019 (1965.6). The increase in deaths during March-April 2020 occurred at a younger age than in the general population. We went on to investige the effect of previous influenza seasons. The 2014/15 season saw the greatest excess all-cause mortality nationally in recent years, and there were 624 deaths in 38,888 people (6472.5 person-years) with vasculitis in our data (crude mortality rate 9640.8 (8913.3-10427.7); The ASMR was 2657.6, which was marginally higher than the ASMR among people with vasculitis recorded during March-April 2020 during the COVID-19 pandemic. Conclusion  People with vasculitis are at increased risk of death during circulating COVID-19 and influenza epidemics. The ASMR among people with vasculitis was high both during the 2014/15 influenza season and during the first wave of the COVID-19 epidemic. COVID-19 vaccination and annual influenza vaccination for people with vasculitis are both important, regardless of patient age. Disclosure  M. Rutter: None. P.C. Lanyon: Grants/research support; PCL has received funding for research from Vifor Pharma.. M.J. Grainge: None. R.B. Hubbard: None. E.J. Peach: Grants/research support; EJP has received funding for research from Vifor Pharma. M. Bythell: None. J. Aston: None. S. Stevens: None. F.A. Pearce: Grants/research support; FAP has received funding for research from Vifor Pharma..


2017 ◽  
Vol 77 (1) ◽  
pp. 85-91 ◽  
Author(s):  
Marie Holmqvist ◽  
Lotta Ljung ◽  
Johan Askling

ObjectiveTo investigate if, and when, patients diagnosed with rheumatoid arthritis (RA) in recent years are at increased risk of death.MethodsUsing an extensive register linkage, we designed a population-based nationwide cohort study in Sweden. Patients with new-onset RA from the Swedish Rheumatology Quality Register, and individually matched comparators from the general population were followed with respect to death, as captured by the total population register.Results17 512 patients with new-onset RA between 1 January 1997 and 31 December 2014, and 78 847 matched general population comparator subjects were followed from RA diagnosis until death, emigration or 31 December 2015. There was a steady decrease in absolute mortality rates over calendar time, both in the RA cohort and in the general population. Although the relative risk of death in the RA cohort was not increased (HR=1.01, 95% CI 0.96 to 1.06), an excess mortality in the RA cohort was present 5 years after RA diagnosis (HR after 10 years since RA diagnosis=1.43 (95% CI 1.28 to 1.59)), across all calendar periods of RA diagnosis. Taking RA disease duration into account, there was no clear trend towards lower excess mortality for patients diagnosed more recently.ConclusionsDespite decreasing mortality rates, RA continues to be linked to an increased risk of death. Thus, despite advancements in RA management during recent years, increased efforts to prevent disease progression and comorbidity, from disease onset, are needed.


Blood ◽  
2012 ◽  
Vol 120 (21) ◽  
pp. 606-606
Author(s):  
Saro H. Armenian ◽  
Can-Lan Sun ◽  
Tabitha Vase ◽  
George Mills ◽  
Liezl Atencio ◽  
...  

Abstract Abstract 606 Background: alloHCT is offered with curative intent to patients with hematologic malignancies, and conventionally-computed survival estimates are offered for prognosticating outcomes. However, conventionally-computed survival estimates do not take into account elapsed time (and changing hazards with time survived); conditional survival overcomes these limitations, by calculating the probability of survival after having already survived a certain period of time – such data are unavailable for alloHCT recipients. We describe cause-specific (relapse-, GvHD-, treatment-related) conditional survival after alloHCT, providing clinically relevant information for patients who have survived 6 mos, 1, 2, and 5y after alloHCT. Methods: From 1976 to 2006, 2,427 consecutive patients received alloHCT for a hematologic malignancy at a single institution (median age: 34.7y [0.6–72.5]). Vital status and cause of death were determined using National Death Index, Social Security Death Index and medical records. Results: As of 12/31/2007, a total of 1413 deaths (58% of the cohort) were observed; 39% attributed to recurrent disease; 34% to GvHD; 12% to infection; 5% to cardiopulmonary disease; 2% to subsequent malignant neoplasm (SMNs); and 8% to other causes. Conventionally-computed probability of survival was 44.6% at 5y and 41.2% at 10y from alloHCT. On the other hand, conditional on survival for 6 mo, 1, 2, and 5y after alloHCT, 5-y survival rates were 62%, 75%, 83%, and 93%, respectively (Figure A). The cohort was at a 40-fold increased risk of any death compared with the general population (95%CI=38.2–42.4); at a 25.6-fold increased risk of death due to pulmonary complications, 3.3-fold risk due to SMNs, and 2.3-fold risk due to cardiovascular complications. Among patients followed for 15+y after HCT, the risk of all-cause mortality was 2.6-fold that of the general population (95%CI=1.8–3.7). Standardized mortality ratios (SMR) and cause-specific conditional mortality rates by primary diagnosis are summarized in the Table. Individuals who survived the first 5y had negligible (≤5%) risk of relapse- and GvHD-related mortality over the subsequent 5y. Treatment-related mortality increased over time; among those who survived 5y, treatment-related mortality rates exceeded relapse-related mortality (Figure B). After adjustment for demographics, underlying diagnosis and treatment era, individuals with chronic GVHD (cGVHD) had a significantly lower risk of relapse-related mortality (RR=0.43, 95%CI=0.4–0.5) compared to those without cGVHD. Conclusions: The projected 5-y survival rates improve conditional on time survived from alloHCT; 5-y survival exceeds 93% for those who have already survived 5y. However, alloHCT recipients who have survived 15+y continue to remain at increased risk of death compared to the general population. cGVHD is associated with decreased risk of relapse-related mortality. Both relapse-related and GvHD-related mortality rates decline with time, such that, among those who have survived 5y, treatment-related mortality exceeds relapse-related mortality. Conditional survival estimates provide clinically relevant prognostic information, helping inform preventive and interventional strategies. Disclosures: No relevant conflicts of interest to declare.


2017 ◽  
Vol 76 (9) ◽  
pp. 1566-1574 ◽  
Author(s):  
Ju Ann Tan ◽  
Natasha Dehghan ◽  
Wenjia Chen ◽  
Hui Xie ◽  
John M Esdaile ◽  
...  

ObjectiveTo determine the magnitude of all-cause mortality risk in patients with antineutrophil cytoplasmic antibodies-associated vasculitis (AAV) compared with the general population through a meta-analysis of observational studies.MethodsWe searched Medline and Embase databases from their inception to April 2015. Observational studies that met the following criteria were assessed by two researchers: (1) clearly defined AAV identified by either the American College of Rheumatology 1990 classification criteria or the 2012 Chapel Hill Consensus Conference disease definitions, and (2) reported standardised mortality ratios (SMR) and 95% CI. We calculated weighted-pooled summary estimates of SMRs (meta-SMRs) for all-cause mortality using random-effects model, tested for publication bias and heterogeneity.ResultsTen studies met the inclusion criteria, comprising 3338 patients with AAV enrolled from 1966 to 2009, and a total of 1091 observed deaths. Overall, we found a 2.7-fold increased risk of death in patients with AAV when compared with the general population (meta-SMR: 2.71 (95% CI 2.26 to 3.24)). Analysis on studies that included only granulomatosis with polyangiitis cases also indicated a similar mortality risk (meta-SMR: 2.63 (95% CI 2.02 to 3.43)). There was no significant publication bias or small-study effect. Subgroup analyses showed that mortality risks were higher in older cohorts, with a trend towards improvement over time (ie, those with their midpoint of enrolment periods that were between 1980–1993 and 1994–1999, vs 2000–2005).ConclusionPublished data indicate there is a 2.7-fold increase in mortality among patients with AAV compared with the general population.


2022 ◽  
Vol 104-B (1) ◽  
pp. 45-52
Author(s):  
Liam Zen Yapp ◽  
Nick D. Clement ◽  
Matthew Moran ◽  
Jon V. Clarke ◽  
A. Hamish R. W. Simpson ◽  
...  

Aims The aim of this study was to determine the long-term mortality rate, and to identify factors associated with this, following primary and revision knee arthroplasty (KA). Methods Data from the Scottish Arthroplasty Project (1998 to 2019) were retrospectively analyzed. Patient mortality data were linked from the National Records of Scotland. Analyses were performed separately for the primary and revised KA cohorts. The standardized mortality ratio (SMR) with 95% confidence intervals (CIs) was calculated for the population at risk. Multivariable Cox proportional hazards were used to identify predictors and estimate relative mortality risks. Results At a median 7.4 years (interquartile range (IQR) 4.0 to 11.6) follow-up, 27.8% of primary (n = 27,474/98,778) and 31.3% of revision (n = 2,611/8,343) KA patients had died. Both primary and revision cohorts had lower mortality rates than the general population (SMR 0.74 (95% CI 0.73 to 0.74); p < 0.001; SMR 0.83 (95% CI 0.80 to 0.86); p < 0.001, respectively), which persisted for 12 and eighteight years after surgery, respectively. Factors associated with increased risk of mortality after primary KA included male sex (hazard ratio (HR) 1.40 (95% CI 1.36 to 1.45)), increasing socioeconomic deprivation (HR 1.43 (95% CI 1.36 to 1.50)), inflammatory polyarthropathy (HR 1.79 (95% CI 1.68 to 1.90)), greater number of comorbidities (HR 1.59 (95% CI 1.51 to 1.68)), and periprosthetic joint infection (PJI) requiring revision (HR 1.92 (95% CI 1.57 to 2.36)) when adjusting for age. Similarly, male sex (HR 1.36 (95% CI 1.24 to 1.49)), increasing socioeconomic deprivation (HR 1.31 (95% CI 1.12 to 1.52)), inflammatory polyarthropathy (HR 1.24 (95% CI 1.12 to 1.37)), greater number of comorbidities (HR 1.64 (95% CI 1.33 to 2.01)), and revision for PJI (HR 1.35 (95% 1.18 to 1.55)) were independently associated with an increased risk of mortality following revision KA when adjusting for age. Conclusion The SMR of patients undergoing primary and revision KA was lower than that of the general population and remained so for several years post-surgery. However, approximately one in four patients undergoing primary and one in three patients undergoing revision KA died within tenten years of surgery. Several patient and surgical factors, including PJI, were associated with the risk of mortality within ten years of primary and revision surgery. Cite this article: Bone Joint J 2022;104-B(1):45–52.


2017 ◽  
Vol 46 (4) ◽  
pp. 343-354 ◽  
Author(s):  
Ngan N. Lam ◽  
Amit X. Garg ◽  
Greg A. Knoll ◽  
S. Joseph Kim ◽  
Krista L. Lentine ◽  
...  

Background: The implications of venous thromboembolism (VTE) for morbidity and mortality in kidney transplant recipients are not well described. Methods: We conducted a retrospective study using linked healthcare databases in Ontario, Canada to determine the risk and complications of VTE in kidney transplant recipients from 2003 to 2013. We compared the incidence rate of VTE in recipients (n = 4,343) and a matched (1:4) sample of the general population (n = 17,372). For recipients with evidence of a VTE posttransplant, we compared adverse clinical outcomes (death, graft loss) to matched (1:2) recipients without evidence of a VTE posttransplant. Results: During a median follow-up of 5.2 years, 388 (8.9%) recipients developed a VTE compared to 254 (1.5%) in the matched general population (16.3 vs. 2.4 events per 1,000 person-years; hazard ratio [HR] 7.1, 95% CI 6.0-8.4; p < 0.0001). Recipients who experienced a posttransplant VTE had a higher risk of death (28.5 vs. 11.2%; HR 4.1, 95% CI 2.9-5.8; p < 0.0001) and death-censored graft loss (13.1 vs. 7.5%; HR 2.3, 95% CI 1.4-3.6; p = 0.0006) compared to matched recipients who did not experience a posttransplant VTE. Conclusions: Kidney transplant recipients have a sevenfold higher risk of VTE compared to the general population with VTE conferring an increased risk of death and graft loss.


Neurology ◽  
2021 ◽  
pp. 10.1212/WNL.0000000000013058
Author(s):  
Teng Hwee Tan ◽  
Huili Zheng ◽  
Timothy Cheo ◽  
Jeremy Tey ◽  
Yu Yang Soon

BackgroundWe aim to determine the risk of stroke and death within 30 days post stroke in nasopharyngeal cancer (NPC) survivors.MethodsWe conducted a population-based cohort study of patients diagnosed with NPC from Jan 1, 2005 to Dec 31, 2017. Using the cancer and stroke disease registries and the Singapore general population as the reference population, we report the age-standardized incidence rate differences (SIRDs) ratios (SIRs) and the cumulative incidence of stroke and the standardized mortality rate differences (SMRDs) and ratios (SMRs) for all causes of death within 30 days post stroke for NPC survivors.FindingsAt a median follow up of 48.4 months (IQR 19.8 – 92.9) for 3849 patients diagnosed with NPC, 96 patients developed stroke. The overall SIRD and SIR for stroke was 3.12 (95% CI 2.09 – 4.15) and 2.54 (95% CI 2.08 – 3.10) respectively. The SIRD was highest for the age group 70 – 79 years old (8.84 cases per 1000 person-years (PY); 0.46 – 17.21) while the SIR was highest for the age group 30 – 39 years old (16.41; 6.01 – 35.82). The SIRD and SIR for stage 1 disease was (6.96 cases per 1000 PY; 2.16 – 11.77) and (4.15; 2.46 – 7.00) respectively. The SMRD and SMR for all cause deaths within 30 days of stroke was (3.20 cases per 100 persons; -3.87 – 10.28) and (1.34; 0.76 – 2.37) respectively.InterpretationThe overall risk of stroke was markedly elevated in survivors of NPC, especially in Stage 1 disease when compared to the general population. The risk of death within 30 days of stroke was not significantly higher for NPC survivors.Classification of EvidenceThis study provides Class II evidence of the increased risk of stroke in survivors of nasopharyngeal cancer compared to general population.


2019 ◽  
Vol 46 (11) ◽  
pp. 1509-1514 ◽  
Author(s):  
Valérie Leclair ◽  
John Svensson ◽  
Ingrid E. Lundberg ◽  
Marie Holmqvist

Objective.Evidence suggests an increased risk of cardiovascular (CV) diseases, including acute coronary syndrome (ACS), in idiopathic inflammatory myopathies (IIM). The aim of this study was to investigate the risk of ACS in an incident IIM cohort compared to the general Swedish population.Methods.A cohort of 655 individuals with incident IIM and 6813 general population comparators were identified from national registries. IIM subjects were diagnosed from 2002 to 2011. Followup started at IIM diagnosis and corresponding date in the general population. ACS, CV comorbidities, and CV risk factors were defined using International Classification of Diseases codes. Incidence rates including 95% CI were calculated. Cox proportional hazards models were used to compare the risk of ACS in patients with IIM and the general population. The competing risk of death was accounted for using competing risk regression models.Results.The incidence rate of ACS in IIM was higher than in the general population, particularly within the first year of diagnosis and in older individuals. The overall ACS incidence rate in IIM was 15.6 (95% CI 11.7–20.4) per 1000 person-years, with an HR of 2.4 (95% CI 1.8–3.2) compared with the general population. When accounting for the competing risk of death, the risk of ACS in IIM remained increased with a cumulative incidence of 7% at 5 years compared to 3.3% in the general population.Conclusion.IIM individuals are at higher risk of ACS, particularly within the first year after diagnosis.


Sign in / Sign up

Export Citation Format

Share Document