scholarly journals Mortality in patients with systemic lupus erythematosus and neuropsychiatric involvement: A retrospective analysis from a tertiary referral center in the Netherlands

Lupus ◽  
2020 ◽  
Vol 29 (14) ◽  
pp. 1892-1901
Author(s):  
Rory C Monahan ◽  
Rolf Fronczek ◽  
Jeroen Eikenboom ◽  
Huub A M Middelkoop ◽  
Liesbeth J J Beaart-van de Voorde ◽  
...  

Objective We aimed to evaluate all-cause and cause-specific mortality in patients with systemic lupus erythematosus (SLE) and neuropsychiatric (NP) symptoms in the Netherlands between 2007–2018. Methods Patients visiting the tertiary referral NPSLE clinic of the Leiden University Medical Center were included. NP symptoms were attributed to SLE requiring treatment (major NPSLE) or to other and mild causes (minor/non-NPSLE). Municipal registries were checked for current status (alive/deceased). Standardized mortality ratios (SMRs) and 95% confidence intervals (CI) were calculated using data from the Dutch population. Rate ratio (RR) and 95% CI were calculated using direct standardization to compare mortality between major NPSLE and minor/non-NPSLE. Results 351 patients were included and 149 patients were classified as major NPSLE (42.5%). Compared with the general population, mortality was increased in major NPSLE (SMR 5.0 (95% CI: 2.6–8.5)) and minor/non-NPSLE patients (SMR 3.7 (95% CI: 2.2–6.0)). Compared with minor/non-NPSLE, mortality was similar in major NPSLE patients (RR: 1.0 (95% CI: 0.5–2.0)). Cause-specific mortality rates demonstrated an increased risk of death due to infections in both groups, whereas death due to cardiovascular disease was only increased in minor/non-NPSLE patients. Conclusion Mortality was increased in both major NPSLE and minor/non-NPSLE patients in comparison with the general population. There was no difference in mortality between major NPSLE and minor/non-NPSLE patients.

2020 ◽  
Vol 79 (Suppl 1) ◽  
pp. 1514.2-1514
Author(s):  
R. Monahan ◽  
R. Fronczek ◽  
J. Eikenboom ◽  
H. Middelkoop ◽  
L. J. J. Beaart- van de Voorde ◽  
...  

Background:Little is known about mortality in patients with systemic lupus erythematosus (SLE) presenting with neuropsychiatric (NP) symptoms.Objectives:We aimed to evaluate all-cause and cause-specific mortality in patients with SLE and NP symptoms.Methods:All patients with the clinical diagnosis of SLE of 18 years and older that visited the tertiary referral NPSLE clinic of the Leiden University Medical Center between 2007-2018 and signed informed consent were included in this study. Patients were classified as NPSLE if NP symptoms were attributed to SLE and immunosuppressive or anticoagulant therapy was initiated, otherwise patients were classified as non-NPSLE. Municipal registries were checked for current status (alive/deceased). Electronical medical files were studied for clinical characteristics and cause of death. Standardized mortality ratios (SMRs) and 95% confidence intervals were calculated using data from the general Dutch population. In addition, a rate ratio (RR) was calculated using direct standardization to compare mortality in NPSLE with non-NPSLE patients.Results:351 patients with the clinical diagnosis of SLE were included, of which 149 patients were classified as NPSLE (42.5%). Compared with the general population, mortality was increased five times in NPSLE (SMR 5.0, 95% CI: 2.6-8.5) and nearly four times in non-NPSLE patients (SMR 3.7, 95% CI: 2.2-6.0), as shown in Table 1. Risk of death due to cardiovascular disease (CVD) was increased in non-NPSLE patients (SMR 6.2, 95% CI: 2.0-14.6) and an increased risk of death to infections was present in both NPSLE and non-NPSLE patients ((SMR 29.9, 95% CI: 3.5 – 105) and SMR 91.3 (95% CI: 18.8 – 266) respectively). However, mortality did not differ between NPSLE and non-NPSLE patients (RR 1.0, 95% CI: 0.5 – 2.0).Table 1.All-cause mortality in SLE patients presenting with neuropsychiatric symptoms attributed to SLE (NPSLE) or to other causes (non-NPSLE)NPSLE(N = 149)Non-NPSLE(N = 202)Deaths (N, %)13 (8.7)17 (8.4)Age at death (median, range)49 (32 – 79)59 (20 – 89)Follow-up time (years)9061047Crude mortality rate (per 1000 PY)14.316.2All-cause mortality*Female5.5 (2.8 – 9.6)3.4 (1.9 – 5.7)Male2.3 (0.1 - 12.8)6.2 (1.3 – 18.2)Combined5.0 (2.6 – 8.5)3.7 (2.2 – 6.0)*Standardized mortality ratio, ratio of the observed and expected number of deathsConclusion:Mortality was increased in both NPSLE and non-NPSLE patients in comparison with the general population, but there was no difference in mortality between NPSLE and non-NPSLE patients. Risk of death due to infections was increased in both groups.Disclosure of Interests:Rory Monahan: None declared, Rolf Fronczek: None declared, Jeroen Eikenboom: None declared, Huub Middelkoop: None declared, L.J.J. Beaart- van de Voorde: None declared, Gisela Terwindt: None declared, Nic van der Wee: None declared, Frits Rosendaal: None declared, Thomas Huizinga Grant/research support from: Ablynx, Bristol-Myers Squibb, Roche, Sanofi, Consultant of: Ablynx, Bristol-Myers Squibb, Roche, Sanofi, Margreet Kloppenburg: None declared, G.M. Steup-Beekman: None declared


2018 ◽  
Vol 77 (7) ◽  
pp. 1063-1069 ◽  
Author(s):  
Dag Leonard ◽  
Elisabet Svenungsson ◽  
Johanna Dahlqvist ◽  
Andrei Alexsson ◽  
Lisbeth Ärlestig ◽  
...  

ObjectivesPatients with systemic lupus erythematosus (SLE) and rheumatoid arthritis (RA) have increased risk of cardiovascular disease (CVD). We investigated whether single nucleotide polymorphisms (SNPs) at autoimmunity risk loci were associated with CVD in SLE and RA.MethodsPatients with SLE (n=1045) were genotyped using the 200K Immunochip SNP array (Illumina). The allele frequency was compared between patients with and without different manifestations of CVD. Results were replicated in a second SLE cohort (n=1043) and in an RA cohort (n=824). We analysed publicly available genetic data from general population, performed electrophoretic mobility shift assays and measured cytokine levels and occurrence of antiphospholipid antibodies (aPLs).ResultsWe identified two new putative risk loci associated with increased risk for CVD in two SLE populations, which remained after adjustment for traditional CVD risk factors. An IL19 risk allele, rs17581834(T) was associated with stroke/myocardial infarction (MI) in SLE (OR 2.3 (1.5 to 3.4), P=8.5×10−5) and RA (OR 2.8 (1.4 to 5.6), P=3.8×10−3), meta-analysis (OR 2.5 (2.0 to 2.9), P=3.5×10−7), but not in population controls. The IL19 risk allele affected protein binding, and SLE patients with the risk allele had increased levels of plasma-IL10 (P=0.004) and aPL (P=0.01). An SRP54-AS1 risk allele, rs799454(G) was associated with stroke/transient ischaemic attack in SLE (OR 1.7 (1.3 to 2.2), P=2.5×10−5) but not in RA. The SRP54-AS1 risk allele is an expression quantitative trait locus for four genes.ConclusionsThe IL19 risk allele was associated with stroke/MI in SLE and RA, but not in the general population, indicating that shared immune pathways may be involved in the CVD pathogenesis in inflammatory rheumatic diseases.


2017 ◽  
Vol 44 (11) ◽  
pp. 1590-1596 ◽  
Author(s):  
Marios Rossides ◽  
Julia F. Simard ◽  
Elisabet Svenungsson ◽  
Mia von Euler ◽  
Elizabeth V. Arkema

Objective.To investigate mortality and functional impairment after stroke in systemic lupus erythematosus (SLE).Methods.Using Swedish nationwide registers, we identified 423 individuals with SLE and 1652 people without SLE who developed a first-ever ischemic or hemorrhagic stroke (1998–2013) and followed them until all-cause death or for 1 year. HR for death after ischemic or hemorrhagic stroke and the risk ratio of functional impairment (dependence in either transferring, toileting, or dressing) 3 months after ischemic stroke were estimated.Results.One year after stroke, 22% of patients with SLE versus 16% of those without SLE died. After ischemic stroke, patients with SLE had an increased risk of death (HR 1.85, 95% CI 1.39–2.45), which was attenuated after controlling for SLE-related comorbidities (HR 1.41, 95% CI 1.04–1.91). Functional impairment at 3 months was increased in SLE by almost 2-fold (risk ratio 1.73, 95% CI 1.16–2.57). After hemorrhagic stroke, patients with SLE had an HR of 2.30 (95% CI 1.38–3.82) for death, which was increased even during the first month.Conclusion.Compared to subjects without SLE, mortality after ischemic stroke increases after the first month in individuals with SLE, and functionality is worse at 3 months. SLE is associated with all-cause death after hemorrhagic stroke even during the first month. A shift of focus to patient functionality and prevention of hemorrhagic strokes is required.


2012 ◽  
Vol 2012 ◽  
pp. 1-7 ◽  
Author(s):  
Sara C. Croca ◽  
Anisur Rahman

Systemic lupus erythematosus is a multisystem, autoimmune disease known to be one of the strongest risk factors for atherosclerosis. Patients with SLE have an excess cardiovascular risk compared with the general population, leading to increased cardiovascular morbidity and mortality. Although the precise explanation for this is yet to be established, it seems to be associated with the presence of an accelerated atherosclerotic process, arising from the combination of traditional and lupus-specific risk factors. Moreover, cardiovascular-disease associated mortality in patients with SLE has not improved over time. One of the main reasons for this is the poor performance of standard risk stratification tools on assessing the cardiovascular risk of patients with SLE. Therefore, establishing alternative ways to identify patients at increased risk efficiently is essential. With recent developments in several imaging techniques, the ultimate goal of cardiovascular assessment will shift from assessing symptomatic patients to diagnosing early cardiovascular disease in asymptomatic patients which will hopefully help us to prevent its progression. This review will focus on the current status of the imaging tools available to assess cardiac and vascular function in patients with SLE.


2012 ◽  
Vol 39 (10) ◽  
pp. 1964-1970 ◽  
Author(s):  
JONAS F. LUDVIGSSON ◽  
ALBERTO RUBIO-TAPIA ◽  
VAIDEHI CHOWDHARY ◽  
JOSEPH A. MURRAY ◽  
JULIA F. SIMARD

Objective.To investigate a possible association between celiac disease (CD) and systemic lupus erythematosus (SLE). Case series have indicated a possible association, but population-based studies are lacking.Methods.We compared the risk of SLE in 29,048 individuals with biopsy-verified CD (villous atrophy, Marsh 3) from Sweden’s 28 pathology departments with that in 144,352 matched individuals from the general population identified through the Swedish Total Population Register. SLE was defined as having at least 2 records of SLE in the Swedish Patient Register. We used Cox regression to estimate hazard ratios (HR) for SLE.Results.During followup, 54 individuals with CD had an incident SLE. This corresponded to an HR of 3.49 (95% CI 2.48−4.90), with an absolute risk of 17/100,000 person-years and an excess risk of 12/100,000. Beyond 5 years of followup, the HR for SLE was 2.54 (95% CI 1.57−4.10). While SLE was predominantly female, we found similar risk estimates in men and women. When we restricted our outcome to individuals who also had a dispensation for a medication used in SLE, the HR was 2.43 (95% CI 1.22−4.87). The HR for having 2 records of SLE diagnoses, out of which at least 1 had occurred in a department of rheumatology, nephrology/dialysis, internal medicine, or pediatrics, was 2.87 (95% CI 1.97−4.17).Conclusion.Individuals with CD were at a 3-fold increased risk of SLE compared to the general population. Although this excess risk remained more than 5 years after CD diagnosis, absolute risks were low.


2020 ◽  
Vol 79 (Suppl 1) ◽  
pp. 880.1-880
Author(s):  
G. Y. Ahn ◽  
J. Lee ◽  
J. M. Shin ◽  
Y. K. Lee ◽  
J. H. Kim ◽  
...  

Background:Systemic lupus erythematosus (SLE) is a chronic systemic autoimmune inflammatory disease with increased risk for mortality and cancers possibly because of the effects of systemic inflammation and immunodeficiency due to disease itself and/or cytotoxic agents for SLE management. Although there has been improvement in the prognosis of SLE over decades with the treatment advance including standardized treatment strategy for lupus nephritis and less cytotoxic agents, the improvement also is fixed nowadays.Objectives:In this study, we we are to investigate the mortality and cancer of SLE patients in a longitudinal SLE cohort and compare the morality ratio and incidence of cancer with general population over time.Methods:This study was conducted in Hanyang BAE lupus cohort during the period of 1998 to 2015. Mortality data and malignancy data were derived in connection with data from the Korean National Statistics Office and the Korea Central Incidence Database, respectively. The Standardized Mortality Ratio (SMR) and Standardized Incidence Ratio (SIR) was estimated yearly by dividing the observed number deaths/cancers by the expected number of deaths/cancers of age- and sex- matched general population from matched year.Results:Mortality data were available in 1284 patients and total 71 deaths were observed. The most common cause of death was SLE itself (52.1%) followed by infection (18.3%), cerebrovascular disease (8.5%) and suicide (7.0%). The total age and sex adjusted SMR was 3.4 [95% CI (Confidential Interval) 2.6-4.1]. When we conduct subgroup analysis by age, the sex-adjusted SMR was significantly increased in young and middle aged adult patients: the SMR in patients younger than 20 was 12.2, but it was not significant due to the small number of young patients (observed death 3, expected death 0.3, 95% CI 0-26.0). The adjusted SMR in patients aged 20-39, aged 40-59 and aged over 60 were 9.8 (observed death 35, expected death 4.8, 95% CI 6.5-13.0), 3.7 (observed death 24, expected death 11.5, 95% CI 2.2-5.2), and 1.0 (observed death 9, expected death 10.3, 95% CI 0.3-1.6), respectively. Compared with alive patients, died patients had more serositis and more neurologic disorder according to American College of Rheumatology classification criteria for SLE despite the shorter observational period (5.6 years vs. 9.4 years).Malignancy data were available in 1,020 patients and 56 primary cancers were diagnosed. Solid tumor was developed in 51 patient and hematologic malignancy was developed in 5 patients (3 non-Hodgkin’s lymphoma, 1 solitary plasmacytoma and 1 acute lymphoblastic leukemia). Thyroid cancer was the most common solid cancer (24 patients) followed by colorectal (5 patients), breast (4 patients), cervical cancer (4 patients) and hepatocellular carcinoma (3 patients). The total age and sex adjusted SIR was 1.1 (95% CI 0.8-1.4).Conclusion:Patients with SLE had higher risk of mortality than general population and the younger patients had the higher risk of mortality. The leading cause of death was SLE itself followed by infection and cerebrovascular disease. The risk for cancer in patient with SLE was similar with that of general population.Disclosure of Interests:None declared


2021 ◽  
Vol 10 (17) ◽  
pp. 3842
Author(s):  
René Cordtz ◽  
Salome Kristensen ◽  
Louise Plank Holm Dalgaard ◽  
Rasmus Westermann ◽  
Kirsten Duch ◽  
...  

Background: Patients with systemic lupus erythematosus (SLE) have an increased risk of infections due to impaired immune functions, disease activity, and treatment. This study investigated the impact of having SLE on the incidence of hospitalisation with COVID-19 infection. Methods: This was a nationwide cohort study from Denmark between 1 March 2020 to 2 February 2021, based on the linkage of several nationwide registers. The adjusted incidence of COVID-19 hospitalisation was estimated for patients with SLE compared with the general population in Cox-regression models. Among SLE patients, the hazard ratio (HR) for hospitalisation was analysed as nested case-control study. Results: Sixteen of the 2533 SLE patients were hospitalised with COVID-19 infection. The age-sex adjusted rate per 1000 person years was 6.16 (95% CI 3.76–10.08) in SLE patients, and the corresponding hazard ratio was 2.54 (95% CI 1.55–4.16) compared with the matched general population group after adjustment for comorbidities. Among SLE patients, hydroxychloroquine treatment was associated with a HR for hospitalisation of 0.61 (95% CI 0.19–1.88), and 1.06 (95% CI 0.3–3.72) for glucocorticoid treatment. Conclusion: Patients with SLE were at increased risk of hospitalisation with COVID-19.


2009 ◽  
Vol 36 (1) ◽  
pp. 50-57 ◽  
Author(s):  
MURAT ICEN ◽  
PAULO J. NICOLA ◽  
HILAL MARADIT-KREMERS ◽  
CYNTHIA S. CROWSON ◽  
TERRY M. THERNEAU ◽  
...  

ObjectiveFeatures of systemic lupus erythematosus (SLE) are commonly observed in patients with rheumatoid arthritis (RA). However, their frequency and clinical significance are uncertain. We examined the frequency of SLE features in RA and their effect on overall mortality.MethodsWe assembled a population-based incidence cohort of subjects aged ≥ 18 years first diagnosed with RA [1987 American College of Rheumatology (ACR) criteria] between 1955 and 1995. Information regarding disease characteristics, therapy, comorbidities, and SLE features (1982 ACR criteria) were collected from the complete inpatient and outpatient medical records. Cox regression models were used to estimate the mortality risk associated with lupus features.ResultsThe study population comprised 603 subjects with incident RA (mean age 58 yrs, 73% women) with a mean followup time of 15 years. By 25 years after RA incidence, ≥ 4 SLE features were observed in 15.5% of the subjects with RA. After adjustment for age and sex, occurrence of ≥ 4 SLE features was associated with increased overall mortality [hazard ratio (HR) 5.54, 95% confidence interval (CI) 3.59–8.53].With further adjustment for RA characteristics, therapy, and comorbidities, the association weakened but remained statistically significant (HR 2.56, 95% CI 1.60–4.08). After adjustment for age, sex, RA characteristics, therapy, and comorbidities, thrombocytopenia (2.0, 95% CI 1.2, 3.1) and proteinuria (1.8, 95% CI 1.3, 2.6) were significantly associated with mortality.ConclusionSLE features were common in RA, given sufficient observation time. Subjects with RA who developed ≥ 4 SLE features had an increased risk of death. Proteinuria and thrombocytopenia were individually associated with an increased mortality risk.


Lupus ◽  
2021 ◽  
pp. 096120332199010
Author(s):  
Rasmus Westermann ◽  
Kristian Zobbe ◽  
René Cordtz ◽  
Jeanette H Haugaard ◽  
Lene Dreyer

Objectives To investigate if patients with cutaneous lupus erythematosus (CLE) or systemic lupus erythematosus (SLE) have an increased risk of cancer compared with the general population, and furthermore to identify specific cancer types associated with increased risk. Methods This is an observational cohort study of 5310 patients with CLE or SLE identified in the Danish National Patient Register from 1 January 1995 to 31 December 2014. The cohort was followed up for cancer by linkage to the Danish Cancer Registry. Based on the age, sex, and calendar specific cancer rates of the general population of Denmark, standardised incidence ratios (SIRs) were calculated. Results The patients with CLE or SLE were followed for 40.724 person-years, each group’s average duration of follow-up being 6.9 and 8.1 years. The SIR for overall cancer (except non-melanoma skin cancer (NMSC)) was increased in patients with CLE 1.35 (95%CI 1.15 to 1.58) and patients with SLE 1.45 (95%CI 1.30 to 1.62). Both groups had high risks of hematological – including a 3–4-fold increased risk of non-Hodgkin lymphoma –, pancreatic, and lung cancers. Several cancers associated with oncogenic viruses as liver and tongue/mouth/pharynx were increased in the SLE group, while the risk of ovarian cancer was increased 2–4-fold only in the CLE group. Conclusion The overall risk of cancer was significantly increased in both patients with CLE and SLE. SIRs for hematological, pancreatic and lung cancers were elevated in both groups. Extra awareness of cancer in patients with SLE and patients with CLE should be considered.


2021 ◽  
pp. jrheum.210058
Author(s):  
Julia G. Harris

Rheumatology patients on immunosuppression are at increased risk of invasive pneumococcal disease. There is a multitude of literature highlighting the risk of pneumococcal infection, hospitalization, and even death in patients with systemic lupus erythematosus (SLE), in whom incidence of invasive pneumococcal infection is 13 times higher than the general population. 1,2,3,4


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