scholarly journals Transition to severe phenotype in systemic lupus erythematosus initially presenting with non-severe disease: implications for the management of early disease

2020 ◽  
Vol 7 (1) ◽  
pp. e000394 ◽  
Author(s):  
Dionysis S Nikolopoulos ◽  
Myrto Kostopoulou ◽  
Antigoni Pieta ◽  
Sofia Flouda ◽  
Katerina Chavatza ◽  
...  
Keyword(s):  
Lupus Erythematosus ◽  
Disease Duration ◽  
Severe Disease ◽  
Irreversible Damage ◽  
Mild Phenotype ◽  
Double Stranded Dna ◽  
Increased Risk ◽  
Damage Accrual ◽  
Baseline Factors

ObjectiveChanges in the care of patients with SLE dictate a re-evaluation of its natural history and risk factors for disease deterioration and damage accrual. We sought to decipher factors predictive of a deterioration in phenotype (‘transition’) in patients initially presenting with non-severe disease.MethodsPatients from the ‘Attikon’ cohort with disease duration ≥1 year were included. Disease at diagnosis was categorised as mild, moderate or severe, based on the British Isles Lupus Assessment Group manifestations and physician judgement. ‘Transition’ in severity was defined as an increase in category of severity at any time from diagnosis to last follow-up. Multivariable logistic regression was performed to identify baseline factors associated with this transition.Results462 patients were followed for a median (IQR) of 36 (120) months. At diagnosis, more than half (56.5%) had a mild phenotype. During disease course, transition to more severe forms was seen in 44.2%, resulting in comparable distribution among severity patterns at last follow-up (mild 28.4%, moderate 33.1%, severe 38.5%). Neuropsychiatric involvement at onset (OR 6.33, 95% CI 1.22 to 32.67), male sex (OR 4.53, 95% CI 1.23 to 16.60) and longer disease duration (OR 1.09 per 1 year, 95% CI 1.04 to 1.14) were independently associated with transition from mild or moderate to severe disease. Patients with disease duration ≥3 years who progressed to more severe disease had more than 20-fold increased risk to accrue irreversible damage.ConclusionAlmost half of patients with initially non-severe disease progress to more severe forms of SLE, especially men and patients with positive anti-double-stranded DNA or neuropsychiatric involvement at onset. These data may have implications for the management of milder forms of lupus.

scholarly journals Damage accrual and mortality over long-term follow-up in 300 patients with systemic lupus erythematosus in a multi-ethnic British cohort

Rheumatology ◽  
2019 ◽  
Author(s):  
Beatriz Tejera Segura ◽  
Brett Sydney Bernstein ◽  
Thomas McDonnell ◽  
Chris Wincup ◽  
Vera M Ripoll ◽  
...  
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Lupus Erythematosus ◽  
High Dose ◽  
Concomitant Disease ◽  
Systemic Lupus ◽  
Increased Risk ◽  
Damage Accrual ◽  
Long Term Follow Up

Abstract Objective Damage in patients with systemic lupus erythematosus is irreversible change in organs due to disease activity, concomitant disease or medication side-effects. It is measured using the Systemic Lupus International Collaborative Clinics Damage Index (SDI) and is associated with increased mortality. Previous reports have suggested associations between damage accrual and various ethnic, disease and treatment factors, but there is a dearth of long-term follow-up data from large multi-ethnic cohorts. We describe a study of damage and mortality in 300 patients from London, UK followed for up to 40 years. Methods We carried out retrospective analysis of medical records and SDI scores of 300 patients followed for up to 40 years (median 13.3 years). Characteristics of the groups who did and did not develop damage and those who died or survived to the end of follow-up were compared using univariable and multivariable analysis. Kaplan-Meier analysis was used to analyse factors affecting mortality and accrual of damage. Results Damage developed in 231/300 (77%) of patients. There was a linear accrual of damage over 40 years follow-up. Factors associated with damage were African/Caribbean ethnicity, renal and cerebral involvement, early use of high-dose corticosteroids or immunosuppressants, anti-RNP and antiphospholipid antibodies. Damage was strongly associated with mortality. Of 87 patients who died, 93% had damage compared with 70% of survivors (P < 0.001). Conclusion Development of damage is strongly associated with increased mortality. We identified groups at increased risk of developing damage, including those treated with high-dose steroids and immunosuppressants within the first two years.

Metabolic syndrome predicts new damage in systemic lupus erythematosus patients: Data from the Almenara Lupus Cohort

Lupus ◽  
2022 ◽  
pp. 096120332110614
Author(s):  
Claudia Elera-Fitzcarrald ◽  
Cristina Reatégui-Sokolova ◽  
Rocío V Gamboa-Cárdenas ◽  
Mariela Medina ◽  
Francisco Zevallos ◽  
...  
Keyword(s):  
Lupus Erythematosus ◽  
Disease Duration ◽  
Cox Regression ◽  
Damage Index ◽  
Immunosuppressive Drugs ◽  
Age At Diagnosis ◽  
Survival Models ◽  
Damage Accrual ◽  
Comorbidity Index

Objectives This study aims to determine whether the MetS predicts damage accrual in SLE patients. Methods This longitudinal study was conducted in a cohort of consecutive SLE patients seen since 2012 at one single Peruvian institution. Patients had a baseline visit and then follow-up visits every 6 months. Patients with ≥ 2 visits were included. Evaluations included interview, medical records review, physical examination, and laboratory tests. Damage accrual was ascertained with the SLICC/ACR damage index (SDI) and disease activity with the SLEDAI-2K. Univariable and multivariable Cox-regression survival models were carried out to determine the risk of developing new damage. The multivariable model was adjusted for age at diagnosis; disease duration; socioeconomic status; SLEDAI; baseline SDI; the Charlson Comorbidity Index; daily dose; and time of exposure of prednisone (PDN), antimalarials, and immunosuppressive drugs. Results Two hundred and forty-nine patients were evaluated; 232 of them were women (93.2%). Their mean (SD) age at diagnosis was 35.8 (13.1) years; nearly all patients were Mestizo. Disease duration was 7.4 (6.6) years. The SLEDAI-2K was 5.2 (4.3) and the SDI, 0.9 (1.3). One hundred and eight patients (43.4%) had MetS at baseline. During follow-up, 116 (46.6%) patients accrued at least one new point in the SDI damage index. In multivariable analyses, the presence of MetS was a predictor of the development of new damage (HR: 1.54 (1.05–2.26); p < 0.029). Conclusions The presence of MetS predicts the development of new damage in SLE patients, despite other well-known risk factors for such occurrence.

scholarly journals SAT0237 THE SYSTEMIC LUPUS INTERNATIONAL COLLABORATING CLINICS (SLICC) FRAILTY INDEX (SLICC-FI) PREDICTS DAMAGE ACCRUAL IN SYSTEMIC LUPUS ERYTHEMATOSUS (SLE) PATIENTS. DATA FROM A LATIN AMERICAN MESTIZO COHORT

2020 ◽  
Vol 79 (Suppl 1) ◽  
pp. 1061.1-1062
Author(s):  
M. F. Ugarte-Gil ◽  
R. V. Gamboa Cárdenas ◽  
C. Reategui Sokolova ◽  
V. Pimentel-Quiroz ◽  
M. Medina Chinchon ◽  
...  
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Latin American ◽  
Lupus Erythematosus ◽  
Disease Duration ◽  
Negative Binomial ◽  
Frailty Index ◽  
Systemic Lupus ◽  
Research Support ◽  
Damage Accrual

Background:The Systemic Lupus International Collaborating Clinics (SLICC) Frailty Index (SLICC-FI) has been developed as a predictor of outcomes in SLE patients1-3. However, it needs to be validated in several populations.Objectives:To evaluate the SLICC-FI as a predictor of future damage accrual in systemic lupus erythematosus (SLE) patients.Methods:Patients from a single-center prevalent cohort were included. Damage accrual was defined as the increase in SLICC/American College of Rheumatology (ACR) damage index (SDI) scores between the baseline and last visits. The SLICC-FI was measured at baseline. Univariable and multivariable negative binomial regression were performed to determine the association between the baseline SLICC-FI (per 0.05 increase) and damage accrual during follow-up, adjusted for sex, age at diagnosis, socioeconomic status, disease duration, SLE Disease Activity Index 2000 (SLEDAI-2K), SDI, prednisone daily dose, antimalarial and immunosuppressive drug use at baseline, and duration of follow-up.Results:Of the 265 patients included, 248 (93.6%) were female with mean (SD) age 35.1 (13.6) years at diagnosis. At baseline, mean (SD) SLE disease duration was 7.3 (6.5) years, SDI was 1.1 (1.3) and SLEDAI-2K was 5.3 (4.6). The mean (SD) baseline SLICC-FI was 0.22 (0.05). After a mean (SD) of 5.2 (2.2) years of follow-up, the SDI increased in 126 (47.5%) patients, and the final mean (SD) SDI score was 1.7 (1.7). Higher SLICC-FI scores at baseline predicted greater damage accrual in the univariable analysis [Incidence Rate Ratio (IRR)=1.283, (CI95% 1.072-1.536); p=0.007]. The SLICC-FI remained associated with damage accrual in the multivariable model, after adjustment for possible confounders [IRR= 1.224 (CI95% 1.007-1.488); p=0.042].Conclusion:The SLICC-FI predicts damage accrual in prevalent SLE, supporting the relevance of this index in the evaluation of SLE patients. This is the first study validating the SLICC-FI in South American populationReferences:[1]Legge A, Kirkland S, Rockwood K, et al. Construction of a Frailty Index as a Novel Health Measure in Systemic Lupus Erythematosus. J Rheumatol. 2020; 47: 72-81[2]Legge A, Kirkland S, Rockwood K, et al. Evaluating the Properties of a Frailty Index and Its Association With Mortality Risk Among Patients With Systemic Lupus Erythematosus. Arthritis Rheumatol. 2019; 71: 1297-107[3]Legge A, Kirkland S, Rockwood K, et al. Prediction of Damage Accrual in Systemic Lupus Erythematosus Using the Systemic Lupus International Collaborating Clinics Frailty Index (SLICC-FI). Arthitis Rheumatol. Epub ahead of print 2019 Oct 21.Disclosure of Interests:Manuel F. Ugarte-Gil Grant/research support from: Jannsen, Pfizer, Rocío Violeta Gamboa Cárdenas Grant/research support from: Pfizer, Cristina Reategui Sokolova: None declared, Victor Pimentel-Quiroz: None declared, Mariela Medina Chinchon: None declared, Claudia Elera-Fitzcarrald Consultant of: Tecnofarma, Jose Alfaro Lozano Speakers bureau: Lilly, Zoila Rodriguez Bellido: None declared, Cesar Pastor Asurza: None declared, Risto Perich Campos Consultant of: Pfizer, Speakers bureau: Pfizer, Graciela S Alarcon: None declared

scholarly journals POS0204 AUTOANTIBODIES AND SYSTEMIC LUPUS ERYTHEMATOSUS INDUCED BY ANTI-TUMOUR NECROSIS FACTOR ALPHA THERAPY IN RHEUMATOID ARTHRITIS

2021 ◽  
Vol 80 (Suppl 1) ◽  
pp. 318.1-318
Author(s):  
D. Santos Oliveira ◽  
A. Martins ◽  
F. R. Martins ◽  
F. Oliveira Pinheiro ◽  
M. Rato ◽  
...  
Keyword(s):  
Lupus Erythematosus ◽  
Disease Duration ◽  
Seroconversion Rate ◽  
Necrosis Factor Alpha ◽  
Malar Rash ◽  
Tnf Α ◽  
Factor Alpha ◽  
Necrosis Factor ◽  
Over Time

Background:Anti-tumour necrosis factor alpha (anti-TNF-α) therapy is commonly used to treat inflammatory conditions such as rheumatoid arthritis (RA). Autoantibodies namely antinuclear antibodies (ANA) induced by these treatments are well established. However, anti-TNF-α-induced systemic lupus erythematosus (SLE) is rarely described and its incidence is yet unknown.Objectives:This study aimed to determine the prevalence of ANA seroconversion and to characterize the development of SLE induced by anti-TNF-α therapy in patients with RA over time.Methods:An observational retrospective cohort study was conducted with at least one year of follow-up. Patients with diagnosis of RA, according to American College of Rheumatology criteria (ACR), and registered on Rheumatic Diseases Portuguese Register (Reuma.pt) who started their first anti-TNFα between 2003 and 2019 were included. Patients with positive ANA (titer ≥100) and/or positive double-strand DNA (dsDNA) antibodies and/or with a diagnosis of SLE at their first visit were excluded. Demographic, clinical and laboratory data were obtained by consulting Reuma.pt. As there are no recognized criteria for drug-induced SLE, the diagnosis of SLE induced by anti-TNF-α was considered if there is a temporal relationship between clinical manifestations and anti-TNF-α-therapy, the presence of at least 1 serologic ACR criteria (ANA or anti-dsDNA) and at least 1 nonserologic ACR criteria (arthritis, serositis, hematologic disorder or malar rash) [1]. Continuous variables are presented with mean, standard deviation, median, quartile 1 and quartile 3. Categorical variables are presented with absolute and relative frequencies.Results:A total of 211 patients (mean age of 49.9±10.9 years old; 84.4% female) were included with a median follow-up time of 6 [3-14] years. We found a seroconversion rate for ANA of 75.4% (n=159) with median treatment duration of 31 [8.5-70.5] months. The most common titre was 1/100 with diffuse and speckled patterns. ANA seroconversion was higher for etanercept (47.8%, n=76) than with adalimumab (23.9%, n=38), infliximab (13.8%, n=22), golimumab (12.6%, n=20) or certolizumab (1.9%, n=3). SLE induced by anti-TNF-α occurred in two patients (0.9%) with erosive and seropositive (rheumatoid factor and anti-citrullinated protein antibodies) RA previously treated with two conventional synthetic disease-modifying antirheumatic drugs, including methotrexate. The first patient, a female with 66 years old and 17 years of disease duration, developed SLE after 16 months of infliximab, with constitutional symptoms, abrupt worsening of polyarthritis, ANA titer of 1/320 diffuse pattern and positive dsDNA (248 UI/mL) antibodies. The second patient, a woman with 43 years old and 11 years of disease duration, developed SLE after 41 months of adalimumab with malar rash and ANA titer of 1/320 diffuse pattern, positive dsDNA (285 UI/mL), positive anti-histone antibodies and hypocomplementemia. In these two cases, anti-TNF-α therapy was stopped and recovery was spontaneous without treatment. The first patient switched to adalimumab and the second switched to golimumab without recurrence of SLE for more than ten years.Conclusion:We found a high rate of ANA seroconversion induced by anti-TNFα therapy in patients with RA. However, similar to previous literature, only 0.9% of patients developed SLE with mild manifestations without major organ involvement. Although the drug with the highest ANA seroconversion rate was etanercept, those responsible for induced SLE were infliximab and adalimumab. Patients improved after discontinuation of therapy and tolerated an alternative anti-TNF-α drug without recurrence of induced SLE over time. Therefore, ANA and SLE induced by anti-TNF-α should be considered and reported in the follow-up of RA patients. Further research is needed to explore the impact of this adverse event on the outcomes of treatment over time.References:[1]Hochberg MC. Arthritis Rheum. 1997;40(9):1725.Disclosure of Interests:None declared

scholarly journals Mitochondrial DNA damage is associated with damage accrual and disease duration in patients with Systemic Lupus Erythematosus

Lupus ◽  
2014 ◽  
Vol 23 (11) ◽  
pp. 1133-1141 ◽  
Author(s):  
L López-López ◽  
M Nieves-Plaza ◽  
M del R Castro ◽  
YM Font ◽  
CA Torres-Ramos ◽  
...  
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Dna Damage ◽  
Mitochondrial Dna ◽  
Lupus Erythematosus ◽  
Disease Duration ◽  
Systemic Lupus ◽  
Mitochondrial Dna Damage ◽  
Damage Accrual

scholarly journals Early Description of Coronavirus 2019 Disease in Kidney Transplant Recipients in New York

2020 ◽  
Vol 31 (6) ◽  
pp. 1150-1156 ◽  
Author(s):  
Keyword(s):  
New York ◽  
Kidney Transplant ◽  
Severe Disease ◽  
Treatment Protocol ◽  
Current Treatment ◽  
Transplant Recipients ◽  
Kidney Transplant Recipients ◽  
Radiographic Findings ◽  
Increased Risk

BackgroundThe novel SARS-CoV-2 virus has caused a global pandemic of coronavirus disease 2019 (COVID-19). Although immunosuppressed individuals are thought to be at an increased risk of severe disease, little is known about their clinical presentation, disease course, or outcomes.MethodsWe report 15 kidney transplant recipients from the Columbia University kidney transplant program who required hospitalization for confirmed COVID-19, and describe their management, clinical course, and outcomes.ResultsPatients presented most often with a fever (87%) and/or cough (67%). Initial chest x-ray most commonly showed bilateral infiltrates, but 33% had no acute radiographic findings. Patients were managed with immunosuppression reduction and the addition of hydroxychloroquine and azithromycin. Although 27% of our patients needed mechanical ventilation, over half were discharged home by the end of follow-up.ConclusionsKidney transplant recipients with COVID-19 have presentations that are similar to that of the general population. Our current treatment protocol appears to be associated with favorable outcomes, but longer follow-up of a larger cohort of patients is needed.

Antiphospholipid syndrome (APS) in patients with systemic lupus erythematosus (SLE) implies a more severe disease with more damage accrual and higher mortality

Lupus ◽  
2020 ◽  
Vol 29 (12) ◽  
pp. 1556-1565
Author(s):  
Leyre Riancho-Zarrabeitia ◽  
Victor Martínez-Taboada ◽  
Iñigo Rúa-Figueroa ◽  
Fernando Alonso ◽  
María Galindo-Izquierdo ◽  
...  
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Antiphospholipid Syndrome ◽  
Lupus Erythematosus ◽  
Severe Disease ◽  
Organ Damage ◽  
Systemic Lupus ◽  
Katz Index ◽  
Damage Accrual ◽  
Major Organ ◽  
Clinical Profiles

Introduction Antiphospholipid antibodies (aPL) have been associated with organ damage and certain features in systemic lupus erythematosus(SLE) patients. Our aim was to investigate the differences between SLE patients according to the presence of aPL and/or clinical antiphospholipid syndrome (APS). Materials and methods Patients from the RELESSER-T registry were included. RELESSER-T is a Spanish multicenter, hospital-based, retrospective, SLE registry. Results We included 2398 SLE patients, 1372 of whom were positive for aPL. Overall 1026 patients were classified as SLE, 555 as SLE-APS and817 as SLE-aPL. Regarding cardiovascular risk factors, SLE-APS patients had higher rates of hypertension, dyslipidemia and diabetes than those with SLE-aPL and SLE ( p < 0.001). SLE-APS patients showed higher rates of neuropsychiatric, cardiac, pulmonary, renal and ophthalmological manifestations than the other groups ( p < 0.001). SLE-APS patients presented greater damage accrual with higher SLICC values (1.9 ± 2.2 in SLE-APS, 0.9 ± 1.4 in SLE-aPL and 1.1 ± 1.6 in SLE, p < 0.001) and more severe disease as defined by the Katz index (3 ± 1.8 in SLE-APS, 2.7 ± 1.7 in SLE-aPL and 2.6 ± 1.6 in SLE, p  < 0.001). SLE-APS patients showed higher mortality rates ( p < 0.001). Conclusions SLE-APS patients exhibited more severe clinical profiles with higher frequencies of major organ involvement, greater damage accrual and higher mortality than SLE-aPL and SLE patients.

scholarly journals Flares in patients with systemic lupus erythematosus

Rheumatology ◽  
2020 ◽  
Author(s):  
Kathleen McElhone ◽  
Janice Abbott ◽  
Margaret Hurley ◽  
Jane Burnell ◽  
Peter Lanyon ◽  
...  
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Lupus Erythematosus ◽  
Trial Design ◽  
Disease Duration ◽  
Clinical Trial Design ◽  
Reference Population ◽  
World Population ◽  
Future Clinical Trial ◽  
Systemic Lupus

Abstract Objective SLE is characterized by relapses and remissions. We aimed to describe the frequency, type and time to flare in a cohort of SLE patients. Methods SLE patients with one or more ‘A’ or ‘B’ BILAG-2004 systems meeting flare criteria (‘new’ or ‘worse’ items) and requiring an increase in immunosuppression were recruited from nine UK centres and assessed at baseline and monthly for 9 months. Subsequent flares were defined as: severe (any ‘A’ irrespective of number of ‘B’ flares), moderate (two or more ‘B’ without any ‘A’ flares) and mild (one ‘B’). Results Of the 100 patients, 94% were female, 61% White Caucasians, mean age (s.d.) was 40.7 years (12.7) and mean disease duration (s.d.) was 9.3 years (8.1). A total of 195 flares re-occurred in 76 patients over 781 monthly assessments (flare rate of 0.25/patient-month). There were 37 severe flares, 32 moderate flares and 126 mild flares. By 1 month, 22% had a mild/moderate/severe flare and 22% had a severe flare by 7 months. The median time to any ‘A’ or ‘B’ flare was 4 months. Severe/moderate flares tended to be in the system(s) affected at baseline, whereas mild flares could affect any system. Conclusion . In a population with active SLE we observed an ongoing rate of flares from early in the follow-up period with moderate–severe flares being due to an inability to fully control the disease. This real-world population study demonstrates the limitations of current treatments and provides a useful reference population from which to inform future clinical trial design.

scholarly journals Risk of benign paroxysmal positional vertigo in patients with depressive disorders: a nationwide population-based cohort study

BMJ Open ◽  
2019 ◽  
Vol 9 (3) ◽  
pp. e026936
Author(s):  
Chiao-Lin Hsu ◽  
Shih-Jen Tsai ◽  
Cheng-Che Shen ◽  
Ti Lu ◽  
Yao-Min Hung ◽  
...  
Keyword(s):  
Cohort Study ◽  
Lupus Erythematosus ◽  
Depressive Disorders ◽  
Benign Paroxysmal Positional Vertigo ◽  
Observation Time ◽  
Research Database ◽  
Positional Vertigo ◽  
Increased Risk ◽  
Paroxysmal Positional Vertigo

ObjectiveThe association between depression and benign paroxysmal positional vertigo (BPPV) remains debated. This study aimed to investigate the risk of BPPV in patients with depressive disorders.DesignLongitudinal nationwide cohort study.SettingNational health insurance research database in Taiwan.ParticipantsWe enrolled 10 297 patients diagnosed with depressive disorders between 2000 and 2009 and compared them to 41 188 selected control patients who had never been diagnosed with depressive disorders (at a 1:4 ratio matched by age, sex and index date) in relation to the risk of developing BPPV.MethodsThe follow-up period was defined as the time from the initial diagnosis of depressive disorders to the date of BPPV, censoring or 31 December 2009. Cox proportional hazard regression analysis was used to investigate the risk of BPPV by sex, age and comorbidities, with HRs and 95% CIs.ResultsDuring the 9-year follow-up period, 44 (0.59 per 1000 person-years) patients with depressive disorders and 99 (0.33 per 1000 person-years) control patients were diagnosed with BPPV. The incidence rate ratio of BPPV among both cohorts calculating from events of BPPV per 1000 person-years of observation time was 1.79 (95% CI 1.23 to 2.58, p=0.002). Following adjustments for age, sex and comorbidities, patients with depressive disorders were 1.55 times more likely to develop BPPV (95% CI 1.08 to 2.23, p=0.019) as compared with control patients. In addition, hyperthyroidism (HR=3.75, 95% CI 1.67–8.42, p=0.001) and systemic lupus erythematosus (SLE) (HR=3.47, 95% CI 1.07 to 11.22, p=0.038) were potential risk factors for developing BPPV in patients with depressive disorders.ConclusionsPatients with depressive disorders may have an increased risk of developing BPPV, especially those who have hyperthyroidism and SLE.

scholarly journals Measuring Disease Activity and Damage with Validated Metrics: A Systematic Review on Mortality and Damage in Systemic Lupus Erythematosus

2018 ◽  
Vol 45 (10) ◽  
pp. 1448-1461 ◽  
Author(s):  
Stephanie O. Keeling ◽  
Ben Vandermeer ◽  
Jorge Medina ◽  
Trish Chatterley ◽  
Tatiana Nevskaya ◽  
...  
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Disease Activity ◽  
Lupus Erythematosus ◽  
Activity Index ◽  
Damage Index ◽  
Disease Activity Index ◽  
Evaluation Methodology ◽  
Systemic Lupus ◽  
Increased Risk ◽  
Damage Accrual

Objective.To identify the effect of disease activity and damage, measured by validated indices, on mortality and damage accrual, in order to inform upcoming Canadian systemic lupus erythematosus (SLE) recommendations.Methods.Following GRADE (Grading of Recommendations Assessment, Development and Evaluation) methodology to fill in evidence-to-decision tables to create recommendations for “minimal investigations needed to monitor SLE patients at baseline and subsequent visits,” a systematic literature review was performed. The effect of disease activity and damage, measured by validated metrics, on mortality and damage was systematically reviewed, with metaanalyses performed when available.Results.A title/abstract screen of 5599 articles identified 816 articles for full paper review, with 102 meeting inclusion criteria and 53 with extractable data. Thirty-three articles describing outcomes related to disease activity and 20 articles related to damage were identified. Mortality was associated with higher SLE Disease Activity Index-2000 scores in 6 studies (HR 1.14, 95% CI 1.06–1.22) and higher Systemic Lupus International Collaborating Clinics/ACR Damage Index scores in 6 studies (HR 1.53, 95% CI 1.28–1.83). Higher SLE Activity Measure scores were associated with increased risk of damage in 3 studies (OR 1.06, 95% CI 1.04–1.08). British Isles Lupus Assessment Group was associated with mortality in 1 study with HR of 1.15.Conclusion.Active SLE disease and damage are associated with and predict greater mortality and damage. The use of validated disease activity and damage metrics is important in the assessment of disease activity and damage and will inform upcoming Canadian recommendations for the assessment of SLE.

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