scholarly journals Evaluating the performance of ACR, SLICC and EULAR/ACR classification criteria in childhood onset systemic lupus erythematosus

2021 ◽  
Vol 19 (1) ◽  
Author(s):  
Reem Abdwani ◽  
Eiman Masroori ◽  
Eiman Abdullah ◽  
Safiya Al Abrawi ◽  
Ibrahim Al-Zakwani
Keyword(s):  
Lupus Erythematosus ◽  
Disease Duration ◽  
Classification Criteria ◽  
Age At Diagnosis ◽  
First Year ◽  
Lower Sensitivity ◽  
Childhood Onset ◽  
Threshold Score ◽  
Study Population

Abstract Background The ACR 1997, SLICC 2012 and EULAR/ACR 2019 classification criteria were validated based on adult patients. To date, there are no classification criteria specific for children with SLE. The aim of the study is to compare the performance characteristics among the three SLE classification criteria (ACR-1997, SLICC-2012 and EULAR/ACR-2019) in childhood onset SLE (cSLE) cohort of Arab ethnicity from Oman. Methods We conducted a retrospective multicenter study in Oman of cSLE patients as cases and patients with other rheumatic disease with a positive ANA titer as controls. The cSLE cases recruited were children diagnosed with SLE before 13 years of age. Data was retrospectively collected to establish the ACR-1997, SLICC-2012 and EULAR/ACR-2019 criteria fulfilled at first visit, first year follow up and last follow up. Results Study population included 113 cSLE cases (mean age at diagnosis of 7.3 ± 3.4 years with disease duration of 6.1 ± 4.6 years) and 51 controls (mean age at diagnosis 5.0 ± 3.4 with disease duration 5.7 ± 3.9). The cSLE cases had higher frequency of familial SLE than controls (38% vs 7.8%; p < 0.001). The performance measures demonstrated that EULAR/ACR-2019 criteria had the highest sensitivity (81, 88, 89%) compared to ACR 1997 (49, 57, 66%) and SLICC 2012 (76, 84,86%); while the ACR 1997 had the highest specificity (96%) compared to SLICC 2012 (94%) and EULAR/ACR 2019 (90%) at first visit, first year and last assessment. When we increased the threshold score to ≥13 rather than the traditional score ≥ 10 for ACR/EULAR 2019, there was increased specificity (96%) at the expense of lower sensitivity (76, 83, and 84%) at first visit, first year and last assessment. Conclusion In this cSLE population, EULAR/ACR 2019 scored better at initial presentation, first year and last assessment follow up. Further multinational studies are needed to validate the appropriate cut off score for the newly proposed ACR/EULAR 2019 classification criteria in cSLE to increase early sensitivity and specificity for cSLE classification.

scholarly journals Evaluating the Performance of ACR, SLICC and EULAR/ACR Classification Criteria in Childhood onset Systemic Lupus Erythematosus

2021 ◽  
Author(s):  
Reem Abdwani ◽  
Eman AlMasroori ◽  
Eiman Abdalla ◽  
Safiya Al Abrawi ◽  
Ibrahim AlZakwani
Keyword(s):  
Lupus Erythematosus ◽  
Disease Duration ◽  
Classification Criteria ◽  
Age At Diagnosis ◽  
First Year ◽  
Lower Sensitivity ◽  
Childhood Onset ◽  
Threshold Score ◽  
Study Population

Abstract Background: The ACR 1997, SLICC 2012 and EULAR/ACR 2019 classification criteria were validated based on adult patients. To date, there are no classification criteria specific for children with SLE. The aim of the study is to compare the performance characteristics among the three SLE classification criterias (ACR-1997, SLICC-2012 and EULAR/ACR-2019) in childhood onset SLE (cSLE) cohort of Arab ethnicity from Oman. Methods: We conducted a retrospective multicenter study of cSLE patients as cases and patients with other rheumatic disease with a positive ANA titer as controls. Data were retrospectively collected to establish the ACR-1997, SLICC-2012 and EULAR/ACR-2019 criteria fulfilled at first visit, first year follow up and last follow up. Results: Study population included 113 cSLE cases (mean age at diagnosis 7.3 ± 3.4 years with disease duration 6.1 ± 4.6 years) and 51 controls (mean age at diagnosis 5.0 ± 3.4 with disease duration 5.7 ± 3.9). The performance measures demonstrated that EULAR/ACR-2019 criteria had the highest sensitivity (81%, 88%, 89%) compared to ACR 1997 ( 49%, 57%, 66%) and SLICC 2012 (76%, 84%,86%); while the ACR 1997 had the highest specificity (96%) compared to SLICC 2012 (94%) and EULAR/ACR 2019 ( 90%) at first visit, first year and last assessment. When we increased the threshold score to ≥ 13 rather than the traditional score ≥ 10 for ACR/EULAR 2019, there was increased specificity (96%) at the expense of lower sensitivity (76%, 83%, and 84%) at first visit, first year and last assessment. Conclusion: In this cSLE population, EULAR/ACR 2019 scored better at initial presentation, first year and last assessment follow up. Further multinational studies are needed to validate the appropriate cut off score for the newly proposed ACR/EULAR 2019 classification criteria in cSLE to increase early sensitivity and specificity for cSLE classification.

scholarly journals P123 Evaluating the performance of ACR, SLICC and EULAR/ACR classification criteria in childhood onset systemic lupus erythematosus

Rheumatology ◽  
2021 ◽  
Vol 60 (Supplement_1) ◽  
Author(s):  
Reem Abdwani ◽  
Eiman Al Masroori ◽  
Eiman Abdalla ◽  
Safiya Al Abrawi ◽  
Ibrahim Al Zakwani
Keyword(s):  
Lupus Erythematosus ◽  
Disease Duration ◽  
Classification Criteria ◽  
First Year ◽  
Childhood Onset ◽  
Acr Criteria ◽  
Cut Score ◽  
History Of ◽  
One Year

Abstract Background/Aims  The aim of the study is to compare the performance characteristics among three SLE classification criteria (ACR-1997, SLICC-2012 and EULAR/ACR-2019) in childhood onset SLE (cSLE) cohort of Arab ethnicity from Oman. Methods  We conducted a retrospective multicenter study among pediatric rheumatology centers in Oman. Cases were cSLE and controls were patients with other rheumatic disease with a positive ANA titer followed up over the past 10 years. Data were retrospectively collected to establish the ACR, SLICC and EULAR/ACR criteria fulfilled at first visit, first year follow up and last year of follow up. Results  Study population included 113 cSLE cases (mean age at diagnosis 7.3 ± 3.4 years with disease duration 6.13± 4.6 years and 38% family history of SLE) and 51 controls (mean age at diagnosis 5.0 ± 3.4 years with disease duration 5.7 ± 3.9 years and 4% family history of SLE). Table 1 demonstrates the result of the performance measures for the ACR, SLICC and EULAR/ACR criteria at first visit, first year and last follow up. P123 Table 1:Performance measures for the ACR 1997, SLICC 2012 and ACR/EULAR 2019 classification criteria according to first visit, first year and latest periods.CriteriaSensitivity (95% CI)Specificity (95% CI)PPV (95% CI)NPV (95% CI)ROC (95% CI)Accuracy (95% CI)ACR 19971st visit49% (40%-59%)96% (87%-99%)97% (88%-99%)46% (36%-56%)0.73 (0.67-0.78)64% (56%-71%)1st year57% (47-66%)96% (87%-99%)97% (90%-99%)50% (40%-60%)0.76 (0.71-0.82)69% (61%-76%)Latest66% (56%-74%)96% (87%-99%)97% (91%-99%)56% (45%-66%)0.81 (0.76-0.86)75% (68%-81%)SLICC1st visit76% (67%-84%)94% (84%-99%)97% (91%-99%)64% (52%-75%)0.85 (0.80-0.90)82% (75%-87%)1st year84% (76%-90%)94% (84%-99%)97% (91%-99%)73% (60%-83%)0.89 (0.84-0.94)87% (81%-92%)Latest86% (78%-92%)94% (84%-99%)97% (92%-99%)75% (63%-85%)0.90 (0.85-0.95)88% (83%-93%)ACR/EULAR1st visit81% (73%-88%)92% (81%-98%)96% (90%-99%)69% (57%-80%)0.87 (0.82-0.92)85% (78%-90%)1st year88% (80%-93%)90% (79%-97%)95% (89%-98%)77% (64%-87%)0.89 (0.84-0.94)88% (83%-93%)Latest89% (82%-94%)90% (79%-97%)95% (89%-99%)79% (67%-89%)0.90 (0.85-0.95)90% (84%-94%)ACR/EULAR*1st visit76% (67%-84%)96% (87%-99%)98% (92%-99%)65% (53%-75%)0.86 (0.81-0.91)83% (76%-88%)1st year83% (74%-89%)96% (87%-99%)98% (93%-99%)71% (59%-81%)0.89 (0.85-0.94)87% (81%-92%)Latest84% (76%-90%)96% (87%-99%)98% (93%-99%)73% (61%-83%)0.90 (0.86-0.94)88% (83%-93%)ACR, American College of Rheumatology 1997; SLICC, Systemic Lupus Erythematosus International Collaborating Clinics; EULAR, European League Against Rheumatism; CI, confidence interval; PPV, positive predictive value; NPV, negative predictive value; ROC, area under the received operating curve. ACR/EULAR*, a score of ≥ 13 as compared to the traditional cut of score of ≥ 10. The median disease duration was 5 (2.5-9) years with a range of 1-19 years. Conclusion  In this cSLE population, the EULAR/ACR criteria scored better in sensitivity at first, one year and last follow up; while the ACR 1997 scored better in specificity at first, one year and last follow up. However, if the EULAR/ACR cut score ≥ 13 (rather than traditional cut-score ≥ 10) then it an equivalent specificity to ACR 1997 at the expense of lower sensitivity. Disclosure  R. Abdwani: None. E. Al Masroori: None. E. Abdalla: None. S. Al Abrawi: None. I. Al Zakwani: None.

scholarly journals A nationwide study of SLE in Japanese identified subgroups of patients with clear signs patterns and associations between signs and age or sex

Lupus ◽  
2014 ◽  
Vol 23 (13) ◽  
pp. 1435-1442 ◽  
Author(s):  
C Terao ◽  
R Yamada ◽  
T Mimori ◽  
K Yamamoto ◽  
T Sumida
Keyword(s):  
Lupus Erythematosus ◽  
Disease Duration ◽  
Renal Involvement ◽  
Disease Status ◽  
First Year ◽  
Clinical Markers ◽  
Autoantibody Production ◽  
Nationwide Study ◽  
Negatively Associated

We performed a nationwide study to determine the distributions of the signs and clinical markers of systemic lupus erythematosus (SLE) and identify any patterns in their distributions to allow patient subclassification. We obtained 256,999 patient-year records describing the disease status of SLE patients from 2003 to 2010. Of these, 14,779 involved patients diagnosed within the last year, and 242,220 involved patients being followed up. Along with basic descriptive statistics, we analyzed the effects of sex, age and disease duration on the frequencies of signs in the first year and follow-up years. The patients and major signs were clustered using the Ward method. The female patients were younger at onset. Renal involvement and discoid eczema were more frequent in males, whereas arthritis, photosensitivity and cytopenia were less. Autoantibody production and malar rash were positively associated with young age, and serositis and arthritis were negatively associated. Photosensitivity was positively associated with a long disease duration, and autoantibody production, serositis and cytopenia were negatively associated. The SLE patients were clustered into subgroups, as were the major signs. We identified differences in SLE clinical features according to sex, age and disease duration. Subgroups of SLE patients and the major signs of SLE exist.

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 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 AB0327 DRUG-INDUCED LUPUS ERYTHEMATOSUS SECONDARY TO ANTI-TNF-Α AGENTS IN PATIENTS WITH SPONDYLOARTHRITIS AND PSORIATIC ARTHRITIS

2021 ◽  
Vol 80 (Suppl 1) ◽  
pp. 1188.2-1189
Author(s):  
A. Martins ◽  
D. Santos Oliveira ◽  
F. R. Martins ◽  
M. Rato ◽  
F. Oliveira Pinheiro ◽  
...  
Keyword(s):  
Psoriatic Arthritis ◽  
Clinical Features ◽  
Lupus Erythematosus ◽  
Disease Duration ◽  
Age At Diagnosis ◽  
Categorical Variables ◽  
Drug Induced ◽  
Laboratory Findings ◽  
Conversion Rates ◽  
Tnf Α

Background:Induction of autoantibodies is frequently observed in patients treated with TNF-α antagonist and the possible development of drug-induced lupus erythematosus (DILE) remains a matter of concern. The prevalence of DILE secondary to anti-TNF-α therapy is estimated around 0.5-1% and clinical features include arthritis/arthralgia, rash, serositis, fever, myalgias, cytopenias, among others. According to the literature, DILE secondary to anti-TNF-α agents differs in several ways from the clinical and laboratory findings typically associated with classic DILE.Objectives:To estimate the incidence of induction of antinuclear antibodies (ANA) and DILE in a monocentric cohort of patients with spondyloarthritis and psoriatic arthritis treated with anti-TNF-α agents. To describe the clinical and laboratorial features and outcomes of patients with DILE.Methods:We performed a retrospective analysis of patients with spondyloarthritis and psoriatic arthritis treated with anti-TNF-α agents, from our University Hospital, who have been registered on the Portuguese Rheumatic Diseases Register (Reuma.pt) between July 2001 and December 2020. Patients with positive ANA (titer > 1/100) before the anti-TNF-α therapy were excluded. Because specific criteria for the diagnosis of DILE have not been established, we considered the diagnosis in case of a temporal relationship between clinical manifestations and anti-TNF-α treatment and fulfillment of ACR/EULAR 2019 classification criteria for SLE. In patients with DILE, clinical features, laboratory findings, systemic therapies and outcome after discontinuation of medication were collected from reuma.pt and medical records. For the clinical and demographic predictors, continuous variables were analyzed using a two-sided t-test and categorical variables using a Fisher’s exact test. P-value <0.05 was considered statistically significant.Results:In the spondyloarthritis group, 290 patients were included (44.8% females, mean age at diagnosis of 33.3 ± 11.5 years and mean disease duration of 15.1 ± 10.4 years) and in the psoriatic arthritis group, 116 patients were included (50.0% females, mean age at diagnosis of 40.1 ± 11.0 years and mean disease duration of 13.1 ± 6.8 years). In our study, we observed high serology conversion rates (positive ANA in 67.9% and 58.6% of patients with Spondyloarthritis and Psoriatic Arthritis, respectively), with similar conversion rates between different anti-TNF drugs. Three patients with spondyloarthritis (1.0%) and 1 patient with psoriatic arthritis (0.9%) developed DILE. Etanercept was the causative agent in 2 cases, infliximab and adalimumab in 1 case, each. Peripheral arthritis (new onset or abrupt worsening) occurred in 2 patients, serositis in 1 patient, constitutional symptoms in 2 patients, subnephrotic proteinuria in 1 patient, lymphopenia in 2 patients and hypocomplementemia in 1 patient. Specific treatment was prescribed to the 4 patients (oral corticosteroids) and they achieved complete recovery. After anti–TNF-α treatment interruption, no patient had recurrent disease. We observed that patients with DILE had a significantly longer disease duration (> 8.4 years; p=0.04) and a significantly longer duration of therapy with anti-TNF (> 4.0 years; p=0.04) when compared to patients without DILE.Conclusion:Despite the frequent induction of autoantibodies, the development of DILE secondary to anti–TNF-α agents is rare. Our study demonstrates an incidence rate similar to other studies reported before. The clinical and laboratorial characteristics of our patients with DILE attributable to anti–TNF-α agents differ significantly from DILE due to more traditional agents, as is described in literature. Overall, patients in this study had mild disease that improved after therapy discontinuation, without recurrence of the disease. It seems that a longer disease duration and a longer period under anti-TNF-α therapy may increase the risk of DILE development.Disclosure of Interests:None declared

THU0271 PERFORMANCE OF THE EULAR/ACR 2019 CLASSIFICATION CRITERIA FOR SYSTEMIC LUPUS ERYTHEMATOSUS IN EARLY DISEASE, ACROSS SEXES AND ETHNICITIES

2020 ◽  
Vol 79 (Suppl 1) ◽  
pp. 362.1-362
Author(s):  
S. Johnson ◽  
R. Brinks ◽  
K. Costenbader ◽  
D. Daikh ◽  
M. Mosca ◽  
...  
Keyword(s):  
Lupus Erythematosus ◽  
Disease Duration ◽  
Classification Criteria ◽  
Classification Systems ◽  
Early Disease ◽  
Research Support ◽  
Becton Dickinson ◽  
Asian Patients ◽  
Acr Criteria ◽  
New Criteria

Background:EULAR/ACR 2019 SLE Classification Criteria were validated in an international cohort.Objectives:To evaluate performance characteristics of SLE classification systems in sex, race/ethnicity, and disease duration subsets.Methods:Sensitivity and specificity of the EULAR/ACR 2019, SLICC 2012 and ACR 1982/1997 criteria were evaluated in the validation cohort.Results:The cohort consisted of female (n=1098), male (n=172), Asian (n=118), Black (n=68), Hispanic (n=124) and White (n=941) patients; and patients with an SLE duration of 1-3 years (n=196), 3-5 years (n=157), and ≥5 years (n=879). Among patients with 1-3 years disease duration, the EULAR/ACR criteria had better sensitivity than the ACR criteria (97% (95%CI 92-99%) vs 81% (95%CI 72-88%). The new criteria performed well in men (sensitivity 93%, specificity 96%) and women (sensitivity 97%, specificity 94%). The new criteria had better sensitivity than the ACR criteria in White (95% vs 83%), Hispanic (100% vs 86%) and Asian patients (97% vs 77%).Conclusion:The EULAR/ACR 2019 criteria perform well in patients with early disease, and across sexes and ethnicities.Disclosure of Interests:Sindhu Johnson Grant/research support from: Boehringer Ingelheim, Corbus Pharmaceuticals, GlaxoSmithKline, Roche, Merck, Bayer, Consultant of: Boehringer Ingelheim, Ikaria, Ralph Brinks: None declared, Karen Costenbader Grant/research support from: Merck, Consultant of: Astra-Zeneca, David Daikh: None declared, Marta Mosca: None declared, Rosalind Ramsey-Goldman: None declared, Josef S. Smolen Grant/research support from: AbbVie, Eli Lilly, Janssen, Merck Sharp & Dohme, Pfizer, Roche – grant/research support, Consultant of: AbbVie, Amgen Inc., AstraZeneca, Astro, Celgene Corporation, Celtrion, Eli Lilly, Glaxo, ILTOO, Janssen, Medimmune, Merck Sharp & Dohme, Novartis, Pfizer, Roche, Samsung, Sanofi, UCB – consultant, Speakers bureau: AbbVie, Amgen Inc., AstraZeneca, Astro, Celgene Corporation, Celtrion, Eli Lilly, Glaxo, ILTOO, Janssen, Medimmune, Merck Sharp & Dohme, Novartis, Pfizer, Roche, Samsung, Sanofi, UCB – speaker, David Wofsy: None declared, Dimitrios Boumpas Grant/research support from: Unrestricted grant support from various pharmaceutical companies, Diane L Kamen Consultant of: Consulted on SLE survey development for Lilly and consulted on SLE trial protocol development for EMD Serono in 2019, David Jayne Grant/research support from: ChemoCentryx, GSK, Roche/Genentech, Sanofi-Genzyme, Consultant of: Astra-Zeneca, ChemoCentryx, GSK, InflaRx, Takeda, Insmed, Chugai, Boehringer-Ingelheim, Ricard Cervera: None declared, Nathalie Costedoat-Chalumeau Grant/research support from: UCB to my institution, Betty Diamond: None declared, Dafna D Gladman Grant/research support from: AbbVie, Amgen Inc., BMS, Celgene Corporation, Janssen, Novartis, Pfizer, UCB – grant/research support, Consultant of: AbbVie, Amgen Inc., BMS, Celgene Corporation, Janssen, Novartis, Pfizer, UCB – consultant, Bevra H. Hahn Grant/research support from: Janssen Research & Development, LLC, Falk Hiepe: None declared, Soren Jacobsen: None declared, Dinesh Khanna Shareholder of: Eicos Sciences, Inc./Civi Biopharma, Inc., Grant/research support from: Dr Khanna was supported by NIH/NIAMS K24AR063120, Consultant of: Acceleron, Actelion, Bayer, Boehringer Ingelheim, Bristol-Myers Squibb, Corbus Pharmaceuticals, Horizon Therapeutic, Galapagos, Roche/Genentech, GlaxoSmithKline, Mitsubishi Tanabe, Sanofi-Aventis/Genzyme, UCB, Kirsten Lerstrom: None declared, Elena Massarotti: None declared, William Joseph McCune: None declared, Guillermo Ruiz-Irastorza: None declared, Jorge Sanchez-Guerrero: None declared, Matthias Schneider: None declared, Murray B Urowitz: None declared, George Bertsias Grant/research support from: GSK, Consultant of: Novartis, Bimba F. Hoyer: None declared, Nicolai Leuchten: None declared, Chiara Tani: None declared, Sara Tedeschi: None declared, Zahi Touma: None declared, Gabriela Schmajuk Grant/research support from: Pfizer, Branimir Anic: None declared, Florence Assan: None declared, Tak Chan: None declared, Ann E Clarke: None declared, Mary K. Crow: None declared, László Czirják Consultant of: Actelion, BI, Roche-Genentech, Lilly, Medac, Novartis, Pfizer, Bayer AG, Andrea Doria Consultant of: GSK, Pfizer, Abbvie, Novartis, Ely Lilly, Speakers bureau: UCB pharma, GSK, Pfizer, Janssen, Abbvie, Novartis, Ely Lilly, BMS, Winfried Graninger: None declared, Bernadett Halda-Kiss: None declared, Sarfaraz Hasni: None declared, Peter Izmirly: None declared, Michelle Jung: None declared, Gabor Kumanovics Consultant of: Boehringer, Teva, Speakers bureau: Roche, Lilly, Novartis, Xavier Mariette: None declared, Ivan Padjen: None declared, Jose M Pego-Reigosa: None declared, Juanita Romero-Diaz Consultant of: Biogen, Iñigo Rua-Figueroa: None declared, Raphaèle Seror Consultant of: BMS, Medimmune, Novartis, Pfizer, GSK, Lilly, Georg Stummvoll: None declared, Yoshiya Tanaka Grant/research support from: Asahi-kasei, Astellas, Mitsubishi-Tanabe, Chugai, Takeda, Sanofi, Bristol-Myers, UCB, Daiichi-Sankyo, Eisai, Pfizer, and Ono, Consultant of: Abbvie, Astellas, Bristol-Myers Squibb, Eli Lilly, Pfizer, Speakers bureau: Daiichi-Sankyo, Astellas, Chugai, Eli Lilly, Pfizer, AbbVie, YL Biologics, Bristol-Myers, Takeda, Mitsubishi-Tanabe, Novartis, Eisai, Janssen, Sanofi, UCB, and Teijin, Maria Tektonidou Grant/research support from: AbbVie, MSD, Novartis and Pfizer, Consultant of: AbbVie, MSD, Novartis and Pfizer, Carlos Vasconcelos: None declared, Edward Vital Grant/research support from: AstraZeneca, Roche/Genentech, and Sandoz, Consultant of: AstraZeneca, GSK, Roche/Genentech, and Sandoz, Speakers bureau: Becton Dickinson and GSK, Daniel J Wallace: None declared, Sule Yavuz: None declared, Pier Luigi Meroni: None declared, Marvin Fritzler: None declared, Raymond Naden: None declared, Thomas Dörner Grant/research support from: Janssen, Novartis, Roche, UCB, Consultant of: Abbvie, Celgene, Eli Lilly, Roche, Janssen, EMD, Speakers bureau: Eli Lilly, Roche, Samsung, Janssen, Martin Aringer Consultant of: Boehringer Ingelheim, Roche, Speakers bureau: Boehringer Ingelheim, Roche

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.

A comparative study on clinical and serological characteristics between patients with rhupus and those with systemic lupus erythematosus and rheumatoid arthritis

Lupus ◽  
2020 ◽  
Vol 29 (10) ◽  
pp. 1216-1226
Author(s):  
Beatriz Frade-Sosa ◽  
Javier Narváez ◽  
Tarek Carlos Salman-Monte ◽  
Raul Castellanos-Moreira ◽  
Vera Ortiz-Santamaria ◽  
...  
Keyword(s):  
Rheumatoid Arthritis ◽  
Systemic Lupus Erythematosus ◽  
Autoimmune Diseases ◽  
Lupus Erythematosus ◽  
Disease Duration ◽  
Renal Involvement ◽  
Clinical Manifestations ◽  
Classification Criteria ◽  
Systemic Lupus ◽  
Cross Sectional

Background The concomitant presence of two autoimmune diseases – systemic lupus erythematosus (SLE) and rheumatoid arthritis (RA) – in the same patient is known as rhupus. We evaluated a group of patients with rhupus to clarify further their clinical, serological and immunogenic features in a multi-centre cohort. In addition, the study aimed to explore the utility of the 2019 European League Against Rheumatism/American College of Rheumatology (EULAR/ACR) SLE classification criteria in our group of patients with rhupus. Methods This was a cross-sectional study. We included rhupus patients from 11 different rheumatology departments, and compared them to SLE and RA patients at a ratio of 2:1. All information was recorded following a pre-established protocol. Results A total of 200 patients were included: 40 rhupus patients and 80 each of SLE and RA patients as controls. Disease duration was similar among SLE and rhupus groups (around 13 years), but the RA group had a significantly lower disease duration. Main clinical manifestations were articular (94.2%), cutaneous (77.5%) and haematological (72.5%). Rhupus patients had articular manifestations similar to those expected in RA. Only 10% of rhupus patients had renal involvement compared with 25% of those with SLE ( p < 0.05), while interstitial lung disease was more common in patients affected by RA. The 2019 EULAR/ACR SLE criteria were met in 92.5% of the rhupus patients and in 96.3% of the SLE cohort ( p > 0.05). Excluding the joint domain, there were no differences between the numbers of patients who met the classification criteria. Conclusion Rhupus patients follow a particular clinical course, with full expression of both SLE and RA in terms of organ involvement, except for a lower prevalence of kidney affection. The new 2019 EULAR/ACR SLE criteria are not useful for differentiating SLE and rhupus patients. A new way of classifying autoimmune diseases is needed to identify overlapping clusters.

scholarly journals No Increase in Rate and Severity of Flares During Pregnancy and Post-Partum Period in Pregnant Patients with Systemic Lupus Erythematosus. A Sex, Age and Disease Duration Matched Controlled Study

2020 ◽  
Author(s):  
Worawit Louthrenoo ◽  
Thananant Trongkamolthum ◽  
Nuntana Kasitatnon ◽  
Antika Wongthanee
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Disease Activity ◽  
Lupus Erythematosus ◽  
Disease Duration ◽  
Post Partum ◽  
Age At Diagnosis ◽  
Controlled Study ◽  
Control Group ◽  
Systemic Lupus ◽  
Post Partum Period

Abstract Background: Flares in pregnant patients with systemic lupus erythematosus (SLE) have shown conflicting results. This study aimed to determine the disease activity, and rate and severity of flares in pregnant SLE patients compared with the non-pregnant SLE controls. Methods: The medical records of pregnant patients in the SLE cohort were identified. Controls were non-pregnant female SLE patients who were matched for age at diagnosis and disease duration prior to conception. Disease activity was determined by mSLEDAI-2K. The definition and severity of flares followed the SELANA-SLEDAI Flare Index. The disease activity was measured from 6 months prior to conception (-6M) until termination of pregnancy or delivery.Results: Ninety pregnancies occurred from 77 patients, of whom 36.67% had active disease at the time of conception. The pregnancy group was slightly, but significantly, younger than the control group at diagnosis (21.63 ± 5.89 years vs. 24.05 ± 7.27 years, p = 0.015). The SLE disease activity (mSLEDAI-2K) score was comparable in both groups from -6M to the post-partum period, with that in the control group being slightly but significantly higher than that in the pregnancy group at conception (3.57 ± 4.28 vs. 1.91 ± 3.44, p = 0.003). Of the 90 pregnancies, the overall incidence of flares in both groups was similar during pregnancy (39 vs. 26, p = 0.070). There also was no difference in the incidence of flare during each trimester, with 19 vs.7 flares among 90 pregnancies (p = 0.588) in the 1st, 12 vs. 12 among 82 (p = 0.682) in the 2nd, and 6 vs. 7 among 71 (p = 0.266) in the 3rd trimester. The incidence of flare during the post-partum period also was similar (42 vs. 30 flares among 90 pregnancies, p = 0.091). There was no difference in the severity of flare in each trimester, overall flare during pregnancy or post-partum flare. Conclusions: This study found no difference in flare rate or severity of flares between pregnant SLE patients and non-pregnant SLE controls, who were matched by sex, age at diagnosis and disease duration prior to pregnancy.

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