Evaluating the Threshold Score for Classification of Systemic Lupus Erythematosus Using the EULAR/ACR Criteria.

BackgroundThe syndrome of incomplete lupus erythematosus (ILE) likely includes individuals at risk for development of systemic lupus erythematosus (SLE). Studies of interventions to lower risk or prevent further disease in...

ObjectivesThe European League Against Rheumatism (EULAR)/American College of Rheumatology (ACR) 2019 Classification Criteria for systemic lupus erythematosus (SLE) have been validated with high sensitivity and specificity. We evaluated the performance...

SAT0430 Application and Validation of New Classification Criteria (Slicc) for Lupus Erythematosus in the Real Life of a Tertiary Referal Hospital in Brazil

BackgroundChronic cutaneous lupus (CCLE) is a group of distinctive cutaneous lupus erythematosus (CLE) subtypes that includes discoid lupus (DLE), lupus profundus (LEP), chilblain lupus (CLE), and lupus tumidus (LET). While...

The objective of the study is to evaluate the sensitivity of the new criteria for the classification of systemic lupus erythematosus (SLE), when applied to real SLE cases. We retrospectively reviewed the electronic medical records of 100 consecutive patients who visited St. Luke's International Hospital. Patients were included if they were clinically diagnosed as having SLE and excluded if they had other autoimmune disease or if they were less than 18years old. Each patient was evaluated if they satisfied the American College of Rheumatology (ACR) 1997 classification criteria (1997 criteria), 2012 Systemic Lupus International Collaborating Clinics criteria (2012 criteria), or 2019 ACR/European League Against Rheumatism (EULAR) criteria. Among the 100 patients, the sensitivity of the 1997, 2012, and 2019 criteria was, 97, 99, and 92%, respectively. The total patient score with the 2019 criteria ranged from 12 to 44 (mean, 27.3). All patients who were classified as non-SLE with the 2019 criteria had an anti-nuclear antibody (ANA) titre of < 1:80. The 2019 criteria for SLE accomplished modestly high sensitivity in the real-world practice, but not as high as the 1997 and 2012 criteria. They possibly misclassify the real SLE cases as non-SLE, especially if patients have a low titre (< 1:80) of ANA.

ObjectiveThe 2019 European Alliance of Associations for Rheumatology (EULAR)/American College of Rheumatology (ACR) systemic lupus erythematosus (SLE) classification criteria score (≥20 points) has been associated with poor outcomes. We aimed...

The clinical and serologic heterogeneity of systemic lupus erythematosus (SLE) presents challenges for diagnosis, particularly in the earliest stages of the disease when there are insufficient signs to make a reliable diagnosis. To make a comparative assessment of sensitivity and specificity of various classification criteria of SLE on a cohort of patients of Nasonova Research Institute of Rheumatology. A total of 252 patients were included in the study; 152 (60%) of 252 patients had reliable SLE (mean age 36 [29.5-46] years, duration of disease 9 [3.4-19] years). Of 252 patients, 26 (11%) had PAPS (mean age 36.5 [31-42] years, duration of disease 4.6 [1-10.4] years). Systemic sclerosis was diagnosed in 74/252 (29%) patients, (mean age 51.5 [42-59] years, duration of disease 9 [5-16] years). The quality of the classification function of the criteria was assessed by ROC analysis. SLE was diagnosed in 131 (86%) of 152 patients using the American College of Rheumatology - ACR)-1997 criteria, in 145 (95%) using the The Systemic Lupus International Collaborating Clinics (SLICC) 2012 criteria, and in 144 (94.7%) using the European League Against Rheumatism (EULAR)/ACR 2019 criteria. ANF positivity was the least statistically significant of all signs in relation to the diagnosis of SLE. The area under the curve (AUC) for ANF≥1/160 titers was AUC 0.654 for the ACR-97 criteria, AUC 0.616 for the SLICC-12 SLE criteria, and AUC 0.609 for the 2019 EULAR/ACR criteria. ROC analysis of the relationship between the number of criteria/points and a reliable diagnosis of SLE revealed a high diagnostic accuracy - the AUC for all SLE criteria was greater than 0.940. In the ROC analysis of patients with SLE and PAFS, indicating the number of diagnostic criteria, sensitivity was 86% for ACR-1997, 95% for SLICC-2012, 95% for EULAR/ACR 2019, and specificity was 100, 62 and 62%, respectively. The classification criteria SLICC-2012 and EULAR/ACR 2019 are more sensitive for the diagnosis of SLE in the Russian population, and the criteria ACR-1997 are more specific. All three variants of the SLE classification criteria have sufficient sensitivity and specificity for their use in real clinical practice.

ObjectiveTo evaluate the usefulness of biomarkers to predict the evolution of patients suspected of systemic lupus erythematosus (SLE), designated as probable SLE (pSLE), into classifiable SLE according to the American College of Rheumatology (ACR) classification criteria.MethodsPatients suspected of SLE were enrolled by lupus experts if they fulfilled three ACR criteria for SLE and were followed for approximately 1‐3 years to evaluate transition into ACR‐classifiable SLE. Individual cell‐bound complement activation products (CB‐CAPs), serum complement proteins (C3 and C4), and autoantibodies were measured by flow cytometry, turbidimetry, and enzyme‐linked immunosorbent assay, respectively. Blood levels of hydroxychloroquine (HCQ) were measured by mass spectrometry. A multianalyte assay panel (MAP), which includes CB‐CAPs, was also evaluated. A MAP of greater than 0.8 reflected the optimal cutoff for transition to SLE. Time to fulfillment of ACR criteria was evaluated by Kaplan‐Meier analysis and Cox proportional hazards model.ResultsOf the 92 patients with pSLE enrolled, 74 had one or two follow‐up visits 9‐35 months after enrollment for a total of 128 follow‐up visits. Overall, 28 patients with pSLE (30.4%) transitioned to ACR‐classifiable SLE, including 16 (57%) in the first year and 12 (43%) afterwards. A MAP score of greater than 0.8 at enrollment predicted transition to classifiable SLE during the follow‐up period (hazard ratio = 2.72; P = 0.012), whereas individual biomarkers or fulfillment of Systemic Lupus International Collaborating Clinics criteria did not. HCQ therapy was not associated with the prevention of transition to SLE.ConclusionApproximately one‐third of patients with pSLE transitioned within the study period. MAP of greater than 0.8 predicted disease evolution into classifiable SLE.

Background M-phase phosphoprotein (MPP-1), also termed kinesin interacting protein (KIF20B), is a 210 kDa protein that is highly expressed during cell division. Autoantibodies to MPP-1 were first described in approximately 25% of patients with idiopathic ataxia, but recent studies have indicated that they are also found in systemic lupus erythematosus (SLE). The goals of this study were to determine the frequency of anti-MPP-1 in a local SLE cohort and then identify demographic, clinical, and serologic correlations. Methods Patients fulfilling the American College of Rheumatology (ACR) or Systemic Lupus International Collaborating Clinics (SLICC) Classification Criteria for SLE were enrolled in the SouThern Alberta Registry for Lupus EryThematosus (STARLET) cohort (Calgary, Canada). Demographic, clinical information (disease activity – SLEDAI-2K; damage – SLICC/ACR DI), and sera were collected at time of enrollment. Antibodies to MPP-1 were determined by an addressable laser bead immunoassay (ALBIA) utilizing an in vitro expressed MPP-1 cDNA construct inserted into a GFP vector (Clontech Laboratories Inc., Saint-Germain-en-Laye, France). ALBIA results were expressed as median florescence units (MFU) and a dilution of ≥1:500 MFU was considered highly positive. Univariable analysis was performed to determine associations between the prevalence of high positive anti-MPP-1 and demographic (age, sex, race/ethnicity), clinical features (SLEDAI-2K and SLICC/ACR DI total scores and subscales and neurological subscale of the ACR and SLICC Classification Criteria), medications, other autoantibodies (anti-dsDNA, extractable nuclear antigens, and anti-phospholipid antibodies). Results One hundred and forty SLE patients were included; 89.3% were female with a mean age of 47.3 years (SD 16.3) and disease duration of 13.9 years (SD 11.6). The prevalence of high titre anti-MPP-1 was 15.0% (21/140) respectively. Univariable analysis demonstrated that high anti-MPP-1 positivity was associated with a higher total SLEDAI-2K score (Odds Ratio (OR), 1.1 [95% CI 1.0 to 1.3]), particularly with the serositis (OR 3.0, [95% CI 1.4 to 6.6]) and immunological subscales (OR 2.0, [95% CI 1.4 to 2.9]). High anti-MPP-1 positivity was also associated with anti-dsDNA (OR 5.5 [95% CI 1.8 to 16.6]), anti-SSA/Ro60 (OR 3.1 [95% CI 1.0 to 8.9]) and anti-phosphotidylserine/prothrombin complex (aPS/PT)-IgG (OR 3.6 [95% CI 1.1 to 11.5]). Conclusions High titer anti-MPP-1 antibodies were common in this SLE cohort (15.0%) and may be associated with greater clinical and serologic SLE disease activity. A larger study is currently underway to more clearly delineate its role as a biomarker in SLE. Acknowledgements The authors are grateful for the technical assistance of Ms. Haiyan Hou and Meifeng Zhang (Mitogen Advanced Diagnostics, University of Calgary).

Evaluation of remission definitions for systemic lupus erythematosus: a prospective cohort study.

84-Year-Old Man With Night Sweats, Weight Loss, and Diarrhea

Specific events that occur during the development of systemic lupus erythematosus (SLE) can be quite variable among individual patients. The aim of this study was to identify patterns that distinguish early clinical events in SLE and to assess whether the presence of associated autoantibodies precedes the fulfillment of clinical criteria. Through a retrospective chart review of military medical records, 130 patients who met the American College of Rheumatology (ACR) criteria for the classification of SLE were identified. The initial time at which each criterion was fulfilled was recorded. Autoantibody analysis was performed on serum samples, using enzyme-linked immunosorbent assays or immunofluorescence. The clinical features that were observed earliest were discoid rash and seizures, which developed a mean 1.74 and 1.70 years, respectively, before the diagnosis of SLE; however, arthritis was the criterion that was most commonly observed before diagnosis. The presence of IgG rheumatoid factor (IgG-RF) preceded the development of arthritis in 15 (94%) of the 16 patients who were positive for IgG-RF and in whom arthritis developed (Z = 10.2, P < 0.0001). Analogously, IgM-RF appeared before the development of arthritis in 13 (76%) of 17 patients. Anti-double-stranded DNA antibodies were associated with renal disease and appeared before evidence of nephritis in most patients (92%) (Z = 13.3, P < 0.0001). An analysis of the appearance of autoantibodies compared with the appearance of clinical criteria not associated with them revealed no significant temporal relationship. Symptoms associated with the ACR criteria for classification of SLE are commonly present before the diagnosis of SLE, and development of organ-associated autoantibodies generally precedes the appearance of their associated clinical features.

&lt;p class="abstract"&gt;&lt;strong&gt;Background:&lt;/strong&gt; Systemic lupus erythematosus (SLE) is an autoimmune disease involving multiple organ systems. Other than cutaneous manifestations, thorough examination and investigations for systemic involvement should also be done. The objective is to study the prevalence of various systemic manifestations in SLE patients in a tertiary care hospital.&lt;/p&gt;&lt;p class="abstract"&gt;&lt;strong&gt;Methods:&lt;/strong&gt; A retrospective study was conducted from April 2009 to August 2018 on the patients who fulfilled the Systemic Lupus International Collaborating Clinics (SLICC) criteria for SLE. A total of 63 cases of SLE were extracted from the case record for the study. Each case was subjected to detailed history, examination, investigations including complete blood count, liver function test, renal function test, blood sugar, urine routine examination, ECG, X-ray of chest and involved joints, USG abdomen, pulmonary function test. Ours being a resource limited set-up, ANA, ENA profile and echocardiography were done whenever patient could afford.&lt;strong&gt;&lt;/strong&gt;&lt;/p&gt;&lt;p class="abstract"&gt;&lt;strong&gt;Results:&lt;/strong&gt; In present study of 63 cases of SLE 94% were females. Mean age was 30 years. Various systemic involvement was present, amongst them musculoskeletal system was the most common system involved with 60 (95%) patients, followed by hematological system 48 (76%), renal system 37 (59%), neuropsychiatric system 29 (46%), gastrointestinal system 28 (44%), reproductive system 27 (43%), cardiovascular system 25 (40%). Other systems with ophthalmic involvement 23 (21%), hepatobiliary system 22 (35%), respiratory system 17 (27%), endocrine involvement 13 (28%) and malignanacy was present in 2 (3%) cases.&lt;/p&gt;&lt;p class="abstract"&gt;&lt;strong&gt;Conclusions:&lt;/strong&gt; SLE is protean in its manifestations. Thorough knowledge about the systemic involvement will help in tailoring the treatment in case of SLE.&lt;/p&gt;

SAT0032 Assessment of the Accrual Clinical Manifestation, Organ Damage, and Disease Activity in 996 Korean Patients with Systemic Lupus Erythematosus

Introduction: Systemic lupus erythematosus (SLE) is a chronic, autoimmune disease wich variable symptoms affecting numerous organs and insidious onset of an unpredictable course, with episodes of activity and remission. The development of the disease is evaluated by a combination of clinical history, physical and laboratory tests to identify risk factors related to the stage or complications of the disease. The American College of Rheumatology (ACR 97) was the first to establish criteria for SLE classification. In 2012, The Systemic Lupus International Collaborating Clinics (SLICC 12) published a new set of criteria aimed at optimizing the classification of SLE. Objectives: Compare the criteria proposed by ACR 97 and SLICC 12 for the diagnosis of SLE and to gather information on clinical characteristics, diagnosis, and treatment. Methodology: Literature review, using the PubMed-NCBI database. The inclusion criteria were: articles published in the last five years; study in humans and selection by the direct relation with the selected theme. Results: SLICC 12 demonstrated a higher sensitivity diagnosed in reports compared with as ACR 97. Conclusion: We found that SLICC 12 is the classification criterion for SLE presenting the most excellent variety of laboratory, cutaneous, immunological and neuropsychiatric findings, allowing a better performance of the classification of patients with SLE and thus the early diagnosis of the disease.