Classification Criteria For Systemic Lupus Erythematosus Research Articles

Prognostic value and predictors of the alteration of the diffusing capacity of the lungs for carbon monoxide in systemic lupus erythematosus.

SLE is a systemic autoimmune disease characterized by heterogeneous manifestations and severity, with frequent lung involvement. Among pulmonary function tests, the measure of the diffusing capacity of the lungs for carbon monoxide (DLCO) is a noninvasive and sensitive tool assessing pulmonary microcirculation. Asymptomatic and isolated DLCO alteration has frequently been reported in SLE, but its clinical relevance has not been established. This retrospective study focused on 232 SLE patients fulfilling the 2019 EULAR/ACR classification criteria for SLE. Data were collected from the patient's medical record, including demographic, clinical and immunological characteristics, while DLCO was measured when performing pulmonary function tests as part of routine patient follow-up. At the end of follow-up, DLCO alteration (<70% of predicted value) was measured at least once in 154 patients (66.4%), and was associated with a history of smoking as well as interstitial lung disease, but was also associated with renal and neurological involvement. History of smoking, detection of anti-nucleosome autoantibodies and clinical lymphadenopathy at diagnosis were independent predictors of DLCO alteration, while early cutaneous involvement with photosensitivity was a protective factor. DLCO alteration, at baseline or any time during follow-up, was predictive of admission in intensive care unit and/or of all-cause death, both mainly due to severe disease flares and premature cardiovascular complications. This study suggests a link between DLCO alteration and disease damage, potentially related to SLE vasculopathy, and a prognostic value of DLCO on death or intensive care unit admission in SLE.

Preconception clinical factors related to adverse pregnancy outcomes in patients with systemic lupus erythematosus or primary Sjögren’s syndrome: a retrospective cohort study

ObjectiveTo identify preconception clinical factors associated with adverse pregnancy outcomes (APO) in patients with systemic lupus erythematosus (SLE) or primary Sjögren’s syndrome (pSS).MethodsA single-centre, retrospective cohort study was conducted, which...

Comparative assessment of sensitivity and specificity of three variants of classification criteria for systemic lupus erythematosus in a cohort of Russian patients

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.

Unusual presentation and rare association of pediatric lupus

Systemic lupus erythematosus (SLE) is a chronic multisystem autoimmune inflammatory disorder with diverse clinical manifestations which can lead to significant morbidity and mortality. Children and adolescents represent 15% to 20% of all patients with SLE. Childhood onset SLE is a rare disease with a prevalence of 3.5 to 8.8 per one lakh children. Localized or generalized lymphadenopathy may be an uncommon presenting feature of SLE. None of the classification criteria for SLE include lymphadenopathy as a criterion including the latest 2019 American college of rheumatology (ACR) / European league against rheumatism (EULAR) criteria. Nine-year-old female child presented with history of swelling on both sides of neck associated with fever and easy fatiguability for 2 months. Clinical examination revealed significant bilateral cervical and axillary lymph nodes with hepatomegaly. Excision biopsy of lymph node was suggestive of Kikuchi Fujimoto disease (KFD). As rare association of KFD with SLE is well described in literature, despite negative clinical features and laboratory findings of SLE, including an initial negative ANA IF test, we did a repeat ANA IF, complement (C3) level and dsDNA testing, all of which turned out to be positive, confirming the diagnosis of SLE. The child was started on hydroxychloroquine and oral steroids with dramatic response. During steroid tapering she developed a flare needing the addition of azathioprine. Currently she is in remission and is under follow up. Although rare, lupus lymphadenopathy may be a presenting feature of SLE. This can even antecede the diagnosis of SLE by many years, when the presence of auto antibodies or low complement levels are not detected and are associated with higher disease activity. This emphasizes the importance of high index of suspicion of SLE in cases of lymphadenopathy, enabling early diagnosis and management which is essential for preventing morbidity and mortality.

Clinical efficacy of belimumab in central nervous system demyelinating syndromes with systemic lupus erythematosus: A case series.

Systemic lupus erythematosus (SLE) is a complex autoimmune disease involving multiple systems. Central nervous system (CNS) demyelinating syndromes are one of the rare neurological manifestations of SLE, whose diagnosis, treatment, and prognosis are rarely reported. Belimumab, an anti-BAFF monoclonal antibody, has been approved by the FDA for the treatment of SLE. We aimed to assess the effects of belimumab on demyelinating syndromes in patients with SLE. Six patients with demyelination in SLE who were managed at Traditional Chinese and Western Medicine Hospital of Wuhan from March 2021 to March 2023, who received belimumab ≥ 5 times, were enrolled. Ten age- and sex-matched SLE patients with noutneuropsychiatric systemic lupus erythematosus (NPSLE) and normal controls were recruited to analyze potential biomarkers. All patients were diagnosed with SLE based on the 2012 Systemic Lupus International Collaborating Clinics (SLICC) SLE classification criteria or the 2019 EULAR/ACR classification criteria. All SLE patients with CNS demyelinating syndromes were diagnosed by rheumatologists, neurologists, and radiologists. These patients were administered belimumab combined with standard treatment (glucocorticoids and/or antimalarials and/or immunosuppressants [cyclophosphamide, mycophenolate, methotrexate, etc.]). Six patients were included in the study (100% female, mean [range] age at first demyelinating episode 42.8 [24-66] years). The most common extra-CNS features in these patients were rash, arthritis, alopecia, leukopenia, and hypocomplementemia. After Belimumab treatment, 3 of 6 (50%) patients achieved complete remission with decreased prednisone, 2 improvements, and 1 relapsed with uterine surgery. Compared with the baseline, 3.5 months post belimumab treatment, the disease activity score SLEDAI (21.5-5.5, P < .001), C3 and C4 increased, and extra-CNS symptoms improved rapidly. Moreover, The expression of lupus susceptibility gene PBX1 in CD19+ B cells was lowest in demyelinating syndromes with lupus patients compared with healthy volunteers and lupus patients without demyelination, and its relative expression negatively correlated with SLE disease activity. Belimumab could be an effective and safe option for the treatment of SLE demyelination. In addition, PBX1 might be a potential biomarker for the clinical diagnosis of lupus in patients with demyelinating syndrome.

Serological and clinical associations of autoantibodies in Chinese patients with new-onset systemic lupus erythematosus

To study the clinical significance of autoantibodies in Chinese patients with new-onset systemic lupus erythematosus (SLE), we enrolled 526 new-onset patients who met the 1997 Updated American College of Rheumatology SLE Classification Criteria for a retrospective cohort study. Chi-square test and Wilcoxon rank-sum test were used to detect the relationship of autoantibodies with clinical manifestations and serological results respectively. Our results demonstrated that the positive rate of anti-ribosomal P protein (anti-P) antibody in female patients was higher than that in male patients (41.2% vs. 22%, P = 0.008). Patients with anti-SSB (43.95 ± 73.12 vs. 40.92 ± 75.75, P = 0.004; 63.93 ± 103.56 vs. 55.06 ± 120.84, P = 0.008 respectively) antibodies had higher levels of alanine aminotransferase (ALT) and aspartate transaminase (AST), whereas those with anti-P antibody (28.90 ± 25.70 vs. 50.08 ± 93.00, P = 0.014; 38.51 ± 48.19 vs. 69.95 ± 142.67, P = 0.047, respectively) had lower levels of them. Anti-dsDNA antibody (P = 0.021) was associated with pulmonary arterial hypertension (PAH). The patients with anti-Ro60 (P = 0.044), anti-P (P = 0.012) and anti-dsDNA (P = 0.013) antibodies were less likely to develop Interstitial lung disease. Anti-SmRNP antibody was correlated to lower prevalence of neuropsychiatric symptoms (P = 0.037), and patients with anti-centromere antibody (ACA) were more likely to develop serositis (P = 0.016).We identified five clusters of SLE-related autoantibodies, confirmed previously reported associations of autoantibodies, and discovered new associations.

Pattern of systemic lupus erythematosus in NIGERIA: a multicentre descriptive hospital-based study.

Systemic lupus erythematosus (SLE) is a chronic autoimmune inflammatory disease with manifestations ranging from mild to life-threatening organ dysfunction. There is wide variability in the reported incidence and prevalence rate globally, particularly in low and middle-income countries. Nigeria had very few isolated reports of SLE from private and public hospitals Therefore, we conducted this large multi-center descriptive study to determine the sociodemographic, clinical profile, laboratory patterns, and treatment among Nigerian lupus patients. A retrospective hospital-based study of all SLE patients seen over 4years (January 2017 to December 2020) was conducted at 20 rheumatology clinics spread across the 6 geopolitical zones of Nigeria. All patients 18years and above satisfying the American College of Rheumatology (ACR) 1997 and/or the Systemic Lupus International Collaboration Clinics (SLICC) 2012 classification criteria for SLE were enrolled. Patients with other Rheumatic and Musculoskeletal Diseases (RMDs) not in keeping with SLE and Patients with incomplete data were excluded. Data was analysed using SPSS version 23.0 software. A total of 896 patients with SLE were included in the final analysis with a mean age ± SD of 34.47 ± 11 and a female to male ratio of 8.1:1. Synovitis was reported by 61.6% of patients, while 51%, 19.9% and11.4% patients reported acute, sub-acute and chronic lupus rashes respectively. ANA was positive in 98.0% with titers ranging from 1:80 to 1:64,000. SLE is not rare in Nigeria. Most patients were female in their 3rd to 4th decades of life. There is a delayed presentation to a rheumatology facility. Arthritis and mucocutaneous manifestations were the most frequent presentation. Key Points •This study presents the first national data on SLE in Nigeria •This study showed that SLE is not rare in Nigeria in contrast to previous reports •There appear to be ethnic disparity in the frequency of lupus among Nigerians •Nigerians with lupus have very high titer of ANA.

Heterogeneity of echocardiographic variables in systemic lupus erythematosus among clinical subgroups according to non-cardiac organ involvement

Systemic lupus erythematosus (SLE) is a heterogeneous autoimmune disease. Cardiac involvement is rare but plays an important prognostic role. The degree of cardiac involvement according to SLE subsets defined by non-cardiac manifestations is unknown. The objective of this study was to identify differences in TTE parameters associated with different SLE subgroups. One hundred and eighty-one patients fulfilling the ACR/EULAR 2019 classification criteria for SLE and had a TTE were included in this cross-sectional study. We defined four subsets of SLE based on the predominant clinical manifestations. A multivariate multinomial regression analysis was performed to determine whether TTE parameters differ between the subsets. The first subset (n = 37) of patients showed features of mixed connective tissue disease (MCTD); the second subset (n = 76) had primarily cutaneous involvement; the third subset (n = 18) exhibited serositis and the last subset (n = 50) had severe disease with significant organ involvement, including renal involvement. Forty TTE parameters were assessed in each patient. Using a multivariate multinomial regression analysis, 3 parameters differed between groups: early diastolic tricuspid annular velocity (RV-E′, P < 0.0001), RV-S′ (RV-pulse DTI systolic peak wave, P = 0.0031), and RV end-diastole diameter (P = 0.0419). The degree of RV dysfunction was lowest in patients with primarily cutaneous involvement. Four distinct clinical subsets based on clinical manifestations differed in terms of TTE parameters of right heart function and diastolic dysfunction. This could help to tailor the cardiac follow-up required by the different clinical subsets of SLE.

LABORATORY BIOMARKERS FOR SYSTEMIC LUPUS ERYTHEMATOSUS

The development of innovative technologies using mono- and multiplex methods of immune analysis contributed to an increase in the sensitivity and specificity of laboratory tests and a significant expansion of the range of laboratory biomarkers of systemic lupus erythematosus (SLE). The article presents modern aspects of laboratory diagnosis of SLE. Screening and confirmatory methods for the study of antinuclear antibodies (ANA), the main immunological marker of SLE, are considered in detail. New classification criteria for SLE are discussed (EULAR/ACR, 2019), which for the first time single out “seropositivity” for the antinuclear factor as a mandatory “incoming” criterion that allows diagnosing the disease as SLE. The characteristics of clinical and laboratory subtypes of SLE associated with the detection of various autoantibodies in serum (antibodies to dsDNA, histones, nucleosomes, Sm, Ro/SSA and La/SSB, U1 ribonucleoprotein,ribosomal protein P, antiphospholipid antibodies, antibodies to C1q) are given. The diagnostic value of defects in the components of the complement system in SLE is indicated. The relationship between ANA and cytokine profiles in SLE patients using multiplex immunoassay of these biomarkers based on xMAP microarray technology is described. It has been shown that the formation of ANA and high activity of SLE are associated with overexpression of the IP-10 and MCP-1 chemokines induced by IFN. The clinical and pathogenetic significance of subpopulations of B-lymphocytes and CD4+ CD25high+ FoxP3+ regulatory T-cells (Treg) of peripheral blood in SLE was noted. SLE is characterized by an increase in the number of double negative memory B cells (CD19+ CD27-IgD-) and plasma cells, a decrease in the levels of naive and transitional B cells in the blood. A positive correlation was found between the number of double negative B cells and the activity of SLE and ESR. The effect of therapy with genetically engineered biological agents (rituximab and belimumab) on B-cell subpopulations in patients with SLE was analyzed. The decrease in the number of Tregs is most pronounced in patients with an acute variant of the course of the disease, high activity of the pathological process, hyperproduction of IgG and expansion of autoreactive B-cells. The study of modern molecularcellular biomarkers allows diagnosing, assessing activity, the nature of the course, clinical and laboratory subtypes of SLE, and predicting the effectiveness of therapy for this disease at a qualitatively new level.

Absolute risk estimation of new-onset proteinuria in patients with systemic lupus erythematosus: a Danish nationwide cohort study.

Kidney involvement and medical compliance are frequent challenges in systemic lupus erythematosus (SLE). Additional data reporting such as absolute risk estimates may strengthen risk stratification and compliance. This study provides absolute risk estimations of risk of new-onset proteinuria among SLE patients. Danish SLE centres provided clinical data on first time observations of proteinuria and other clinical parameters listed in the 1997 American College of Rheumatology Classification Criteria for SLE. Time from first occurring non-renal manifestation to new-onset proteinuria or censoring defined time at risk. Multivariate Cox-regression models were used to identify risk factors for new-onset proteinuria and to calculate risk of proteinuria stratified by risk factor debut age, duration, and sex. The patient population consisted of 586 patients with SLE, mainly Caucasian (94%) women (88%), mean age at inclusion of 34.6 years (standard deviation, SD=14.4 years), observed for a mean of 14.9 years (SD=11.2 years). The cumulative prevalence of proteinuria was 40%. Discoid rash, HR =0.42 (p=0.01) and lymphopenia HR=1.77 (p=0.005) were associated with new-onset proteinuria. Male patients with lymphopenia had the highest predictive risks of proteinuria with a 1-, 5- and 10-year risk of proteinuria ranging from 9-27%, 34-75% and 51-89%, depending on the age at presentation (debut at 20, 30, 40 or 50 years). The corresponding risk profiles for women with lymphopenia were 3-9%, 8-34% and 12-58%, respectively. Large differences in absolute risk estimates for new-onset proteinuria were identified. The differences may aid risk stratification and patient compliance among high-risk individuals.

Lupus low disease activity state and remission and risk of mortality in patients with systemic lupus erythematosus: a prospective, multinational, longitudinal cohort study.

Treat-to-target goals for patients with systemic lupus erythematosus (SLE) have been validated to protect against organ damage and to improve quality of life. We aimed to investigate the association between lupus low disease activity state (LLDAS) and remission and risk of mortality in patients with SLE. We hypothesised that LLDAS has a protective association with mortality risk. In this prospective, multinational, longitudinal cohort study, we used data from patients with SLE in the Asia Pacific Lupus Collaboration cohort collected between May 1, 2013, and Dec 31, 2020. Eligible patients were adults (aged ≥18 years) who met either the 1997 American College of Rheumatology modified classification criteria for SLE or the 2012 Systemic Lupus International Collaborating Clinics classification criteria. The primary outcome was all-cause mortality, and LLDAS, remission, and variations of remission with lower glucocorticoid thresholds were the primary exposure variables. Survival analyses were used to examine longitudinal associations between these endpoints and risk of mortality. This study is registered with ClinicalTrials.gov, NCT03138941. Among a total of 4106 patients in the cohort, 3811 (92·8%) patients were included in the final analysis (median follow-up 2·8 years [IQR 1·0-5·3]; 3509 [92·1%] women and 302 [7·9%] men), of whom 80 died during the observation period (crude mortality rate 6·4 deaths per 1000 person-years). LLDAS was attained at least once in 43 (53·8%) of 80 participants who died and in 3035 (81·3%) of 3731 participants who were alive at the end of the study (p<0·0001); 22 (27·5%) participants who died versus 1966 (52·7%) who were alive at the end of the study attained LLDAS for at least 50% of observed time (p<0·0001). Remission was attained by 32 (40·0%) of 80 who died and in 2403 (64·4%) of 3731 participants who were alive at the end of the study (p<0·0001); 14 (17·5%) participants who died versus 1389 (37·2%) who were alive at the end of the study attained remission for at least 50% of observed time (p<0·0001). LLDAS for at least 50% of observed time (adjusted hazard ratio 0·51 [95% CI 0·31-0·85]; p=0·010) and remission for at least 50% of observed time (0·52 [0·29-0·93]; p=0·027) were associated with reduced risk of mortality. Modifying the remission glucocorticoid threshold (<5·0 mg/day prednisolone) was more protective against mortality than current remission definitions (0·31 [0·12-0·77]; p=0·012), and glucocorticoid-free remission was the most protective (0·13 [0·02-0·96]; p=0·046). LLDAS significantly reduced the risk of mortality in patients with SLE. Remission did not further reduce the risk of mortality compared with LLDAS, unless lower glucocorticoid thresholds were used. The Asia-Pacific Lupus Collaboration received funding from Janssen, Bristol Myers Squibb, Eli Lilly, and UCB for this study.

Performance of the New 2019 European Alliance of Associations for Rheumatology/American College of Rheumatology Systemic Lupus Erythematosus Classification Criteria in a Large Unicentric Cohort.

The recently published 2019 American College of Rheumatology/European Alliance of Associations for Rheumatology (ACR/EULAR) classification criteria for systemic lupus erythematosus (SLE) were developed to increase the reliability and identification of SLE, especially in early disease. With the emergence of several new drugs for SLE, identifying and treating patients early are more important than ever. Data of 446 SLE patients evaluated in our center between 1996-2019 and 226 controls with other autoimmune diseases evaluated between 2001-2022 were retrospectively analyzed. The sensitivity and specificity of the 2019 ACR/EULAR criteria were compared to the 2012 SLICC and the 1997 ACR criteria. The 2019 ACR/EULAR criteria showed very good sensitivity (86.6%) compared to the 1997 ACR criteria (76.7%), p &lt; 0.001, with a trend toward significance compared to the 2012 SLICC criteria (83.6%), p = 0.072. Their sensitivity remained high (87.6%) in patients with a short disease duration. The specificity of the 2019 ACR/EULAR criteria (91.2%) was statistically lower than the 2012 SLICC (96.0%) and 1997 ACR criteria (95.1%), p = 0.007 and p = 0.012, respectively, but still had a very high value. A total of 22 controls (9.7%) fulfilled at least one set of criteria (15 patients with MCTD, 5 with UCTD, and 2 with SSc). In this large real-life cohort, the 2019 ACR/EULAR criteria had very good performance compared to the 2012 SLICC and 1997 ACR criteria.

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

To evaluate whether a change in the European Alliance of Associations for Rheumatology (EULAR)/American College of Rheumatology (ACR) systemic lupus erythematosus (SLE) classification criteria threshold score affects accurate classification of SLE cases compared to disease-based control subjects. We evaluated a range of threshold scores to determine the score that maximizes the accurate classification of early SLE. We conducted a cross-sectional study comparing SLE cases and control patients. A EULAR/ACR criteria score was calculated using baseline information. Sensitivity, specificity, positive likelihood ratios (+LRs), and negative likelihood ratios (-LRs) with 95% CIs were used to evaluate operating characteristics. Threshold scores of 6 to 12 were evaluated in subjects with early disease (ie, disease duration of ≤ 5 years). +LRs > 10 and -LRs < 0.1 provide evidence to rule in or rule out SLE. A total of 2764 patients were included: 1980 SLE cases who fulfilled either the ACR or Systemic Lupus International Collaborating Clinics criteria and 784 control subjects. The EULAR/ACR SLE criteria had a sensitivity of 98% (95% CI 97-98), a specificity of 99% (95% CI 98-100), a +LR of 95.5 (95% CI 48.0-190), and a -LR 0.03 (95% CI 0.02-0.03). The criteria operated well in those with early disease, in women, in men, and in White, Black, Chinese, and Filipino people. A score of 10 maximized the accurate classification of patients with early disease (+LR 174.4, 95% CI 43.8-694.6; -LR 0.03, 95% CI 0.02-0.04). An increase in the threshold score from 10 to 11 resulted in significant worsening in the -LR (threshold score 10: -LR 0.03, 95% CI 0.02-0.03 vs threshold score 11: -LR 0.05, 95% CI 0.04-0.06). The EULAR/ACR SLE classification criteria threshold score of 10 performs well, particularly among those with early disease and across sexes and ethnicities.

Association of the Systemic Lupus International Collaborating Clinics Frailty Index and Damage Accrual in Longstanding Systemic Lupus Erythematosus.

To externally validate the Systemic Lupus International Collaborating Clinics Frailty Index (SLICC-FI) in a prevalent systemic lupus erythematosus (SLE) cohort and to assess the ability of the SLICC-FI to predict organ damage accrual among individuals with longstanding SLE. This was a secondary analysis of data from the Study of Lupus Vascular and Bone Long-Term Endpoints (SOLVABLE) cohort, which consists of adult women from the Chicago Lupus Database who met the 1997 revised American College of Rheumatology (ACR) classification criteria for SLE. There were 185 patients with SLE enrolled, of whom 149 patients were included in a 5-year follow-up analysis. The SLICC-FI and SLICC/ACR Damage Index (SDI) scores were calculated at baseline and 5-year follow-up. Unadjusted and adjusted logistic regression models estimated the association of baseline SLICC-FI scores (per 0.05 increase) with damage accrual at 5-year follow-up. At enrollment the mean ± SD age of the 149 patients was 43.30 ± 10.15 years, the mean ± SD disease duration was 11.93 ± 8.46 years, and the mean ± SD SDI score was 1.64 ± 1.83. At baseline, the mean ± SD SLICC-FI score was 0.18 ± 0.08, and 36% of participants were categorized as frail (SLICC-FI score >0.21). In a model adjusted for age, race, and disease duration, each 0.05-unit increase in the baseline SLICC-FI score was associated with 28% higher odds of subsequent damage accrual (odds ratio 1.28, 95% confidence interval 1.01-1.63). In a prevalent cohort of women with established SLE, higher baseline SLICC-FI scores were associated with a higher risk of subsequent damage accrual at 5-year follow-up.

81 Juvenile RHUPUS syndrome: a case reports

BackgroundRHUPUS syndrome is a rare association with rheumatoid arthritis and systemic lupus erythematosus (SLE) in adult patients. Its pediatric presentation is very rare and underdiagnosed. The mechanism is not well understood yet, but most theories accepted a real overlap between SLE and juvenile idiopathic arthritis. In children. Patients with Rhupus have clinical symptoms of SLE with positive ANA, anti-DNA or anti Sm associated with clinical symptoms of JIA.ObjectiveIllustrate an unusual presentation of lupus in childrenMethodswe present a case of juvenile rhupus syndrome; we describe the clinical presentation, the serological results, the diagnostic criteria for SLE (ACR 1997) and JIA (ILLA 2001) and the treatment installed.Case reportsWe recently diagnosed Rhupus syndrome in an 11-year-old girl who presented with polyarthritis deformans of the bilateral joints of the wrists, hands and feet for 24 months. she also had an onset of profound asthenia, recurrent oral aphtosis and massive hair loss over the past few months. Initial investigation showed anaemia, increased erythrocyte sedimentation rate (ESR) and C-reactive protein (CRP). Radiographic examinations showed juxtra-articular osteopenia and chronic synovitis of the wrists. The autoimmune assessment was contributory with a positive rheumatoid factor (RF), antinuclear antibodies (ANA) (1/1000) and positive anti-Sm antibodies. Anticardiolipin and anti-RNP antibodies were negative. Our patient met >4 ACR criteria for SLE classification. She was treated with methotrexate and hydroxychloroquine, under close medical supervision in order to watch for the appearance of other organic damage to lupus disease, in particular renal and neurological. This type of joint damage is considered to be either lupus joint damage, lupus with chronic arthritis, or overlapping lupus with JIA. Children with Rhupus initially present with JIA and later develop lupus. Previous reports have shown female predominance, polyarticular involvement, non-erosive arthritis, and years of diagnostic wandering. Our patient had polyarthritis deformans with a two-year delay in diagnosis of SLE.ConclusionAlthough rare, the infantile Rhupus syndrome must be evoked in front of a deforming arthropathy.

Health Care Utilization in Systemic Lupus Erythematosus in the Community: The Lupus Midwest Network.

The aim of this study was to determine inpatient health care utilization in an incident cohort of patients with systemic lupus erythematosus (SLE) compared with the general population. This was a population-based cohort study in the upper Midwest, United States. We included patients fulfilling the European League Against Rheumatism/American College of Rheumatology SLE classification criteria between 1995 and 2018. They were 1:1 age-, sex-, county-matched with individuals without SLE. All hospital admissions and emergency department (ED) visits were electronically retrieved for 1995-2020. Rates for hospital admission, length of stay, readmission, ED visits, and discharge destination were compared between groups. Three hundred forty-one patients with SLE and 341 comparators without SLE were included (mean age, 48.6 years at diagnosis; 79.2% female). Rates of hospitalization for patients with SLE and comparators were 29.8 and 9.9 per 100 person-years, respectively. These differences were present across sexes and age groups. Hospitalization rates were higher in patients with SLE after diagnosis and remained higher than comparators for the first 15 years of the disease. Patients with SLE were more likely than comparators to visit the ED (hazard ratio, 2.71; 95% confidence interval, 2.05-3.59). Readmission rates (32% vs. 21%, p = 0.017) were higher in patients with SLE. Length of stay and discharge destination were similar between both groups. Patients with SLE were more likely to be hospitalized and to visit the ED than individuals without SLE, highlighting important inpatient care needs. Increased hospitalization rates were observed in both male and female patients and all age groups.

Erythrocyte complement receptor 1 (ECR1) and erythrocyte-bound C4d (EC4d) in the prediction of poor pregnancy outcomes in systemic lupus erythematosus (SLE)

BackgroundComplement activation has been associated with adverse pregnancy outcomes (APO) in SLE. Pregnant women with SLE were studied to evaluate whether complement dysregulation within the first two pregnancy trimesters predicts...

Neutrophil to lymphocyte ratio and platelet to lymphocyte ratio reflect disease activity and flares in patients with systemic lupus erythematosus-A prospective study.

To determine the association between neutrophil to lymphocyte ratio (NLR) and platelet to lymphocyte ratio (PLR) with disease activity and flares in an inception cohort of patients with systemic lupus erythematosus (SLE) using a prospective study design. Consecutive adult patients (age≥21) who fulfilled the 1997 American College of Rheumatology (ACR) or the 2012 Systemic Lupus International Collaboration Clinic Classification (SLICC) Criteria for SLE were followed every 3 months, with SLE disease activity assessed by using SLEDAI-2K, and disease flares defined and captured by the SELENA-SLEDAI Flare Index (SFI). NLR and PLR were computed from the automated machine-counted blood count differentials. Linear mixed model and generalized estimating equation model were constructed to analyze the associations between NLR/PLR and SLEDAI-2K and disease flares, with multivariate adjustments. Of 290 patients recruited, the median (IQR) duration of follow-up and baseline SLEDAI-2K were 4.7 (3.2-6.1) years and 2 (0.5-3.5), respectively. On multivariable analyses, NLR was shown to be positively and significantly associated with SLEDAI-2K (estimate of coefficient (β)=0.05, P<0.01) and severe disease flares (odds ratio [OR] 1.05, P<0.05), but not with overall disease flares [OR 1.02, non-significant]. While PLR was shown to be positively associated with SLEDAI-2K [β=0.09, P<0.05], no statistically significant association between PLR and overall or severe disease flares was found [OR 1.00 and OR 1.06 respectively, non-significant]. Derived readily from automated blood count differentials, the NLR potentially serves as a surrogate prospective marker of disease activity and severe disease flares in SLE patients.

Skin lesions in systemic lupus erythematosus. Part 1: classification, etiology, pathogenesis

Skin and mucous membranes lesions in systemic lupus erythematosus (SLE) significantly impair the quality of life of patients, although they are not a formidable manifestation of the disease. Skin manifestations of SLE can occur both at the onset and on the late stage of the disease. Although skin and mucous membranes lesions are clearly grouped in the latest classification criteria for SLE, verification of the diagnosis requires a multidisciplinary approach. In the etiology of SLE, environmental factors, hormonal factors, and genetic predisposition play a role. Further research will reveal differences in subtypes of cutaneous lupus erythematosus and will facilitate the development of new therapies.

Pre-Clinical Autoimmunity in Lupus Relatives: Self-Reported Questionnaires and Immune Dysregulation Distinguish Relatives Who Develop Incomplete or Classified Lupus From Clinically Unaffected Relatives and Unaffected, Unrelated Individuals.

Systemic lupus erythematosus (SLE) is propelled by pathogenic autoantibody (AutoAb) and immune pathway dysregulation. Identifying populations at risk of reaching classified SLE is essential to curtail inflammatory damage. Lupus blood relatives (Rel) have an increased risk of developing SLE. We tested factors to identify Rel at risk of developing incomplete lupus (ILE) or classified SLE vs. clinically unaffected Rel and healthy controls (HC), drawing from two unique, well characterized lupus cohorts, the lupus autoimmunity in relatives (LAUREL) follow-up cohort, consisting of Rel meeting <4 ACR criteria at baseline, and the Lupus Family Registry and Repository (LFRR), made up of SLE patients, lupus Rel, and HC. Medical record review determined ACR SLE classification criteria; study participants completed the SLE portion of the connective tissue disease questionnaire (SLE-CSQ), type 2 symptom questions, and provided samples for assessment of serum SLE-associated AutoAb specificities and 52 plasma immune mediators. Elevated SLE-CSQ scores were associated with type 2 symptoms, ACR scores, and serology in both cohorts. Fatigue at BL was associated with transition to classified SLE in the LAUREL cohort (p≤0.01). Increased levels of BLyS and decreased levels of IL-10 were associated with type 2 symptoms (p<0.05). SLE-CSQ scores, ACR scores, and accumulated AutoAb specificities correlated with levels of multiple inflammatory immune mediators (p<0.05), including BLyS, IL-2Rα, stem cell factor (SCF), soluble TNF receptors, and Th-1 type mediators and chemokines. Transition to SLE was associated with increased levels of SCF (p<0.05). ILE Rel also had increased levels of TNF-α and IFN-γ, offset by increased levels of regulatory IL-10 and TGF-β (p<0.05). Clinically unaffected Rel (vs. HC) had higher SLE-CSQ scores (p<0.001), increased serology (p<0.05), and increased inflammatory mediator levels, offset by increased IL-10 and TGF-β (p<0.01). These findings suggest that Rel at highest risk of transitioning to classified SLE have increased inflammation coupled with decreased regulatory mediators. In contrast, clinically unaffected Rel and Rel with ILE demonstrate increased inflammation offset with increased immune regulation, intimating a window of opportunity for early intervention and enrollment in prevention trials.