Search

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.

OBJECTIVE. Physical activity is known to improve depressive symptoms. The present study was undertaken to examine physical inactivity as a predictor of incident depression in systemic lupus erythematosus (SLE). METHODS. Data derive from the California Lupus Epidemiology Study (CLUES), a longitudinal cohort with confirmed SLE diagnoses. Physical inactivity was assessed from a single item, “I rarely or never do any physical activities,” and depressive symptoms by the 8-item Patient Health Questionnaire (PHQ-8). Analysis included those not depressed at baseline (PHQ-8 score 3-fold increased risk of incident depression among the sedentary group (HR 3.88 [95% Cl 1.67–9.03]). CONCLUSION. In this diverse SLE cohort, a simple question about physical inactivity was highly predictive of incident depression over the subsequent 2 years. Results suggest an urgent need for approaches to reduce sedentary behavior in this high-risk population.

Background: Urinary biomarkers may help in identification and assessment of the activity of lupus nephritis (LN).Urinary osteoprotegerin may reflect renal disease activity better. Objective: To investigate urinary osteoprotegerin level as a potential marker of lupus nephritis activity. Patients and Methods: The study included 90 individuals classified into three equal groups: Group (I): clinically and laboratory free, Group (II): patients without renal involvement (non active renal group) and Group (III): patients with active lupus nephritis. Measurement of the level of urinary osteoprotegerin has been done by ELISA from January 2018 till June 2019. Results: All patients with systemic lupus erythromatosus SLE in group II and III had significant higher levels in urinary Osteoprotegerin compared to the control group (I). Moreover, SLE patients with lupus nephritis activity (Group III) had marked increase in urinary osteoprotegerin more than SLE patients without renal involvement (Group II). The study showed also that, with advance of renal histopathological class in lupus nephritis patients, the urine OPG level showed more increase. Conclusion: Urinary level of osteoprotegerin may be used as a biomarker for early prediction of lupus nephritis. Also, it may be used for monitoring the progression and follow up of lupus nephritis as well as with shifting from one class to another.

SLE disease activity score (SLE-DAS) is a novel, rapid, continuous and comprehensive score that overcomes the drawbacks of SLEDAI-2 K. Low lupus disease activity state (LLDAS) has been targeted as an endpoint in many clinical trials and as a favourable outcome in clinical practice. Therefore, our objective in the current study is to evaluate the validity of SLE-DAS for defining LLDAS as an objective in the treat-to-target strategy. A cross-sectional study was carried out on 117 SLE patients who were diagnosed according to Systemic Lupus International Collaborating Clinics (SLICC) classification criteria for SLE. Patients were evaluated for disease activity by both SLEDAI-2 K and SLE-DAS. Additionally, patients were divided according to the SLEDAI-2 K-derived LLDAS definition into two groups: low disease activity (LDA) group and high disease activity (HDA) group. The validity of SLE-DAS for the definition of LLDAS was evaluated in comparison to SLEDAI-2 K. SLE-DAS shows highly significant positive correlation (r = 0.743, p

ObjectiveThe Systemic Lupus International Collaborating Clinics (SLICC) frailty index (FI) predicts mortality and damage accrual in systemic lupus erythematosus (SLE), but its association with hospitalizations has not been described. Our objective was to estimate the association of baseline SLICC-FI values with future hospitalizations in the SLICC inception cohort.MethodsBaseline SLICC-FI scores were calculated. The number and duration of inpatient hospitalizations during follow-up were recorded. Negative binomial regression was used to estimate the association between baseline SLICC-FI values and the rate of hospitalizations per patient-year of follow-up. Linear regression was used to estimate the association of baseline SLICC-FI scores with the proportion of follow-up time spent in the hospital. Multivariable models were adjusted for relevant baseline characteristics.ResultsThe 1,549 patients with SLE eligible for this analysis were mostly female (88.7%), with a mean ± SD age of 35.7 ± 13.3 years and a median disease duration of 1.2 years (interquartile range 0.9-1.5) at baseline. Mean ± SD baseline SLICC-FI was 0.17 ± 0.08. During mean ± SD follow-up of 7.2 ± 3.7 years, 614 patients (39.6%) experienced 1,570 hospitalizations. Higher baseline SLICC-FI values (per 0.05 increment) were associated with more frequent hospitalizations during follow-up, with an incidence rate ratio of 1.21 (95% confidence interval [95% CI] 1.13-1.30) after adjustment for baseline age, sex, glucocorticoid use, immunosuppressive use, ethnicity/location, SLE Disease Activity Index 2000 score, SLICC/American College of Rheumatology Damage Index score, and disease duration. Among patients with ≥1 hospitalization, higher baseline SLICC-FI values predicted a greater proportion of follow-up time spent hospitalized (relative rate 1.09 [95% CI 1.02-1.16]).ConclusionThe SLICC-FI predicts future hospitalizations among incident SLE patients, further supporting the SLICC-FI as a valid health measure in SLE.

Secondary forms of immune thrombocytopenia (ITP) represent approximately 20% of all ITP cases in adulthood and this rate increases with age. Since some causes may influence both the prognosis and outcome but also the management of ITP, a minimal workup must be performed at ITP diagnosis to look for an associated or underlying cause. Among adults, B-cell lymphomas and mainly chronic lymphocytic leukemia, systemic auto-immune diseases such as systemic lupus or primary immunodeficiencies mainly represented by common variable immunodeficiency are the most frequent causes of secondary ITP. Whereas first-line therapy used for secondary ITP is usually similar to the one commonly used in primary ITP and relies mostly on corticosteroids±intravenous immunoglobulin according to the severity of bleeding, second and third-line treatments must take into account the type and degree of activity of the underlying disease.

Objective.Systemic lupus erythematosus (SLE) is a chronic autoimmune disease. The American College of Rheumatology (ACR) 1997, Systemic Lupus International Collaborating Clinics (SLICC) 2012, and European League Against Rheumatism (EULAR)/ACR 2019 SLE classification criteria are formed based on data mainly from adult patients. We aimed to test the performances of the SLE classification criteria among pediatric patients with SLE.Methods.Pediatric patients with SLE (n = 262; 80.9% female) were included from 3 different centers in Turkey. As controls, 174 children (60.9% female) with other diseases who had ANA (antinuclear antibody) test results were included. The gold standard for SLE diagnosis was expert opinion.Results.The sensitivities of the ACR 1997, SLICC 2012, and EULAR/ACR 2019 criteria were 68.7%, 95.4%, and 91.6%, respectively. The specificities of the ACR 1997, SLICC 2012, and EULAR/ACR 2019 criteria were 94.8%, 89.7%, and 88.5%, respectively. Eighteen patients with SLE met the SLICC 2012 but not the EULAR/ACR 2019 criteria. Among these, hematologic involvement was prominent (n = 13; 72.2%). Eight patients with SLE fulfilled the EULAR/ACR 2019 but not the SLICC 2012 criteria. Among these, joint involvement was prominent (n = 6; 75%).Conclusion.To our knowledge, this is the largest cohort study of pediatric SLE to test the performances of all 3 classification criteria. The SLICC 2012 criteria yielded the best sensitivity, whereas the ACR 1997 criteria had the best specificity. SLICC 2012 criteria performed better than EULAR/ACR 2019 criteria. Separation of different hematological manifestations in the SLICC 2012 criteria might have contributed to the higher performance of this criteria set.

To explore the association between the creatinine clearance rate (Ccr) and the prognosis of patients, and compared with estimated glomerular filtration rate (eGFR). We retrospectively collected information of patients with SLE who were first hospitalized between 1999 and 2009 in Jiangsu Province, China, and followed up in 2010 and 2015. Ccr was calculated and dichotomized into normal group (Ccr ≥ 70) and decreasing group (Ccr < 70). The clinical characteristics of the two groups were compared and Cox proportional-hazards regression models were used to calculate hazard ratio (HR) and 95% confidence interval (CI). Among 1990 SLE patients, we observed 437 (22.0%) with decreased Ccr, including 237 cases (11.9%) with mild renal dysfunction, 136 cases (6.8%) with moderate renal dysfunction, and 64 cases (3.2%) with severe renal dysfunction. Compared to normal Ccr, decreasing Ccr had a higher risk for mortality with adjusted HR (95% CI) of 2.21 (1.59–3.06). Dose-response relationships were significantly found between increased mortality of SLE and decreased Ccr (p for trend < 0. 001), as well as eGFR. Positive associations were consistently observed in subgroups, such as systemic lupus disease activity index (SLEDAI) ≥ 15, without comorbidities and abnormal laboratory indexes. Decreasing Ccr was positively associated with mortality from infection and renal failure with HR (95% CI) of 1.80 (1.02–3.19) and 6.84 (3.05–15.36). A significant association has been observed between decreased Ccr and increased risk for mortality of SLE patients. Early clinical interventions to modulate the Ccr of SLE patients may be beneficial to their survival.

OBJECTIVE Different classification criteria for systemic lupus erythematosus (SLE) have been proposed for many years. The most widely used and accepted criteria has been the 1997 American College of Rheumatology (ACR) criteria. In 2012, the Systemic Lupus International Collaborating Clinics (SLICC) criteria were published in an attempt to improve the clinical relevance of SLE criteria. In 2017, weighted criteria were proposed that included entry criteria, something the 1997 ACR and the 2012 SLICC criteria did not identify. The aim of the present study was to validate the 2017 weighted criteria, the 1997 ACR criteria, and the 2012 SLICC criteria and compare the sensitivities and specificities in pediatric SLE. METHODS For the past 15 years, retrospective chart review of patients diagnosed with SLE before age 19 years was conducted. The controls were patients referred for serologies positive for antinuclear antibodies but did not fulfill criteria for diagnosis of SLE at the initial visit or were diagnosed with another autoimmune disease. The 3 classification criteria sets were applied to these patients and compared against a gold standard of physician diagnosis. RESULTS A total of 156 patients were diagnosed with SLE. The sensitivity for the 2017 weighted criteria was 0.974 (95% confidence interval [95% CI] 0.936-0.993) and the specificity was 0.984 (95% CI 0.966-0.994). The sensitivity for the 1997 ACR criteria was 0.872 (95% CI 0.809-0.920) and the specificity was 1.00 (95% CI 0.990-1.000). The sensitivity for the 2012 SLICC criteria was 0.974 (95% CI 0.936-0.993) and the specificity was 0.997 (95% CI 0.985-1.000). CONCLUSION The 2017 weighted criteria and the 2012 SLICC criteria were more sensitive than the 1997 ACR criteria. There were no significant differences in sensitivity and specificity between the 2012 SLICC and the 2017 weighted criteria.

Autoimmune systemic diseases (ASD) may show impaired immunogenicity to COVID-19 vaccines. Our prospective observational multicenter study aimed to evaluate the seroconversion after the vaccination cycle and at 6-12-month follow-up, as well the safety and efficacy of vaccines in preventing COVID-19. The study included 478 unselected ASD patients (mean age 59 ± 15 years), namely 101 rheumatoid arthritis (RA), 38 systemic lupus erythematosus (SLE), 265 systemic sclerosis (SSc), 61 cryoglobulinemic vasculitis (CV), and a miscellanea of 13 systemic vasculitis. The control group included 502 individuals from the general population (mean age 59 ± 14SD years). The immunogenicity of mRNA COVID-19 vaccines (BNT162b2 and mRNA-1273) was evaluated by measuring serum IgG-neutralizing antibody (NAb) (SARS-CoV-2 IgG II Quant antibody test kit; Abbott Laboratories, Chicago, IL) on samples obtained within 3 weeks after vaccination cycle. The short-term results of our prospective study revealed significantly lower NAb levels in ASD series compared to controls [286 (53–1203) vs 825 (451–1542) BAU/mL, p < 0.0001], as well as between single ASD subgroups and controls. More interestingly, higher percentage of non-responders to vaccine was recorded in ASD patients compared to controls [13.2% (63/478), vs 2.8% (14/502); p < 0.0001]. Increased prevalence of non-response to vaccine was also observed in different ASD subgroups, in patients with ASD-related interstitial lung disease (p = 0.009), and in those treated with glucocorticoids (p = 0.002), mycophenolate-mofetil (p < 0.0001), or rituximab (p < 0.0001). Comparable percentages of vaccine-related adverse effects were recorded among responder and non-responder ASD patients. Patients with weak/absent seroconversion, believed to be immune to SARS-CoV-2 infection, are at high risk to develop COVID-19. Early determination of serum NAb after vaccination cycle may allow to identify three main groups of ASD patients: responders, subjects with suboptimal response, non-responders. Patients with suboptimal response should be prioritized for a booster-dose of vaccine, while a different type of vaccine could be administered to non-responder individuals.

Antecedentes. Los pacientes con lupus eritematoso sistémico (LES) tienen alto riesgo de compromiso cardíaco con elevada mortalidad, por lo que el diagnóstico subclínico podría mejorar su sobrevida. Se ha observado que el strain (deformación) miocárdico longitudinal (SGL) puede ser de utilidad para la evaluación de la función cardíaca en estos pacientes. Objetivos. Evaluar la función miocárdica mediante el análisis del strain longitudinal bidimensional (2D), comparar el strain longitudinal entre pacientes con LES y controles, y determinar la correlación con el índice de actividad del LES. Material y métodos. Se estudiaron a 44 pacientes con LES (50 ±13 años) y 50 controles (49 ±12 años) pareados por edad y sexo, a quienes se les realizó un ecocardiograma transtorácico bidimensional. Se valoró el strain longitudinal mediante el método de speckle tracking, y la actividad del LES se estimó mediante el Systemic Lupus Erythematous Disease Activity Index (SLEDAI). Se definió como LES activo con una puntuación ≥ 4. Resultados. El strain longitudinal global2D fue menor en pacientes con LES que los controles (– 17,3% ± 1,9% vs. – 20%, ± 1,9%; p=0,00). No hubo diferencias en la fracción de eyección de ventrículo izquierdo entre ambos grupos, ni en 2D (p=0,650), ni en 3D (p=0,718). En los pacientes lúpicos, el SLEDAI varió entre 0 y 10, y el 63,8% estuvieron inactivos. Se halló correlaciones negativas significativas entre el puntaje de SLEDAI y la FEVI bidimensional (r de Pearson=-0,372, p=0,017); no se encontró correlación entre el puntaje de SLEDAI y el strain longitudinal global 2D (rho de Spearman=- 0,091 p =0,582). Conclusiones. El strain longitudinal global 2D se encontró disminuida en aquellos afectados con LES, lo que sugiere que podría ser una herramienta útil para evaluar la función cardíaca en estos pacientes.

Objectives Thrombotic thrombocytopenia purpura (TTP) associated with systemic lupus erythematous (SLE) (i.e., SLE-TTP) is a rare life-threatening disease often requiring intensive immunosuppressive agents, in addition to high-dose corticosteroids and plasma exchange (PEX). The optimal therapy of rituximab is unclear, but 375 mg/m2 weekly for 4 weeks is the usual practice, adopted from regimens for non-Hodgkin’s lymphoma. We reported two cases of refractory SLE-TTP that showed good efficacy and prognosis with combination of methylprednisolone (MP) pulse, plasma exchange and low-dose rituximab (100 mg weekly for 4 weeks) treatment. Methods Clinical data and treatment outcomes were reviewed of two patients diagnosed with refractory SLE-TTP at Peking Union Medical College Hospital between July 2017 and July 2018. Results Both patients had SLE and presented with microangiopathic anemia and thrombocytopenia. Laboratory assays revealed high anti-nuclear antibody titers, reduced complement 3 and 4 levels, proteinuria, significantly elevated lactate dehydrogenase, schistocytes on peripheral blood smear, low ADAMTS13 activity, and the presence of ADAMTS13 inhibitor. In both patients, platelet counts remained below 50 × 109/L after MP pulse and 6 PEXs, confirming the diagnosis of refractory SLE-TTP. Low-dose rituximab (100 mg weekly for 4 weeks) was administered in both cases, resulting in normalization of platelet counts and significant reductions in B-lymphocyte counts. No TTP relapse or SLE flare occurred during 24 months of follow-up. Conclusions Our cases confirmed the efficacy and good follow-up outcomes of low-dose rituximab treatment (100 mg weekly for 4 weeks) for refractory SLE-TTP.

Aim of the work: This study was undertaken to evaluate cataract risk factors in Egyptian systemic lupus erythematosus (SLE) patients. Patients and methods: A cohort of 150 consecutive adult SLE patients was included. None of the patients was diabetic or had cataract before steroids treatment for SLE. Detailed medical and ophthalmological histories were taken. Thorough clinical examination was performed with assessment of SLE disease activity index (SLEDAI) and systemic lupus international collaborating clinics damage index (SLICC DI), along with routine blood tests. Slit lamp examination was done to assess the anterior segment of the eye especially the lens to detect the type and degree of cataract. Results: 150 SLE patients were studied with a mean age of 37.16 ± 11 years; 129 females and 21 males and a mean disease duration of 11 ± 5.7 years. Their mean SLEDAI was 4.1 ± 3.78 and SLICC/DI 2.1 ± 1.3. The frequency of cataract was 18% (n = 27); 22.2% of them were cortical and 77.8% were posterior subcapsular with mild-moderate degree. Glaucoma was present in only 2%. A significant increase in age, disease duration, age at onset, SLICC/DI, cumulative steroid dose and hydroxychloroquine (HCQ) dose was found in those with cataract compared to non-cataract patients (p

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 18 years 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

Objective This study aimed to investigate how septicaemia, non-septicaemia infection and the disease itself are associated with disease activity and mortality in inpatients with systemic lupus erythematosus (SLE) in Taiwan. Methods We retrospectively reviewed 1115 patients and enrolled 427 with SLE admitted for lupus flare-ups and co-morbidities. Disease activity and infection type/site were recorded and categorized according to the causes of admission and mortality into three categories, of which two were specified as follows: (a) septicaemia admissions, non-septicaemia admissions; and (b) septicaemia mortality, non-septicaemia infection mortality and non-infection mortality. The relationships between lupus flare-ups and mortality in different groups were analysed using an unpaired t-test, Mann–Whitney U-test and logistic regression. Results Septicaemia was the major cause of mortality in SLE inpatients. There were 98 (22.95%) mortality patients among all 427 SLE patients. The septicaemia admissions had higher disease activity (SLE Disease Activity Index 2000 = 13.00 ± 7.98) than the non-septicaemia admissions (9.77 ± 5.72; p Conclusion Septicaemia relates to increased lupus disease activity and is associated with a greater risk of mortality in the SLE patients than other causes of admission. Fewer previous septicaemia admissions decrease the risk of septicaemia mortality.

Objectives This study aims to evaluate the frequency and clinical and laboratory features of interstitial lung disease (ILD) in Chinese patients with systemic lupus erythematosus (SLE) and to evaluate the association of ILD with the clinical features. Patients and methods The study included 505 SLE patients (64 males, 441 females; mean age 35.3±15.3 years; range, 14 to 87 years) who were categorized into two groups as 449 patients without ILD and 56 patients with ILD based on evidence obtained from high-resolution computed tomography images. The demographic data, clinical and laboratory findings, SLE disease activity index score, and Systemic Lupus International Collaborating Clinics/American College of Rheumatology Damage Index of all patients were also recorded and statistically analyzed. Results The ILD frequency in patients with SLE was 11.1%. Compared to the group of SLE patients without ILD, the group of SLE patients with ILD possessed the following statistical differences: elderly age, longer illness duration, lower level of anti-double-stranded deoxyribonucleic acid, and higher level of serum complement 3, increased ratios of Raynaud's phenomenon, moist rales and tachypnea. Multivariate logistic regression results suggested that elderly age (≥60 years), long illness duration (1-10 years, ≥10 years), Raynaud's phenomenon, and tachypnea were statistically associated with the occurrence of ILD in SLE patients. Conclusion Chinese SLE patients who possessed the factors that were statistically associated with ILD, namely, elderly age (≥60 years old), long illness duration (≥1 years), Raynaud's phenomenon, and tachypnea, were recommended to be monitored for the possibility of ILD.

Aim of the work: To investigate the relation between lymphopenia and clinical manifestations, laboratory findings, disease activity and damage index in systemic lupus erythematosus (SLE) patients. Patients and methods: 124 SLE patients were recruited from the Rheumatology Department in-patient and outpatient clinic, Faculty of medicine, Cairo University Hospitals. SLE disease activity index (SLEDAI) and the systemic lupus international collaborating clinics damage index (SLICC-DI) were assessed. Patients were divided into two groups according to presence or absence of lymphopenia (lymphocyte count ≤1500 cells/mm3), and were compared as regards different disease parameters. Results: The 124 patients were 113 females (91.1%) and 11 males (8.9%). Their mean age was 28.54 ± 8.25 years and median disease duration was 48 (24–105) months. 86 (69.4%) had renal involvement, complement C3 was consumed in 84 (67.7%) and C4 in 44 (35.5%). All were receiving steroids and a high dose was more frequent in those with lymphopenia (17 (29.8%) compared to those without (in 9 (13.4%). Lymphopenia was found in 57 (46%) patients. Lymphopenic patients had higher frequency of renal involvement (p = 0.002) (OR = 3.37, CI = 1.5–7.8), complement consumption (p = 0.03), 24 h urinary proteins (p = 0.034), higher steroid doses (p = 0.012) and cyclophosphamide administration (p = 0.03). SLEDAI was severe in 29.8% of lymphopenic patients (p = 0.16), and SLICC-DI median was 2 in both groups (p = 0.4). Conclusion: Lymphopenia is a common finding in SLE patients and was significantly associated with lupus nephritis, complement consumption, higher steroid doses and cyclophosphamide administration. Lymphopenia might be a promising marker for renal involvement in SLE. Keywords: Lymphopenia, Systemic lupus erythematosus, Lupus nephritis, Proteinuria, C3

Supported by both the European League Against Rheumatism (EULAR) and the American College of Rheumatology (ACR) from the beginning, a large transatlantic, and in many phases worldwide, project has led to new classification criteria for systemic lupus erythematosus (SLE). The project had ambitious goals, particularly maintaining the high specificity of the ACR criteria, while reaching a sensitivity close to the Systemic Lupus International Collaborating Clinics (SLICC) 2012 criteria. The validation data suggest that the new criteria reached this goal, with a specificity of 93% (as for the ACR criteria) and a sensitivity of 96% (vs 97% for the SLICC criteria). The new EULAR/ACR classification criteria use positive antinuclear antibodies (ANA) as an entry criterion and have weighted items, ranging from 2 (for delirium, non-infectious fever and anti-phospholipid antibodies) to 10 (for class III or IV lupus nephritis). The cut-off for classification is 10, reached by class III/IV nephritis alone. Items are organized in domains, within which only the highest item is to be counted. Instead of many defined exclusions, one rule is used for all, namely that items are to be attributed to SLE and counted only if there is no more likely alternative diagnosis. These criteria are now expected to be externally validated in various other cohorts. The team spirit and collegiality characteristic for this EULAR/ACR criteria effort will hopefully facilitate future international projects on SLE.

Background: Migraine is common in systemic lupus erythematosus.It is a significant source of patient disability. Objective: To determine the rate of migraine in patients with systemic lupus erythematosus, to assess migraine type, severity, and the association between migraine and patient’s characteristics. Type of the study: Cross-sectional study. Methods: 100 subjected were recruited and divided into two groups; fifty patients with the diagnosis of systemic lupus erythematosus were recruited from the Rheumatologic department of medicine,and another 50 normal subjects, then complete medical and drugs history were taken from them. Results: Fifty patients completed the questionnaire. Thirty percent of systemic lupus erythematosus patients and 12% of normal subjects had migraine. Of the patients with migraine 80%, 13.3% and 6.7% met criteria for migraine without aura, migraine with aura and retinal migraine respectively. The moderately severe migraine was commonly observed (53.3%). There were significant associations between migraine and systemic lupus erythematosus patients who have Raynaud’s phenomenon, and cardiolipin antibodies.There were no statistically significant associations between migraine, systemic lupus erythematosus duration and patient’s age,sex, and anti-dsDNA. Conclusions:A high rate of migraine in patients with systemic lupus erythematosus.Migraine associated with Raynaud’s phenomenon, and cardiolipin antibodies.

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.

Aim of the work: To determine the risk factors of bilateral and severe (advanced) osteonecrosis femoral head (ONFH) as assessed by magnetic resonance imaging (MRI) staging in Egyptian SLE patients. Patients and methods: Sixty SLE patients were studied and subdivided into two groups according to the presence or absence of symptomatic osteonecrosis. Full medical history, musculoskeletal examination, laboratory and serologic tests were done to all patients. SLE disease activity index (SLEDAI), systemic lupus international collaboration clinics damage index (SLICC/DI), health assessment questionairre (HAQ) disability index and the visual analogue scale (VAS-pain) were assessed. 30 patients with symptomatic ONFH were identified and staged by MRI according to Ficat and Arlet classification. Dual energy x-ray absorptiometry (DEXA) scan was performed. Results: The mean age at-onset of patients with ONFH was significantly lower than those without (20.7 ± 5.9 vs 30.4 ± 7.9 years; p

Objectives This study aimed to test the performance of the new ACR and EULAR criteria, that include ANA positivity as entry criterion, in JSLE. Methods Performance of the ACR/EULAR-2019 criteria were compared with Systemic Lupus International Collaborating Clinics (SLICC-2012), using data from children and young people (CYP) in the UK JSLE Cohort Study (n = 482), with the ACR-1997 criteria used as reference standard. An unselected cohort of CYP positive for ANA (n = 129) was used to calculate positive/negative predictive values of the criteria. Results At both first and last visits, the number of patients fulfilling the different classification criteria varied significantly (P < 0.001). The sensitivity of the SLICC-2012 criteria was higher when compared with that of the ACR/EULAR-2019 criteria at first and last visits (98% vs 94% for first visit, and 98% vs 96% for last visit; P < 0.001), when all available CYP were considered. The ACR/EULAR-2019 criteria were more specific when compared with the SLICC-2012 criteria (77% vs 67% for first visit, and 81% vs 71% for last visit; P < 0.001). Significant differences between the classification criteria were mainly caused by the variation in ANA positivity across ages. In the unselected cohort of ANA-positive CYP, the ACR/EULAR-2019 criteria produced the highest false-positive classification (6/129, 5%). Conclusion In CYP, the ACR/EULAR-2019 criteria are not superior to those of the SLICC-2012 or ACR-1997 criteria. If classification criteria are designed to include CYP and adult populations, paediatric rheumatologists should be included in the consensus and evaluation process, as seemingly minor changes can significantly affect outcomes.

As treatment options in advanced systematic lupus erythematosus (SLE) are limited, there is an urgent need for new and effective therapeutic alternatives for selected cases with severe disease. Bortezomib (BTZ) is a specific, reversible, inhibitor of the 20S subunit of the proteasome. Herein, we report clinical experience regarding efficacy and safety from all patients receiving BTZ as therapy for SLE in Sweden during the years 2014-2020. 8 females and 4 males were included with a mean disease duration at BTZ initiation of 8.8 years (range 0.7-20 years). Renal involvement was the main target for BTZ. Reduction of global disease activity was recorded by decreasing SLEDAI-2K scores over time and remained significantly reduced at the 6-month (p=0.007) and the 12-month (p=0.008) follow-up visits. From BTZ initiation, complement protein 3 (C3) levels increased significantly after the 2(nd) treatment cycle (p=0.05), the 6-month (p=0.03) and the 12-month (p=0.04) follow-up visits. The urine albumin/creatinine ratio declined over time and reached significance at the 6-month (p=0.008) and the 12-month follow-up visits (p=0.004). Seroconversion of anti-dsDNA (27%), anti-C1q (50%) and anti-Sm (67%) was observed. 6 of 12 patients experienced at least one side-effect during follow-up, whereof the most common adverse events were infections. Safety parameters (C-reactive protein, blood cell counts) mainly remained stable over time. To conclude, we report favorable therapeutic effects of BTZ used in combination with corticosteroids in a majority of patients with severe SLE manifestations irresponsive to conventional immunosuppressive agents. Reduction of proteinuria was observed over time as well as seroconversion of some autoantibody specificities. In most patients, tolerance was acceptable but mild adverse events was not uncommon. Special attention should be paid to infections and hypogammaglobinemia. Funding Agencies|Swedish Rheumatism Association; Region Ostergotland; Gustafsson Foundation; King Gustaf Vs 80-year Anniversary foundation; King Gustaf V and Queen Victorias Freemasons foundation

Objective: The Systemic Lupus International Collaborating Clinics (SLICC) 2012 systemic lupus erythematosus (SLE) classification criteria and the revised American College of Rheumatology (ACR) 1997 criteria are list based, counting each SLE manifestation equally. We derived a classification rule based on giving variable weights to the SLICC criteria and compared its performance to the revised ACR 1997, the unweighted SLICC 2012, and the newly reported European Alliance of Associations for Rheumatology (EULAR)/ACR 2019 criteria sets. Methods: The physician-rated patient scenarios used to develop the SLICC 2012 classification criteria were reemployed to devise a new weighted classification rule using multiple linear regression. The performance of the rule was evaluated on an independent set of expert-diagnosed patient scenarios and compared to the performance of the previously reported classification rules. Results: The weighted SLICC criteria and the EULAR/ACR 2019 criteria had less sensitivity but better specificity compared to the list-based revised ACR 1997 and SLICC 2012 classification criteria. There were no statistically significant differences between any pair of rules with respect to overall agreement with the physician diagnosis. Conclusion: The 2 new weighted classification rules did not perform better than the existing list-based rules in terms of overall agreement on a data set originally generated to assess the SLICC criteria. Given the added complexity of summing weights, researchers may prefer the unweighted SLICC criteria. However, the performance of a classification rule will always depend on the populations from which the cases and non-cases are derived and whether the goal is to prioritize sensitivity or specificity.

Introduction Systemic lupus erythematosus (SLE) is a systemic disease with clinically heterogeneous outcomes. Lupus nephritis (LN) is a common complication of SLE. LN impacts clinical SLE outcomes both directly, in the form of target organ damage, and indirectly, through the adverse effects of immunosuppressive therapy. Patients & methods The study included 402 SLE cases with biopsy-proven lupus nephritis who were under follow-up for the past 13 years at Mansoura Urology and Nephrology Center, Egypt. We studied the differences in outcome among various LN classes and between 275 proliferative cases and 102 non-proliferative cases. Results Class IV was the main LN class in our series with renal survival of 60% at 10 years. The major induction regimen after the first biopsy was cyclophosphamide. Mycophenolate mofetil was the main induction and adjunctive regimen after the second biopsy. The mean follow-up period was 6.7 + 5.2 years. Higher serum creatinine, proteinuria, activity, and chronicity indices were noted in proliferative LN. Patients suffering from proliferative lesions received higher immunosuppression and demonstrated higher morbidity than those with non-proliferative lesions. Remission was higher among the non-proliferative compared to the proliferative group. Conclusions Serum creatinine, proteinuria, and LN class were the most relevant prognostic factors for renal survival among Egyptian LN patients.

Background: Systemic Lupus Erythematosus (SLE) is an autoimmune disease with unknown aetiology. SLE attacks multiple organs with diverse clinical manifestations. Most patients get immunosuppressant therapy that suppresses immune system, causing the body to be susceptible to infection. Objective: to describe clinical manifestations, laboratory abnormalities, and incidence of infections in SLE patients hospitalized at Dr. Soetomo General Academic Hospital, Surabaya, Indonesia in 2016. Materials and Methods: Cross-sectional descriptive observational study used medical records of 273 SLE patients hospitalized at Dr. Soetomo General Academic Hospital, Surabaya, Indonesia in 2016. Results: Clinical manifestations found in this study were malar rash 7.33%, discoid rash 2.93%, oral ulcer 8.42%, allopecia 16.48%, arthritis 26.74%, serositis 13.19%, kidney 35.9%, neurology 24.91%, anemia 73.71%, leucopenia 32.67%, lymphopenia 76.89%, and thrombocytopenia 33.86%. Laboratory abnormalities found in this study were hematology (anemia 73.71%, leucopenia 32.67%, lymphopenia 76.89%, thrombocytopenia 33.86%), kidney function (high serum creatinine levels 39.66%, high BUN levels 41.2%, hypoalbuminemia 62.6%), urine (proteinuria 68.21%, hematuria 51.79%) and liver function (high ALT levels 36.65%, high AST levels 29.86%). Infection occurred in 33.7% patients. The most common infections were pneumonia (70.65%), urinary tract infections (51.09%), and sepsis (35.87%). Conclusion: The most common clinical manifestations experienced by SLE patients are hematological disorder, kidney disorder, and arthritis. Prominent laboratory abnormalities are anemia, lymphopenia, and proteinuria. Infection is a common complication, with the most common types pneumonia, urinary tract infection, and sepsis.

Context The management of systemic auto-immune diseases (SAID) -associated thrombotic microangiopathies (TMA) [SAID-TMA] remains debated. Objectives To provide a demographic, clinical and therapeutic picture of SAID-TMA. Methods A cross-sectional analysis was conducted on adult patients presenting with SAID and TMA from the French National TMA Registry over a 20-year period. Clinical features were extracted and compared to those from a historical cohort of atypical haemolytic and uremic syndrome (aHUS) patients. Results Forty-one patients with SAID-TMA were compared to 78 patients with aHUS from a historical cohort. Connective tissue diseases (CTD) were systemic lupus erythematosus (n=18), primary Sjogren's syndrome (n=7), systemic sclerosis (n=11), mixed CTD (n=2) and 2 cases of vasculitides, including 7 overlapping forms and 8 cases of primary antiphospholipid syndromes (APLS). Patients with SAID-TMA generally had pre-existing chronic kidney failure (OR= 3.17, 95%CI: 1.204 to 7.923; p= 0.016) compared to aHUS patients, though creatinine levels were significantly lower (216 [IQR, 108-334] µmol/L vs. 368 [IQR, 170-722] µmol/L; p= 0.002). Patients were less likely to recover if renal replacement therapy was needed at onset (OR= 0.07; 0.02 to 0.34; p 0.05). Conclusion The management of SAID-TMA implies an early initiation of immunosuppressive drugs for flares of the associated SAID, whereas TPE seem ineffective. Key messages • Systemic auto-immune diseases (SAID) in thrombotic microangiopathies (TMA) [SAID-TMA] include mainly systemic lupus erythematosus, systemic sclerosis and primary Sjogren's syndrome. • SAID-TMA improves with the treatment of the underlying SAID • Therapeutic plasma exchange does not seem to have an early effect on TMA features at Day-7 nor Day-15

ObjectiveHere, we assess the usage of high throughput sequencing (HTS) in rheumatic research and the availability of public HTS data of rheumatic samples.MethodsWe performed a semiautomated literature review on PubMed, consisting of an R-script and manual curation as well as a manual search on the Sequence Read Archive for public available HTS data.ResultsOf the 699 identified articles, rheumatoid arthritis (n=182 publications, 26%), systemic lupus erythematous (n=161, 23%) and osteoarthritis (n=152, 22%) are among the rheumatic diseases with the most reported use of HTS assays. The most represented assay is RNA-Seq (n=457, 65%) for the identification of biomarkers in blood or synovial tissue. We also find, that the quality of accompanying clinical characterisation of the sequenced patients differs dramatically and we propose a minimal set of clinical data necessary to accompany rheumatological-relevant HTS data.ConclusionHTS allows the analysis of a broad spectrum of molecular features in many samples at the same time. It offers enormous potential in novel personalised diagnosis and treatment strategies for patients with rheumatic diseases. Being established in cancer research and in the field of Mendelian diseases, rheumatic diseases are about to become the third disease domain for HTS, especially the RNA-Seq assay. However, we need to start a discussion about reporting of clinical characterisation accompany rheumatological-relevant HTS data to make clinical meaningful use of this data.

India is a country with different beliefs like religious, cultural, variation and economic diversity in the health care setting. SLE is a long term disease which involves multisystem, leading to loss of immune tolerance to own antigens. The study aims to assess the knowledge regarding the risk of cardiovascular diseases among systemic lupus erythematous patient at Saveetha Medical College and Hospital. A quantitative research approach was adopted with convenient sampling technique to select 60 patients with systemic lupus erythematosus who attend at Saveetha Medical College and Hospital. Data was collected using a semi-structured questionnaire. The data was organized and analyzed in term of descriptive statistics. The study findings revealed that among 60 samples, 10 (16.7%) had inadequate knowledge; 16 (26.7%) had moderate knowledge, 34 (56.7%) had adequate knowledge on systemic lupus erythematous risk. The majority of the patients with systemic lupus erythematous had moderate knowledge regarding the risk of cardiovascular.

Objective: to assess changes in health-related quality of life (HRQoL) in patients with systemic lupus erythematosus (SLE) during performed therapy.Subjects and methods. The investigation included 128 patients with reliable SLE who fulfilled the 2012 SLICC criteria. Sixty-eight patients received standard therapy; 60 used biological agents (BA). During the first visit and at 12 months, all the patients were assessed for disease activity using by SLEDAI-2K and for organ damage employing the SLICC damage index (DI). HRQoL was assessed by the LupusQoL questionnaire that was filled out by the patients independently.Results and discussion. At Visit 1, the disease activity index averaged 8.0±7.3 in the standard therapy group and 15.4±7.6 in the BA group (p≤0.001); DI was 1.2±0.9 and 1.8±1.8, respectively (p=0.008). BA-treated patients had significantly lower HRQoL scores on all questionnaire scales, except «dependence on others» and «emotional health» ones Both the standard therapy and BA groups showed significantly lower disease activity during Visit 2 at 12 months. HRQoL assessment revealed significant improvements in the scales «pain», «planning», «intimate relationships», «emotional health», «body image», and «fatigue» in the BA group. The standard therapy group had a statistically significant improvement in the scale «dependence on others». All the other scales of the questionnaire displayed a positive change in HRQoL; however, it did not reach statistical significance.

In this case series we present two young female patients presenting in the peripartum period with cardiogenic shock. Both patients had underlying autoimmune diseases, one with systemic lupus erythematosus (SLE) and the other with antiphospholipid syndrome (APS). In both cases cardiogenic shock was a direct manifestation of their autoimmune condition, and with prompt diagnosis and management both patients were able to recover. This case series illustrates the importance of early recognition of cardiogenic shock as a rare manifestation both of SLE and APS.

Aim of the work: To evaluate three subtypes of CD4+Foxp3+ T cells, interleukin-35 (IL-35) and IL-10 in systemic lupus eryhtematosus (SLE) patients and study their relation to disease activity. Patients and methods: Fifty SLE patients were included and divided according to the SLE disease activity index (SLEDAI) into 2 equal groups with activity or in remission. Twenty healthy subjects were included as controls. All subjects underwent flow cytometric analysis of CD4, CD25, CXCR5 and Foxp3 expression on T cells. Serum IL-35 and IL-10 levels were measured by ELISA. Results: Patients were 46 females and 4 males with a mean age of 38.0 ± 10.0 years, disease duration of 9.2 ± 6.0 years. The mean SLEDAI was 6.8 ± 3.7 in active ones. SLE patients especially those with activity had significantly reduced percents of CD4+CD25+Foxp3+ and CD4+CXCR5+Foxp3+ T cells, but increased percents of CD4+CD25−Foxp3+ T cells. This was accompanied by significant higher levels of serum IL-35 and IL-10 (p

Background Screening for pulmonary arterial hypertension (PAH), a leading cause of death in systemic sclerosis, facilitates earlier treatment. The aim of this work was to study connective tissue disease (CTD)-associated PAH guided by ‘DETECT’ algorithm. Patients and methods This study was a prospective cross-sectional study conducted on 30 patients with CTDs, including 16 cases with systemic sclerosis, nine cases with systemic lupus erythromatosis, and five cases with rheumatoid arthritis. Results According to right heart catheterization finding, estimated total sensitivity and specificity of step 1 and step 2 in diagnosis of PAH among all cases were 80 and 64%, respectively. Conclusion The novel, evidence-based DETECT algorithm for PAH detection in CTDs is a sensitive, noninvasive tool and addresses resource usage.

Aim of the work: To study the clinical characteristics and health related quality of life (HRQoL) in systemic lupus erythematosus patients. Patients and methods: 94 adult SLE patients were included from those attending Zagazig University Hospitals. SLE disease activity index (SLEDAI) and Systemic Lupus International Collaborative Clinics damage Index (SLICC/DI) were recorded. The health-related quality of life (HRQoL) was assessed using the lupus-QoL (LQoL) questionnaire. Results: The mean age of the patients was 36.9 ± 14.1 years and disease duration 5.8 ± 4 years. All LqoL domains were reduced. LQoL was significantly related to the gender, SLEDAI, SLICC/DI, erythrocyte sedimentation rate (ESR), anti-nuclear antibody (ANA) and anti-double stranded deoxyribonucleic acid (ds-DNA) (p

Objective.Currently there are 2 different classification criteria for systemic lupus erythematosus (SLE): American College of Rheumatology (ACR) and Systemic Lupus International Collaborating Clinics (SLICC). The aim of this study was to compare the sensitivities of ACR and SLICC criteria in childhood-onset SLE (cSLE) using a large, multiethnic cohort.Methods.We conducted a retrospective study of 722 patients diagnosed with cSLE at The Hospital for Sick Children (SickKids). Prospectively collected data from SickKids’ Lupus Database were reviewed/validated against medical records prior to ACR and SLICC scoring based on cumulative symptoms up to the last visit. Sensitivities were compared using McNemar’s test. Descriptive statistics were used to identify SLE features unique to each set of criteria and autoantibodies not included in either.Results.ACR and SLICC sensitivities were as follows: 92.4% and 96.3% overall (p = 0.001); 82.5% and 91.3% (p = 0.01) in those scored ≤ 1 year from diagnosis; 92.7% and 97.9% (p = 0.02) in those scored 2–3 years from diagnosis. Forty-eight of 55 (87.3%) patients who did not meet ACR criteria met SLICC criteria through SLICC-specific criterion or renal biopsy. Twenty of 27 (74.1%) patients who did not meet SLICC criteria met ACR criteria as a result of photosensitivity (73.9%) and ACR lymphopenia criteria (26.1%). Six of 7 patients (85.7%) who were clinically diagnosed with cSLE but did not meet either SLICC or ACR criteria had anti-Ro antibodies.Conclusion.SLICC criteria were significantly more sensitive than ACR criteria in cSLE classification, especially early in the disease course. Because of the extreme rarity of primary Sjögren syndrome in children, one may consider adding anti-Ro antibodies to the classification criteria for cSLE because they are present in ∼40% of patents with cSLE.

Cutaneous manifestations of systemic lupus erythematosus (SLE) are important diagnostic criteria as described by the revised American College of Rheumatology and Systemic Lupus International Collaborating Clinics. Patients usually present with various lupus-specific cutaneous manifestations such as malar rash, discoid rash, photosensitivity, and alopecia. Acneiform presentation of SLE is extremely rare. Review of the literature using PubMed has found only nine other reports so far. Here, we report a case who was initially treated as acne and later diagnosed as SLE.

Systemic lupus erythematosus (SLE) is a chronic autoimmune, multisystem rheumatic disease with significant impact on health-related quality of life (HRQoL). Patient-reported outcomes (PROs) provide valuable data on patient perceptions across a variety of domains, such as HRQoL, pain, fatigue, and depression. The measurement and results of PROs with respect to HRQoL in randomized controlled trials (RCTs) on belimumab (B-lymphocyte stimulator inhibitor) in SLE are reviewed here, including BLISS-52 and BLISS-76, as well as publications related to belimumab trials that included HRQoL data. Other trials that evaluated belimumab did not include HRQoL data and were therefore not included in the analysis. The BLISS-52 and BLISS-76 RCTs met their primary endpoints and demonstrated improvements in PROs, measured by the 36-item Short Form Health Survey, EuroQol 5 Dimensions, and Functional Assessment of Chronic Illness Therapy-Fatigue Scale. Belimumab was shown overall to improve PROs in adult autoantibody-positive lupus patients.

Mixed connective tissue disease (MCTD) is a rare connective tissue disorder with clinical features that overlap with systemic lupus erythematous, systemic sclerosis, and polymyositis. We report the case of a patient who presented with dysphagia, 25-lb weight loss, difficulty opening her mouth, and joint pain. Dysphagia workup showed a normal barium swallow and normal-appearing EGD but esophageal manometry consistent with severe dysmotility. Through further laboratory and imaging studies, the patient met the diagnostic criteria for MCTD. She had marked improvement in her dysphagia with steroids, biologic therapy, and intravenous immunoglobulin.

Introduction: Systemic lupus erythematosus (SLE) is a chronic disease that results in variable and occasionally life-threatening manifestations. persons with SLE may experience a wide range of physical, psychological and social problems that affect quality of life. Aim of the work: Assess quality of life in the patients suffering from systemic lupus erythromatosus. Patients and Methods:The study was conducted on forty patients, with systemic lupus erythematosus [32 females (80%) and 8 males (20%)] and forty healthy adults of matched age and sex, as control group [30 female (80%) and 10 males (20%)]. The patients selected from the rheumatology outpatient clinic of Sohag University hospital. An informed written consent had been taken from all patients. Results: The increase in the disease activity, as assessed bysystemic lupus erythematosus disease activity index (SLEDAI) was significantly associated with more impaired quality of life as assessed by SF-36 and systemic lupus erythematosus quality of life questionnaire (SLEQOL). Conclusion:SLE patients have more significantly impaired quality of life, as assessed by short form-36(SF-36), and systemic lupus erythematosus quality of life questionnaire (SLEQOL) than the controls.

Objectives SLE is a chronic autoimmune disease, which can affect the level of bone metabolism and increase the risk of osteoporosis and fracture. The purpose of this research is to study the effect of SLE on bone turnover markers without the influence of glucocorticoids. Methods A total of 865 female subjects were recruited from Zhejiang Provincial People’s Hospital and the First Hospital of Jiaxing, including 391 SLE patients without the influence of glucocorticoids and 474 non-SLE people. We detected Bone turnover markers including amino-terminal propeptide of type 1 procollagen (P1NP), C-terminal turnover of β - I collagen (β-CTX), N-terminal midfragment of osteocalcin (NMID) and 25(OH)D, and analyzed the difference in Bone turnover markers between the SLE group and the control group, as well as the influence of age and season on bone metabolism in female SLE patients. Results In the SLE group, the average age was 43.93±13.95 years old. In the control group, the average age was 44.84±11.42 years old. There was no difference between the two groups (t = 1.03, P = 0.30). P1NP, NMID and 25(OH)D in the SLE group were significantly lower than those in the control group (Z = 8.44, p Conclusion The bone formation markers of female SLE patients without glucocorticoid were lower than those of normal people and the bone resorption marker was higher than that of normal people. The 25 (OH) D of female SLE patients without glucocorticoid was lower than that of normal people. The risk of osteoporosis and fracture may be higher in elderly women with SLE. The bone resorption level of female SLE patients is high in summer and the bone formation level is low in winter.

Lupus nephritis is a common and serious manifestation of systemic lupus erythematosus,its diagnosis is established by a combination of clinical ,laboratory, serological data and histology of the kidney. Plasma complement 4 d can be used to predict flare of lupus nephritis. Aim: The aim of our work was to assess the relation between plasma complement 4 d and lupus nephritis.. Methods: This study was carried out on 100 Lupus Nephritis patients fulfilled the Systemic Lupus International Collaborating Clinics (SLICC) classification criteria and 100 apparently healthy age & sex matched controls. Plasma complement 4 d was detected by specific sandwich ELISA according to the instruction of the manufacturer (SunRed, Shanghi). Results: Our work showed that 24 hour urine protein,RBCs in urine, serum albumin, pus in urine were (significantly associated) with plasma C4d (unstandardized β=0.001, -0.213, 0.441, 0.041 and 0.024 respectively) (p < 0.05). Our results also revealed that there was statistically significant relations between plasma c4d and lupus nephritis in SLE patients (p < 0.05). Conclusion: The study evaluated the importance of Plasma complement 4d in lupus nephritis and confirmed that plasma complement 4d can be used as a very good marker of the flare of lupus nephritis.

OBJECTIVE: To determine the difference in outcomes in patients who achieved or did not achieve the 2019 European Alliance of Associations for Rheumatology (EULAR)/American College of Rheumatology (ACR) criteria. METHODS: Patients from the LUpus in MInorities, NAture versus nurture (LUMINA) cohort were included. For these analyses, we compared those patients who achieved the 2019 EULAR/ACR criteria any time during follow-up to those who did not. The predefined outcomes were the last Systemic Lupus International Collaborating Clinics/ACR Damage Index (SDI) scores and survival. Univariable and multivariable negative binomial regression models were performed; adjustment models were based on a forward selection process. RESULTS: In total, 98 of 640 patients never achieved the 2019 EULAR/ACR criteria. There was no difference in mean baseline SDI score among the patients who did not achieve the criteria compared to those who did. Conversely, the mean ± SD SDI score at last visit was lower for those who never achieved the criteria (1.2 ± 1.7 versus 2.0 ± 2.3, P = 0.0004). In the final adjusted model, the SDI score at last visit was 31% lower for those who never achieved the criteria (P = 0.0077). These patients were also more likely to survive, but this was not statistically significant. CONCLUSION: In our cohort, patients who did not achieve the 2019 EULAR/ACR criteria accrued less damage, suggesting that these criteria could allow us to identify a subset of patients with more severe disease than allowed by previous criteria.

Steroids are the cornerstone in treatment of idiopathic nephrotic syndrome (INS) and systemic lupus erythematosus (SLE). Steroids are used for their anti-inflammatory and immunosuppressive effects. Although, they are known to cause serious adverse effects including glucose intolerance.The mechanisms by which steroid-induced glucose intolerance (SIGI) include: increased gluconeogenesis in the liver, reduced glucose uptake in adipose tissue and skeletal muscle, and induced pancreatic β-cell dysfunction. The exact prevalence of SIGI in patients on chronic steroid therapy is unknown. The prevalence was reported to vary between 0% and 65% and several risk factors for the developing SIGI have been identified. THE AIM OF THIS WORK The aim of this work was to study steroid-induced glucose intolerance in patients with idiopathic nephrotic syndrome and systemic lupus erythematosus on long term steroid treatment and to determine the risk factors associated with development of steroid-induced glucose intolerance.SUBJECTSSubjects were recruited directly from outpatient Pediatric Nephrology Clinic. Sixty children aged from 2 to 18 years were enrolled; including 2 groups: thirty patients with INS and thirty patients with SLE on long-term steroids use for at least 6 months.

We thank Ronnelid et al for their comments on our paper, ‘Performance of the 2019 EULAR/ACR classification criteria for systemic lupus erythematosus in early disease, across sexes, and ethnicities.’1 In 2019, the authors comparatively evaluated the ‘diagnostic accuracy’ of the 2019 European League Against Rheumatism (EULAR)/American College of Rheumatology (ACR) systemic lupus erythematosus (SLE) criteria2 3 against the 2012 Systemic Lupus International Collaborating Clinics (SLICC) criteria. In referring to their paper,4 we stated that it is inappropriate to evaluate the 2019 EULAR/ACR criteria as diagnostic criteria. Ronnelid et al now point out that diagnostic use of the criteria was not their intention. They make the point that they used the term diagnostic, as diagnostic sensitivity …

The aim of our study was to assess the regulatory response of the chemokine CXCL13 in the serum of systemic lupus erythematosus (SLE) patients with disease activity and to evaluate its influence on the inflammatory process in SLE. Serum samples from 97 SLE patients, 49 non-SLE patients (23 patients with other autoimmune diseases and 26 patients with rheumatoid arthritis) and 50 healthy controls were analyzed for the concentration of CXCL13 using ELISA. The results indicated that the serum levels of CXCL13 were significantly higher in SLE patients than in non-SLE patients and healthy controls (p

Hydroxychloroquine (HCQ) is a key systemic lupus erythematosus (SLE) drug, making concerns of drug shortages grave. Our objective was to evaluate factors associated with poor outcomes after HCQ taper or discontinuation in SLE.We studied 5 Canadian SLE cohorts between 1999 and 2019, following patients from the date of HCQ tapering (cohort 1) or discontinuation (cohort 2). A composite outcome was defined as any of the following: a need for therapy augmentation, an increase (of at least 4 points) in the Systemic Lupus Erythematosus Disease Activity Index 2000 score, or hospitalization for SLE. In each cohort, multivariable Cox regression was used to identify demographic and clinical factors associated with time to the earliest of these events. A third cohort continuing to receive HCQ was also studied, to assess whether the same factors influenced the outcome even when the HCQ dose was unchanged.The poor outcome rate, per 100 person-years, was 35.7 (95% confidence interval [95% CI] 31.6-40.3) in the HCQ taper cohort (n = 398), 29.0 (95% CI 25.5-33.0) in the discontinuation cohort (n = 395), and 16.1 (95% CI 13.2-19.6) in the maintenance cohort (n = 395). In patients tapering HCQ, baseline prednisone use was independently associated with greater risk of poor outcomes. In the discontinuation cohort, the risk of poor outcomes was greater for Black patients and those diagnosed with SLE at age ≤25 years. Among those maintaining HCQ, baseline immunosuppressive use and First Nations ethnicity were associated with poor outcomes.We identified demographic and clinical factors associated with poor outcomes after HCQ taper/discontinuation. This information is critical in the current setting of potential shortages, but over the long term, such information could inform personalized therapies.