Addition of constitutional symptoms to the SLEDAI-2K improves overall disease activity assessment: A pilot study.

OBJECTIVE: Constitutional symptoms (fatigue, lymphadenopathy, and weight loss) are not included in the SLE disease activity index-2000 (SLEDAI-2K). In this pilot study, we assessed the concurrent and construct validity of a revised SLEDAI-2K (SLED-R) that included these symptoms with the original SLEDAI-2K (SLED-O), using the physician global assessment of disease activity (PGA) as the reference. METHODS: Our revised SLED-R substituted the SLED-O's fever descriptor with a constitutional descriptor that included fever, fatigue, lymphadenopathy, and/or weight loss. SLED-O, SLED-R, PGA and patient global assessment (PtGA) scores were collected prospectively. Bland-Altman correlations for repeated measures were calculated and Meng's z-test was used to compare correlations between dependent and overlapping correlation coefficients. Associations between constitutional symptoms and disease activity measures were analyzed using Mann-Whitney U, Kruskal-Wallis, Chi-square tests and repeated measures correlations. RESULTS: 1123 SLED-O, SLED-R, PGA, and 1066 PtGA were collected in 239 subjects. The new descriptor was scored in 45 subjects (18.8%) and 92 instances (8.1%), while the original descriptor, fever, was scored in only 4 subjects (1.7%) and 5 instances (0.4%). Mean SLED-O, PGA and PtGA scores were higher when the constitutional descriptor was scored versus not (p < .001). The correlation between SLED-R and PGA was marginally higher than between SLED-O and PGA (p < .001). Fatigue contributed most to this increase (p = .001) and associated with both higher PGA and PtGA scores (p < .001). Mean SLED-O and PGA scores were higher when ≥1 constitutional symptom(s) were scored versus not (p < .002). Correlations between PGA and PtGA when the new descriptor was scored versus not were similar (p = .860). The frequency of concordance between PGA and PtGA was lower when the new descriptor was scored (55%) versus not (72.5%), with PGA > PtGA when the new descriptor was scored (p < .001). CONCLUSION: The addition of constitutional symptoms to SLEDAI-2K, particularly fatigue, resulted in a marginal increase in its correlation with PGA, and new constitutional symptoms associated with higher SLED-O and PGA scores. As fatigue is subjective and difficult to attribute to SLE, its validity and inter-rater reliability in scoring remains uncertain. The clinical utility of SLED-R remains unclear, and further studies of its validity and reliability are needed.

Thrombotic risk assessment in patients with systemic lupus erythematosus: Validation of the adjusted-Global Antiphospholipid Syndrome Score (aGAPSS) in Thai patients.

BACKGROUND: The adjusted-Global Antiphospholipid Syndrome Score (aGAPSS) has been validated and used to predict antiphospholipid antibodies (aPL) related to vascular thrombosis (VT). OBJECTIVE: To validate aGAPSS for predicted aPL-related VT and pregnancy complications (PC) in Thai systemic lupus erythematosus (SLE) patients. METHODS: A cross-sectional study was performed among Thai SLE patients with clinical manifestations; history of VT and PC, cardiovascular risk factors, and aPL profiles were collected. The aGAPSS was calculated from the sum of the risk factors (hyperlipidemia = 3.0, arterial hypertension = 1.0, anti-cardiolipin antibody = 5.0, anti-b2 glycoprotein I antibody = 4.0, and lupus anticoagulant = 4.0). RESULTS: Of 132 SLE patients, 12 (9.1%) had VT and 5 (4.1%) had PC. When comparing the aGAPSS (median; interquartile range [IQR]) of patients with events (VT and/or PC) (6.5; IQR 3.3-9.0), VT (8.0; IQR 4.0-9.0), arterial thrombosis (3.5; IQR 1.0-5.8), and PC (9.0; IQR 8.0-11.5), and the aGAPSS of patients without an event (3.0; IQR 0-4.0), aGAPSS of patients with events was significantly higher, except in patients with arterial thrombosis. An aGAPSS of 4.5 or more was associated with risk of aPL-related VT (sensitivity 71.4%, specificity 76.7%), and an aGAPSS of 6.0 or more was associated with risk of aPL-PC (sensitivity 100%, specificity 84.0%). CONCLUSION: The aGAPSS could predict the risk of aPL-PC and aPL-related VT in Thai SLE patients.

Childhood-onset systemic lupus erythematosus with trisomy X and the increased risk for bone complications: a case report.

BACKGROUND: Systemic lupus erythematosus is a multi-organ inflammatory autoimmune disease; immune complexes are part of the pathogenesis, but not entirely responsible. Trisomy X is the most common female chromosomal abnormality and the role of an additional X chromosome in the development of systemic lupus erythematosus is well recognized. However, the potential complications and optimal management of childhood lupus with trisomy X remain unclear. Herein, we describe a case of childhood-onset systemic lupus erythematosus associated with severe bone complications presumably secondary to trisomy X. CASE PRESENTATION: A 16-year-old Japanese girl was diagnosed with childhood-onset systemic lupus erythematosus and trisomy X. A chromosomal abnormality (47, XXX) was incidentally identified on bone marrow examination initially done to determine the cause of pancytopenia. She had a persistent headache, fever　for six days, diffuse hair loss, mucosal ulcers, butterfly eruptions, and palmar erythema. Furthermore, thrombocytopenia, anemia, and erythrocyte fragmentation were detected, suggesting secondary thrombotic microangiopathy. She was initially treated with intravenous methylprednisolone pulse therapy and prescribed monthly cyclophosphamide for severe disease activity, prednisolone, mycophenolate mofetil, and hydroxychloroquine as remission maintenance drugs. She developed generalized extremity pain that had been worsening throughout the disease. Extremity magnetic resonance imaging performed 12 months after the treatment onset revealed multifocal avascular necrosis, and dual-energy X-ray absorptiometry revealed further decreased bone mineral density. High plasma levels of factor VIII were detected by additional tests for coagulation functions, and we suspected the possibility that factor VIII might cause avascular necrosis due to thrombosis. Currently, she is being treated with prednisolone and MMF for SLE. However, her extremity pain has not been managed effectively even under the administration of non-steroidal anti-inflammatory drugs and pregabalin. CONCLUSIONS: An additional X chromosome has been reported to be associated with factor VIII and osteoporosis. Additionally, elevated plasma levels of FVIII is the risk factors for thrombosis, which leads to the risk of avascular necrosis. Patients with systemic lupus erythematosus complicated by trisomy X might be at a higher risk of avascular necrosis and osteoporosis that can also manifest in childhood systemic lupus erythematosus.

Structural Retinal Assessment Using Optical Coherence Tomography and Fundus Fluorescein Angiography in Systemic Lupus Erythematosus Patients.

BACKGROUND: Ocular manifestations in systemic lupus erythematosus (SLE) can be the presenting symptom of the disease or a sight-threatening complication. OBJECTIVES: To detect different structural retinal changes in patients with SLE who had no ophthalmological symptoms and investigate the relationship between different retinal changes and the disease activity assessed by the Systemic Lupus Erythromatosus Disease Activity Index score. STUDY DESIGN: A descriptive pilot study from January 2016 to January 2017. METHODS: Fifty-two eyes of 26 patients diagnosed to have SLE were examined using visual acuity assessment, fundus examination, optical coherence tomography (OCT), and fundus fluorescein angiography (FFA). RESULTS: Fundus fluorescein angiography showed different changes in the form of venular occlusion and optic nerve leakage. There were also degenerative changes in the form of alternating hyperfluorescent and hypofluorescent areas outside the arcades as well as peripapillary areas and capillary dropout. Optical coherence tomography detected signs of degenerative thinning, incomplete posterior vitreous detachment, and epiretinal membrane. A significant correlation was found between SLE activity and the changes detected by FFA (p = 0.017). However, there was no significant correlation between disease activity and changes detected by OCT. Optical coherence tomography changes were significantly correlated with the duration of hydroxychloroquine use of more than 5 years (p = 0.032). There was no correlation between FFA or OCT changes and proteinuria or antiphospholipid antibodies. CONCLUSIONS: Fundus fluorescein angiography is more sensitive in detecting early subclinical retinal changes in patients with SLE, which correlates with disease activity, whereas OCT is more sensitive in detecting changes resulting from hydroxychloroquine use.

Identifying co-morbid fibromyalgia in patients with systemic lupus erythematosus using the Multi-Dimensional Health Assessment Questionnaire.

OBJECTIVE: Fibromyalgia (FM) is prevalent but often under-recognized in patients with systemic lupus erythematosus (SLE). Patient-reported outcomes (PROs) from the Multi-Dimensional Health Assessment Questionnaire (MDHAQ) can identify co-morbid FM in patients with rheumatic diseases. The present study examined the utility of the MDHAQ in recognizing FM in patients with SLE during routine consultations. METHODS: Patients with SLE completed an MDHAQ. FM status was determined by the validated 2016 revision of the ACR 2010/2011 preliminary FM criteria. Individual PROs from the MDHAQ and composite Fibromyalgia Assessment Tool (FAST) indices of the discriminatory PROs were compared between patients with and without FM using Student's unpaired t-test and receiver operating characteristic curve analysis to determine the area under the curve (AUC). The physician's clinical impression of FM was recorded, and the SLE Disease Activity Index was used to assess disease activity. RESULTS: Of 88 patients with SLE, 23 (26%) satisfied the 2016 FM criteria. The FAST3 composite measure of two out of three of pain (≥6/10), joint count (≥16/48) and symptom checklist (≥16/60) correctly classified 89% of patients (AUC=0.90, kappa=0.71). Physician diagnosis demonstrated moderate agreement with the 2016 FM criteria (kappa=0.43) but missed 43% of patients with FM. In the presence of active disease, the FAST3 correctly classified 91% of patients. CONCLUSIONS: Co-morbid FM is prevalent in SLE yet often underdiagnosed by physicians. The simple FAST3 index of the MDHAQ provides an easy-to-use self-reported tool to improve identification of FM in patients with SLE.

Influence of disease severity and cardiac autonomic tone on ventricular repolarization and dispersion in electrocardiographic assessment of patients with systemic lupus erythematosus.

BACKGROUND: There are no data on the influence of disease severity and cardiac autonomic tone on ventricular repolarization and dispersion in 24-hour Holter monitoring in systemic lupus erythematosus (SLE). METHODS: Consecutive 92 SLE and 51 healthy subjects were studied. The standard 12-lead electrocardiography (ECG), Holter monitoring with heart rate turbulence (HRT) and QT, Tp-e and Tp-e/QT ratio assessment (including corrected values) were performed. Subjects with conditions causing repolarization abnormalities or insufficient number of beats suitable for QT evaluation were excluded (17 SLE and 8 controls). RESULTS: Finally, 75 SLE and 43 sex- and age-matched controls were included to the study. In SLE patients, the median disease severity score (Systemic Lupus International Collaborating Clinics/American College of Rheumatology Damage Index (SLICC/ACR-DI)) was 3.0. The mean values of QTc, cTp-e and cTp-e/QTc were significantly higher in SLE patients than in controls. QTc ≥ 460 ms was observed in 18.7% of patients using standard ECG and in 58.7% using Holter monitoring. With Holter monitoring, patients with SLICC/ACR-DI >3.0 presented longer QTc than those with SLICC/ACR-DI ≤3.0 (418±15 vs. 409 ± 16, p = 0.04), while cTp-e and cTp-e/QTc values were similar. Patients with abnormal HRT presented longer cTp-e and higher cTp-e/QTc than those with normal HRT (92 ± 52 vs. 71 ± 16 ms, p = 0.04; 0.244 ± 0.126 vs. 0.187 ± 0.035, p = 0.03), while QTc values were similar. No differences in QT and Tp-e parameters were observed according to disease duration. CONCLUSION: In SLE patients, Holter monitoring revealed QTc prolongation more frequently than standard ECG. Longer QTc values were observed in patients with more advanced disease, while increased cTp-e and cTp-e/QTc were related to cardiac autonomic dysfunction expressed by abnormal HRT.

Therapeutic peptides for the treatment of systemic lupus erythematosus: a place in therapy.

INTRODUCTION: Studies in vitro and in vivo have identified several peptides that are potentially useful in treating systemic lupus erythematosus (SLE). The rationale for their use lies in the cost-effective production, high potency, target selectivity, low toxicity, and a peculiar mechanism of action that is mainly based on the induction of immune tolerance. Three therapeutic peptides have entered clinical development, but they have yielded disappointing results. However, some subsets of patients, such as those with the positivity of anti-dsDNA antibodies, appear more likely to respond to these medications. AREAS COVERED: This review evaluates the potential use of therapeutic peptides for SLE and gives an opinion on how they may offer advantages for SLE treatment. EXPERT OPINION: Given their acceptable safety profile, therapeutic peptides could be added to agents traditionally used to treat SLE and this may offer a synergistic and drug-sparing effect, especially in selected patient populations. Moreover, they could temporarily be utilized to manage SLE flares, or be administered as a vaccine in subjects at risk. Efforts to ameliorate bioavailability, increase the half-life and prevent immunogenicity are ongoing. The formulation of hybrid compounds, like peptibodies or peptidomimetic small molecules, is expected to yield renewed treatments with a better pharmacologic profile and increased efficacy.

High disease activity status suggests more severe disease and damage accrual in systemic lupus erythematosus.

OBJECTIVE: Disease severity in SLE is an important concept related to disease activity, treatment burden and prognosis. We set out to evaluate if high disease activity status (HDAS), based on ever attainment of a Systemic Lupus Erythematosus Disease Activity Index (SLEDAI) disease activity score of ≥10, is an indicator for disease severity in SLE. METHODS: Using prospectively collected data, we assessed the association of HDAS with sociodemographic and disease characteristics and adverse clinical outcomes using logistic regression or generalised estimating equations. RESULTS: Of 286 patients with SLE, who were observed for a median (range) of 5.1 years (1-10.8 years), 43.7% experienced HDAS at least once during the observational period. Autoantibody positivity, particularly anti-dsDNA and anti-Sm positivity, were associated with increased likelihood of HDAS. Age ≥45 years at diagnosis was associated with reduced likelihood of HDAS (p=0.002). Patients with HDAS had higher Physician Global Assessment score (>1: OR 8.1, p<0.001) and were more likely to meet criteria for flare (mild/moderate flare: OR 4.4, p<0.001; severe flare: OR 17.2, p<0.001) at the time of experiencing HDAS. They were also more likely to have overall higher disease activity, as defined by time-adjusted mean SLEDAI-2K score in the highest quartile (OR 11.7, 95% CI 5.1 to 26.6; p>0.001), higher corticosteroid exposure (corticosteroid dose in highest quartile: OR 7.7, 95% CI 3.9 to 15.3; p<0.001) and damage accrual (OR 2.3, 95% CI 1.3 to 3.9; p=0.003) when compared with non-HDAS patients. CONCLUSIONS: HDAS is associated with more severe disease, as measured by higher disease activity across time, corticosteroid exposure and damage accrual. The occurrence of HDAS may be a useful prognostic marker in the management of SLE.

Systemic lupus erythematosus gastrointestinal involvement: a computed tomography-based assessment.

Systemic lupus erythematosus (SLE) gastrointestinal (GI) complication is characterized by multi-segment and multi-compartment involvement. The aim of this study is to develop a computed tomography (CT) image-based system for disease evaluation. SLE patients with GI involvement from two independent cohorts were retrospectively included. Baseline abdominal CT scan with intravenous and oral contrast was obtained from each individual. A CT scoring system incorporating the extent of GI tract involvement and intestinal wall thickness, along with extra-GI compartment involvement, was developed and validated. The outcome measurement was the time to GI functional recovery, defined as the time to tolerable per os (PO) intake ≥50% of ideal calories (PO50). A total of 54 and 37 patients with SLE GI involvement were enrolled in the derivation and validation cohorts, respectively. The CT scores for SLE GI involvement were positively correlated with patients' time to PO50 (r = 0.57, p < 0.0001, derivation cohort; r = 0.42, p = 0.0093, validation cohort). Patients with a CT score ≤ 3 had a shorter time to PO50 (median time of 0 day) in pooled cohort, whereas those with a CT score > 3 incurred a significantly prolonged recovery with a median time to PO50 of 13 days (p < 0.0001). The CT-based scoring system may facilitate more accurate assessment and individualized management of SLE patients with GI involvement.

Electrocardiogram and heart rate variability assessment in patients with common autoimmune diseases: a methodological review.

The aim of this article was to summarize current knowledge about the potential clinical utility of electrocardiogram (ECG) and heart rate variability (HRV) measures in patients with 4 common autoimmune diseases (ADs): rheumatoid arthritis (RA), systemic lupus erythematosus (SLE), Behcet's disease (BD), and systemic sclerosis (SSc). A search was conducted of the PubMed, Embase, and Scopus databases using terms and a controlled vocabulary associated with these ADs, ECG, and HRV. The available, full-text articles published in English were considered. In all, 20 publications that examined the direct effect of these diseases on the heart were selected according to a systematic review protocol. Time-frequency domain analysis revealed that HRV parameters were lower in patients with the selected ADs in comparison with control groups. An increased QT dispersion and heart rate corrected QT, which are well-known as risk factors for sudden cardiac death, were observed in the patient group. In some studies, a correlation was seen between the duration of the disease and its activity, while others did not report such an association. Heart rate turbulence parameters were also examined. Turbulence onset was increased in SLE and SSc patients, while the turbulence slope was decreased in SLE patients. There was no significant change in these parameters in BD patients. Patients with ADs demonstrate abnormal HRV and ECG parameters, which indicates an autonomic cardiac functional impairment. Measurement of these parameters can be a useful clinical tool in the diagnosis and prediction of some disorders in patients with ADs. Both of these signals can provide helpful information for physicians to trace the efficacy of prescribed medicines.

Assessment of superficial and deep retinal vessel density in systemic lupus erythematosus patients using optical coherence tomography angiography.

PURPOSE: The aim of this study is to evaluate the retinal microvascular density in SLE patients using optical coherence tomography angiography (OCTA) and to correlate vascular density with the disease activity and damage risk. METHODS: Twenty eyes of 20 SLE patients were compared with 20 eyes of normal subjects. The retinal capillary plexuses were examined by OCTA. The disease activity and damage risk were evaluated by the SLEDAI-2 K and SLICC/ACR SDI scoring systems. RESULTS: No difference was found between SLE patients' central foveal thickness (CFT) and foveal avascular zone (FAZ) area and the normal (P > 0.05). SLE patients had slightly lower superficial vessel densities than normal in the upper and lower macular regions (P < 0.05), sparing the middle sectors (P > 0.05). In the deep plexus, vessel density loss was detected in all sectors (P < 0.001). The vessel density in 300-µm-wide region around the FAZ (FD-300) and the acircularity index (AI) were affected in the SLE in comparison to the normal group (P < 0.05). No significant correlation was found between the SLEDAI-2 k and the retinal vessel density in either layer, while the SLICC/SDI had moderate inverse correlation with vessel density in some sectors (P < 0.05). Receiver operating characteristic (ROC) curve analysis showed that the deep capillary plexus had high sensitivity and specificity for detecting vascular damage in SLE patients. CONCLUSIONS: OCTA permits noninvasive quantitative assessment of retinal vessel density in SLE, allowing early detection of altered retinal circulation. Vessel density could be included in future assessment of SLE activity and damage scores.

Patient-Reported Lupus Flare Symptoms Are Associated with Worsened Patient Outcomes and Increased Economic Burden.

BACKGROUND: Lupus flares significantly contribute to health resource utilization and hospitalizations. Identification of flare activity may be hindered since validated assessment scales are rarely used in clinical practice and flare severity may fall below clinician-assessed thresholds. Therefore, patient-reported outcomes of lupus flare frequency are important assessment tools for lupus management. OBJECTIVE: To better understand the relationship between lupus flares as reported by persons with lupus and specific direct and indirect costs, including hospital admission, unplanned urgent care (UC)/emergency department (ED) visits, work productivity loss, and nonwork activity impairment. METHODS: In this cross-sectional analysis, persons with lupus were drawn from 2 enriched sampling sources. Data were collected via an online survey and included individuals with self-reported physician's diagnosis of systemic lupus erythematosus, skin or discoid lupus, or lupus nephritis. Respondents were asked the total number of hospitalizations and ED/UC visits for any reason and for lupus-related hospitalizations and ED/UC visits. Work productivity loss and nonwork activity impairment were measured via the Work Productivity and Activity Impairment - General Health scale. The sample was stratified into those with 0 flares, 1-3 flares, 4-6 flares, and 7 or more flares, with 0 flares used as the reference. Chi-square tests for trend and analyses of variance were used to evaluate differences among flare frequency groups. Multivariable regression modeling was conducted to evaluate the independent relationship of flare frequency to health care use and productivity loss. RESULTS: We studied 1,288 survey respondents with known flare frequency in the past 12 months. Flare frequency increased with duration of illness. The mean number of lupus-related hospital admissions was significantly associated with increasing flare frequency for the total sample (F = 3.9; P < 0.009). Compared to patients with no flare, those who reported flare activities had 1.72-3.13 times higher rates of hospitalizations. The mean number of lupus-related ED/UC visits were also found to be significantly associated with increasing flare frequency for the total sample (F = 23.4; P < 0.001), and rates were increased by 6.98- to 16.12-fold for unplanned ED/UC visits depending on flare frequency. Rates of employment were significantly related to increasing flare frequency. With respect to work-related impairment, absenteeism increased with greater lupus flare frequency (F = 6.2; P < 0.001), as did presenteeism (F = 31.5; P < 0.001) and the combined value of total work productivity loss (F = 30.4; P < 0.001). Mean work-related activity impairment was 12%-32% more among patients who reported flare activities compared to those who reported no flares. CONCLUSIONS: Increased lupus-related flare frequency is associated with worsened patient outcomes as measured by increased hospitalizations, visits to the ED/UC, work productivity loss, and activity impairment. This association may be an important indicator of disease severity and resource burden and therefore suggests an unmet need among patients experiencing lupus-related flares. DISCLOSURES: This study was sponsored by Mallinckrodt Pharmaceuticals via grants to Vedanta Research and The Lupus Foundation of America. Katz received consulting fees from Vedanta Research, which received grant support from Mallinckrodt Pharmaceuticals to support data collection and analysis. Nelson and Connolly-Strong are employees of Mallinckrodt Pharmaceuticals and are stockholders in the company. Reed is an employee of Vedanta Research. Daly and Topf are employees of the Lupus Foundation of America, which received grant funding to support data collection. This study was a podium presentation at The American College of Rheumatology (ACR) Annual Meeting 2018; October 19-24, 2018; Chicago, IL.

Diet and lupus: what do the patients think?

OBJECTIVES: Cardiovascular disease is the leading cause of mortality in patients with systemic lupus erythematosus. Therefore, using diet to control blood lipid levels and modify cardiovascular disease risk could be a promising therapeutic strategy to control disease symptoms. The primary objective of this study was to learn about systemic lupus erythematosus patient experiences with diet, including their opinion on considering diet as a therapeutic option. The secondary objective was to obtain this information in a cost- and time-effective manner. METHODS: A lay summary and a 15-question diet-based online survey were publicly available for 3 weeks. Social media was used to promote the survey through relevant charities, hospitals and research groups. RESULTS: A total of 300 responses were received, 284 from patients with systemic lupus erythematosus. Patients reported that there was a lack of clinical counselling regarding diet, with only 24% stating their doctor had spoken to them about diet. Despite this, 100% of patients stated they would change their diet if they knew it would help their symptoms and 83% would take part in a future diet-based clinical trial. Text analysis of patient research suggestions identified a particular interest in using diet to treat fatigue and manage disease flares. CONCLUSIONS: This project successfully gathered patient information regarding diet and systemic lupus erythematosus over a short timeframe using an anonymous social media platform. The survey provided evidence that patients support further research and potential diet intervention studies investigating the effect of diet on the symptoms of systemic lupus erythematosus.

Health-related quality of life in patients with systemic lupus erythematosus: a Spanish study based on patient reports.

INTRODUCTION AND OBJECTIVES: Systemic lupus erythematosus (SLE) is a disease that significantly affects the quality of life and welfare of patients. SLE patients can be classified into multimorbidity levels using Clinical Risk Groups (CRGs) to help to incorporate predictive models of health needs. The goal of this study was to correlate CRGs with health-related quality of life (HR-QoL) and costs in SLE patients. METHODS: A questionnaire was administered to SLE patients in four hospital centers of the Valencian Community (Spain) between October 2015 and March 2016. The factors studied included HR-QoL (EQ-5D-5L and VAS), disease activity (SLAI/SELENA), damage (SLICC/ACR), and severity (IGK). RESULTS: The patients (N = 190, 92.06% female, age (mean ± SD) 47.23 ± 13.43 years) were sorted according to health status in nine CRGs. We found that most SLE patients (> 70%) were in CRGs 5 and 6. The main HR-QoL issues in these patients were related to mobility, ability to perform usual activities, and pain/discomfort. The scores (mean ± SD) for EQ-5D-5L and VAS were 0.74 ± 0.25 and 65.67 ± 23.52, respectively. We found that the age of the patients negatively affected their HR-QoL (r = - 0.266). SLE direct costs per patient increased with each CRG group, representing 71.92% of the total costs, while indirect costs were highly variable. The average cost per patient with SLE amounted to €8432.85 (year 2014). CONCLUSIONS: Patients' quality of life is related with age, disease activity, damage, and severity. Age was the parameter which most affects HR-QoL. Most costs of SLE are concentrated in two CRGs in which the HR-QoL deteriorates sharply.

The Fatigue Assessment Scale as a simple and reliable tool in systemic lupus erythematosus: a cross-sectional study.

BACKGROUND: The vast majority of patients with systemic lupus erythematosus (SLE) complain about fatigue. They also report fatigue as one of their most debilitating symptoms. Yet, in clinical practice, fatigue is only rarely assessed and remains poorly understood. The purpose of this study is to validate the Fatigue Assessment Scale (FAS) and assess the impact of disease activity on fatigue in SLE. METHODS: A cross-sectional single-center study of patients was included in the Swiss SLE Cohort Study. The FAS and the Short Form 36 (SF-36) were administered to SLE patients and controls with primary Sjogren's syndrome (pSS) and healthy volunteers (HV) attending our clinic. Disease activity in SLE was captured at the same time as patient-reported outcomes using the SLE Disease Activity Index score with the Safety of Estrogens in SLE National Assessment modification (SELENA-SLEDAI) and the physician's global assessment. We explored the internal consistency, reproducibility, construct validity, and convergence of the FAS, in comparison to the vitality subscale (VT) of the SF-36. We examined the association of FAS with demographics, disease type, SLE disease activity, and clinical features. RESULTS: Of the 73 SLE subjects, 89% were women and 77% were Caucasians. The median age was 43 years, and 23 (32%) patients had active SLE. Demographics in pSS and HV were similar. Within the SLE group, FAS displayed good internal consistency (Cronbach's alpha = 0.93), unidimensionality, and test-retest reliability (ICC = 0.90). FAS and VT correlated well. The total FAS was highest in active SLE and pSS and higher in non-active SLE compared to HV. CONCLUSION: The FAS is a promising tool to measure fatigue in SLE. Patients with SLE display a significantly higher level of fatigue than HV, which is even more pronounced in active disease and comparable to fatigue levels measured in pSS.

Usefulness of composite indices in the assessment of joint involvement in systemic lupus erythematosus patients: correlation with ultrasonographic score.

Specific indices are not available to evaluate systemic lupus erythematosus (SLE) joint involvement; indeed, the application of indices validated for rheumatoid arthritis has been suggested. We evaluated the usefulness of organ specific composite indices, i.e. the Disease Activity Score on 28 joints (DAS28), Simplified Disease Activity Index (SDAI), Clinical Disease Activity Index (CDAI), and the ratio of swollen to tender joints (STR), to assess SLE joint activity by analyzing the correlation between these indices and ultrasonography (US) inflammatory status. We evaluated SLE patients with arthralgia and/or arthritis: the above-mentioned indices were calculated and the SLE Disease Activity Index 2000 (SLEDAI-2k) was applied to assess global disease activity. US of I-V metacarpophalangeal, I-V proximal interphalangeal, wrist, and knee bilateral was performed. Synovial effusion/hypertrophy and power Doppler findings were scored according to a semi-quantitative scale (0-3) to obtain an inflammatory total score (0-216). One hundred and six patients (M/F 7/99, median age 49.5 years (IQR 17.0), median disease duration 8.5 years (IQR 17.0)) were enrolled. We identified a positive correlation between US score and DAS28-CRP ( r = 0.3, p = 0.007), STR ( r = 0.42, p = 0.0005), SDAI ( r = 0.33, p = 0.02), CDAI ( r = 0.29, p = 0.03); US score reflected different levels of clinimetric joint activity. In conclusion, we suggest the ability of composite indices in detecting SLE joint inflammation and their possible real-life use.

Comparison of non-invasive assessment of arrhythmias, conduction disturbances and cardiac autonomic tone in systemic sclerosis and systemic lupus erythematosus.

Systemic sclerosis (SSc) and systemic lupus erythematosus (SLE) are connective tissue diseases presenting cardiac complications including different arrhythmias, then direct electrocardiographic comparison may be useful in everyday clinical decision making. We examined 86 adult SSc patients, 76 with SLE and 45 healthy controls. Among other examinations all subjects underwent 24-h Holter monitoring with time-domain heart rate variability and heart rate turbulence evaluation. Patients with various co-existing conditions which might markedly influence arrhythmias and autonomic modulation were excluded from further analysis (SSc n = 12, SLE n = 6). Finally, 76 SSc and 70 SLE subjects were eligible for this study, mean age 51.9 ± 13.1 and 46.5 ± 12.7 years (p = 0.11), with median disease duration 6.0 and 8.5 years (p = 0.15), respectively. As compared to SLE, patients with SSc were characterised by more frequent incidence of various supraventricular and ventricular arrhythmias. As compared to SSc, patients with SLE presented prolonged corrected QT intervals and also significant correlations between corrected QT length and heart rate variability indices. Both SSc and SLE subjects presented impaired sympathetic cardiac autonomic modulation, while indices associated with parasympathetic activity in SLE were not diminished. Disease duration was not associated with arrhythmias' occurrence (except for ventricular tachycardia in SSc, p = 0.02) and also with autonomic function in both groups of patients. Patients with SSc and SLE differ in terms of arrhythmias, conduction disturbances and cardiac autonomic tone. Regular Holter monitoring should be considered as a part of routine evaluation in connective tissue diseases patients, especially in systemic sclerosis.

Relación entre los factores sociodemográficos y la calidad de vida en pacientes con lupus eritematoso sistémico / Relationship between sociodemographic factors and the quality of life in patients with systemic lupus erythematosus

El lupus eritematoso sistémico (LES) es una enfermedad autoinmune que puede tener repercusiones negativas en la calidad de vida (CV) de quienes la padecen. La CV puede verse influida por factores sociodemográficos (FSD) relacionados con la enfermedad en sí. Objetivo: Determinar la relación entre los FSD y la CV en pacientes con LES. Métodos: Estudio descriptivo, de corte transversal, de campo y observacional, donde se evaluaron 100 pacientes con diagnóstico de LES. Se emplearon dos encuestas: una para la recolección de datos sociodemográficos y el formulario de la Escala GENCAT. Resultados: La edad promedio fue 42,9 años ± 13,5, el sexo más afectado fue femenino con 93%, los años de estudio promedio fueron 11,4 años ± 4,4 y el tiempo de evolución del LES promedio fue 11,1 años ± 9,5. La CV según el formulario de la Escala GENCAT fue aceptable; la edad avanzada y mayor tiempo de evolución de LES se asoció con menor CV, y mayor escolaridad se asoció con mayor CV. Conclusiones: La CV fue aceptable según la Escala GENCAT, y se encontraron asociaciones entre la CV y edad, años de estudio y tiempo de evolución del LES que fueron estadísticamente significativas(AU)

Systemic lupus erythematosus (SLE) is an autoimmune disease that can have a negative impact on the quality of life (QoL) of those who suffer from it. QoL may be influenced by sociodemographic factors (SDF) related to the disease itself. Objective: Determine the relationship between the SDF and the QoL in patients with SLE. Methods: Descriptive, cross-sectional, field and observational study, where 100 patients with a diagnosis of SLE were evaluated. Two surveys were used: one for the collection of sociodemographic data and the GENCAT Scale form. Results: The average age was 42.9 years ± 13.5, the most affected sex was female with 93%, the average study years were 11.4 years ± 4.4 and the evolution time of the average SLE was 11.1 year ± 9.5. TheQoL according to the GENCAT Scale form was acceptable; the advanced age and longer time of evolution of SLE was associated with lower QoL, and higher schooling was associated with higher QoL. Conclusions: The QoL was acceptable according to the GENCAT Scale, and associations were found between the QoL and age, years of study and time of evolution of the disease, which were statistically significant(AU)

Image enhancement effect on inter and intra-observer reliability of nailfold capillary assessment.

BACKGROUND: Nailfold capillaroscopy (NC) is a diagnostic imaging technique that is used to assess the blood capillary network in the nailfold area. NC is routinely used for patients with microcirculation problems, such as systemic sclerosis and other connective tissue diseases. Experts commonly use subjective evaluation as a reference point in images of nailfold video capillaroscopy, so it is important to reduce the inherent ambiguities in human judgment and diagnosis. Image quality is an important factor that affects measurement error and assessment time of NC images. OBJECTIVE: In this study, a new image enhancement technique was introduced and evaluated subjectively. METHODS: In total, 475 nailfold video capillaroscopy images from 18 healthy subjects and 41 systemic lupus erythematosus patients were used. The images were randomly divided into two sets, one each with 275 and 200. Eight independent observers who were familiar with the capillaroscopy technique participated in this study. The set of 275 images was evaluated by three observers with the forced-choice pairwise comparison method. Elliptic broken line (EBL) was used to count the number of capillaries. The intra- and inter-observer reliability of the original and enhanced images was evaluated on 200 images by five observers. RESULT: Except for eight images, all observers preferred the enhanced images in the visual quality comparison method. The intra-class correlation coefficient (ICC) of intra- and inter-observer reliability increased from 0.76-0.84 to 0.82-0.89, respectively, when using the enhancement method. CONCLUSION: By improving the image quality, more capillary details will be visible, and an observer can document more details that may not be visible in the original image and can do so more efficiently.

Early Assessment of Right Ventricular Function in Systemic Lupus Erythematosus Patients using Strain and Strain Rate Imaging / Avaliação Precoce da Função Ventricular Direita em Pacientes com Lúpus Eritematoso Sistêmico pelo Strain e Strain Rate

Abstract Background: Right ventricular function is a crucial factor of the prognosis of systemic lupus erythematosus (SLE). Objectives: To evaluate the right ventricular function in SLE patients with different degrees of pulmonary hypertension (PH) by strain and strain rate imaging. Methods: A total of 102 SLE patients and 30 healthy volunteers were studied between October 2015 and May 2016. Patients were divided into three groups according to pulmonary artery systolic pressure (PASP) estimated by echocardiography: group control (A); PASP ≤ 30 mmHg (group B, n = 37); PASP 30-50 mmHg (mild PH; group C, n = 34); and PASP ≥ 50 mmHg (moderate-to-severe PH; group D, n = 31). Longitudinal peak systolic strain (ε) and strain rate (SR), including systolic strain rate (SRs), early diastolic strain rate (SRe) and late diastolic strain rate (SRa) were measured in the basal, middle and apical segments of the right ventricular free wall in participants by two-dimensional speckle tracking echocardiography (2D-STE) from the apical four-chamber view. A p < 0.05 was set for statistical significance. Results: The parameters of ε, SRs, SRe, and SRa were significantly decreased in groups C and D compared with groups A and B. The ε of each segments was significantly lower in group D than in group C, while there were no differences in SRs, SRe and SRa between groups C and D. Conclusions: Strain and strain rate imaging could early detect the right ventricular dysfunction in SLE patients with PH, and provide important value for clinical therapy and prognosis of these patients. (Arq Bras Cardiol. 2018; [online].ahead print, PP.0-0)

Resumo Fundamento: A função ventricular direita é fator crucial do prognóstico do lúpus eritematoso sistêmico (LES). Objetivos: Avaliar a função ventricular direita em pacientes com LES e diferentes graus de hipertensão pulmonar (HP) por avaliação do strain e do strain rate. Métodos: Um total de 102 pacientes com LES e 30 voluntários sadios foram estudados entre outubro de 2015 e maio de 2016. Os pacientes foram divididos em três grupos de acordo com a pressão sistólica da artéria pulmonar (PSAP) estimada por ecocardiografia: grupo controle (A); PSAP ≤ 30 mmHg (grupo B, n = 37); PSAP 30-50 mmHg (HP leve; grupo C, n = 34); e PSAP ≥ 50 mmHg (HP moderada a grave; grupo D, n = 31). Foram medidos, nos segmentos basal, medial e apical da parede livre do ventrículo direito, o pico sistólico longitudinal (ε) e o strain rate (SR), incluindo o SR sistólico (SRs), o SR diastólico precoce (SRp) e o SR diastólico tardio (SRt) pela técnica de ecocardiografia bidimensional com rastreamento de "pontos" (two-dimensional speckle tracking echocardiography, 2D-STE) pela visualização apical de quatro câmaras. Um p < 0,05 foi adotado como estatisticamente significativo. Resultados: Os parâmetros ε, SRs, SRp e SRt foram significativamente menores nos grupos C e D em comparação aos grupos A e B. O ε de cada segmento foi significativamente menor no grupo D que no grupo C, ao passo que não houve diferença no SRs, SRp ou SRt entre os grupos C e D. Conclusões: O strain e o SR obtidos por 2D-STE puderam detectar precocemente disfunção ventricular direita em pacientes com LES e HP, contribuindo para o tratamento clínico e prognóstico desses pacientes. (Arq Bras Cardiol. 2018; [online].ahead print, PP.0-0)