Subclinical left ventricular dysfunction during systemic lupus erythematosus activity with follow-up after remission – A speckle tracking echocardiographic study

Aim of the workTo investigate the subclinical left ventricular (LV) dysfunction in patients with active systemic lupus erythematosus (SLE) using speckle tracking echocardiography (STE). The echocardiographic parameters were followed up when the disease activity was controlled.Patients and methodsThis prospective study included 63 patients with active SLE and LV ejection fraction (EF) ≥50%.Safety of Estrogens in Lupus Erythematosus: National Assessment – Systemic Lupus Erythematosus Disease Activity Index (SELENA-SLEDAI) was assessed and categorized as mild/moderate (≤12) or severe (>12). Fifty SLE patients continued follow-up after 3–6 months of the disease remission. Fifty age- and gender-matched healthy individuals acted as the control group. The measured STE parameters included LV deformation (global longitudinal strain [GLS] and global circumferential strain [GCS]) and rotational parameters (rotation, twist, and torsion).ResultsThe patients were 56 females and 7 males (F:M 8:1) and median age 26 years (IQR: 21–31 years) and a disease duration of 3 years (IQR 2–5 years. Active SLE patients showed worse strain parameters than controls (mean GLS ?19.9%±2.1 vs ?22.7%±1.3 and mean GCS ?21.2 ± 2.5% vs ?25.1 ± 1.7% respectively; p < 0.001 for both). Patients had lower LV rotational parameters (p < 0.001 for all). STE parameters were similar in patients with mild-moderate and severe activity and improved after remission in both groups.ConclusionActive SLE patients had modest LV dysfunction by STE despite having normal function by traditional echocardiography. Disease remission resulted in the improvement of STE parameters. STE is a simple tool to use in SLE activity scores to detect early cardiac dysfunction.     

Increased serum β2-microglobulin is associated with clinical and immunological markers of disease activity in systemic lupus erythematosus patients

The objective of this study was to explore the relationship between serum levels of β2-microglobulin (β2MG), which some studies suggest reflect disease activity in systemic lupus erythematosus (SLE), and various clinical and immunological markers of disease activity in SLE. Twenty-six SLE patients and 10 healthy controls were included. Disease activity was assessed by: SLEDAI, 24?hr-proteinuria, circulating levels of complement C3, anti-double-stranded DNA (anti-dsDNA), β2MG and various pro-inflammatory and anti-inflammatory cytokines (IL-6, IL-8, IL-10, IL-18) measured with a multiplex assay, IFN-α assessed with a reporter gene assay, and a combined expression score of 12 IFN-α inducible genes in peripheral blood mononuclear cells. Median serum levels of β2MG were significantly higher in SLE patients vs controls (2.8?mg/L, range: 1.1-21.6 and 1.2?mg/L, range: 0.9-1.7, respectively, p?相似文献   

Exercise therapy in patients with idiopathic inflammatory myopathies and systemic lupus erythematosus – A systematic literature review

Idiopathic inflammatory myopathies (IIM) and systemic lupus erythematosus (SLE) are inflammatory connective tissue diseases (CTDs) with common features of arthritis, muscle impairment, skin rash, and heart- and lung involvement. Exercise is becoming an important part of the treatment in patients with IIM and SLE; however, there is a need for evidence-based exercise recommendations on patient-relevant outcomes. To evaluate the evidence and to present evidence-based exercise recommendations on patient-relevant outcomes in patients with IIM and SLE. A systematic literature search of five databases was performed at two time points, 2016 going back all years, and an update in 2019. Inclusion criteria: RCTs including exercise, physical activity intervention, and patient-relevant outcomes. Systematic reviews and meta-analysis was also included. Grading of evidence was done according to the GRADE system. Five RCTs and 1 systematic review were identified in patients with IIM and eight RCTs, 6 systematic reviews, and 2 meta-analysis for patients with SLE. Aerobic exercise and resistance training on moderate-high intensity can improve aerobic capacity, muscle impairment, activity limitation, quality of life, and disease activity (limited evidence) in patients with established polymyositis (PM) and dermatomyositis (DM). Moderate-high intensity aerobic exercise can improve aerobic capacity (moderately strong evidence) and improve fatigue and depressive symptoms (limited evidence) without changing disease activity in patients with mild/inactive SLE with low/no organ damage. There is insufficient evidence for effects of exercise in patients with recent onset PM/DM and IBM. Exercise performed in line with American College of Sports Medicine recommendations can improve aerobic capacity, patient-reported outcomes in patients with nonactive PM/DM and mild/inactive SLE. More well-designed studies are needed to increase the scientific evidence. Studies with additional focus on evaluating effects of exercise in patients with higher disease activity, in patients with vital-organ involvement and in patients with IBM are needed.     

Gout in systemic lupus erythematosus and overlap syndrome – a hospital-based study

The negative association between gout and rheumatoid arthritis is widely accepted, and gout is also speculated to be rare in systemic lupus erythematosus (SLE), as only a few sporadic cases have been reported. From 1985 to 2001 we encountered 15 lupus patients at Chang-Gung Memorial Hospital, including two with lupus–scleroderma and one with lupus–scleroderma–polymyositis overlap syndrome coexisting with gout. This study retrospectively analyses the clinical and laboratory characteristics of these patients. A lower female predominance is found, and most patients developed gout after the onset of SLE, although gout preceded SLE in two cases. Measurement of serum uric acid and 24-h urine uric acid found all of the patients to be hyperuricaemic and underexcretors of uric acid. Furthermore, most of the patients (14/15) were receiving diuretics. Also, many had hypertension and serious cardiovascular diseases. Renal impairment during gouty attacks seemed to be a predisposing factor for developing end-stage renal disease. Gouty arthritis usually occurred during relative SLE inactivity, podagra was frequent, and tophi were found in a few patients. Compared with the unselected population of SLE patients, the cases studied here had a higher incidence of chronic arthritis, malar rash, haematologic disorder, photosensitivity, serositis and neurologic disorder. Renal disease in the patients sampled was frequently membranous nephropathy.     

Anti-C1q antibodies and antiendothelial cell antibodies in systemic lupus erythematosus – relationship with disease activity and renal involvement

The aim of this study was to investigate the relationship between the presence and titre of antibodies against C1q (anti-C1q Ab) and disease activity and renal involvement in patients with systemic lupus erythematosus (SLE). Anti-C1q Ab were measured in 79 patients with SLE (70 women and 9 men; mean age 41.7 years; mean disease duration 8.4 years): 19 patients had active disease with lupus nephritis, 8 active disease without nephritis, 26 inactive disease with nephritis and 26 inactive disease without nephritis. Anti-dsDNA antibodies (EIA and immunofluorescence), antiendothelial cell antibodies (AECA) and complement levels (C3, C4, total haemolytic complement activity) were determined in parallel. Anti-C1q Ab were positive in 49%, anti-dsDNA Ab in 61% and AECA in 19% of the patients, respectively. Significantly higher titres of anti-C1q Ab were found in patients with active disease compared with those with inactive SLE (P < 0.01). Serum levels of anti-C1q Ab showed a positive correlation with anti-dsDNA Ab and SLEDAI score (P < 0.01) and a negative correlation with C3 (P < 0.05), C4 (P < 0.01) and CH50U (P < 0.01). The presence of anti-C1q Ab was not different between patients with or without nephritis. In patients with (P < 0.05) and without nephritis (P < 0.01) the frequency of anti-C1q Ab was significantly higher in active patients compared with inactive patients. Both anti-C1q and anti-ds-DNA Ab were detectable in 74% of patients with active nephritis but only in 30% of all other patients (P=0.001). None of the patients with active nephritis was negative for anti-C1q and anti-dsDNA Ab, whereas 37% of the patients without active nephritis were negative for both antibodies (P < 0.01). Sensitivity, specificity, positive and negative predictive values for active lupus nephritis among SLE patients were 100%, 50%, 51.9% and 100% for anti-dsDNA Ab (EIA) and 74%, 70%, 57% and 89.4% for positive findings of both anti-dsDNA and anti-C1q Ab. The presence and titre of anti-C1q-Ab in SLE are related to disease activity. Absence of anti-dsDNA Ab excludes active nephritis; positive findings of both anti-dsDNA Ab and anti-C1q Ab are of relatively high specificity for active nephritis.     

Skin lesions--an indicator of disease activity in systemic lupus erythematosus?

Cutaneous manifestations have great diagnostic value for systemic lupus erythematosus (SLE). In this study we tried to establish a correlation between lupus erythematosus LE-specific and LE-nonspecific cutaneous lesions and disease activity measured by the Systemic Lupus Erythematosus Disease Activity Index (SLEDAI). Sixty-six patients with SLE were evaluated. They were divided into three groups having: (1) only LE-specific lesions (38 or 58.46%); (2) only LE-nonspecific lesions (4 or 6.15%); and (3) both types of lesions (23 or 35.38%). Results were analyzed using the Student t-test. Patients with LE-nonspecific skin manifestations had significantly increased disease activity compared to those with only LE-specific lesions. The number of different skin lesion types also correlated with disease activity. It was significantly increased in a group with three different types of lesion, either specific or nonspecific. Patients with only one type of lesion had mild disease. An intermediate disease activity was found in the group with two different lesion types. Lupus-specific skin manifestations serve primarily as an important diagnostic clue. In conclusion, patients with LE-nonspecific lesions have significantly more active SLE than those with LE-specific lesions and may therefore require more intensive therapy and disease monitoring.     

How does one assess and monitor patients with systemic lupus erythematosus in daily clinical practice?

Systemic lupus erythematosus (SLE) is a multisystem autoimmune rheumatic disease (ARD) characterized by flares and remissions. SLE has protean and often complex manifestations, necessitating careful clinical assessment. However, it is important to remember that not all clinical problems reported by a lupus patient are due to the disease. Some may be a consequence of therapy and others may be unrelated to lupus. Therefore it is important to understand the totality of the effect of the disease on the patient. In order to do this measures are needed which distinguish current, potentially reversible disease activity, permanent organ damage and the effect of the disease on the patients' health status. Several measures are in current use in clinical trials, but not all are suitable for use in the routine clinical setting. This chapter discusses the current measures available to assess disease activity and damage in SLE.     

Symptomatic knee osteonecrosis in patients with systemic lupus erythematosus: a case–control study

To explore the associated risk factors of symptomatic knee osteonecrosis (KON) in patients with systemic lupus erythematosus (SLE), we conducted a retrospective case–control study to compare the clinical and laboratory features between SLE patients with and without symptomatic KON matched by age and gender. Univariate and multivariate regression analyses were used to evaluate possible associated risk factors. Twenty (one male, nineteen females) out of 3941 lupus patients were identified as symptomatic KON, which was confirmed by magnetic resonance imaging. The mean age at KON onset was 34.4 (range 12–67) years, and the median course of lupus at KON onset was 72.5 (range 8–123) months. Univariate and multivariate analyses identified that the prevalence of cutaneous vasculitis (OR 5.23; 95 % CI 1.11–24.70), hyperfibrinogenemia (OR 4.75; 95 % CI 1.08–20.85), and elevated IgG levels (OR 6.05; 95 % CI 1.58–23.16) were statistically higher in KON group, and hydroxychloroquine (HCQ) usage was statistically lower in KON group (OR 0.27; 95 % CI 0.07–0.97). Glucocorticoid usage, in terms of maximal dose, duration of treatment, and the percentage of receiving methylprednisolone pulse therapy, did not show statistical difference between the two groups (p > 0.05). Symptomatic KON is a relatively rare complication of SLE. Cutaneous vasculitis, hyperfibrinogenemia, and elevated IgG levels are possible risk factors, whereas HCQ may provide a protective effect. Our results suggest that lupus activity as well as hypercoagulation status may play a role in the pathogenesis of KON in lupus.     

Serum salusin-β levels in patients with systemic lupus erythematosus

Clinical Rheumatology - Endothelial dysfunction (ED) has an important role in the pathogenesis of systemic lupus erythematosus (SLE). Studies on other inflammatory diseases show that salusin-β...     

How can we define low disease activity in systemic lupus erythematosus?

Background: In recent years, low disease activity emerged as a state that is associated with improved long-term outcomes in systemic lupus erythematosus (SLE). Our aim was to review the current concepts for low disease activity in SLE in order to serve as the basis of a future consensus for standardization.Methods: The PubMed database was searched for relevant articles from inception up to July 2018. Medical Subject Headings (MeSH terms) included “lupus” AND “low disease activity” OR “minimal disease activity”.Results: Three different definitions of low disease activity in lupus have been proposed. Minimal disease activity (MDA) is defined as a clinical SLE Disease Activity Index 2000 (SLEDAI-2K)≤1 on antimalarials, immunosuppressives in standard doses and prednisone ≤5 mg/day. Low disease activity (LDA) allows for a clinical SLEDAI-2K≤2 maintained on antimalarials only. Lupus Low Disease Activity State (LLDAS) accepts a SLEDAI-2K≤4 with no activity from major organ systems, a Physician's Global Assessment of ≤1 with no new activity, prednisone dose ≤7.5 mg/day and standard doses of antimalarials, immunosuppressives and biologics. Active serology (anti-dsDNA and complement C3/C4) is not included in the MDA and LDA but counts towards disease activity in the LLDAS definition. All definitions were associated with less damage-accrual and mortality in the long-term that were comparable to those of clinical remission.Conclusions: There is solid evidence that low disease activity is associated with improved outcomes in SLE and could serve as a therapeutic target in daily practice and clinical trials. Future research should focus on advancing a consensus for the best possible definition.     

Association of serum MIP-1α, MIP-1β, and RANTES with clinical manifestations,disease activity,and damage accrual in systemic lupus erythematosus

The aim of this study was to determine if macrophage inflammatory protein (MIP) 1α, MIP-1β, and RANTES (regulated upon activation normally T-cell expressed and secreted) serum concentrations are associated with clinical manifestations, disease activity, and damage accrual in patients with systemic lupus erythematosus (SLE). A cross-sectional study was performed in 62 SLE patients (per American College of Rheumatology criteria) participating in a longitudinal study and 20 healthy subjects. MIP-1α, MIP-1β, and RANTES serum concentrations were determined by enzyme-linked immunosorbent assay. Demographic parameters, clinical manifestations, serologic features, pharmacologic treatments, disease activity, and damage accrual were determined at study visit. Disease activity was assessed with the Systemic Lupus Erythematosus Activity Measure (SLAM), and disease damage was assessed with Systemic Lupus International Collaborating Clinic Damage Index (SDI). The relation between the variables was studied with the Student t test and the Pearson r correlation test. SLE patients were more likely to have higher concentrations of MIP-1β and RANTES than healthy individuals. In addition, they had a trend to have higher concentrations of MIP-1α. Patients with discoid lupus were more likely to have higher levels of MIP-1α. Elevation of MIP-1β correlated with higher SDI score. No association was found between serum chemokines levels and disease activity. In conclusion, SLE patients have higher serum levels of MIP-1β and RANTES than healthy individuals. MIP-1α is associated with discoid lupus, and MIP-1β correlates with damage accrual in SLE. This study suggests that chemokines may have a role in the pathogenesis of SLE.     

Mortality of patients with systemic lupus erythematosus admitted to the intensive care unit – A retrospective single-center study

Systemic lupus erythematosus (SLE) is a heterogeneous autoimmune disease that results in increased morbidity and mortality. Under certain conditions, patients with SLE may be admitted to the intensive care unit (ICU) secondary to infectious disease flare-ups or other non-SLE disease conditions that are aggravated by SLE. The aim of our study was to investigate the causes and outcomes of ICU-admitted patients with SLE.This is a retrospective cohort study involving paitents with SLE that were admitted to the general ICU at Sheba Medical Center between 2002 and 2015. Outcome was measured by the 30-day mortality and the Acute Physiology and Chronic Health Evaluation (APACHE) II score. Demographic, diagnostic, physiological, and laboratory variables of survivors and nonsurvivors were compared using univariate and multivariate Cox regression analyses. A receiver operating characteristic curve was plotted for significant variables to illustrate their diagnostic capabilities.Twenty-seven patients were admitted to the ICU (female: 21 [77%], mean age ± SD: 51.1 ± 15.4 years). The mean ± SD APACHE II score and 30-day mortality rate were 23.4 ± 8.3 and 29.6%, respectively. Infections, especially lower respiratory tract infections, were the cause of 66.7% of admissions and accounted for 87.5% of deaths. APACHE II scores, bacteremia, and gram-negative infections were significantly associated with mortality (p = 0.033, p = 0.022, and p = 0.01, respectively).An APACHE II score of 27 and above was the strongest predictor of mortality with a sensitivity and specificity of 83.3% and 84.2%, respectively (AUC = 0.82, p = 0.022).Patients with SLE that were admitted to the ICU with gram-negative infections, sepsis, or an APACHE II score of 27 and above have a higher mortality rate and thus should be promptly identified and treated accordingly.