Chronic Systemic Lupus Erythematosus Research Articles

Extrafollicular CD19lowCXCR5−CD11c− double negative 3 (DN3) B cells are significantly associated with disease activity in females with systemic lupus erythematosus

ObjectiveB cells play a major role in the development and maintenance of systemic lupus erythematosus (SLE). Double negative (DN) B cells defined by the lack of surface expression of IgD and CD27 have attracted recent interest for their sensitivity to Toll-like receptor 7 (TLR7) ligands and their potential role in the production of autoantibodies. Here we aimed at investigating the possible association of DN B cells and their subsets with SLE disease activity specifically in female patients, in which TLR7 gene has been reported to escape X chromosome inactivation. MethodsPeripheral blood mononuclear cells were purified from woman participating to the clinically well-characterized Swiss SLE Cohort Study (SSCS). PBMC from age-matched healthy females were used as controls. PBMC were stained for cell surface markers, intracellular Tbet and analyzed by multicolor cytofluorimetry. Single nucleotide TLR7 polymorphisms were assessed by polymerase chain reaction. ResultsThe median SLE disease activity index of the 86 females was 2, IQR [0–6], all but 8 were under chronic SLE treatment. B cells co-expressing CD11c and Tbet were increased, the mean fluorescence intensity (MFI) of CD19 was considerably reduced and we observed a large increase in CD11c + CXCR5-and CD11c-CXCR5-concomitantly with a reduction of CD11c-CXCR5+ B cells in SLE compared to 40 healthy donors (HD). When focusing on the DN B cell subset, we found a reduction of DN1 (CD11c-CXCR5+) and an increase of DN2 (CD11c + CXCR5-) and most impressively of DN3 (CD11c-CXCR5-) cells. The DN subset, particularly DN3, showed the lowest level of CD19 expression. Both DN1 and DN3 percentages as well as the CD19 MFI of DN cells were associated with SLE disease activity. The use of glucocorticoids, immunosuppressants, and antimalarials impacted differentially on the frequencies of DN B cell subsets. CD19 MFI in B cells and the percentage of DN3 were the strongest biomarkers of disease activity. The TLR7 snp3853839 G allele was associated with increased percentages of B cells and CD19+CD11c-CXCR5+ and decreased CD19+CD11c-CXCR5-. ConclusionsDN3 B cells are strongly associated with SLE clinical activity pointing to their potential involvement in disease pathogenesis, and CD19 expression level performs accurately as disease activity biomarker.

Clinical and laboratory assessment of the state of periapical tissues in systemic lupus erythematosus.

Aim: To evaluate changes in clinical and laboratory parameters in patients with SLE depending on the severity and activity of the disease. Materials and Methods: The study included 50 patients with SLE of different age groups, 10 men and 40 women. Results: Systemic lupus erythematosus (SLE) is a chronic disease that belongs to the group of rheumatic diseases and is characterised by autoimmune tissue damage. Chronic inflammatory periodontal diseases remain one of the most common dental pathologies. Chronic gingivitis and periodontitis in SLE are described as one of the earliest and most striking symptoms of the disease. Approximately the same trend was found in patients with SLE depending on the degree of clinical and laboratory activity of the disease. Patients with minimal, 1 degree of activity (characterized by the longest duration of SLE) showed the greatest decrease in bone mineral density (up to 2.25 points), and patients with higher activity had a significantly shorter duration of SLE and, accordingly, a smaller decrease in bone mineral density: in patients with 2 degrees of activity (1.79 points), with 3 degrees of activity (1.94 points). Conclusions: In patients with acute SLE, the maximum value of the Muhlemann-Cowell index was 2.31 points, in patients with subacute SLE - 1.89 points, and in patients with chronic SLE - 1.58 points. CPITN values increase inversely with the nature of the course of SLE, which is associated with the duration of the underlying disease.

Risk factors for severe COVID-19 among patients with systemic lupus erythematosus: a real-world analysis of a large representative US administrative claims database, 2020–2021

ObjectivesTo identify risk factors for progression to severe COVID-19 and estimate the odds of severe COVID-19 associated with vaccination among patients with systemic lupus erythematosus (SLE).MethodsThis retrospective cohort study identified...

Mental disorders in patients with systemic lupus erythematosus: association with activity and the course of rheumatic disease

To clarify the relationship between the clinical and psychopathological features of mental disorders, clinical and laboratory manifestations of activity and the nature of the course of systemic lupus erythematosus (SLE). The study included 119 patients - 98 (82.4%) women, mean age 36.5±12.4 years (M±SD) - with a reliable diagnosis of SLE (EULAR/ACR 2019 criteria), 51 (29.5%) of them - with secondary antiphospholipid syndrome - APS (International criteria of 2006). Among patients with SLE a high frequency of anxiety-depressive spectrum disorders (ADSD) and cognitive impairment (CI) was revealed. There was an association of greater severity of depression with high SLE activity index, acute/subacute onset of the disease course according to the classification of V.A. Nasonova, relapsing-remitting and chronic active current disease activity patterns of SLE according to the classification of S. Barr - M. Petri. Anxiety disorders were associated with subacute onset and relapsing-remitting disease activity patterns of SLE and were not associated with SLE activity index. Bipolar disorder was detected more often in patients with chronic SLE. Acute psychosis/delirium was associated with acute onset of SLE. Organic CI was associated with APS, chronic onset and long quiescent disease activity patterns of SLE. The episindrome and schizotypal disorder in patients with SLE are more often caused by concomitant APS. Patients with high SLE activity index should be of particular concern to rheumatologists regarding the diagnosis of depressive disorders. Patients with concomitant APS need timely diagnosis and treatment of CI and episindrome in order to improve the prognosis of the disease and the overall quality of life.

Does periodontitis play a causal role in the Systemic lupus erythematosus? A systematic review

Systemic lupus erythematosus (SLE) is an autoimmune disease, with a relatively unclear etiology. Some evidence confirmed the effects of chronic infections on the development of autoimmune conditions. The association between periodontitis and rheumatoid arthritis as an autoimmune disease is assessed in several studies, which guides the readers to the possible role of periodontitis and oral dysbiosis in other autoimmune diseases, such as SLE. This study aimed to determine the relationship between SLE and periodontitis. For the purpose of the study, the articles focused mainly on the relationship between SLE and periodontitis published up to 2019 were searched in three databases, including Google Scholar, PubMed, and Scopus. Moreover, some articles were found using manual search. Finally, 18 studies were selected after the detailed evaluation of the full-text versions. The majority of the entered studies confirmed a high prevalence of periodontitis diseases (PD) and the destruction of periodontal tissues in patients with SLE, which was estimated between 60% and 93.8%. This finding can be attributed to the differences in genetic and environmental backgrounds. The relationship between chronic periodontitis (CP) and SLE depends on the common multiple factors. Periodontal and inflammatory diseases have a common origin, and both of them have several components of the immune system. In general, two main predisposing factors of SLE and periodontitis diseases are similar genetic vulnerability and environmental background. On the other hand, it should not be neglected to consider the role of immune dysfunction and immunosuppressive treatment in the initiation and development of CP.

Bio-inspired adhesive porous particles with human MSCs encapsulation for systemic lupus erythematosus treatment

Mesenchymal stem cells (MSCs) therapy is a promising treatment for Systemic lupus erythematosus (SLE) patients. However, this method is encumbered by suboptimal phenotype of MSCs used in clinical settings, and a short in vivo persistence time. Herein, inspired by the natural microstructure of the sand tower worm nest, we proposed novel adhesive porous particles with human MSCs encapsulation via microfluidic electrospray technology for SLE treatment. The porous microparticles were formed by immediate gelation reaction between sodium alginate (ALG) and poly-d-lysine (PDL), and then sacrificed polyethylene oxide (PEO) to form the pores. The resultant microparticles could protect MSCs from immune cells while maintain their immune modulating functions, and achieve rapid exchange of nutrients from the body. In addition, owing to the electrostatic adsorption and covalent bonding between PDL and tissues, the porous microparticles could adhere to the bowel surfaces tightly after intraperitoneal injection. Through in vivo imaging system (IVIS) methods and in vivo study, it was demonstrated that the MSCs-encapsulated porous adhesive microparticles could significantly increase the cellular half-life, turn activated inflammatory macrophages into an anti-inflammatory profile, and ameliorate disease progression in MRL/lpr mice. Thus, the MSCs-encapsulated porous microparticles showed distinctive functions in chronic SLE treatment, with additional potential to be used in a variety of biomedical applications.

A case report of heart transplant for ischaemic cardiomyopathy from lupus coronary vasculitis.

BackgroundCoronary vasculitis is a rare, life-threatening complication of systemic lupus erythematosus (SLE).Case summaryA 23-year-old woman with SLE presented with typical angina and worsening dyspnoea on exertion. Coronary angiography revealed severe triple vessel disease with a ‘string of beads’ appearance classic for coronary vasculitis. Transthoracic echocardiogram revealed ejection fraction of 25–30% with a severely hypokinetic distal septum and distal anterior wall and an akinetic apical wall. Despite vasculitis treatment with cyclophosphamide and pulse-dose steroids, her coronary vasculitis did not improve. She was refractory to anti-anginal and guideline-directed medical therapy for heart failure and successfully underwent orthotopic heart transplant (OHT).DiscussionThis is the first reported case of OHT in the case of SLE coronary vasculitis. Chronic SLE coronary vasculitis is caused by lymphocyic infiltration leading to inflammation and fibrosis of the major epicardial coronary arteries but can be successfully managed with OHT when refractory to medical SLE and heart failure therapies. It can affect patients of all ages with SLE, emphasizing the importance of thorough history taking and clinical evaluation in young patients presenting with cardiac symptoms to establish an appropriate diagnosis and treatment plan.

P4353Global longitudinal strain is a measure of subclinical left ventricular dysfunction in chronic inflammatory autoimmune conditions

Abstract Background Myocardial deformation indices are proposed to be a more sensitive marker of subclinical dysfunction compared to standard measures of left ventricular (LV) systolic function. We hypothesize that subclinical myocardial dysfunction is present in chronic inflammatory autoimmune diseases such as Systemic Lupus Erythematosus (SLE) and Rheumatoid Arthritis (RA), despite both conditions being mediated by different pro-inflammatory modulators. Purpose Identify subclinical myocardial dysfunction through assessment of global longitudinal strain in two different chronic autoimmune conditions, SLE and RA. Methods Consecutive patients admitted to our institution with known history of SLE or RA (>1 year disease activity) were examined. Patients with preexisting cardiac disease, LVEF <50% and those without comprehensive transthoracic echocardiograms (TTE) were excluded. Mean longitudinal LV strain was performed offline using vendor-independent software (TomTec v4.6) and compared to age- and gender-matched controls with normal LV function and no history of cardiac disease. Results Of the 86 patients examined (mean age 53.01±21.74, 85.4% female), 51 (59.3%) had SLE and 35 (40.7%) had RA. No significant difference in BMI, hypertension, hypercholesterolemia, diabetes, obesity, obstructive sleep apnea and stroke was observed between controls and patients with SLE or RA. While there was no significant difference in LVEF between RA patients and matched controls, there was a significantly lower GLS in the RA cohort. Conversely, patients with SLE had significantly lower LVEF and GLS when compared to matched controls, despite LVEF being in the normal range. See Table 1. Receiver operator curve analysis revealed that mean GLS is a better discriminator for autoimmune disease with an area under the curve of 0.829 (95% CI, 0.77 to 0.89; p<0.01) compared to LVEF with an area under the curve of 0.632 (95% CI, 0.55 to 0.72; p<0.01). Echocardiographic Parameters SLE (n=51) Controls (n=51) Sig (p value) RA (n=35) Controls (n=35) Sig (p value) LVEDV (mls) 102±30 85±20 <0.01 84±28 89±30 0.43 LVESV (mls) 36±17 29±9 0.02 26±14 30±12 0.24 Biplane LVEF % 59±6 63±4 <0.01 62±6 62±5 0.81 LV Mass (grams/m2) 96±34 72±20 <0.01 79±26 82±23 0.67 LV Mean GLS % 16.7±2.8 21.3±2 <0.01 17.8±1.7 19.1±2.5 0.02 Conclusions Our results suggest that chronic inflammatory conditions (SLE and RA) are associated with subclinical cardiac dysfunction. Impaired GLS may reflect early myocardial damage and be used as a tool for screening of patients with inflammatory conditions. Acknowledgement/Funding None

Levamisole-induced vasculitis/vasculopathy in the setting of chronic systemic lupus erythematosus

Levamisole-induced vasculitis/vasculopathy in the setting of chronic systemic lupus erythematosus

Therapeutic potential of allogeneic mesenchymal stromal cells transplantation for lupus nephritis.

Animal and human studies have suggested the potential of mesenchymal stromal cells (MSCs) to treat systemic lupus erythematosus (SLE). Here, we present the results of compassionate MSC treatments for three SLE patients to provide the proof of concept for a randomized and controlled clinical trial. Three patients of different ethnicities who suffer from chronic SLE, and who presented with class IV active proliferative nephritis confirmed by biopsy, were treated with allogeneic MSCs from healthy donors. Ninety million cells were infused intravenously into each patient during high and very high activity disease flare-ups and follow-up was continued for 9 months. Multi-organic affectation was quantified by the SLE disease activity index (SLEDAI), and indicators of lupus nephritis activity, such as proteinuria, as well as lymphocyte and monocyte antigens and anti-HLA antibodies were measured at 1, 3, 6, and 9 months after treatment. Proteinuria levels improved dramatically during the 1st month after treatment and the ameliorations were sustained throughout the follow-up period. SLEDAI scores revealed early, durable, and substantial remissions that were complete for two patients and partial for the third patient and that permitted medication doses to be reduced 50-90%. These favourable outcomes support completion of the randomized and controlled MSC trial for SLE.

The association between metabolic syndrome and its components with systemic lupus erythematosus: a comprehensive systematic review and meta-analysis of observational studies.

Based upon inflammatory-related factors in chronic systemic lupus erythematosus (SLE), as well as the long-term prescription of corticosteroids, metabolic syndrome (MetS) prevalence is expected to be higher in SLE patients than among those without SLE. The aim of this study was to systematically analyze: (1) the worldwide prevalence of MetS in patients with SLE using different criteria, (2) the risk of MetS in patients with SLE compared with those without SLE, and (3) the risk of MetS component in patients with SLE compared with healthy controls. We searched international databases, such as: Web of Science, Medline, PubMed, Scopus, Embase, CABI, CINAHL, DOAJ and Google Scholar. The articles which reported the prevalence of MetS in SLE patients, between 2006 and 2017, were included in the study if they had a: clear study design, study time and location, sound sampling approach and appropriate statistical analyses. Studies without sufficient data to determine the prevalence of MetS were excluded. Also, studies in patients suffering from other clinical diseases were not included. The meta-analyses of the prevalence (40 studies (n = 6085)) and risk (20 studies (n = 2348)) of MetS in SLE patients were conducted separately. The pooled prevalence of MetS among SLE patients was found to be 26% (95% confidence interval (CI): 22-30%), but varied from 18% (95% CI: 11-25%) to 34% (95% CI: 25-42%), depending upon the diagnostic criteria used. The overall pooled odds ratio (OR) of MetS in SLE patients, compared with healthy controls, was (OR = 2.50; 95% CI: 1.86-3.35), but this ranged from (OR = 1.23; 95% CI: 0.61-2.49) to (OR = 10.71; 95% CI: 1.33-86.48), depending upon the criteria used. Also, the risk of high fasting blood sugar (FBS; OR = 1.59; 95% CI: 1.05-2.40), low high-density lipoprotein cholesterol (HDL-C; OR = 1.43; 95% CI: 1.02-2.01), high blood pressure (BP; OR = 2.76; 95% CI: 2.19-3.47), high triglycerides (TG; OR = 2.85; 95% CI: 2.05-3.95) and high waist circumference (WC; OR = 1.37; 95% CI: 0.97-1.94) were all found to be higher in SLE patients compared with healthy controls. The risk of MetS was significantly higher in SLE patients, compared with healthy controls, even after adjusting for publication bias. Among MetS components, high TG and high BP were most strongly associated with SLE. Considering that high TG and high BP are preventable, there is an international need to implement effective interventions to reduce MetS components in SLE patients in order to prevent serious outcomes such as cardiovascular diseases and mortality.

Altered frequencies of memory B cells in new-onset systemic lupus erythematosus patients.

Systemic lupus erythematosus (SLE) is a chronic systemic autoimmune disease, characterized by B cell hyperactivity and pathogenic autoantibodies formation. The objective of this study is to evaluate the distribution of B cell subsets in patients with SLE. We included patients with SLE followed in First Affiliated Hospital of Xi'an JiaoTong University, Xi'an, China. Flow cytometry was used to measure frequencies of B cell subsets, including memory B cells, switched memory B cells, non-switched memory B cells, double-negative memory B cells, and naïve B cells in 130 patients with SLE and 55 healthy controls. The different distributions of B cell subsets were further evaluated by their associations with disease activity and clinical manifestation. SLE patients showed significant alteration of B cell subsets, including lower frequency of non-switched memory B cells and higher double-negative memory B cells. The frequencies of B cell subsets also varied between new-onset SLE patients and chronic SLE patients. Frequencies of total memory B cells, switched memory B cells, and non-switched memory B cells were lower in new-onset SLE patients and frequency of naïve B cells was higher compared with healthy controls. Chronic SLE patients showed increased switched memory B cells and double-negative memory B cells. In addition, switched memory B cells and double-negative B cells were higher in patients with lupus nephritis (LN) regardless of disease activity. Our findings suggested that abnormalities of the B cell subsets homeostasis might contribute to the pathogenesis of SLE.

Levels of C-terminal propeptide of type I procollagen and oxyproline in patients with systemic lupus erythematosus: The relationship between the course of the disease and the structural and functional state of bone tissue

The high prevalence of osteoporosis in patients with systemic lupus erythematosus (SLE) makes it necessary to study the abnormalities in bone metabolism, its relationship with reduced bone mineral density (BMD) and the impact of the disease on it. The aim of this study was to determine serum levels of C-terminal propeptide of type I procollagen (PICP) and oxyproline in patients with SLE, their comparison with the structural and functional state of the patients’ bone tissue and the course of the disease. We examined 58 female SLE patients. The mean age of the patients was 45.1 ± 1.0 years. The control group included 29 healthy individuals,corresponding in age and sex with the researched group. For every patient, data were recorded on age, body mass index (BMI), menstrual history, smoking, chronic SLE damage (SLICC/ACR DI) and disease activity score (SLEDAI), cumulative glucocorticoid dose, serum concentrations of interleukin-6 (IL-6) and C-reactive protein (CRP), bone formation marker (C-terminal propeptide of type I procollagen) and bone resorption marker (oxyproline). In all patients BMD was measured by DXA (Dual-energy X-ray absorptiometry) at two sites. To determine vertebral compression fractures, female SLE patients were examined with an x-ray of the thoracic and lumbar spine. We established that bone turnover markers showed a significant difference between the SLE patients and the control group, with lower levels of PICP and higher levels of oxyproline in the SLE patients. Alterations of bone metabolism were associated with the severity of the disease, active inflammation (high levels of CRP and IL-6), the age of the patients, and the high cumulative glucocorticoid dose but no correlation was found with disease duration, BMI and smoking. Patients with osteopenia, osteoporosis and fractures were significantly more frequently found among patients with reduced bone formation and increased bone resorption rate. Thus, our findings showed that female SLE patients have alterations of bone metabolism in the form of increasing serum oxyproline and reducing serum C-terminal propeptide of type I procollagen, the correction of which would slow the progression of adverse structural and functional changes in the bone tissue.

Catastrophic antiphospholipid syndrome (CAPS): Descriptive analysis of 500 patients from the International CAPS Registry

To analyze the clinical and immunologic manifestations of patients with catastrophic antiphospholipid syndrome (CAPS) from the "CAPS Registry". The demographic, clinical and serological features of 500 patients included in the website-based "CAPS Registry" were analyzed. Frequency distribution and measures of central tendency were used to describe the cohort. Comparison between groups regarding qualitative variables was undertaken by chi-square or Fisher exact test while T-test for independent variables was used to compare groups regarding continuous variables. 500 patients (female: 343 [69%]; mean age 38±17) accounting for 522 episodes of CAPS were included in the analysis. Forty percent of patients had an associated autoimmune disease, mainly systemic lupus erythematosus (SLE) (75%). The majority of CAPS episodes were triggered by a precipitating factor (65%), mostly infections (49%). Clinically, CAPS was characterized by several organ involvement affecting kidneys (73%), lungs (60%), brain (56%), heart (50%), and skin (47%). Lupus anticoagulant, IgG anticardiolipin and IgG anti-β2-glycprotein antibodies were the most often implicated antiphospholipid antibodies (83%, 81% and 78% respectively). Mortality accounted for 37% of episodes of CAPS. Several clinical differences could be observed based on the age of presentation and its association to SLE. Those cases triggered by a malignancy tended to occur in older patients, while CAPS episodes in young patients were associated with an infectious trigger and peripheral vessels involvement. Additionally, CAPS associated with SLE were more likely to have severe cardiac and brain involvement leading to a higher mortality (48%). Although the presentation of CAPS is characterized by multiorgan thrombosis and failure, clinical differences among patients exist based on age and underlying chronic diseases, e.g. malignancy and SLE.

Hypersplenism in liver disease and SLE revisited: current evidence supports an active rather than passive process

BackgroundActive and passive theories have been advanced to explain splenomegaly and cytopenias in liver disease. Dameshek proposed active downregulation of hematopoiesis. Doan proposed passive trapping of blood components in a spleen enlarged by portal hypertension. Recent findings do not support a passive process.DiscussionCytopenias and splenomegaly in both liver disease and systemic lupus erythematosus (SLE) poorly correlate with portal hypertension, and likely reflect an active process allocating stem cell resources in response to injury. Organ injury is repaired partly by bone-marrow-derived stem cells. Signaling would thus be needed to allocate resources between repair and routine marrow activities, hematologic and bone production. Granulocyte-colony stimulating factor (G-CSF) may play a central role: mobilizing stem cells, increasing spleen size and downregulating bone production. Serum G-CSF rises with liver injury, and is elevated in chronic liver disease and SLE. Signaling, not sequestration, likely accounts for splenomegaly and osteopenia in liver disease and SLE. The downregulation of a non-repair use of stem cells, bone production, suggests that repair efforts are prioritized. Other non-repair uses might be downregulated, namely hematologic production, as Dameshek proposed.SummaryRecognition that an active process may exist to allocate stem-cell resources would provide new approaches to diagnosis and treatment of cytopenias in liver disease, SLE and potentially other illnesses.

P103. Relationship between severe preeclampsia onset with IUGR incidence at Dr. Soetomo General Hospital in 2013

Introduction Intrauterine growth restriction can be a serious problem in neonates because it is associated with increased morbidity and mortality and long-term sequel in the future. One of the main maternal risk factors of intrauterine growth restriction is severe preeclampsia in pregnancy. As we know preeclampsia has two types, first is early onset of severe preeclampsia that caused by failed of trophoblast invasion to the spiral artery and the second is late onset of severe preeclampsia that caused by such as chronic hypertension, diabetes mellitus, obese, tuberculosis, SLE ( systemic lupus erythematosus) and other autoimmune disease. Objectives We are curious about relationship onset of severe preeclampsia with intrauterine growth restriction. This study analyzed the relationship between the onset of severe preeclampsia and the incidence of intrauterine growth restriction in Dr. Soetomo General Hospital Surabaya. The aim of this study was to prove whether early onset severe preeclampsia is a risk factor for intrauterine growth restriction compared with late-onset severe preeclampsia. Methods This was an analytic observational cross-sectional study. Sampling technique we used in this study was random sampling. The data in this study were taken from the medical records at the Department of Obstetrics and Gynecology Dr. Soetomo General Hospital Surabaya in January 2013 until December 2013, which include in these data are gestational age, preeclampsia onset, sex, birth weight, and intrauterine growth restriction incidence. The onset of severe preeclampsia was divided into two categories: first, early onset (⩽34 weeks) and second, late onset (>34 weeks). Data analysis was performed in two stages, the univariate and bivariate analysis. Data analysis used was cross tabulation and chi-square test with level of significance of 95% ( p Results Results showed that prevalence rate of IUGR in early-onset severe preeclampsia was 1.32 times higher compared with late-onset severe preeclampsia. However, there was no statistically significant association between early-onset preeclampsia and the incidence of IUGR. ( p = 0.53; PR=0.71; 95% CI = 0.25–2.07). Late-onset severe preeclampsia also had no effect on the incidence of intrauterine growth restriction ( p = 0.53; PR = 1.40; 95% CI = 0.48–4.08). Conclusion It can be concluded that the onset of severe preeclampsia had no effect on the incidence of intrauterine growth restriction.

Polyomavirus Reactivation in Pediatric Patients with Systemic Lupus Erythematosus

Polyomavirus (PyV) infection usually persists without any symptoms in normal individuals. In immunocompromised patients including patients with systemic lupus erythematosus (SLE), PyV reactivation occurs with a high prevalence and can cause severe clinical complications. In this study, reactivation of six PyV [JC, BK, WU, KI, merkel cell (MC) and trichodysplasia spinulosa (TS)] was investigated in terms of prevalence, clinical implications and correlation with urine transforming growth factor (TGF)-β1 expression in 50 SLE patients aged less than 18 years. Clinical characteristics were obtained from medical record review. PyV viruria was assessed by nested polymerase chain reaction. Urine TGF-β1 was measured with ELISA. The mean age was 13 ± 2.8 years. The prevalence of JC and BK viruria was 16% and 32%, respectively. WU, KI, MC and TS were not isolated from any urine specimens. Co-reactivation of 2 PyV was not detected. Urine TGF-β1 levels in patients with JC viruria, with BK viruria and without PyV viruria were 0.27 ± 0.09, 0.10 ± 0.05 and 0.13 ± 0.09 ng/mg of urine creatinine, respectively. Cumulative doses of cyclophosphamide per body weight and urine TGF-β1 levels were higher in JC viruria than in other groups (p < 0.05). The prevalence of JC and BK reactivation was higher in pediatric patients with SLE than in the normal population. JC reactivation in pediatric patients with SLE was correlated with the administration of high-dose cyclophosphamide and increased urine TGF-β1 levels. Surveillance of JC reactivation is recommended in pediatric patients with chronic and severe SLE receiving high-dose cyclophosphamide.

B cells twist and shout

B cells twist and shout

Concepts important to persons with systemic lupus erythematosus and their coverage by standard measures of disease activity and health status

To explore the array of concepts important to patients with chronic systemic lupus erythematosus (SLE) and to compare these with instruments assessing disease activity, damage, and health status. We conducted a qualitative focus-group study of patients with SLE concerning their problems in daily functioning. The group sessions were tape recorded, transcribed, and divided into meaning units. The concepts contained in these meaning units were extracted and linked to the International Classification of Functioning, Disability and Health (ICF). We then compared the concepts from the focus groups with those concepts covered by SLE activity scores, the Systemic Lupus International Collaborating Clinics/American College of Rheumatology Damage Index (SDI), and the Short Form 36 Health Survey (SF-36). A total of 92 concepts emerged from 5 focus groups; of these, 28 related to body functions and structures, 24 to activities and participation, and 25 to environmental factors. Two concepts were linked to the health condition itself and 6 to personal factors. Seven were not covered by the ICF. Of the 28 concepts regarding body functions and structures, 24 (86%) were covered by the combination of activity scores and the SDI. The SF-36 also addressed 3 of these concepts and contained 9 (38%) of 24 concepts in activities and participation. Although the combination of SLE activity scores, SDI, and SF-36, as suggested for SLE studies, well covers body functions and structures and includes a significant portion of problems regarding activities and participation, neither environmental nor personal factors are covered at all.

Emergence of panniculitis and haemophagocytic syndrome in a patient with chronic systemic lupus erythematosus

Panniculitis rarely occurs in the course of systemic lupus erythematosus (SLE). When it occurs, it is thought to be mainly lupus erythematosus panniculitis (LEP). Here we describe a 32-year old Japanese woman with chronic SLE, who simultaneously presented facial lymphocytic lobular panniculitis and pancytopenia due to haemophagocytic syndrome. She showed several auto-antibodies against trilineage haematopoetic cells, an anti-cardiolipin antibody, and no evidence of viral infection, indicating that her haemophagocytic syndrome might be autoimmune-associated haemophagocytic syndrome. The panniculitis and haemophagocytic syndrome presented simultaneously, and these manifestations were dramatically improved with corticosteroid therapy; therefore, the lymphocytic lobular panniculitis could be linked to autoimmune-associated haemophagocytic syndrome in this case.