Baseline Clinical and Serological Findings in Pediatric-Onset Discoid Lupus Erythematosus: Analysis of a Multicenter Retrospective Cohort Study
Nnenna Ezeh, Kevin Buhr, Cordellia Nguyen et. al.

Nov 13th, 2019 - Background/Purpose: DLE is a rare, disfiguring disorder in children. Small retrospective studies suggest 20-25% of patients progress to SLE. Progression risk factors are poorly understood, but DLE has been associated with delay in SLE diagnosis and reduced access to care. This multicenter retrospective cohort study aimed to describe baseline characteristics and clinical phenotypes of pediatric ...

Nocturnal Blood Pressure Dipping as a Marker of Endothelial and Cardiac Function in Pediatric-onset Systemic Lupus Erythematosus
Joyce Chang, Taylor Goldberg, Sarah McGuire et. al.

Nov 13th, 2019 - Background/Purpose: There is a need for clinically useful measures of cardiovascular (CV) risk in pediatric-onset SLE (pSLE). Nocturnal blood pressure (BP) non-dipping (loss of the physiologic nocturnal decline in BP) is a reversible marker of CV risk and potential treatment target that can be reproducibly measured by ambulatory blood pressure monitoring (ABPM). We assessed the frequency of non...

CD6-ALCAM Signaling Is Upregulated in Kidneys with Lupus Nephritis and Is Associated with Disease Activity
Evan Der, Ting Zhang, Chi Chiu Mok et. al.

Nov 13th, 2019 - Background/Purpose: Lupus nephritis (LN) is a leading cause of morbidity and mortality in Systemic lupus erythematosus (SLE) patients. However, the pathogenesis of renal disease in lupus patients is not yet fully understood. CD6 is a co-stimulatory receptor, predominantly expressed on T cells, that binds to activated leukocyte cell adhesion molecule (ALCAM), a ligand expressed on antigen presen...

Combined Trajectories of Fatigue and Disease Activity in an Inception Cohort of Lupus Patients over 10 Years
Mitra Moazzami, Vibeke Strand, Jiandong Su 3 and Zahi Touma et. al.

Nov 13th, 2019 - Background/Purpose: Fatigue is very common in SLE patients. We aim to: 1) determine if different trajectories of fatigue associate with specific latent classes of disease activity and 2) define the patient characteristics and associated factors in different latent classes. Methods: This is a retrospective analysis on a prospectively collected dataset from an inception cohort and followed from 1...

Complement Protein CL-K1 at High Concentrations Protects Against the Development of Lupus Nephritis
Anne Troldborg, Anne Voss, Søren Werner Karlsjov Hansen et. al.

Nov 13th, 2019 - Background/Purpose: Nephritis is a severe manifestation of systemic lupus erythematosus (SLE) that develops in 30 to 50 percent of the patients. At the time of diagnosis, it is not possible to predict which patients will develop nephritis. CL-K1 (collectin kidney-1, collectin-11) is a soluble C-type lectin, with collagenous stalks like C1q, that through recognition of suitably spaced patterns o...

Cell-bound Complement Activation Products in Combination with Low Complement C3 or C4 Have Superior Diagnostic Performance in Systemic Lupus Erythematosus
Sonali Narain, Daniel Wallace, Chaim Putterman et. al.

Nov 13th, 2019 - Background/Purpose: Cell-bound complement activation products (CB-CAPs) are stable forms of classical complement activation ex-vivo, with high sensitivity and specificity for systemic lupus erythematosus  (SLE). We sought to compare the performance of CB-CAPs to the gold standard low complement C3 or C4 in distinguishing SLE from other rheumatic diseases and healthy individuals. Methods: Multip...

The Role of Alterations in the Splicing Machinery in the Pathogenesis of Lupus: Does It Impact Lupus Nephritis?
Chary Lopez-Pedrera, Alejandro Ibañez-Costa, Mª Ángeles Aguirre-Zamorano et. al.

Nov 13th, 2019 - Background/Purpose: The aim of this study was to evaluate whether alterations in the splicing machinery of immune cells could influence the development and activity of the disease and the kidney involvement in systemic lupus erythematosus (SLE) patients. Methods: Monocytes, lymphocytes and neutrophils from 43 SLE patients and 34 healthy donors (HD) were purified by immunomagnetic selection. The...

Prospective Demonstration That Attainment of the Lupus Low Disease Activity State Is Associated with Improved Health Related Quality of Life
Vera Golder, Molla Huq, Rangi Kandane-Rathnayake et. al.

Nov 13th, 2019 - Background/Purpose: The Lupus Low Disease Activity State (LLDAS) is a treatment endpoint for SLE that has been shown in prospective validation studies to be associated with protection against disease flares and damage accrual. In addition to improved physician-measured outcomes, attainment of treatment endpoints should also result in improvement in patient-reported outcomes such as health relat...

Factors Associated with Cardiac Dysfunction in a Longitudinal Follow-Up of Neonatal Lupus
Amit Saxena, Peter Izmirly, Rebecca Bomar et. al.

Nov 13th, 2019 - Background/Purpose: Cardiac manifestations of neonatal lupus (NL) have been associated with significant morbidity and mortality, however there has been minimal information on long-term outcomes of affected individuals.  This study was initiated to evaluate the presence of and risk factors associated with cardiac dysfunction in NL after birth in multiple age groups to improve counseling, further...

Evaluation of the Transcriptome of Non-Lesional, Non-Sun Exposed Skin in Patients with Lupus Nephritis
Hemant Suryawanshi, Robert Clancy, Evan Der et. al.

Nov 13th, 2019 - Background/Purpose: The impact of renal injury in lupus nephritis (LN) is widespread with consequences to resident cells in other tissue beds, even non-lesional, non-sun exposed skin. Faithful reflection of a relevant renal tissue pathway in a more readily accessible compartment would allow for less invasive diagnostic alternatives. While ongoing studies are exploiting single cell RNA sequencin...

Increased CD69+ Tissue-resident Memory T (TRM) Cells and STAT3 Expression in Cutaneous Lupus Erythematosus Patients Recalcitrant to Antimalarials
Majid Zeidi, Kristen Chen, Krisha Desai et. al.

Nov 13th, 2019 - Background/Purpose: Cutaneous lupus erythematosus (CLE) is an autoimmune disease with various subsets and clinical manifestations. T lymphocytes are the predominant cell type found in lesional skin, but plasmacytoid dendritic cells (pDCs) and myeloid dendritic cells (mDCs) also play an important role in the pathogenesis. Oral antimalarials, including hydroxychloroquine (HCQ) and quinacrine (QC)...

Transcriptomic Meta-analysis of Lupus Affected Tissues Reveals Shared Immune, Metabolic, and Biochemical Dysregulation
Kathryn Kingsmore, Sarah Heuer, Erika Hubbard et. al.

Nov 13th, 2019 - Background/Purpose: Systemic lupus erythematosus (SLE) affects various organs and tissues, but whether pathologic processes in each organ are distinct or whether dysregulated molecular functions are found in common in all tissues is not known. We, therefore, conducted a meta-analysis of gene expression profiles in four affected SLE tissues to identify commonly dysregulated pathways. Methods: Ge...

Efficacy and Safety of Intravenous Belimumab in Children with Systemic Lupus Erythematosus: An Across-Trial Comparison with the Adult Belimumab Studies
Antonio Nino, Damon L Bass, Gina Eriksson et. al.

Nov 13th, 2019 - Background/Purpose: Belimumab (BEL) is the first treatment approved for children with systemic lupus erythematosus (SLE) from 5 years of age and older.1 This approval was based on the PLUTO trial (NCT01649765), which was designed to assess the safety and efficacy of BEL in children with SLE.2 Additional evidence to support the consistency of effect of BEL in children included an across-trial co...

Systemic Lupus Erythematous Risk Alleles Drive Autoimmune Features in a Population Without Diagnosed Autoimmune Diseases
April Barnado, Lee Wheless, Leslie Crofford 2 and Joshua Denny et. al.

Nov 13th, 2019 - Background/Purpose: Systemic lupus erythematosus (SLE) is a heterogeneous disease with > 100 known risk alleles. Some of these risk alleles associate with ACR SLE criteria such as renal disease. We examined if nine of these SLE risk alleles would identify autoimmune features in a population without diagnosed autoimmune diseases. Methods: We investigated subjects from BioVU, a databank that link...

Prevalence of Systemic Lupus Erythematosus in the United States: Preliminary Estimates from a Meta-Analysis of the Centers for Disease Control and Prevention Lupus Registries
Emily Somers, Lu Wang, W Joseph McCune et. al.

Nov 13th, 2019 - Background/Purpose: The heterogeneity of the clinical manifestations of systemic lupus erythematosus (SLE) and lack of a diagnostic test make SLE difficult for epidemiologists to study. The Centers for Disease Control and Prevention (CDC) supported five population-based SLE surveillance registries, using harmonized methodology, to better estimate incidence and prevalence of SLE in diverse areas...

BATF2 Contributes to Interferon Dysregulation in SLE Keratinocytes
Jessica Turnier, Celine Berthier, Lam Tsoi et. al.

Nov 12th, 2019 - Background/Purpose: Skin inflammation can drive systemic disease in SLE, thus it is essential to understand the key regulators of the aberrant inflammatory response in lupus skin. We have previously identified BATF2 as a transcription factor that is highly overexpressed in lupus keratinocytes following type I IFN exposure. The objective of this study was to determine the role of BATF2 in regula...

Cytokine and Autoantibody Profiles During Treatment with Belimumab in Patients with Systemic Lupus Erythematosus
Ioannis Parodis, Emil Åkerström, Christopher Sjöwall et. al.

Nov 12th, 2019 - Background/Purpose: The anti-BAFF monoclonal belimumab is approved for the treatment of systemic lupus erythematosus (SLE) since 2011. Effects of belimumab on anti-double stranded (ds)DNA levels, as well as serum levels of B cell activating factor (BAFF) and a proliferation-inducing ligand (APRIL), both belonging to the tumour necrosis factor (TNF) ligand superfamily, have been demonstrated in ...

Clinical Evidence Supporting Therapeutic Potential of Activating the Immune Checkpoint Receptor BTLA in SLE
Matthew Linnik, Adam Godzik, Lukasz Jaroszewski et. al.

Nov 12th, 2019 - Background/Purpose: Background/Purpose:  Immune checkpoint inhibitors have demonstrated durable benefit in some cancer patients.  However, checkpoint inhibitors are also associated with new onset autoimmune adverse events.  The link between checkpoints and autoimmune disease suggests that activating immune checkpoint receptors might be useful in treating autoimmune diseases like SLE.  As the ro...

Burden of Systemic Lupus Erythematosus Among Korean Women in Childbearing Years Based on the National Health Insurance Service Data
Min Kyung Chung, Jin Su Park, Hyun Sun Lim et. al.

Nov 12th, 2019 - Background/Purpose: Most women with systemic lupus erythematosus (SLE) are diagnosed with the disease in their reproductive ages, but the burden of SLE among women in childbearing years remains incompletely understood. We aimed to investigate the burden of SLE among Korean women in childbearing years in terms of prevalence and incidence of disease, prevalence of comorbidity, medication dispensi...

Clinical Impact of Decreasing Hydroxychloroquine Dose According to the 2016 American Academy of Ophthalmology Guidelines in Patients with Systemic Lupus Erythematosus
Nicolle Medina-Cintrón, Ileana Vázquez-Otero, Mariangelí Arroyo-Ávila et. al.

Nov 12th, 2019 - Background/Purpose: Hydroxychloroquine (HCQ) is one of the main drugs used for the treatment of systemic lupus erythematosus (SLE). Nonetheless, HCQ-induced retinal toxicity remains a major concern. To reduce the risk of HCQ-induced retinopathy, in 2016 the American Academy of Ophthalmology recommended a maximum daily HCQ dose of ≤5.0 mg/kg real weight. However, it is uncertain if this recommen...