Lupus

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The prevalence of systemic lupus erythematosus (SLE) varies significantly based on ethnicity rather than geographic distribution; thus, the prevalence is higher in Asian, Hispanic, and Black African populations than in European populations. The risk of developing lupus nephritis (LN) is the highest among Asian populations. Therefore, we hypothesize that human genetic diversity between races has occurred through the early human migration and human genetic adaptation to various environments, with a particular focus on pathogens...

The objective of this study was to analyze complement activation in antiphospholipid antibody (aPL)-positive patients without other systemic autoimmune rheumatic diseases, using C3/C4 and cell-bound complement activation products (CB-CAPs) (B-lymphocytes [BC4d], erythrocytes [EC4d], and platelets [PC4d]). Persistently aPL-positive patients with or without aPL-related clinical manifestations (thrombotic APS [TAPS], microvascular APS [MAPS], obstetric APS, thrombocytopenia [TP], and/or hemolytic anemia [HA]) were enrolled in a single center study...

Tertiary lymphoid structures (TLSs) are formed in tissues targeted by chronic inflammation processes, such as infection and autoimmunity. In Sjögren's disease, the organization of immune cells into TLS is an important part of disease progression. Here, we investigated the dynamics of tissue resident macrophages in the induction and expansion of salivary gland TLS. We induced Sjögren's disease by cannulation of the submandibular glands of C57BL/6J mice with LucAdV5. In salivary gland tissues from these mice, we analyzed the different macrophage populations prior to cannulation on day 0 and on day 2, 5, 8, 16 and 23 post-infection using multicolored flow cytometry, mRNA gene analysis, and histological evaluation of tissue specific macrophages...

Longitudinally extensive transverse myelitis (LETM) is a rare but severe neurological complication of systemic lupus erythematosus (SLE). The existing literature contains only limited information about this condition. We present a case of a 38-year-old female with SLE who presented with quadriparesis. Magnetic resonance imaging (MRI) of the brain and spinal cord showed T2-weighted high signal intensity involving the brainstem, bilateral middle and inferior cerebellar peduncles, and C1-C7 spinal cord segments...

INTRODUCTION: Antimalarials (AMs) are old drugs with a wide range of beneficial effects in systemic lupus erythematosus (SLE) beyond the control of lupus activity, including protection against damage accrual and improved survival. Nowadays, hydroxychloroquine (HCQ) is considered the background therapy for every patient with SLE. Despite this well-proven long-term efficacy, the most recent debate is focused on defining the optimal doses to assure the best benefit/risk ratio. AREAS COVERED: We have reviewed the pharmacological basis underlying the various therapeutic effects of AMs, with special attention put on the pharmacokinetics, in order to better understand the conflicting results regarding HCQ dosing...

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We analysed the change in the positioning of belimumab (BLM) in systemic lupus erythematosus (SLE) treatment in the first decade of real-life use, by providing data about patients treated by this biological drug in the Sapienza Lupus Cohort. We evaluated SLE patients treated by BLM according to the current clinical practice. Data of each patient were collected, focusing on previous and concomitant treatments. Finally, the drug retention rate was assessed. Since August 2013, 138 SLE patients started BLM (M/F 7/131; median age 49 years, IQR 13...

OBJECTIVES: Chorea, characterised by involuntary, irregular movements, is a rare neurological manifestation of antiphospholipid syndrome (APS). The specific clinical features remain unclear. This study aimed to summarise the available evidence on antiphospholipid antibody (aPL)-associated chorea. METHODS: We used a mixed-methods approach, combining data from patients with chorea with aPL positivity admitted to Peking Union Medical College Hospital (PUMCH) from 2014 to 2024, with cases identified in public databases since 1983...

The article by Tani et al, "Anifrolumab in Refractory Systemic Lupus Erythematosus: A Real-World, Multicenter Study,"1 evaluates the efficacy of anifrolumab (ANI) in patients with systemic lupus erythematosus (SLE) who failed to respond to other treatments, including biologics.There are many unmet needs in the treatment of SLE. Despite recent advances, remission and low disease activity (LDA) rates remain suboptimal; therefore, some patients with SLE are at high risk of developing endstage renal disease, organ damage, drug-related toxicities-particularly related to the prolonged use of glucocorticoids (GCs)-and premature mortality...

BACKGROUND: The association between connective tissue diseases (CTDs), including rheumatoid arthritis (RA), systemic lupus erythematosus (SLE), and scleroderma, and complications following aesthetic surgery is under-investigated. We hypothesized that the risk of complications following aesthetic surgery was higher in patients with these connective tissue disorders compared to matched non-CTD patients. METHODS: All patients diagnosed with RA, SLE, and scleroderma who underwent aesthetic surgery at our institution from 2003-2022 were reviewed...

Five mammalian Toll-like receptors (TLR 3, 7, 8, 9, and 13) recognize nucleic acids (NA) and induce signals that control the function of multiple immune cell types and initiate both innate and adaptive immune responses. While these receptors enable recognition of diverse microbial threats, in some instances, they respond inappropriately to self-NA released from host cells and drive the development of autoimmune diseases. Specifically, activation of TLR7 and TLR8 by self-RNA and TLR9 by self-DNA has been linked to development of a collection of systemic autoimmune or autoinflammatory disorders, including systemic lupus erythematosus, systemic juvenile idiopathic arthritis, and macrophage activation syndrome...

Monoclonal antibody therapies for cancer have demonstrated extraordinary clinical success in recent years. However, these strategies are thus far mostly limited to specific cell surface antigens, even though many disease targets are found intracellularly. Here we report studies on the humanization of a full-length, nucleic acid binding, monoclonal lupus-derived autoantibody, 3E10, which exhibits a novel mechanism of cell penetration and tumor specific targeting. Comparing humanized variants of 3E10, we demonstrate that cell uptake depends on the nucleoside transporter ENT2, and that faster cell uptake and superior in vivo tumor targeting are associated with higher affinity nucleic acid binding...

BACKGROUND/OBJECTIVE: Despite advances in clinical care and treatment options, adolescents with lupus continue to experience adverse health outcomes. Poor adherence to medication regimens is a major contributor to these negative outcomes. The utility of short message service (SMS) in tracking barriers to adherence prospectively remains untested for adolescents with lupus. Our objectives were (1) feasibility of incorporating a Health Insurance Portability and Accountability Act (HIPAA)-compliant SMS text messaging application into REDCap and (2) acceptability of using SMS text messaging to track barriers to medication adherence in adolescents with lupus...

INTRODUCTION: Connective tissue diseases (CTDs) are autoimmune diseases with multiorgan involvement. CTDs present with a heterogeneous clinical manifestation, especially in the cutaneous system. This study aimed to describe the common cutaneous manifestations of CTDs, to determine the association with antinuclear antibody (ANA) and other associated antibodies, and to assess the impact of CTDs on patient's quality of life (QOL). MATERIAL AND METHODS: This was a cross-sectional study conducted among patients 18 years and above, with a confirmed diagnosis of systemic lupus erythematosus (SLE), systemic sclerosis, dermatomyositis, mixed connective tissue disease (MCTD) or overlap syndrome, who attended the rheumatology clinic at Hospital Sultan Ismail Johor Bahru between March 2023 to June 2023...

BACKGROUND: Although high serum levels of interleukin (IL)-17 and its producing cells have been found in rheumatoid arthritis (RA) and systemic lupus erythematosus (SLE) in earlier research, it is still unclear how these findings relate to disease activity. OBJECTIVES: This study examines the link between serum levels of IL-17 and the activity of both RA and SLE. DESIGN: This pilot case-control study included 100 patients with RA, 100 with SLE, and 100 healthy controls...

BACKGROUND: Previous studies have suggested an association between Type 1 diabetes (T1D) and autoimmune diseases (AIDs), but the causal relationship remains unclear. Therefore, this study utilizes publicly available Genome-Wide Association Studies (GWAS) databases and employs a two-sample Mendelian Randomization (MR) approach to explore the causal relationships between T1D and systemic lupus erythematosus (SLE), rheumatoid arthritis (RA), and inflammatory bowel disease (IBD). METHODS: Summary GWAS data for T1D, SLE, RA, and IBD were downloaded from open GWAS databases and the International Inflammatory Bowel Disease Genetics Consortium (IIBDGC)...

Transient receptor potential canonical 6 (TRPC6) variants, which were initially detected in adult-onset familial focal segmental glomerulosclerosis (FSGS), were also identified in pediatric-onset one. Here, we present a patient with adult-onset steroid-resistant nephrotic syndrome (SRNS) who harbored a likely pathogenic TRPC6 variant and partially responded to calcineurin inhibitors (CNIs). A 44-year-old woman with stable rheumatoid arthritis, systemic lupus erythematosus, and Sjögren's syndrome was presented with nephrotic syndrome...

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Tyk2 is a member of the JAK kinase family. It is an important mediator in pro-inflammatory signalling, implicated in both innate and adaptive immune system. Activation of Tyk2 is believed to be integral to cellular processes that contribute to the development and progression of autoimmune disorders. Selective targeting of Tyk2 may reduce the number of adverse events as compared to non-selective JAK inhibitors. Therefore, in recent years there has been a growing body of research examining the inhibition of Tyk2 as a therapeutic intervention in autoimmune disease...