Lupus

Systemic lupus erythematosus (SLE) is a systemic autoimmune disease with a dramatic sex bias, affecting 9-times more women than men. Activation of toll-like receptor 7 (TLR7) by self-RNA is a central pathogenic process leading to aberrant production of type-I interferon (IFN) in SLE, but the specific RNA molecules that serve as TLR7 ligands have not been defined. We therefore sought to identify female-specific endogenous RNAs containing canonical TLR7 stimulatory motifs. By leveraging gene expression data and the known sequence specificity of TLR7, we identified the female-specific X-inactive specific transcript (XIST) long non-coding RNA as a uniquely rich source of TLR7 ligands in SLE...

Abnormal macrophage polarization is generally present in autoimmune diseases. Overwhelming M1 macrophage activation promotes the continuous progression of inflammation, which is one of the vital reasons for the development of autoimmune diseases. However, the underlying mechanism is still unclear. Here we explore the function of RFX1 in macrophage polarization by constructing colitis and lupus-like mouse models. Both in vivo and in vitro experiments confirmed that RFX1 can promote M1 and inhibit M2 macrophage polarization...

Systemic autoimmune and autoinflammatory diseases are characterized by genetic and cellular heterogeneity. While current single-cell genomics methods provide insights into known disease subtypes, these analysis methods do not readily reveal novel cell-type perturbation programs shared amongst distinct patient subsets. Here, we performed single-cell RNA-Seq of PBMCs of systemic juvenile idiopathic arthritis (SJIA) patients with diverse clinical manifestations, including macrophage activation syndrome (MAS) and lung disease (LD)...

OBJECTIVE: The primary objective of this review is to examine which disease-modifying antirheumatic drugs (DMARDs) and biologics used to treat pregnant individuals with rheumatic conditions have been reported in observational studies using population-based health administrative data. The secondary objective is to describe which adverse pregnancy outcomes (both maternal and neonatal) have been reported, their definitions, and corresponding diagnostic and/or procedural codes. INTRODUCTION: Pregnant individuals are typically excluded from drug trials due to unknown potential risks to both mother and fetus, leaving most antirheumatic drugs understudied for use in pregnancy...

In-vitro fertilization clinics across the world currently use the body mass index (BMI) to assess risk for and determine access to in-vitro fertilization (IVF); however, clinics vary widely in both setting specific BMI limits for access to IVF and articulating the reasons for their policies. Given that scholars have begun to question the usefulness of BMI for individual health risk assessment, it is striking that ethicists have not yet systematically evaluated the reasons given for using BMI in assessing individuals' risk in IVF treatments...

A common criticism of the classification of lupus nephritis is the relative scarcity of information regarding tubular, interstitial, and vascular changes compared to the available information regarding glomerular changes, even though their potential for independent progression is known. This study reviewed the importance of less explored lesions by the current and widely used 2003 classification of lupus nephritis of the International Society of Nephrology/Renal Pathology Society (ISN/RPS), with emphasis on the tubulointerstitial, podocyte, and vascular lesions, increasingly recognised as being important in the pathogenesis and prognosis of the disease...

Castleman disease is an infrequent disease that affects the lymph nodes and related tissues. The condition may manifest with lymphadenopathy, characterized by the enlargement of the lymph nodes, alongside additional symptoms such as high fever, nocturnal sweating, exhaustion, and loss of body mass. The diagnosis of Castleman disease typically entails a multifaceted approach that includes a physical examination, imaging modalities, and a biopsy of the lymph nodes that are affected. The selection of treatment modalities is contingent upon the classification and extent of the disease...

The association between systemic lupus erythematosus (SLE) and tophaceous gout is rarely documented. It can remain unnoticed if gout peculiarly lacks clinical symptoms. This may be attributed to treating SLE with immunosuppressive agents and steroids, which can mask the inflammation caused by gout. In this case report, we will discuss the case of a 35-year-old female from an indigenous Asian ethnic group called the Hmong community living in Fresno, California. She was diagnosed with lupus nephritis (LN) and was incidentally found to have tophi in the kidney with no gout symptoms clinically...

Harnesses have become increasingly popular and whilst there are benefits to harnesses, the impact of harness design on canine biomechanics, and thus physical health and welfare is largely unknown. The purpose of this study was to assess the effect of three popular commercially available harnesses on canine locomotion in 66 domestic dogs. Dogs were filmed moving on a loose lead over a Tekscan Strideway gait analysis system. Stride length as a proportion of limb length (calculated as distance from the elbow to the floor), body weight distribution in the front versus the hind limbs (%), and minimum and maximum apparent angles of the lateral epicondyle of humerus (LEH) and greater tubercle of humerus (GTH) during the motion cycle were measured...

This study aimed to understand the profile of hydroxychloroquine-treated patients, referral patterns, and dosing and to assess the adherence of eye care providers to the latest 2016 screening guidelines provided by the American Academy of Ophthalmology. Patients were identified using electronic health records (EHR) taking hydroxychloroquine and were seen by optometrists, retinal specialists, and non-retinal ophthalmologists. Review of EHR data includes demographic characteristics, indications, and dosing profile of hydroxychloroquine use, eye care provider managing the patient, and imaging modalities performed...

Systemic lupus erythematosus (SLE) is a chronic autoimmune disease characterized by the dysregulation of B cell subpopulation and function. Recent studies have suggested a potential role of ferroptosis, an iron-dependent form of regulated cell death, in the pathogenesis of SLE. Here, we demonstrate that B-cell ferroptosis occurs both in lupus patients and MRL/lpr mice. Treatment with liproxstatin-1, a potent ferroptosis inhibitor, could reduce autoantibody production, improve renal damage, and alleviate lupus symptoms in vivo...

A 73-year-old man with dementia was referred to our clinic with hypernatremia and volume depletion. New-onset neurogenic dysphagia was likely the reason for both. The patient had chronic embolic strokes on the computed tomography (CT) images. Documentation from previous hospitalizations in different hospitals revealed a repeatedly prolonged activated partial thromboplastin time (aPTT); 5 years prior, antiphospholipid antibody syndrome had already been suspected, but the necessary workup was never completed...

Membranoproliferative glomerulonephritis (MPGN) represents a heterogeneous group of diseases. The common feature of a membranoproliferative lesion pattern in the kidney biopsy can either be idiopathic/primary or-much more frequently-have a secondary cause. The historical classification into MPGN types I to III has largely been abandoned and replaced in recent years by a pathogenesis-oriented classification. A MPGN with C1q, C3 and/or C4 deposits on light microscopy is referred to as immune complex GN (IC-GN), while a MPGN with dominant C3 deposits is referred to as C3 glomerulopathy (C3G)...

The manuscript summarizes the consensus of the Austrian Society of Nephrology on the diagnosis and therapy of lupusnephritis, which is built on existing studies and literature. We discuss in detail the immunosuppressive treatment in proliferative forms of lupusnephritis (III and IV ± V) and in pure lupusnephritis V with nephrotic-range proteinuria. Furthermore, the supportive medication in lupusnephritis is summarized in the consensus. The figures were designed to provide the reader a guidance through the therapeutical approach in lupusnephritis for the daily practice...

OBJECTIVE: The diminished expression of miR-146a in SLE contributes to the aberrant activation of the interferon pathway. Despite its significance, the underlying mechanism driving this reduced expression remains elusive. Considering the integral role of enhancers in steering gene expression, our study seeks to pinpoint the SLE-affected enhancers responsible for modulating miR-146a expression. Additionally, we aim to elucidate the mechanisms by which these enhancers influence miR-146a's contribution to the activation of interferon pathway...

INTRODUCTION: Immune-mediated skin lesions (IMSL) can be very disabling leading to treatment discontinuation. Although these lesions have rarely been previously described, the true incidence is unknown. OBJECTIVE: To explore the cumulative incidence, management and outcomes of IMSL related to bDMARD in a large cohort of patients with chronic inflammatory rheumatic diseases. To explore possible associations and risk factors for IMSL development. METHODS: A retrospective single-center study of patients with rheumatoid arthritis (RA), spondylarthritis (SpA) and psoriatic arthritis (PsA) that had been treated with at least one bDMARD for at least 6 months was conducted...

OBJECTIVES: We previously revealed the role of prolactin (PRL) in antibody production and disease activity in patients with systemic lupus erythematosus. In this study, we sought to determine whether inhibition of PRL could improve lupus-like disease in MRL/lpr mice. METHODS: The expression levels of PRL in various cell types of lupus patients were measured by flow cytometry. The effects of anti-PRL on animal survival, renal histopathology, creatinine, proteinuria, anti-dsDNA antibody, cytokine production, splenomegaly and lymphadenopathy were assessed...

IL-32 is a recently described cytokine that performs a variety of functions under inflammatory conditions. Serum IL-32 has been shown to be elevated in several diseases, including type 2 diabetes, cancer, systemic lupus erythematosus, HIV infection, and atopic diseases including atopic dermatitis. There are nine different isoforms of IL-32, with IL-32γ being the most biologically active one. The following review summarizes the different roles of the various IL-32 isoforms in the context of skin inflammation, with a focus on atopic dermatitis...

INTRODUCTION: The role of lasers in the treatment of standard therapy-resistant inflammatory dermatoses and connective tissue disorders has been controversial and evidence supporting the role of lasers in this setting is scarce. OBJECTIVE: To assess the efficacy of lasers in the management of inflammatory dermatoses and connective tissue disorders (CTD). MATERIALS AND METHODS: A retrospective case review of all inflammatory dermatoses/connective tissue diseases treated in a tertiary laser clinic between March 2010 and 2020 was undertaken...

BACKGROUND: One of the most prevalent problems of patients with systemic lupus erythematosus (SLE) is the uncertainty over an indefinite future. Uncertainty has significant effects on quality of life. The aim of this study was to explore uncertainty and personal strategies to cope with it among patients with SLE. MATERIALS AND METHODS: This qualitative study was conducted in 2020-2021 using conventional content analysis. Participants were 21 patients with SLE who were purposefully selected from rheumatology clinics in Kashan, Kerman, and Bandar Abbass in Iran...