Lupus

BACKGROUND: Thalidomide has been used successfully in a variety of chronic refractory inflammatory dermatological conditions with underlying autoimmune or infectious pathogenesis. It was first used for refractory discoid lupus erythematosus (DLE) in 1983 and has steadily grown since then. METHOD: In this review, we describe the therapeutic benefits of thalidomide for DLE treatment and its biological properties. We explain how new discoveries in DLE pathogenesis are relevant to understand thalidomide's mechanism of action and the need to find an alternative safe drug with similar therapeutic effects...

Here, we report a family with two children (the elder son and younger daughter) diagnosed with juvenile-onset systemic lupus erythematosus (SLE) and the father diagnosed with hereditary angioedema. Serum C1 inhibitor (C1-INH) levels were low, and clinical exome next-generation sequencing detected a frameshift mutation in the SERPING-1 gene in all three patients. The mother had neither of the clinical phenotypes. The son had cutaneous symptoms, fever and polyarthralgia, along with lupus nephritis, and thus required rituximab therapy as well as mycophenolate mofetil and low-dose steroids to control disease activity...

INTRODUCTION: Autoimmune hemolytic anemia (AIHA) is a serious manifestation of systemic lupus erythematosus (SLE) associated with significant morbidity and mortality. In order to more fully understand the causative pathways, we utilized sera from subjects with SLE and active AIHA, or a history of AIHA, to evaluate the classical complement pathway, anti-erythrocyte antibodies, and immune complexes. METHODS: To evaluate antibody-mediated complement activation on the surface of erythrocytes, as occurs in AIHA, blood type O erythrocytes were incubated with sera from 19 subjects with SLE and a history of AIHA...

BACKGROUND: Nephrotic syndrome is associated with an increased risk of venous thromboembolism (VTE). However, the risk factors of VTE in nephrotic syndrome, other than hypoalbuminemia and severe proteinuria, are not well established. Therefore we aimed to investigate the risk factors of VTE in patients with nephrotic syndrome. METHODS: This retrospective cohort study used data from a Japanese nationwide claims database. We identified patients ≥18 years of age hospitalized with nephrotic syndrome...

No abstract text is available yet for this article.

Harvest can affect vital rates such as reproduction and survival, but also genetic measures of individual and population health. Gray wolves (Canis lupus) live and breed in groups and effective population size is a small fraction of total abundance. As a result, genetic diversity of wolves may be particularly sensitive to harvest. We evaluated how harvest affected genetic diversity and relatedness in wolves. We hypothesized that harvest would 1) reduce relatedness of individuals within groups in a subpopulation but increase relatedness of individuals between groups due to increased local immigration, 2) increase individual heterozygosity and average allelic richness across groups in subpopulations and, 3) add new alleles to a subpopulation and decrease the number of private alleles in subpopulations due to an increase in breeding opportunities for unrelated individuals...

Systemic lupus erythematosus (SLE) is a multifactorial autoimmune disease without an effective and safe treatment. Besides, macrophages are the major components of the innate immune system and play a critical role in the inflammation process in SLE. Secoiridoids from olive tree are phenolic compounds which have shown important pharmacological effects. Particularly, oleuropein (OL) has shown antioxidant, anti-inflammatory and immunomodulatory properties suggesting a potential application in a large number of inflammatory and reactive oxygen species (ROS)-mediated diseases...

Intractable epilepsy remains a significant medical challenge, resulting in recurrent and prolonged intensive care unit (ICU) admissions. Autoimmune encephalitis is emerging as a treatable cause of intractable epilepsy. It is characterized by antibodies against cerebral antigens, such as potassium channels such as leucine-rich, glioma inactivated 1 (LGI1) and contactin-associated protein 2 (CASPR2), calcium channels such as the voltage-gated calcium channel (VGCC), or neurotransmitter receptors such as the α -amino-3-hydroxy-5-methyl-4-isoxazolepropionic acid receptor (AMPAR), gamma aminobutyric acid receptor (GABAR), and N -methyl-D-aspartate receptor (NMDAR)...

Tinea incognito is a variant of dermatophyte infection of the skin modified by an erroneously applied topical or systemic steroid. Aim of the study: to describe the various clinic-epidemiological aspects of tinea incognito found among our patients. A prospective study was carried out in the Department of Dermatology, Basra Teaching Hospital, Basra, Iraq. The clinical diagnosis was confirmed by mycological tests. Clinical data were described in more detail and the lesions were classified according to the site, shape, and extent...

Systemic Lupus Erythematosus (SLE), among many other auto-immune diseases, is known to be more prevalent in females than in males. This observation has served as the foundation for studies into how sex hormones may interact with the immune system to either drive or inhibit immune activation. Early studies using castration in lupus mouse models showed the potential protective effect of testosterone against lupus development. These studies were later corroborated by observational studies in lupus patients, who upon treatment with testosterone therapy, displayed decreased disease burden...

Peptidylarginine deiminase 4 (PAD4), encoded by PADI4 , plays critical roles in the immune system; however, its contribution to the pathogenesis of lupus nephritis remains controversial. The pathological roles of PAD4 were investigated in lupus model mice. An imiquimod (IMQ)-induced lupus model was analyzed in wild-type (WT) and Padi4 -knockout (KO) mice. Proteinuria, serum anti-double stranded DNA (anti-dsDNA) antibody, and renal infiltrated cells were evaluated. Neutrophil migration and adhesion were assessed using adoptive transfer and adhesion assay...

Cutaneous lupus erythematosus (CLE) is an autoimmune disease with a broad range of cutaneous manifestations. In skin lesions of CLE, keratinocytes primarily undergo apoptosis. Interferon-κ(IFN-κ) is belonged to type I interferons (type I IFNs) and is selectively produced by keratinocytes. Recently, keratinocytes selectively produced IFN-κ is identified to be a key to trigger type I interferon responses in CLE. Other immune cells such as plasmacytoid dendritic cells (pDCs) are identified to be relevant origin of type I interferons (type I IFNs) which are central to development of CLE lesions and responsible for mediating Th1 cell activity...

OBJECTIVES: We wanted to estimate the magnitude of the risk from all-cause, cause-specific and sex-specific mortality in patients with SLE and relative risks compared with matched controls and to evaluate the influence of exposure to medication on risk of mortality in SLE. METHODS: We conducted a population-based cohort study using the Clinical Practice Research Datalink, Hospital Episode Statistics and national death certificates (from 1987 to 2012). Each SLE patient (n = 4343) was matched with up to six controls (n = 21 780) by age and sex...

Systemic lupus erythematosus (SLE) is an autoimmune disorder with prevalent hypertension and renal disease. To avoid side effects of immunosuppressive drugs, alternative therapies are needed. Curcumin has been used in Eastern medicine for its anti-inflammatory and antioxidant properties. This study tested whether oral curcumin administration attenuates autoimmunity and renal injury during SLE. Female NZBWF1 (model of SLE) and NZW/LacJ (control) mice were administered curcumin (500 mg kg-1  day-1 , oral gavage) for 14 days in two separate groups beginning at either 26 or 32 weeks of age...

Bcl-3 is an atypical member of the IκB family that modulates NF-κB activity in nuclei. lpr mice carry the lpr mutation in Fas, resulting in functional loss of this death receptor; they serve as models for lupus erythematosus and ALPS. To explore the biologic roles of Bcl-3 in this disease model we generated BL6/lpr mice lacking Bcl-3. Unlike lpr mice on an MRL background, BL6/lpr mice present with very mild lupus- or ALPS-like phenotypes. Bcl-3 KO BL6/lpr mice, however, developed severe splenomegaly, dramatically increased numbers of double negative T cells - a hallmark of human lupus, ALPS and MRL/lpr mice - and exhibited inflammation in multiple organs, despite low levels of autoantibodies, similar to those in BL6/lpr mice...

OBJECTIVE: The goal of these studies is to discover novel urinary biomarkers of lupus nephritis (LN). METHODS: Urine from systemic lupus erythematosus (SLE) patients was interrogated for 1000 proteins using a novel, quantitative planar protein microarray. Hits were validated in an independent SLE cohort with inactive, active non-renal (ANR) and active renal (AR) patients, in a cohort with concurrent renal biopsies, and in a longitudinal cohort. Single-cell renal RNA sequencing data from LN kidneys were examined to deduce the cellular origin of each biomarker...

Leprosy is an infectious disease with chronic evolution, caused by Mycobacterium leprae, an acid-fast bacillus that mainly affects the skin and peripheral nervous tissue. Many of the clinical manifestations of leprosy can mimic connective tissue diseases. The authors present the case of a 49-year-old woman who had been treated for four years for systemic lupus erythematosus in a rheumatological service. Skin biopsy of a plaque on the inguinal region was compatible with borderline lepromatous leprosy associated with a type 1 lepra reaction...

BACKGROUND: The long-term outcome of optic neuritis (ON) is heterogeneous, and it appears to vary among different populations. Identification of determinants of natural history of ON would be helpful in therapeutic planning and prognostication, while relevant investigations in Asian patients were less reported. This study aims to clarify whether comorbidities and treatment in ON patients are associated with differential risks of subsequent development of multiple sclerosis (MS) or neuromyelitis optica (NMO) in Taiwan...

Systemic lupus erythematosus (SLE) involves excessive autoimmune reactions, with pathogenesis characterized by autoantibody production. Although the specific mechanism underlying the development of neuropsychiatric syndromes in SLE (NPSLE) is still unclear, recent studies indicate the involvement of autoimmune pathophysiology. We previously identified the presence of anti- N -methyl-d-aspartate receptor subunit GluN2 antibody (anti-GluN2) as a functional autoantibody which is able to impair neurons and is essential for the diagnosis of diffuse psychiatric/neuropsychological syndromes in NPSLE (dNPSLE)...

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