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Astragaloside IV Alleviates Systemic Lupus Erythematosus by Modulating ITGB1/PTK2/p38 Pathway: Integrated Network Pharmacology and Experimental Validation.

PURPOSE: Systemic lupus erythematosus (SLE) features immune cell dysfunction, causing immune - complex and inflammatory - factor formation that damages organs. Astragaloside IV (AS-IV), a cyclic triterpene saponin from Astragalus membranaceus, has strong anti-inflammatory and immunomodulatory effects. This study aimed to evaluate AS-IV's therapeutic potential for SLE and uncover its mechanism. METHODS: MRL/lpr mice were divided into MRL/lpr, low - medium - high - dose AS-IV, and Pred groups, with C57BL/6 mice as controls. Renal damage was assessed by histopathology and electron microscopy. Im

Markers of Antiviral Response in SLE Patients After Vaccination Against SARS-CoV-2.

Patients with systemic lupus erythematosus (SLE) and lupus nephritis (LN) are at increased risk of severe infections, making effective vaccination strategies essential. While antibody responses to SARS-CoV-2 vaccination have been studied in SLE, less is known about innate immune correlates. Therefore, we evaluated cytokines with a particular emphasis on interferon and chemokine profiles. To fulfill the immunological picture, we also assessed neutralizing antibodies against SARS-CoV-2 variants, lymphocyte subpopulations, and selected gene expression signatures in 33 patients stratified by vacci

ANA-positive versus ANA-negative Antiphospholipid Antibody-positive Patients: Results from the Antiphospholipid Syndrome Alliance for Clinical Trials and International Networking (APS ACTION) Clinical Database and Repository ("Registry").

OBJECTIVES: This study focused on the prevalence and impact of ANA in aPL-positive patients without concomitant systemic autoimmune rheumatic diseases (SARDs). METHODS: Data from aPL-positive patients with or without Revised Sapporo APS classification criteria were retrieved from the Antiphospholipid Syndrome Alliance for Clinical Trials and International Networking (APS ACTION) Registry. Patients with concomitant SARDs were excluded. RESULTS: A total of 430 aPL-positive patients were included in the analysis, 56% ANA-positive (ANA+) and 44% ANA-negative (ANA-). ANA positivity was significan

Association of the energy-adjusted dietary inflammatory index and Sjögren's syndrome: a cross-sectional study.

There are no studies in the literature examining the inflammatory content and effects of the diets of patients with primary Sjögren’s syndrome (PSS). This study aimed to investigate the relationship between the Energy-Adjusted Dietary Inflammatory Index (E-DII) and anthropometric measurements, disease activity, inflammatory markers, and blood lipid concentrations in female patients with PSS. A cross-sectional study was conducted between November 2020 and November 2021, including 102 female patients with a confirmed diagnosis of PSS. Dietary intake was assessed using the multiple-pass, 3-day fo

Isolated thrombocytopenia as the initial manifestation of childhood-onset systemic lupus erythematosus: A case report.

RATIONALE: Systemic lupus erythematosus (SLE) is a rare but serious autoimmune disorder that can present with diverse clinical manifestation … autoimmune testing revealed elevated antinuclear antibodies, antidouble-stranded DNA, and decreased complement C4 levels, leading to a diagnosis of childhood-onset SLE with isolated hematologic involvement. INTERVENTION: The patient was initially treated with intravenous immunoglobulin for the management of ITP. Following the diagnosis of SLE, he was started o …

Anti-CD38 monoclonal antibody CM313 for systemic lupus erythematosus: a randomized, double-blind, placebo-controlled phase Ib/IIa trial.

CD38 is highly expressed on various immune cells, including long-lived plasma cells, making it a potential therapeutic target in autoimmune diseases. This phase Ib/IIa study aimed to explore the safety, pharmacokinetics, pharmacodynamics, and preliminary efficacy of CM313, an anti-CD38 antibody, in patients with systemic lupus erythematosus (SLE). Eligible patients were sequentially enrolled in four ascending dose groups (2, 4, 8, and 16 mg/kg) and randomized 4:1 to receive CM313 or placebo intravenously at days 1, 29, 36, 43, and 50. The primary endpoint was safety, and efficacy was explorato

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… Fig. 4 Mean percentage changes from baseline in immune biomarkers and concentrations of immunoglobulins. a – c Mean percentage changes in anti-double-stranded deoxyribonucleic acid (ds-DNA) antibodies, complement C3, and complement C4. d , e Concentrations and mean percentage changes in IgG. f , g Concentrations … Efficacy outcomes also included serologic response of biomarkers encompassing anti-double-stranded deoxyribonucleic acid (ds-DNA) antibodies, IgG, IgA, IgM, IgE, complement C3, and complement C4. Details of the assessments of the endpoints are described in Supplementary Methods . …

Overlap of membranous nephropathy and IgA nephropathy in a patient with Kimura's disease: a case report and literature review.

INTRODUCTION: Kimura's disease (KD) is a rare chronic inflammatory disorder characterized by subcutaneous lymphoid hyperplasia with peripheral eosinophilia. Kidney involvement is reported in 15%-18% of adult patients with KD, in many cases as nephrotic syndrome. We present a case of overlapping membranous nephropathy and IgA nephropathy associated with KD. CASE REPORT: A 27-year-old man was admitted with a history of bilateral leg edema for the last 2 months and concomitant progressive increase of cervical mass and fever. Laboratory findings were as follows: peripheral leukocyte count, 10,080

Association between peripheral activated naive and double negative 2 B-cell subsets and clinical parameters in lupus nephritis patients.

Altered composition of B-cell compartments is a known feature in patients with systemic lupus erythematosus (SLE). However, deep characterisation of B-cell subsets and their relation to clinical manifestations and disease activity in patients is limited. In this study, we analysed peripheral B-cell subsets phenotype in SLE (n = 35) and healthy controls (HCs, n = 15) by spectral flow cytometry. Disease activity was stratified as inactive (SLEDAI-2 K score 0, n = 2), mild (SLEDAI-2 K score 1-5, n = 12), moderate (SLEDAI-2 K score 6-10, n = 6) or high (SLEDAI-2 K > 10, n = 15). An elevated propor

Autoimmune screening panel in patients with multiple sclerosis: A Vienna multiple sclerosis database study.

BACKGROUND AND PURPOSE: Autoimmune screening panels (ASPs) are often ordered as a part of the diagnostic workup in people with suspected multiple sclerosis (MS). However, data on the significance of ASP seropositivity in MS are scarce. This study aimed to investigate whether routine implementation of ASPs is viable in MS diagnostic workup. METHODS: In this retrospective study, we included patients from the Vienna Multiple Sclerosis Database who were diagnosed with MS according to current McDonald criteria between 2014 and 2021 and had an ASP performed. RESULTS: We analyzed 212 patients (mean

Prevalence of primary immunodeficiency syndromes in tuberculous meningitis: A case-control study.

BACKGROUND: Only a proportion of patients with tuberculosis develop tuberculous meningitis. We hypothesize that inherent abnormalities in the host's innate or adaptive immune system may affect the outcome in tuberculous meningitis. In this study, we evaluated the proportion of underlying primary immunodeficiency in patients with tuberculous meningitis and its impact on the outcome. METHODS: Newly-diagnosed cases with tuberculous meningitis and healthy controls were included. Patients with HIV disease were excluded. Blood specimen were subjected to immunological assessment to detect primary im

The Panorama of Primary Angioedema in the Brazilian Population.

BACKGROUND: Primary angioedema (PA) is a complex disorder, presenting multiple hereditary (hereditary angioedema) and acquired subtypes (acquired angioedema). Despite a very similar clinical presentation among subtypes, the differential diagnosis is limited by the difficulty to identify bradykinin-mediated PA and the lack of specific biomarkers. OBJECTIVES: To report the clinical and genetic features of Brazilian patients with PA. METHODS: Brazilian patients referred from 50 centers were diagnosed on the basis of clinical symptoms, C1 inhibitor (C1-INH) and C4 plasma measurements, and DNA se

[Common variable immune deficiency in adult patients: analysis of 13 cases and literature review].

OBJECTIVE: To investigate the clinical and immunological characteristics, treatment and prognosis of common variable immune deficiency (CVID) in adult patients. METHODS: We retrospectively analyzed the clinical data of 13 adult patients hospitalized in our hospital for CVID diagnosed according to the criteria in International Consensus Document (2016), and analyzed their clinical manifestations, laboratory test results, imaging findings, pathological examinations and treatments. RESULTS: The mean age of onset was 24.46±16.82 years in these patients, who had a mean age of 32.54±14.86 years at

Secondary antibody deficiency is associated with development of infection in kidney transplantation: Results of a multicenter study.

BACKGROUND: We performed a multicenter study to assess the association between secondary antibody deficiency (immunoglobulin G [IgG] hypogammaglobulinemia combined with low levels of specific antibodies) and development of infection in kidney transplantation. METHODS: We prospectively analyzed 250 adult kidney recipients at four centers. The assessment points were before transplantation and 7 and 30 days after transplantation. The immune parameters were as follows: IgG, IgA, and IgM and complement factors C3 and C4 tested by nephelometry; specific IgG antibodies to cytomegalovirus (CMV) and I

Hereditary angioedema with F12 mutation: Clinical features and enzyme polymorphisms in 9 Southwestern Spanish families.

BACKGROUND: Information on F12 mutation hereditary angioedema (HAE) is still limited, but Spain is now recognized as having one of the highest concentrations of cases in Western Europe. OBJECTIVE: To describe unique features of HAE in Spanish carriers of the F12 mutation and investigate a potential role for angiotensin-converting enzyme (ACE) and aminopeptidase-P polymorphisms in disease expression. METHODS: This was a prospective observational cohort study of 35 individuals (80% females) from 9 unrelated families carrying the p.Thr309Lys mutation. We analyzed detailed medical records and co

Genital warts treated by photodynamic therapy.

The patient, a 30-year-old man with numerous genital condylomata acuminata (CA), has had unsuccessful treatment with liquid nitrogen, 20% podophyllin, and repeated 0.5% podophyllotoxin solution with 5% imiquimod (Figure 1). Before the appearance of CA, he experienced acute orchiepididimitis and a Candida infection. The patient was immunologically examined, and the lower level of lymphocytes, slightly reduced level of IgM, and C4 complement were revealed. Results from a human immunodeficiency virus examination were negative. After the therapeutic failure mentioned above, photodynamic therapy (P

[Lupus profundus and myelopathy].

BACKGROUND: We report a case of myelopathy during the course of lupus profundus that is unique to our knowledge. CASE-REPORT: A 29-year-old woman had lupus profundus since 1999, initially associated with thrombopenia (28,000 platelets/mm3) treated with corticosteroids for 6 months. Several nodular eruptions occurred from 1999 to 2004. Antinuclear antibodies were positive at 1/320 to 1/640 and complement C4 fraction was low. While being treated with hydroxychloroquine for a recent flare-up of nodular lesions of lupus profundus, she complained of paraesthesia of the abdominal wall and of the up…

Repeated renal biopsy in proliferative lupus nephritis--predictive role of serum C1q and albuminuria.

OBJECTIVE: Proliferative (WHO III/IV) nephritis in systemic lupus erythematosus (SLE) is a severe disease manifestation for which treatment with cyclophosphamide and high dose corticosteroids is generally recommended. We investigated the effect of this standard treatment on renal histopathology and clinical and serological findings to determine if the therapeutic response could be predicted by these variables. METHODS: We studied 18 patients with SLE and proliferative nephritis in whom repeated renal biopsy was performed after termination of induction therapy with cyclophosphamide and cortico

Enhanced tryptophan degradation in systemic lupus erythematosus.

In vitro and in vivo, tryptophan degradation was found to be associated with T cell functional loss and tolerance induction. In systemic lupus erythematosus (SLE) besides the Th2-type cytokine interleukin-10, Th1-type cytokines including interferon-gamma (IFN-gamma) are expressed especially during exacerbation of the disease. IFN-gamma stimulates the enzyme indoleamine (2,3)-dioxygenase (IDO) converting tryptophan to the metabolite kynurenine which in macrophages is subsequently degraded to other, partly neurotoxic compounds like quinolinic acid, and finally to nicrotinamides. We measured kynu

[Serum levels of soluble interleukin-2 receptor in alcoholic patients].

OBJECTIVES: Contribution of cellular immunity to the onset and perpetuation of alcohol-induced liver damage remains controversial. The aim of this work was to know whether T-cells participate in the pathogenesis of alcoholic liver injury by measuring the serum levels of sIL-2R in alcoholic patients with different degree of hepatic damage. PATIENTS AND METHODS: Fifty two patients and eighteen healthy subjects (Control group) were included. All patients were active drinkers of at least 100 grams/day of ethanol over a ten-years period. Serum sIL-2R was determined by ELISA. Liver biopsy was perfo

Expert Perspectives On Clinical Challenges: Expert Perspectives: Challenges in Scleroderma.

You are consulted to evaluate a 56-year-old woman with known Raynaud's phenomenon, finger swelling of several; months' duration, and new hypertension with a blood pressure of 160/100 mm/Hg. She also reports progressive shortness of breath. Physical examination reveals telangiectasias, sclerodactyly, and proximal skin sclerosis (thick shiny skin on the chest and upper arms), and bibasilar crackles are found on chest examination. Laboratory tests reveal evidence of microangiopathic hemolytic anemia, thrombocytopenia, and elevation of the serum creatinine level (previously normal), and chest comp