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Systemic Lupus Erythematosus

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181. Paediatric systemic lupus erythematosus as a manifestation of constitutional mismatch repair deficiency. (Abstract)

Paediatric systemic lupus erythematosus as a manifestation of constitutional mismatch repair deficiency. Biallelic mutations in any of the four mismatch repair genes MSH2, MSH6, MLH1 and PMS2 result in one of the most aggressive childhood cancer predisposition syndromes, termed constitutional mismatch repair deficiency (CMMRD) syndrome. In addition to a very high tumour risk, the CMMRD phenotype is often characterised by the presence of signs reminiscent of neurofibromatosis type 1. Although (...) paediatric systemic lupus erythematosus (pSLE) has been reported so far in three patients with CMMRD, it has not been considered a diagnostic feature of the syndrome. We report here two additional female patients with pSLE and CMMRD due to biallelic pathogenic variants in MSH6 Hence, there are a total of five out of approximately 200 (2.5%) currently reported patients with CMMRD that also have pSLE, suggesting pSLE should raise the suspicion of a diagnosis of CMMRD, especially if supported by additional

2019 Journal of Medical Genetics

182. Multicenter double-blind randomized controlled trial to evaluate the effectiveness and safety of bortezomib as a treatment for refractory systemic lupus erythematosus. (Abstract)

Multicenter double-blind randomized controlled trial to evaluate the effectiveness and safety of bortezomib as a treatment for refractory systemic lupus erythematosus. The objective of this study is to evaluate the efficacy and safety of bortezomib for treating systemic lupus erythematosus (SLE), in patients whose disease activity could not be controlled.Fourteen SLE patients with persistent disease activity were selected, who required prednisolone doses of >10 mg/d despite concomitant

2019 Modern Rheumatology Controlled trial quality: predicted high

183. Monoclonal antibody targeting BDCA2 ameliorates skin lesions in systemic lupus erythematosus. Full Text available with Trip Pro

Monoclonal antibody targeting BDCA2 ameliorates skin lesions in systemic lupus erythematosus. Plasmacytoid DCs (pDC) produce large amounts of type I IFN (IFN-I), cytokines convincingly linked to systemic lupus erythematosus (SLE) pathogenesis. BIIB059 is a humanized mAb that binds blood DC antigen 2 (BDCA2), a pDC-specific receptor that inhibits the production of IFN-I and other inflammatory mediators when ligated. A first-in-human study was conducted to assess safety, tolerability (...) blood and skin biopsies were measured. Skin disease activity was determined using the Cutaneous Lupus Erythematosus Disease Area and Severity Index Activity (CLASI-A).Single doses of BIIB059 were associated with favorable safety and PK profiles. BIIB059 administration led to BDCA2 internalization on pDCs, which correlated with circulating BIIB059 levels. BIIB059 administration in patients with SLE decreased expression of IFN response genes in blood, normalized MxA expression, reduced immune

2019 The Journal of clinical investigation Controlled trial quality: uncertain

184. The study of interactions between genome and exposome in the development of systemic lupus erythematosus. Full Text available with Trip Pro

The study of interactions between genome and exposome in the development of systemic lupus erythematosus. Systemic lupus erythematosus (SLE) is a systemic inflammatory autoimmune disease characterized by a broad spectrum of clinical and serological manifestations. This may reflect a complex and multifactorial etiology involving several identified genetic and environmental factors, though not explaining the full risk of SLE. Established SLE risk genotypes are either very rare or with modest

2019 Autoimmunity reviews

185. Psychoanalytic psychotherapy improves quality of life, depression, anxiety and coping in patients with systemic lupus erythematosus: a controlled randomized clinical trial. Full Text available with Trip Pro

Psychoanalytic psychotherapy improves quality of life, depression, anxiety and coping in patients with systemic lupus erythematosus: a controlled randomized clinical trial. Systemic Lupus Erythematosus (SLE) is an autoimmune disease which impairs the quality of life. The objective of study was to evaluate the effectiveness of Brief Group Psychoanalytic Psychotherapy to improve quality of life, depression, anxiety and coping strategies in SLE patients.In a randomized clinical trial, 80 female

2019 Advances in rheumatology (London, England) Controlled trial quality: uncertain

186. Increased Incidence of Resistant Hypertension in Patients with Systemic Lupus Erythematosus: A Retrospective Cohort Study. (Abstract)

Increased Incidence of Resistant Hypertension in Patients with Systemic Lupus Erythematosus: A Retrospective Cohort Study. To compare the risk of resistant hypertension (RHTN) in systemic lupus erythematosus (SLE) patients and controls without SLE; to define factors associated with RHTN in SLE patients.We studied 1,044 SLE patients and 5,241 control subjects using de-identified electronic health records from a tertiary care center. SLE was defined as ≥ 4 ICD codes for SLE and ANA ≥ 1:160. RHTN

2019 Arthritis care & research

187. Interleukin-17/Interleukin-21 and Interferon-g producing T cells specific for β2 Glycoprotein I in atherosclerosis inflammation of systemic lupus erythematosus patients with antiphospholipid syndrome. Full Text available with Trip Pro

Interleukin-17/Interleukin-21 and Interferon-g producing T cells specific for β2 Glycoprotein I in atherosclerosis inflammation of systemic lupus erythematosus patients with antiphospholipid syndrome. Systemic lupus erythematosus is frequently associated with antiphospholipid syndrome. Patients with lupus-antiphospholipid syndrome are characterized by recurrent arterial/venous thrombosis, miscarriages, and persistent presence of autoantibodies against phospholipid-binding proteins, such as β2 (...) -Glycoprotein I. We investigated the cytokine production induced by β2-Glycoprotein I in activated T cells that infiltrate in vivo atherosclerotic lesions of lupus-antiphospholipid syndrome patients. We examined the helper function of β2-Glycoprotein I-specific T cells for tissue factor production, as well as their cytolytic potential and their helper function for antibody production. Lupus-antiphospholipid syndrome patients harbor in vivo activated CD4+ T cells that recognize β2-Glycoprotein I

2019 Haematologica

188. Osteopontin and Disease Activity in Patients with Recent-onset Systemic Lupus Erythematosus: Results from the SLICC Inception Cohort. Full Text available with Trip Pro

Osteopontin and Disease Activity in Patients with Recent-onset Systemic Lupus Erythematosus: Results from the SLICC Inception Cohort. In cross-sectional studies, elevated osteopontin (OPN) levels have been proposed to reflect, and/or precede, progressive organ damage and disease severity in systemic lupus erythematosus (SLE). We aimed, in a cohort of patients with recent-onset SLE, to determine whether raised serum OPN levels precede damage and/or are associated with disease activity or certain (...) disease phenotypes.We included 344 patients from the Systemic Lupus International Collaborating Clinics (SLICC) Inception Cohort who had 5 years of followup data available. All patients fulfilled the 1997 American College of Rheumatology (ACR) criteria. Baseline sera from patients and from age- and sex-matched population-based controls were analyzed for OPN using ELISA. Disease activity and damage were assessed at each annual followup visit using the SLE Disease Activity Index 2000 (SLEDAI-2K

2019 Journal of Rheumatology

189. Epidemiologic Profile of Erectile Dysfunction in Patients with Systemic Lupus Erythematosus: The Latin American Landscape. (Abstract)

Epidemiologic Profile of Erectile Dysfunction in Patients with Systemic Lupus Erythematosus: The Latin American Landscape. The aim of this study was to describe the prevalence of erectile dysfunction (ED), as well as associated demographic and clinical features, in men with systemic lupus erythematosus (SLE), by means of a systematic, standardized evaluation.We performed a transversal study in 8 tertiary care centers in Latin America. We included male patients ≥ 16 years who fulfilled ≥ 4 (...) was 69%. Mean age in that group was 36.3 ± 1.03 years. Among SLE patients with and without ED, these factors were significantly different: the presence of persistent lymphopenia (p = 0.006), prednisone dose (9.3 ± 1.2 vs 5.3 ± 1.3 mg, p = 0.026), and the Systemic Lupus International Collaborating Clinics damage score (1.25 ± 0.14 vs 0.8 ± 0.16 points, p = 0.042). Independent risk factors for ED in patients with SLE were persistent lymphopenia (OR 2.79, 95% CI 1.37-5.70, p = 0.001) and corticosteroid

2019 Journal of Rheumatology

190. Long-Term Prognosis of Patients with Systemic Lupus Erythematosus-Associated Pulmonary Arterial Hypertension: CSTAR-PAH Cohort Study. Full Text available with Trip Pro

Long-Term Prognosis of Patients with Systemic Lupus Erythematosus-Associated Pulmonary Arterial Hypertension: CSTAR-PAH Cohort Study. This study aimed to identify the long-term clinical outcomes and prognostic factors of patients with systemic lupus erythematosus (SLE)-associated pulmonary arterial hypertension (PAH) confirmed by right heart catheterisation.A multicentre prospective cohort of SLE-associated PAH was established. Baseline and follow-up records were collected. The primary end

2019 European Respiratory Journal

191. Trichoscopic signs in systemic lupus erythematosus: a comparative study with 109 patients and 305 healthy controls. (Abstract)

Trichoscopic signs in systemic lupus erythematosus: a comparative study with 109 patients and 305 healthy controls. Hair and scalp involvement in systemic lupus erythematosus (SLE) can manifest as scarring alopecia, non-scarring alopecia or scalp/hair shaft changes without apparent hair loss. While trichoscopic signs in chronic cutaneous lupus are well established, data on SLE patients with normal-looking or non-scarring scalp are limited.To investigate trichoscopic features of SLE patients (...) without chronic cutaneous scalp lesions and compare the findings with normal controls, as well as determine which feature associates with systemic disease. Furthermore, we aim to explore different clinical presentations of the scalp in SLE patients and their association with disease activity.Trichoscopic photographs were taken from patients and healthy controls and evaluated by one blinded hair specialist. For SLE patients, their clinical presentations and evaluations for cutaneous, extracutaneous

2019 Journal of the European Academy of Dermatology and Venereology

192. Take a look at the eyes in Systemic Lupus Erythematosus: A novel point of view. (Abstract)

Take a look at the eyes in Systemic Lupus Erythematosus: A novel point of view. Systemic Lupus Erythematosus (SLE) is a connective tissue disease that involves multiple organs. Ocular structures and visual pathways can be affected in SLE because of disease-related eye involvement or drug toxicity. All the part of the eye may be interested with an external, anterior involvement, responsible of the dry eye disease, or posterior (retina) and neuro-ophtalmic manifestations. Retinopathy in SLE

2019 Autoimmunity reviews

193. Mechanisms of action and historical facts on the use of intravenous immunoglobulins in systemic lupus erythematosus. (Abstract)

Mechanisms of action and historical facts on the use of intravenous immunoglobulins in systemic lupus erythematosus. The current existing therapies for severe cases of systemic lupus erythematosus (SLE) patients are still limited. Intravenous immunoglobulin (IVIGs), which are purified from the plasma of thousands of healthy human donors, have been profiled as efficacious and life-saving options for SLE patients refractory to conventional therapy. The specific mechanism of action by which IVIGs

2019 Autoimmunity reviews

194. Human Low-Affinity IgG Receptor FcγRIIA Polymorphism H131R Associates with Subclinical Atherosclerosis and Increased Platelet Activity in Systemic Lupus Erythematosus. Full Text available with Trip Pro

Human Low-Affinity IgG Receptor FcγRIIA Polymorphism H131R Associates with Subclinical Atherosclerosis and Increased Platelet Activity in Systemic Lupus Erythematosus. Essentials Systemic lupus erythematosus (SLE) patients are at increased risk for premature CVD. Platelet activity, vascular dysfunction and carotid artery plaque are associated with FcγRIIA genotype in SLE. FcγRIIA genotype was not associated with platelet activity or carotid plaque in healthy controls. FcγRIIA represents a link (...) that connects platelet activity, vascular health and CVD in SLE. SUMMARY: Background Systemic lupus erythematosus (SLE) is a complex autoimmune disease associated with an elevated risk of premature cardiovascular disease. Platelets express receptors contributing to inflammation and immunity, including FcγRIIA, the low affinity receptor of the Fc portion of IgG antibodies. The variation at a single amino acid substitution, H131R, in the extracellular binding domain alters the affinity for IgG, which may

2019 Journal of Thrombosis and Haemostasis

195. Metabolic and structural skeletal muscle health in systemic lupus erythematosus related fatigue: a multi-modal magnetic resonance imaging study. Full Text available with Trip Pro

Metabolic and structural skeletal muscle health in systemic lupus erythematosus related fatigue: a multi-modal magnetic resonance imaging study. This study aimed to investigate the potential structural and metabolic role of skeletal muscle in SLE related fatigue.A case control, multi-modal MRI study was conducted. Cases were inactive SLE patients who reported chronic fatigue. Controls were age/sex matched healthy members of the general population. Participants were clinically characterised

2019 Arthritis care & research

196. Objectively-assessed foot and ankle characteristics in people with systemic lupus erythematosus: a comparison with age- and sex-matched controls. Full Text available with Trip Pro

Objectively-assessed foot and ankle characteristics in people with systemic lupus erythematosus: a comparison with age- and sex-matched controls. To objectively identify foot and ankle characteristics in people with SLE compared to age- and sex-matched controls.54 SLE and 56 control participants attended a study visit designed to comprehensively assess the foot and ankle. Objectively-assessed foot characteristics included muscle strength, joint motion, foot posture, foot problems, protective

2019 Arthritis care & research

197. Multicriteria decision analysis process to develop new classification criteria for systemic lupus erythematosus. Full Text available with Trip Pro

Multicriteria decision analysis process to develop new classification criteria for systemic lupus erythematosus. European League Against Rheumatism and are jointly supporting multiphase development of systemic lupus erythematosus (SLE) classification criteria based on weighted criteria and a continuous probability scale. Prior steps included item generation, item reduction and hierarchical organisation of candidate criteria using an evidence-based approach. Our objectives were to determine

2019 Annals of the Rheumatic Diseases

198. Understanding Non-Adherence with Hydroxychloroquine Therapy in Systemic Lupus Erythematosus. (Abstract)

Understanding Non-Adherence with Hydroxychloroquine Therapy in Systemic Lupus Erythematosus. Hydroxychloroquine (HCQ) is a cornerstone to managing systemic lupus erythematosus (SLE), yet adherence to medication is poor. We sought to measure the association of adherence with five 'dimensions of adherence' as articulated by the World Health Organization for chronic conditions: the patient's socioeconomic status, and patient-, condition-, therapy-, and healthcare-system-related factors. Our long

2019 Journal of Rheumatology

199. Atherosclerotic Vascular Events in Systemic Lupus Erythematosus - an Evolving Story. (Abstract)

Atherosclerotic Vascular Events in Systemic Lupus Erythematosus - an Evolving Story. Atherosclerotic vascular events (AVEs) are a major cause of mortality and morbidity in systemic lupus erythematosus (SLE). We aimed to determine the impact of early recognition and therapy for both classic risk factors for AVE and for SLE on the burden of AVEs in lupus in recent decades.Inception patients who entered the University of Toronto lupus Clinic between 1975 and 1987 followed to 1992 (Cohort 1 (...) ) and between 1999 and 2011 followed to 2016 (Cohort 2) were studied. AVEs, attributed to atherosclerosis, and occurring during the 17 years were identified. Lupus disease activity and therapy as well as hypertension, hypercholesterolemia, hyperglycemia and smoking were assessed. Analysis included descriptive statistics on baseline characteristics, traditional risk factors over the follow up, outcome rates by each 100 person years; Kaplan-Meier cumulative AVE curves, as well as competing risk Cox models

2019 Journal of Rheumatology

200. Fatigue Measurements in Systemic Lupus Erythematosus. (Abstract)

Fatigue Measurements in Systemic Lupus Erythematosus. Fatigue is a frequent, disabling issue in SLE. It is, however, difficult to quantify. The Ad Hoc Committee on SLE Response Criteria for Fatigue in 2007 recommended using the Krupp Fatigue Severity Scale (FSS). Since then, the Functional Assessment of Chronic Illness Therapy (FACIT) Fatigue scale has also been validated in SLE. We performed a review of instruments used to measure fatigue in adult SLE patients from 2007 onward.We searched

2019 Journal of Rheumatology

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