30,776 results match your criteria systemic sclerosis


Clinical features, visual outcome, and poor prognostic factors in occlusive retinal vasculitis.

Can J Ophthalmol 2021 Apr 7. Epub 2021 Apr 7.

Massachusetts Eye Research and Surgery Institution, Waltham, Mass.; Ocular Immunology and Uveitis Foundation, Waltham, Mass.; Harvard Medical School, Boston, Mass.. Electronic address:

Objective: To investigate the clinical features, treatment, and visual outcome of occlusive retinal vasculitis (ORV), with a focal analysis on prognostic factors associated with poor visual outcome.

Methods: We conducted a retrospective cohort study in patients diagnosed with ORV with at least 6 months of follow-up. Demographic data, ocular features, best corrected visual acuity (BCVA), fluorescein angiography, therapy regimens, and outcomes were collected from the Massachusetts Eye Research and Surgery Institution database from 2006 to 2017. Read More

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Insights into the regulatory role of Plexin D1 signalling in cardiovascular development and diseases.

J Cell Mol Med 2021 Apr 9. Epub 2021 Apr 9.

Institute for Translational Medicine, The Affiliated Hospital of Qingdao University, College of Medicine, Qingdao University, Qingdao, China.

Plexin D1 (PLXND1), which was previously thought to mediate semaphorin signalling, belongs to the Plexin family of transmembrane proteins. PLXND1 cooperates mostly with the coreceptor neuropilin and participates in many aspects of axonal guidance. PLXND1 can also act as both a tumour promoter and a tumour suppressor. Read More

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Author Correction: Network-based modeling of drug effects on disease module in systemic sclerosis.

Sci Rep 2021 Apr 9;11(1):8238. Epub 2021 Apr 9.

Department of Computer Science, University of California, Davis, CA, USA.

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Secukinumab and metformin ameliorate dermal fibrosis by decreasing tissue interleukin-17 levels in bleomycin-induced dermal fibrosis.

Int J Rheum Dis 2021 Apr 9. Epub 2021 Apr 9.

Department of Rheumatology, Faculty of Medicine, Firat University, Elazig, Turkey.

Although the pathogenesis of systemic sclerosis is not exactly known, it is thought that immune activation has prominent roles in pathogenesis. Secukinumab is a monoclonal antibody against interleukin (IL)-17A. Metformin, a widely used antidiabetic medication, has anti-proliferative, immunomodulating and anti-fibrotic activities. Read More

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Interleukin-1 and Systemic Sclerosis: Getting to the Heart of Cardiac Involvement.

Front Immunol 2021 23;12:653950. Epub 2021 Mar 23.

Department of Experimental and Clinical Medicine, University of Florence, and Division of Rheumatology AOUC, Florence, Italy.

Systemic sclerosis (SSc) is rare, severe connective tissue disease characterized by endothelial and vascular damage, immune activation, and resulting in inflammation and fibrosis of skin and internal organs, including the heart. SSc is associated with high morbidity and mortality. Cardiac involvement is frequent in SSc patients, even though often asymptomatic at early stages, and represents one of the major causes of SSc-related mortality. Read More

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Therapeutic prospects of MicroRNAs carried by mesenchymal stem cells-derived extracellular vesicles in autoimmune diseases.

Life Sci 2021 Apr 5:119458. Epub 2021 Apr 5.

Department of Rheumatology and Immunology, West China Hospital, Sichuan University, 37 Guoxue Xiang, Chengdu, Sichuan 610041, China. Electronic address:

Autoimmune diseases (ADs) are a class of chronic disease conditions with impaired tolerance to autoantigens. Currently, there is no effective treatment for ADs, and the existing medications have limitations due to non-specific targets and side effects. Accumulating evidence has shown that mesenchymal stem cells (MSCs) play a role in ADs treatment. Read More

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Neurologic manifestations in hospitalized patients with COVID-19 in Mexico City.

PLoS One 2021 8;16(4):e0247433. Epub 2021 Apr 8.

Department of Neurology and Psychiatry, Instituto Nacional de Ciencias Médicas y Nutrición Salvador Zubirán, Mexico City, Mexico.

Background: The coronavirus disease 2019 (COVID-19) is a systemic entity that frequently implies neurologic features at presentation and complications during the disease course. We aimed to describe the characteristics and predictors for developing in-hospital neurologic manifestations in a large cohort of hospitalized patients with COVID-19 in Mexico City.

Methods: We analyzed records from consecutive adult patients hospitalized from March 15 to June 30, 2020, with moderate to severe COVID-19 confirmed by reverse transcription real-time polymerase chain reaction (rtRT-PCR) for the severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2). Read More

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Ophthalmic manifestations in patients with collagen vascular disorders: a hospital-based retrospective observational study.

Int Ophthalmol 2021 Apr 8. Epub 2021 Apr 8.

Department of Dermatology, Venereology & Leprosy, Dr. Rajendra Prasad Govt. Medical College, Kangra (Tanda), 176001, Himachal Pradesh, India.

Aim: To study frequency and characteristics of ocular manifestations in Indian patients with collagen vascular disorders.

Methods: The medical records of 73 patients (Males: Females 16:57) aged between 22 and 78 years (mean ± SD = 43.5 ± 12. Read More

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Vascular endothelial injury assessed with functional techniques in systemic sclerosis patients with pulmonary arterial hypertension versus systemic sclerosis patients without pulmonary arterial hypertension: a systematic review and meta-analysis.

Rheumatol Int 2021 Apr 8. Epub 2021 Apr 8.

Department of Respiratory Medicine, G. Papanikolaou Hospital, Thessaloniki, Greece.

Microvascular dysfunction is one of the hallmarks of systemic sclerosis (SSc). The presence of pulmonary-arterial-hypertension (PAH) in SSc-patients is associated with poor prognosis. This is a systematic review and meta-analysis of studies assessing microvascular and endothelial injury with functional techniques in SSc-patients with PAH (SSc-PAH) compared to those without PAH (SSc-non-PAH) (PROSPERO: CRD42021236212). Read More

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Editorial: Etiopathogenesis of Systemic Sclerosis: An Update.

Front Immunol 2021 22;12:663381. Epub 2021 Mar 22.

Dipartimento di Medicina Interna e Specialità Mediche (DIMI), University of Genova, Genova, Italy.

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Fibrotic expression profile analysis reveals repurposed drugs with potential anti-fibrotic mode of action.

PLoS One 2021 7;16(4):e0249687. Epub 2021 Apr 7.

Department of Bioinformatics, The Cyprus Institute of Neurology and Genetics, Nicosia, Cyprus.

Fibrotic diseases cover a spectrum of systemic and organ-specific maladies that affect a large portion of the population, currently without cure. The shared characteristic these diseases feature is their uncontrollable fibrogenesis deemed responsible for the accumulated damage in the susceptible tissues. Idiopathic Pulmonary Fibrosis, an interstitial lung disease, is one of the most common and studied fibrotic diseases and still remains an active research target. Read More

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Peripheral nervous system manifestations of rheumatological diseases.

J Neurol Sci 2021 Mar 27:117421. Epub 2021 Mar 27.

University of Virginia, Department of Neurology, Charlottesville, VA, USA.

Rheumatological diseases result in immune-mediated injury to not only connective tissue, but often components of the peripheral nervous system. These overlap conditions can be broadly categorized as peripheral neuropathies and overlap myositis. The peripheral neuropathies are distinctive as many have unusual presentations such as non-length-dependent, small fiber neuropathies and sensory neuronopathies (both due to dorsal root ganglia dysfunction), multiple mononeuropathies (e. Read More

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Effect of combined pulmonary fibrosis and emphysema on patients with connective tissue diseases and systemic sclerosis: a systematic review and meta-analysis.

Arthritis Res Ther 2021 04 6;23(1):100. Epub 2021 Apr 6.

Department of Rheumatology, Hanyang University Hospital for Rheumatic Diseases, Seoul, South Korea.

Background: This study aimed to analyze the literature systematically to determine the clinical characteristics and prognosis of patients with connective tissue disease (CTD) with combined pulmonary fibrosis and emphysema (CPFE) compared to those of patients with CTD-interstitial lung disease (CTD-ILD) without emphysema.

Methods: We searched MEDLINE, EMBASE, Cochrane Library, and KoreaMed for relevant articles published before July 2019. Studies meeting all the following criteria were included: (1) original research studies evaluating the effect of CPFE on CTD, (2) studies that compared patients with CTD-CPFE to those with CTD-ILD without emphysema, and (3) studies providing data on physical capacity, pulmonary function, or death in patients with CTD. Read More

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The Role of Pro-fibrotic Myofibroblasts in Systemic Sclerosis: from Origin to Therapeutic Targeting.

Curr Mol Med 2021 Mar 24. Epub 2021 Mar 24.

Department of Experimental and Clinical Medicine, Section of Anatomy and Histology, University of Florence, Florence. Italy.

Systemic sclerosis (SSc, scleroderma) is a complex connective tissue disorder characterized by multisystem clinical manifestations resulting from immune dysregulation/autoimmunity, vasculopathy and, most notably, progressive fibrosis of the skin and internal organs. In recent years, it has emerged that the main drivers of SSc-related tissue fibrosis are myofibroblasts, a type of mesenchymal cells with both the extracellular matrix-synthesizing features of fibroblasts and the cytoskeletal characteristics of contractile smooth muscle cells. The accumulation and persistent activation of pro-fibrotic myofibroblasts during SSc development and progression result into elevated mechanical stress and reduced matrix plasticity within the affected tissues and may be ascribed to a reduced susceptibility of these cells to pro-apoptotic stimuli, as well as their increased formation from tissue-resident fibroblasts or transition from different cell types. Read More

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The catch-22 of limited Food and Drug Administration approval for connective tissue disease therapies.

J Am Acad Dermatol 2021 Apr 3. Epub 2021 Apr 3.

Department of Dermatology, Brigham and Women's Hospital, Harvard Medical School (Boston, MA). Electronic address:

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Autoantibody profiles in systemic sclerosis; a comparison of diagnostic tests.

Autoimmunity 2021 Apr 5:1-8. Epub 2021 Apr 5.

Laboratory for Medical Immunology, Radboud University Medical Center, Nijmegen, The Netherlands.

Objectives: Autoimmune antibody profiling plays a prominent role in both classification and prognosis of systemic sclerosis (SSc). In the last years novel autoantibodies have been discovered and have become available in diagnostic assays. However, standardization in autoimmune serology is lacking, which may have a negative impact on the added value of autoantibodies in diagnosis and prognosis of SSc. Read More

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An elevated polyclonal free light chain level reflects a strong interferon signature in patients with systemic autoimmune diseases.

J Transl Autoimmun 2021 2;4:100090. Epub 2021 Mar 2.

Laboratory of Immunology and Immunotherapy, CHRU Morvan, Brest, France.

High amount of polyclonal free light chains (FLC) are reported in systemic autoimmune diseases (SAD) and we took advantage of the PRECISESADS study to better characterize them. Serum FLC levels were explored in 1979 patients with SAD (RA, SLE, SjS, Scl, APS, UCTD, MCTD) and 614 healthy controls. Information regarding clinical parameters, disease activity, medications, autoantibodies (Ab) and the interferon α and/or γ scores were recorded. Read More

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The PREdictor of MAlnutrition in Systemic Sclerosis (PREMASS) Score: A Combined Index to Predict 12 Months Onset of Malnutrition in Systemic Sclerosis.

Front Med (Lausanne) 2021 17;8:651748. Epub 2021 Mar 17.

National Institute for Health Research (NIHR) Leeds Biomedical Research Centre (BRC), Leeds Teaching Hospitals National Health Service (NHS) Trust and Leeds Institute of Rheumatic and Musculoskeletal Medicine, University of Leeds, Leeds, United Kingdom.

Malnutrition is a severe complication in Systemic Sclerosis (SSc) and it is associated with significant mortality. Notwithstanding, there is no defined screening or clinical pathway for patients, which is hampering effective management and limiting the opportunity for early intervention. Here we aim to identify a combined index predictive of malnutrition at 12 months using clinical data and specific serum adipokines. Read More

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Change of the microvascularization in systemic sclerosis, a matter of air.

Best Pract Res Clin Rheumatol 2021 Apr 1:101683. Epub 2021 Apr 1.

Department of Rheumatology, Radboud University Medical Center, Nijmegen, the Netherlands. Electronic address:

Systemic sclerosis (SSc) is a rare complex disease, characterized by microvascular damage, auto-immunity, and fibrosis. Nailfold capillary microscopy (NCM), a safe and noninvasive imaging technique, can be used to visualize specific microvascular alterations in SSc. In this review, we discuss an interesting case of a patient with changes in microvascular pattern on NCM after pulmonary transplantation. Read More

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Quantitative susceptibility mapping in the thalamus and basal ganglia of systemic lupus erythematosus patients with neuropsychiatric complaints.

Neuroimage Clin 2021 Mar 22;30:102637. Epub 2021 Mar 22.

Department of Radiology, Leiden University Medical Center, Leiden, The Netherlands. Electronic address:

Systemic lupus erythematosus (SLE) is an auto-immune disease characterized by multi-organ involvement. Although uncommon, central nervous system involvement in SLE, termed neuropsychiatric SLE (NPSLE), is not an exception. Current knowledge on underlying pathogenic mechanisms is incomplete, however, neuroinflammation is thought to play a critical role. Read More

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Calcinosis in systemic sclerosis.

Joint Bone Spine 2021 Mar 31:105180. Epub 2021 Mar 31.

Univ. Lille, Inserm, CHU Lille, Service de Médecine Interne et Immunologie Clinique, Centre de référence des maladies autoimmunes systémiques rares du Nord et Nord-Ouest de France (CeRAINO), U1286 - INFINITE - Institute for Translational Research in Inflammation, F-59000 Lille, France.

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QT Interval Prolongation in Patients with Systemic Sclerosis-Are the Holter ECG Recordings a Better Option for QT Interval Evaluation?

Medicina (Kaunas) 2021 Mar 22;57(3). Epub 2021 Mar 22.

Department of Internal Medicine, Gr T Popa University of Medicine and Pharmacy, 700115 Iasi, Romania.

Cardiac involvement in systemic sclerosis has important consequences on patient survival. Myocardial fibrosis and microcirculation involvement can generate arrhythmic complications, which can be associated with a higher death risk. QT interval prolongation is considered as a risk factor for ectopic ventricular events and can be evaluated using standard short ECG recordings or 24-h Holter ECG recordings. Read More

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Muscle Damage in Systemic Sclerosis and CXCL10: The Potential Therapeutic Role of PDE5 Inhibition.

Int J Mol Sci 2021 Mar 12;22(6). Epub 2021 Mar 12.

Leeds Institute of Rheumatic and Musculoskeletal Medicine, University of Leeds, Leeds LS9 7TF, UK.

Skeletal muscle damage is a common clinical manifestation of systemic sclerosis (SSc). C-X-C chemokine ligand 10 (CXCL10) is involved in myopathy and cardiomyopathy development and is associated with a more severe SSc prognosis. Interestingly, the phosphodiesterase type 5 inhibitor (PDE5i) sildenafil reduces CXCL10 sera levels of patients with diabetic cardiomyopathy and in cardiomyocytes. Read More

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Analysis of PI3K Pathway Associated Molecules Reveals Dysregulated Innate and Adaptive Functions of B Cells in Early Diffuse Cutaneous Systemic Sclerosis.

Int J Mol Sci 2021 Mar 12;22(6). Epub 2021 Mar 12.

Clinical Center, Department of Immunology and Biotechnology, University of Pécs Medical School, H-7624 Pécs, Hungary.

B cell activation is an early event in the development of systemic sclerosis (SSc). The classical activation of B cells downstream of the B-cell receptor (BCR) involves the phosphatidylinositol-3 kinase (PI3K) pathway that integrates the effects of multiple co-stimulatory receptors. Our analysis of PI3K pathway associated molecules in peripheral blood B cells of early diffuse cutaneous SSc (dcSSc) patients showed altered mRNA expression of Toll-like receptor (TLR) homolog CD180, TLR4, complement component 3, IL-4 receptor and secreted phosphoprotein 1 (SPP1). Read More

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Progression in the Management of Non-Idiopathic Pulmonary Fibrosis Interstitial Lung Diseases, Where Are We Now and Where We Would Like to Be.

J Clin Med 2021 Mar 23;10(6). Epub 2021 Mar 23.

Laboratory of Respiratory Diseases and Thoracic Surgery (BREATHE), Department CHROMETA, KU Leuven, B-3000 Leuven, Belgium.

A significant proportion of patients with interstitial lung disease (ILD) may develop a progressive fibrosing phenotype characterized by worsening of symptoms and pulmonary function, progressive fibrosis on chest computed tomography and increased mortality. The clinical course in these patients mimics the relentless progressiveness of idiopathic pulmonary fibrosis (IPF). Common pathophysiological mechanisms such as a shared genetic susceptibility and a common downstream pathway-self-sustaining fibroproliferation-support the concept of a progressive fibrosing phenotype, which is applicable to a broad range of non-IPF ILDs. Read More

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Butyrate Improves Skin/Lung Fibrosis and Intestinal Dysbiosis in Bleomycin-Induced Mouse Models.

Int J Mol Sci 2021 Mar 9;22(5). Epub 2021 Mar 9.

Department of Internal Medicine and Institute of Health Science, College of Medicine, Gyeongsang National University and Hospital, Jinju 52727, Korea.

Systemic sclerosis (SSc) is an autoimmune disorder characterized by fibrosis of the skin and internal organs. Despite several studies on SSc treatments, effective treatments for SSc are still lacking. Since evidence suggests an association between intestinal microbiota and SSc, we focused on butyrate, which has beneficial effects in autoimmune diseases as a bacterial metabolite. Read More

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Pharmacological Interventions for Pulmonary Involvement in Rheumatic Diseases.

Pharmaceuticals (Basel) 2021 Mar 10;14(3). Epub 2021 Mar 10.

Division of Rheumatology, Department of Internal Medicine, Seoul National University Hospital, Seoul 03080, Korea.

Among the diverse forms of lung involvement, interstitial lung disease (ILD) and pulmonary arterial hypertension (PAH) are two important conditions in patients with rheumatic diseases that are associated with significant morbidity and mortality. The management of ILD and PAH is challenging because the current treatment often provides only limited patient survival benefits. Such challenges derive from their common pathogenic mechanisms, where not only the inflammatory processes of immune cells but also the fibrotic and proliferative processes of nonimmune cells play critical roles in disease progression, making immunosuppressive therapy less effective. Read More

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New Autoantibody Specificities in Systemic Sclerosis and Very Early Systemic Sclerosis.

Antibodies (Basel) 2021 Mar 28;10(2). Epub 2021 Mar 28.

Istituto Superiore di Sanita', National Centre for Pre-Clinical and Clinical Drug Research and Evaluation, Pharmacological Research and Experimental Therapy Unit, 00166 Rome, Italy.

Chemokine (C-X-C motif) ligand 4 (CXCL4) is a biomarker of unfavorable prognosis in Systemic Sclerosis (SSc), a potentially severe autoimmune condition, characterized by vasculitis, fibrosis and interferon (IFN)-I-signature. We recently reported that autoantibodies to CXCL4 circulate in SSc patients and correlate with IFN-α. Here, we used shorter versions of CXCL4 and CXCL4-L1, the CXCL4 non-allelic variant, to search for autoantibodies exclusively reacting to one or the other CXCL4 form. Read More

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