28,089 results match your criteria Scleroderma


Oxidative stress-induced senescence mediates inflammatory and fibrotic phenotypes in fibroblasts from systemic sclerosis patients.

Rheumatology (Oxford) 2021 Jun 11. Epub 2021 Jun 11.

Faculty of Pharmacy, Université de Montréal, Québec, Canada.

Objective: Systemic sclerosis (SSc) is an autoimmune connective tissue disorder characterized by inflammation and fibrosis. Although constitutive activation of fibroblasts is proposed to be responsible for the fibrotic and inflammatory features of the disease, the underlying mechanism remains elusive and, effective therapeutic targets are still lacking. The aim of this study was to evaluate the role of oxidative stress-induced senescence and its contribution to the pro-fibrotic and pro-inflammatory phenotypes of fibroblasts from SSc patients. Read More

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A new molecular classification to drive precision treatment strategies in primary Sjögren's syndrome.

Nat Commun 2021 06 10;12(1):3523. Epub 2021 Jun 10.

Institut de Recherches Internationales Servier, Departments of Translational Medicine and Immuno-Inflammatory Diseases Research and Development, Suresnes, France.

There is currently no approved treatment for primary Sjögren's syndrome, a disease that primarily affects adult women. The difficulty in developing effective therapies is -in part- because of the heterogeneity in the clinical manifestation and pathophysiology of the disease. Finding common molecular signatures among patient subgroups could improve our understanding of disease etiology, and facilitate the development of targeted therapeutics. Read More

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Integration of With Disease-Gene Network Reveals Common Cellular Mechanisms Between IMIDs and Drug Repurposing Strategies.

Front Immunol 2021 24;12:669400. Epub 2021 May 24.

Division Internal Medicine and Dermatology, University Medical Center Utrecht, Utrecht, Netherlands.

Objective: Development and progression of immune-mediated inflammatory diseases (IMIDs) involve intricate dysregulation of the disease-associated genes (DAGs) and their expressing immune cells. Identifying the crucial disease-associated cells (DACs) in IMIDs has been challenging due to the underlying complex molecular mechanism.

Methods: Using transcriptome profiles of 40 different immune cells, unsupervised machine learning, and disease-gene networks, we constructed the Disease-gene IMmune cell Expression (DIME) network and identified top DACs and DAGs of 12 phenotypically different IMIDs. Read More

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Multimodality imaging of the gastrointestinal manifestations of scleroderma.

Clin Radiol 2021 Jun 6. Epub 2021 Jun 6.

Radiology Department, Mater Misericordiae University Hospital, Dublin, Ireland.

Scleroderma is a complex multisystem connective tissue disorder. Early visceral disease, such as gastrointestinal (GI) involvement, is associated with significant morbidity and a poorer prognosis. Prompt diagnosis is crucial to allow disease modifying therapies be initiated early in the course of the disease. Read More

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Cutaneous manifestations related to the COVID-19 pandemic: a review article.

Cutan Ocul Toxicol 2021 Jun;40(2):168-174

Dermatology Department of Razi Hospital, Tehran University of Medical Sciences, Tehran, Iran.

Background: Skin lesions are either caused by COVID-19 disease or they can be due to other driving forces related to the COVID-19 pandemic.

Aim: Considering the fact that the reported data in different articles for the type and prevalence of skin manifestations related to the COVID-19 pandemic are inconsistent, we have described the mechanism and type of skin lesions related to the COVID-19 pandemic.

Methods: In this review article, we have searched the Medline database (PubMed) for the combination of the following key terms "Dermatological Manifestation", "cutaneous Manifestation", "Skin Manifestation", "COVID-19", "SARS-CoV-2". Read More

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Phenotyping Angiotensin-Converting Enzyme in Blood: A Necessary Approach for Precision Medicine.

J Appl Lab Med 2021 Jun 7. Epub 2021 Jun 7.

Medical Center, Moscow University, Moscow, Russia.

Background: Angiotensin-converting enzyme (ACE) metabolizes a number of important peptides participating in blood pressure regulation and vascular remodeling. Elevated ACE expression in tissues (which is generally reflected by ACE in blood) is associated with increased risk of cardiovascular diseases. Elevated ACE in blood is also a marker for granulomatous diseases. Read More

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Comprehensive analysis of the major histocompatibility complex in systemic sclerosis identifies differential HLA associations by clinical and serological subtypes.

Ann Rheum Dis 2021 Apr 1. Epub 2021 Apr 1.

Department of Cell Biology and Immunology, Institute of Parasitology and Biomedicine López-Neyra, CSIC, Granada, Andalucía, Spain.

Objective: The greatest genetic effect reported for systemic sclerosis (SSc) lies in the major histocompatibility complex (MHC) locus. Leveraging the largest SSc genome-wide association study, we aimed to fine-map this region to identify novel human leucocyte antigen (HLA) genetic variants associated with SSc susceptibility and its main clinical and serological subtypes.

Methods: 9095 patients with SSc and 17 584 controls genome-wide genotyped were used to impute and test single-nucleotide polymorphisms (SNPs) across the MHC, classical HLA alleles and their composite amino acid residues. Read More

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Clinical and psychosocioeconomic impact of COVID-19 pandemic on patients of the Indian Progressive Systemic Sclerosis Registry (IPSSR).

Rheumatol Adv Pract 2021 23;5(2):rkab027. Epub 2021 Apr 23.

Centre for Arthritis and Rheumatism Excellence (CARE), Cochin, Kerala, India.

Objectives: The aim was to determine the impact of the coronavirus disease 2019 (COVID-19) pandemic on access to health care among patients with scleroderma and to analyse the economic and psychosocial impacts and the infection prevention measures taken by them during the pandemic.

Methods: A 25-item questionnaire designed to assess the components of the objectives was tele-administered between October 2020 and January 2021 to the patients enrolled in the Indian Progressive Systemic Sclerosis Registry.

Results: Of the 428 patients in the registry, 336 took part in the study. Read More

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Microvascular Hand Surgery for Digital Ischemia in Scleroderma.

J Scleroderma Relat Disord 2020 Jun 29;5(2):130-136. Epub 2019 Jun 29.

Division of Plastic Surgery, Department of Surgery, The Robert A. Chase Hand Center, Stanford University Medical Center, Palo Alto, CA, USA.

Periarterial sympathectomy and arterial bypass are microsurgical techniques which the literature suggests can provide improvement in digital pain and ulceration in patients with systemic sclerosis (SSc) who have persistent symptoms despite medication management. This review summarizes the relevant anatomy, medical therapies, operative techniques, and surgical outcomes and complications associated with the management of the vascular manifestations of SSc in the hand. Multidisciplinary collaboration between dermatology, rheumatology, and hand surgery can facilitate optimal medical and surgical management for SSc patients. Read More

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Narrow-Band Reflectance Spectrophotometry for the Assessment of Erythematous and Hyperpigmented Skin Lesions in Localized Scleroderma: A Preliminary Study.

Clin Cosmet Investig Dermatol 2021 28;14:575-580. Epub 2021 May 28.

Department of Dermatology, School of Medicine in Katowice, Medical University of Silesia, Katowice, Poland.

Background: Localized scleroderma (LoS) is an inflammatory fibrosing disease of the connective tissue, whose esthetic sequelae are atrophic skin lesions with hyperpigmentation. The key element of diagnostic and therapeutic procedures is the assessment of the disease's severity and damage. The study objective was to analyze the usefulness of narrow-band reflectance spectrophotometry (NBRS) to assess erythema and hyperpigmentation in LoS lesions. Read More

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BMSC-derived extracellular vesicles intervened the pathogenic changes of scleroderma in mice through miRNAs.

Stem Cell Res Ther 2021 Jun 5;12(1):327. Epub 2021 Jun 5.

Department of Dermatology, Tongji Hospital, School of Medicine, Tongji University, Shanghai, 200065, China.

Background: Systemic sclerosis (SSc) is a disease that features severe fibrosis of the skin and lacks effective therapy. Bone marrow mesenchymal stem cell (BMSC)-derived extracellular vesicles (EVs) are potential stem cell-based tools for the treatment of SSc.

Methods: BMSCs were isolated from the bone marrow of mice and identified with surface markers according to multilineage differentiation. Read More

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Association between gastroprotective agents and risk of incident interstitial lung disease in systemic sclerosis.

Respir Med 2021 May 27;185:106482. Epub 2021 May 27.

Department of Medicine, Université de Montréal, Montreal, Quebec, Canada; Division of Rheumatology, Centre hospitalier de l'Université de Montréal, Montreal, Quebec, Canada. Electronic address:

Objectives: Although interstitial lung disease (ILD) occurs in over half of systemic sclerosis (SSc) patients and represents a leading cause of mortality, there are currently no preventative strategies. We evaluated if gastroprotective agents were associated with a lower incident risk of SSc-ILD.

Methods: An SSc cohort without clinically apparent ILD at baseline was constructed from the Canadian Scleroderma Research Group registry. Read More

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Assessment of Nail Findings in Children with Atopic Dermatitis.

Clin Exp Dermatol 2021 Jun 5. Epub 2021 Jun 5.

Department of Dermatology, Firat University Hospital, Elazig, Turkey.

Background: Cutaneous findings are well known in atopic dermatitis (AD), but nail changes are generally overlooked.

Objective: In the present study, the aim was to determine the clinical and disease-related capillaroscopic findings of nail findings in pediatric patients with AD.

Methods: A total of 100 participants aged 2-16 years, admitted to the dermatology outpatient clinic, were included in the study. Read More

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Clinical outcomes of asymptomatic cardiac involvement in systemic sclerosis patients after a 2-year follow-up (extended study).

Am J Med Sci 2021 Jun 1. Epub 2021 Jun 1.

Division of Rheumatology, Department of Medicine, Faculty of Medicine, Khon Kaen University, Khon Kaen, Thailand. Electronic address:

Background: Asymptomatic cardiac involvement in systemic sclerosis (SSc) has been reported. Long-term follow-up might elucidate the clinical implications of these abnormalities. The aim was to identify the clinical outcomes of asymptomatic cardiac involvement in SSc patients after 2 years of follow-up. Read More

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Application of a novel finger temperature device in the assessment of subjects with Raynaud's phenomenon.

Skin Res Technol 2021 Jun 3. Epub 2021 Jun 3.

Department of Health Management and Health Economics, Faculty of Public Health, Medical University of Plovdiv, Plovdiv, Bulgaria.

Introduction: Finger skin thermometry is one of the most commonly used methods for evaluating the response of the digital vessels to cold stimulation. The aim of this study was to evaluate the applicability of a novel finger skin temperature device for performing cold-stimulation test (CST) in subjects with primary and secondary Raynaud's phenomenon (RP).

Methodology: A total of 155 consecutive subjects were studied. Read More

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Sildenafil Counteracts the In Vitro Activation of CXCL-9, CXCL-10 and CXCL-11/CXCR3 Axis Induced by Reactive Oxygen Species in Scleroderma Fibroblasts.

Biology (Basel) 2021 May 31;10(6). Epub 2021 May 31.

Unit of Endocrinology, Department of Movement, Human and Health Sciences, University of Rome "Foro Italico", 00135 Rome, Italy.

Oxidative stress plays a key role in systemic sclerosis (SSc) pathogenesis, and an altered redox homeostasis might be responsible for abnormal inflammatory status, fibrosis and tissue damage extension. In this study, we explored the effect of the phosphodiesterase type 5 inhibitor sildenafil in modulating the activation of the CXCL-9, -10, -11/CXCR3 axis, which is fundamental in the perpetuation of inflammation in different autoimmune diseases, in the cell culture of SSc human dermal fibroblasts exposed to a pro-oxidant environment. We observed that sildenafil significantly reduced gene expression and release of CXCL-9, -10 and -11, inhibited the CXCR3 action and suppressed the activation of STAT1-, JNK- and p38MAPK pathways. Read More

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Prevalence of Oral and Maxillofacial Disorders in Patients with Systemic Scleroderma-A Systematic Review.

Int J Environ Res Public Health 2021 May 14;18(10). Epub 2021 May 14.

Department of Oral Surgery and Policlinical Ambulance, Faculty of Health, Witten/Herdecke University, Alfred-Herrhausen-Str. 45, 58448 Witten, Germany.

Background: Systematic scleroderma is a rare chronic autoimmune disease of unknown aetiology. The aim of this study was to identify the prevalence of orofacial pathognomonic conditions in patients with systemic scleroderma using only randomised prospective studies that investigated the treatment of oral and maxillofacial changes, highlighted associations between the disease and Sjogren's syndrome, and/or analysed the effect of oral hygiene.

Methods: The literature was systematically reviewed based on Cochrane Library, EMBASE, PubMed, Scopus, and Web of Science articles published up to March 2020. Read More

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The Risk of Conducting Overgrafting of Fat for Treating Facial Atrophy Induced by Localized Scleroderma.

Facial Plast Surg 2021 Jun 1;37(3):412-414. Epub 2021 Jun 1.

Department of Plastic Surgery, Peking Union Medical College Hospital, Beijing, China.

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New risk model is able to identify patients with a low risk of progression in systemic sclerosis.

RMD Open 2021 May;7(2)

Department of Rheumatology, Leiden University Medical Center, Leiden, The Netherlands.

Objectives: To develop a prediction model to guide annual assessment of systemic sclerosis (SSc) patients tailored in accordance to disease activity.

Methods: A machine learning approach was used to develop a model that can identify patients without disease progression. SSc patients included in the prospective Leiden SSc cohort and fulfilling the ACR/EULAR 2013 criteria were included. Read More

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Recognition and Management of Cutaneous Connective Tissue Diseases.

Med Clin North Am 2021 Jul;105(4):757-782

Autoimmune Skin Disease Program, Department of Dermatology, Brigham and Women's Hospital, Harvard Medical School, 221 Longwood Avenue, Boston, MA 02115, USA. Electronic address:

Connective tissue diseases (CTDs) encompass a broad spectrum of clinical presentations that involve multidisciplinary management. Cutaneous findings are common in CTD and careful examination of these features aids in appropriate diagnosis and subsequent evaluation. Thorough work-up of CTD is crucial to properly identify disease subtypes and systemic involvement. Read More

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Factors influencing patient decision-making concerning treatment escalation in Raynaud's phenomenon secondary to systemic sclerosis.

Arthritis Care Res (Hoboken) 2021 May 31. Epub 2021 May 31.

Department of Internal Medicine, Division of Rheumatology, Scleroderma Program, University of Michigan, Ann Arbor, USA.

Objective: To explore patient priorities and ranking of factors influencing patient decision-making concerning treatment escalation in the management of Raynaud's phenomenon (RP) secondary to systemic sclerosis (SSc).

Methods: SSc patients were invited to participate in an online survey disseminated through patient-led organisations and social media platforms.

Results: Responses from 747 people with self-reported SSc-RP were evaluable with broad international representation. Read More

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Considerations for Hand Surgery in Patients With Scleroderma.

Hand (N Y) 2021 May 31:15589447211017211. Epub 2021 May 31.

The Ohio State University Wexner Medical Center, Columbus, USA.

Systemic sclerosis (scleroderma, SSc) is an autoimmune disease that causes significant dysfunction to multiple organ systems, including the musculoskeletal system. It poses significant challenges to the hand surgeon, including calcinosis, ischemic changes, Raynaud phenomenon, tendinopathies, synovitis, and joint contractures. Patients with SSc also suffer from multiorgan dysfunction, which makes them high-risk surgical patients. Read More

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Epilepsy in Parry-Romberg syndrome and linear scleroderma en coup de sabre: Case series and systematic review including 140 patients.

Epilepsy Behav 2021 May 27;121(Pt A):108068. Epub 2021 May 27.

Department of Neurology, University of Colorado, CU Anschutz Research Complex II, 12700 East 19th Avenue, Aurora, CO 80045, USA; Mayo Clinic Neurology and Neurosurgery, 13400 E Shea Blvd, Scottsdale, AZ 85259, USA. Electronic address:

Parry-Romberg syndrome (PRS) and linear sclerosis en coup de sabre (LScs) are rare, related, autoimmune conditions of focal atrophy and sclerosis of head and face which are associated with the development of focal epilepsy. The scarcity of PRS and LScs cases has made an evidence-based approach to optimal treatment of seizures difficult. Here we present a large systematic review of the literature evaluating 137 cases of PRS or LScs, as well as three new cases with epilepsy that span the spectrum of severity, treatments, and outcomes in these syndromes. Read More

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Mechanisms of Immunothrombosis in Vaccine-Induced Thrombotic Thrombocytopenia (VITT) Compared to Natural SARS-CoV-2 Infection.

J Autoimmun 2021 07 19;121:102662. Epub 2021 May 19.

Leeds Institute of Rheumatic and Musculoskeletal Medicine (LIRMM), University of Leeds, Leeds, UK. Electronic address:

Herein, we consider venous immunothrombotic mechanisms in SARS-CoV-2 infection and anti-SARS-CoV-2 DNA vaccination. Primary SARS-CoV-2 infection with systemic viral RNA release (RNAaemia) contributes to innate immune coagulation cascade activation, with both pulmonary and systemic immunothrombosis - including venous territory strokes. However, anti-SARS-CoV-2 adenoviral-vectored-DNA vaccines -initially shown for the ChAdOx1 vaccine-may rarely exhibit autoimmunity with autoantibodies to Platelet Factor-4 (PF4) that is termed Vaccine-Induced Thrombotic Thrombocytopenia (VITT), an entity pathophysiologically similar to Heparin-Induced Thrombocytopenia (HIT). Read More

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SARS-COV-2 vaccination after stem cell transplantation for scleroderma.

Ann Rheum Dis 2021 May 28. Epub 2021 May 28.

Department of Hematology and Bone Marrow Transplantation, Rambam medical center, Haifa, Israel.

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[The role Epstein-Barr virus played in the outcome of skin diseases].

Authors:
J W Lu Y Lu

Zhonghua Yi Xue Za Zhi 2021 Jun;101(20):1458-1462

Department of Dermatology, the First Affiliated Hospital of Nanjing Medical University, Nanjing 210029, China.

Since the discovery of Epstein-Barr (EB) virus from the cultured cells in Burkitt lymphoma tissue in African children in 1964, we have gradually taken in its oncogenicity and the link between this virus and multiple mucocutaneous diseases. This review exhibits the process of EB virus infection and summarizes the clinical manifestations and varied prognosis of EB virus-associated skin diseases. The cases of common skin diseases including psoriasis, dermatomyositis, pemphigus, pemphigoid, parapsoriasis, atopic dermatitis, urticaria, systemic lupus erythematosus, scleroderma, panniculitis,erythema multiforme and vasculitis coinciding with EB virus infection were collected for the analysis of their EB virus-associated complications, treatments and outcomes. Read More

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A Practical Approach to the Management of Digital Ulcers in Patients With Systemic Sclerosis: A Narrative Review.

JAMA Dermatol 2021 May 26. Epub 2021 May 26.

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

Importance: Digital ulcers (DUs) occurring on the fingers in patients with systemic sclerosis (SSc) are associated with substantial pain and disability and are often challenging to treat. However, careful clinical assessment and prompt intervention (wound bed management and systemic pharmacologic treatment) may modify the clinical course.

Objectives: To provide a practical approach to the assessment and management of SSc-DUs and highlight unmet needs and research priorities. Read More

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Hematopoietic stem cell transplantation for autoimmune diseases in the time of COVID-19: EBMT guidelines and recommendations.

Bone Marrow Transplant 2021 May 24. Epub 2021 May 24.

Department of Haematology, Sheffield Teaching Hospitals NHS Foundation Trust, Sheffield, UK.

Coronavirus disease-19 (COVID-19), caused by Severe Acute Respiratory Syndrome Coronavirus 2 (SARS-CoV-2), represents one of the biggest challenges of 21st century, threatening public health around the globe. Increasing age and presence of co-morbidities are reported risk factors for severe disease and mortality, along with autoimmune diseases (ADs) and immunosuppressive treatments such as haematopoietic stem cell transplantation (HSCT), which are also associated with adverse outcomes. We review the impact of the pandemic on specific groups of patients with neurological, rheumatological, and gastroenterological indications, along with the challenges delivering HSCT in adult and pediatric populations. Read More

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The Efficacy of a Home-Based, Self-Administered Hand Exercise Program for Patients With Systemic Sclerosis: A Randomized Controlled, Evaluator-Blind, Clinical Trial.

J Clin Rheumatol 2021 May 6. Epub 2021 May 6.

From the Division of Rheumatology, Department of Physical Medicine and Rehabilitation Division of Rheumatology, Department of Internal Medicine Department of Physical Medicine and Rehabilitation Department of Biostatistics and Medical Informatics, Faculty of Medicine, Cukurova University, Adana, Turkey.

Background: For patients with systemic sclerosis (SSc), hand involvement is an underrated clinical manifestation. Therefore, the aim of this study was to investigate the efficacy of a hand exercise program and to demonstrate its effect on hand function, quality of life, anxiety, and depression in patients with SSc.

Methods: This study was designed as a single blind, randomized controlled comparative study. Read More

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