25,668 results match your criteria Diffuse Sclerosis


Subcorneal Pustular Dermatosis: A Case Report of a Patient with Diffuse Scleroderma.

Adv Biomed Res 2018 23;7:83. Epub 2018 May 23.

Department of Dermatology, Skin Diseases and Leishmaniasis Research Center, Isfahan University of Medical Sciences, Isfahan, Iran.

Subcorneal pustular dermatosis (SPD) or Sneddon-Wilkinson disease is a rare, benign, chronic, sterile pustular eruption which is associated with various systemic diseases including immunoglobinopathies, neoplasms, and autoimmune disorders. This paper reports a case of SPD in a patient with diffuse scleroderma in a 37-year-old woman. The hypothesis that immune dysregulation may play a role in the pathogenesis of SPD was supposed by the coexistence of diffuse scleroderma and SPD in our patient. Read More

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May 2018
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[Correlation between nailfold capillaroscopic findings and presence of interstitial lung disease in systemic sclerosis patients].

Beijing Da Xue Xue Bao Yi Xue Ban 2018 Jun;50(3):501-506

Department of Rheumatology and Immunology, Peking University First Hospital, Beijing 100034, China.

Objective: To evaluate the correlation between nailfold capillaroscopic (NC) findings and the presence of interstitial lung disease (ILD) in systemic sclerosis (SSc) patients.

Methods: We retrospectively involved 71 SSc patients, 45 patients with ILD. NC was performed in all the patients according to the standard method. Read More

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Prospective evaluation of frequency of genital lichen sclerosus in 79 patients with systemic sclerosis.

Br J Dermatol 2018 Jun 21. Epub 2018 Jun 21.

Clinique dermatologique des hôpitaux universitaires et faculté de médecine, Université de Strasbourg, 1, place de l'Hôpital, 67091, Strasbourg cedex, France.

Skin sclerosis is a hallmark of morphea (localized scleroderma), lichen sclerosus (LiS) and systemic sclerosis (SSc). Genital LiS is significantly more frequent in patients with morphea [1], but its prevalence in patients with SSc is unknown. The aim of this prospective multicenter study was to determine the prevalence of LiS in a cohort of patients with SSc, and to compare it to an age-and sex-matched control group. Read More

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Case series: rheumatological manifestations attributed to exposure to Libby Asbestiform Amphiboles.

J Toxicol Environ Health A 2018 Jun 21:1-14. Epub 2018 Jun 21.

e Icahn School of Medicine at Mt Sinai , New York NY.

An increased risk for Systemic Autoimmune Diseases (SAID) has been reported in Libby, Montana, where extensive exposures to fibrous amphiboles occurred due to mining and use of asbestos-laden vermiculite. In addition, positive antinuclear autoantibody tests are associated with exposure to Libby Asbestiform Amphiboles (LAA) in both humans and mice. Among 6603 subjects who underwent health screening at the Center for Asbestos Related Diseases (CARD, Libby MT), 13. Read More

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[Scleromyxedema as a systemic disease of glycosaminoglycan accumulation].

Arkh Patol 2018 ;80(3):53-58

V.A. Nasonova Research Institute of Rheumatology, Moscow, Russia.

Scleromyxedema is a rare mucinosis with a primary skin lesion due to diffuse mucin deposition, sclerosis, and lichenoid eruptions in the absence of hypothyroidism. The paper describes scleromyxedema cases and gives recommendations for the histological diagnosis of the disease by histochemical reactions to detect acid and neutral glycosaminoglycans. Read More

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January 2018

Novel classification for global gene signature model for predicting severity of systemic sclerosis.

PLoS One 2018 20;13(6):e0199314. Epub 2018 Jun 20.

Department of Health Promotions and Development, University of Pittsburgh School of Nursing, Pittsburgh, PA, United States of America.

Progression of systemic scleroderma (SSc), a chronic connective tissue disease that causes a fibrotic phenotype, is highly heterogeneous amongst patients and difficult to accurately diagnose. To meet this clinical need, we developed a novel three-layer classification model, which analyses gene expression profiles from SSc skin biopsies to diagnose SSc severity. Two SSc skin biopsy microarray datasets were obtained from Gene Expression Omnibus. Read More

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June 2018
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Systemic Sclerosis in the Intensive Care Unit: A Case Series and Literature Review.

J Clin Rheumatol 2018 Jun 15. Epub 2018 Jun 15.

Introduction: Systemic sclerosis (SSc) is a multisystemic autoimmune disease. Few studies have focused on the outcomes of SSC patients who require intensive care unit (ICU) admission, largely due to the absence of protocols for the optimal management of this disease during an ICU stay.

Objectives: This study aimed to describe the outcomes of a series of SSc patients admitted to the ICU at a single center in Cali, Colombia. Read More

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June 2018
2 Reads

Focal and diffuse cervical spinal cord damage in patients with early relapsing-remitting MS: A multicentre magnetisation transfer ratio study.

Mult Scler 2018 Jun 1:1352458518781999. Epub 2018 Jun 1.

IRISA, UMR CNRS 6074, VisAGeS U1228, INSERM, INRIA, Université Rennes I, Rennes, France.

Background: Studies including patients with well-established multiple sclerosis (MS) have shown a significant and disability-related reduction in the cervical spinal cord (SC) magnetisation transfer ratio (MTR).

Objectives: The objectives are to (1) assess whether MTR reduction is already measurable in the SC of patients with early relapsing-remitting multiple sclerosis (RRMS) and (2) describe its spatial distribution.

Methods: We included 60 patients with RRMS <12  months and 34 age-matched controls at five centres. Read More

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Lymphedema secondary to limited cutaneous systemic sclerosis.

BMJ Case Rep 2018 Jun 15;2018. Epub 2018 Jun 15.

Internal Medicine, Centro Hospitalar e Universitário de Coimbra, Coimbra, Portugal.

Systemic sclerosis (SSc) is an autoimmune connective tissue disease characterised by vascular abnormalities, immune system activation and fibrosis. Lymphatic involvement in SSc was described more recently and starts in early stages. This report describes a 46-year-old patient who developed over the last 2 years asymmetric lymphedema in lower extremities. Read More

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June 2018
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Symptoms of Autonomic Dysfunction in Systemic Sclerosis Assessed by the COMPASS-31 Questionnaire.

J Rheumatol 2018 Jun 15. Epub 2018 Jun 15.

From the Department of Rheumatology, Johns Hopkins University School of Medicine; the departments of Neurology, Anatomy, and Neurobiology, University of Maryland School of Medicine; the Veterans Affairs Medical Center, Baltimore, Maryland, USA. Supported by the Rheumatology Research Foundation Scientist Development Award, the Jerome L. Greene Foundation (90057213), the Scleroderma Research Foundation (ZHM), the Johns Hopkins Clinician Scientist Research Career Development Award (ZHM), and the US National Institute of Arthritis and Musculoskeletal and Skin Diseases (BLA, T32AR048522). JWR was supported in part by the US National Institute of Diabetes and Digestive and Kidney Diseases, the National Institutes of Health and Office of Research Development (1R01DK107007-01A1), the Department of Veterans Affairs (Biomedical and Laboratory Research Service and Rehabilitation Research and Development, 101RX001030). B.L. Adler, MD, Rheumatology Fellow, Department of Rheumatology, Johns Hopkins University School of Medicine; J.W. Russell, MD, Professor of Neurology, departments of Neurology, Anatomy and Neurobiology, University of Maryland School of Medicine and Veterans Affairs Medical Center; L.K. Hummers, MD, MSc, Associate Professor of Medicine, Department of Rheumatology, Johns Hopkins University School of Medicine; Z.H. McMahan, MD, MHS, Assistant Professor of Medicine, Department of Rheumatology, Johns Hopkins University School of Medicine. Drs. Hummers and McMahan contributed equally to this work. Author correspondence to Dr. B.L. Adler, 5200 Eastern Ave., MFL Center Tower, Suite 4100, Baltimore, Maryland 21224, USA. E-mail: Accepted for publication March 2, 2018.

Objective: Autonomic dysfunction is a known complication of systemic sclerosis (SSc) that can affect vascular tone, gastrointestinal (GI) motility, heart rate, and blood pressure control. We sought to quantify autonomic symptom burden in SSc, and to define the characteristics of patients with SSc and autonomic dysfunction.

Methods: Patients with SSc were consecutively recruited during routine clinical visits at the Johns Hopkins Scleroderma Center and asked to complete the Composite Autonomic Symptom Score (COMPASS)-31 questionnaire, a validated tool to assess symptoms of autonomic dysfunction. Read More

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June 2018
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Ictal signs in tuberous sclerosis complex: Clinical and video-EEG features in a large series of recorded seizures.

Epilepsy Behav 2018 Jun 12;85:14-20. Epub 2018 Jun 12.

Epilepsy Center-Child Neuropsychiatric Unit, ASST Santi Paolo e Carlo, Milan, Italy; Department of Health Sciences, University of Milan, Italy.

Epilepsy is the most common neurological symptom in tuberous sclerosis complex (TSC), occurring in 72-85% of affected individuals. Despite the large number of patients reported, their electroclinical phenotype has been rarely described. We analyzed seizure semiology through ictal video-electroencephalography (V-EEG) recordings in a large series of patients. Read More

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June 2018
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[Non-infective endocarditis].

Rev Med Interne 2018 Jun 11. Epub 2018 Jun 11.

Service des maladies infectieuses et de réanimation médicale, CHU Pontchaillou, 2, rue Henri-Le-Guilloux, 35033 Rennes cedex, France. Electronic address:

Non-infective endocarditis, also referred to as non-bacterial thrombotic endocarditis, represent a wide range of rare pathologies, often severe. This review gathered the data available in the literature, to decipher the major information collected on the pathophysiology, the diagnosis and the treatment of these heterogeneous diseases, often misdiagnosed. Characteristics of non-infective endocarditis are similar to infective endocarditis in terms of valvular lesions (mostly left-sided, with regurgitations and vegetations), and their complications (embolism). Read More

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Antinuclear antibodies and cancer: A literature review.

Crit Rev Oncol Hematol 2018 Jul 26;127:42-49. Epub 2018 May 26.

Infanta Sofía University Hospital, SS de los Reyes, Madrid, Spain. Electronic address:

Antinuclear antibodies (ANAs) are a spectrum of autoantibodies targeted to various nuclear and cytoplasmic components of the cells. They are very useful as serological markers for different autoimmune disease, like systemic lupus erythematosus (SLE), Sjögren's syndrome (SS), scleroderma, polymyositis, or mixed connective tissue disease. In these years, an increasing attention has been focussed in the relationship between tumours and autoimmunity. Read More

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Is laser speckle contrast analysis (LASCA) the new kid on the block in systemic sclerosis? A systematic literature review and pilot study to evaluate reliability of LASCA to measure peripheral blood perfusion in scleroderma patients.

Autoimmun Rev 2018 Jun 6. Epub 2018 Jun 6.

Department of Rheumatology, Ghent University Hospital, Department of Internal Medicine, Ghent University, Corneel Heymanslaan 10, Ghent, Belgium. Electronic address:

Objectives: A reliable tool to evaluate flow is paramount in systemic sclerosis (SSc). We describe herein on the one hand a systematic literature review on the reliability of laser speckle contrast analysis (LASCA) to measure the peripheral blood perfusion (PBP) in SSc and perform an additional pilot study, investigating the intra- and inter-rater reliability of LASCA.

Methods: A systematic search was performed in 3 electronic databases, according to the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) guidelines. Read More

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Predictors of morbidity and mortality in early systemic sclerosis: Long-term follow-up data from a single-centre inception cohort.

Autoimmun Rev 2018 Jun 6. Epub 2018 Jun 6.

First Department of Propaedeutic and Internal Medicine and Joint Academic Rheumatology Program, National and Kapodistrian University, Medical School of Athens, Greece. Electronic address:

Objectives: To determine predictors of morbidity and mortality in systemic sclerosis (SSc) in a long-term follow-up of an inception cohort of early SSc patients.

Methods: We evaluated clinical manifestations, laboratory and lung function tests at disease onset as predictors of morbidity and mortality in 3rd, 6th and 9th year in SSc patients recruited within 12 months of disease onset.

Results: A total of 115 SSc patients (97 women, mean age 48. Read More

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Reliability of digital ulcer definitions as proposed by the UK Scleroderma Study Group: A challenge for clinical trial design.

J Scleroderma Relat Disord 2018 Jun 27;3(2):170-174. Epub 2018 Mar 27.

Centre for Musculoskeletal Research, The University of Manchester, Salford Royal NHS Foundation Trust, Manchester Academic Health Science Centre, Manchester, UK.

Introduction: The reliability of clinician grading of systemic sclerosis-related digital ulcers has been reported to be poor to moderate at best, which has important implications for clinical trial design. The aim of this study was to examine the reliability of new proposed UK Scleroderma Study Group digital ulcer definitions among UK clinicians with an interest in systemic sclerosis.

Methods: Raters graded (through a custom-built interface) 90 images (80 unique and 10 repeat) of a range of digital lesions collected from patients with systemic sclerosis. Read More

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June 2018
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Prediction of progression of interstitial lung disease in patients with systemic sclerosis: the SPAR model.

Ann Rheum Dis 2018 Jun 6. Epub 2018 Jun 6.

Department of Rheumatology, University Hospital Zurich, Zurich, Switzerland.

Objectives: To identify the predictive clinical characteristics and establish a prediction model for the progression of mild interstitial lung disease (ILD) in patients with systemic sclerosis (SSc).

Methods: Patients with SSc from two independent prospective cohorts were included in this observational study. All patients fulfilled the 2013 American College of Rheumatology/European League Against Rheumatism criteria, had mild ILD at baseline diagnosed by High-Resolution Computed Tomography (HRCT), available baseline and ≥1 annual follow-up pulmonary function tests and no concomitant pulmonary hypertension or airflow obstruction. Read More

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June 2018
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The contemporary management of systemic sclerosis.

Expert Rev Clin Immunol 2018 Jun 20:1-10. Epub 2018 Jun 20.

a Division of Rheumatology, St. Joseph's Health Care, Department of Medicine , University of Western Ontario , Ontario , Canada.

Introduction: Systemic sclerosis (SSc) is a rare autoimmune connective tissue disease characterized by vascular dysfunction, fibrosis, inflammation and autoantibodies. The pathophysiology of SSc is not completely understood, and many patients acquire organ or tissue damage despite advances in treatment. Current treatments target affected organs with modest improvements. Read More

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June 2018
4 Reads

Overall and sex- and disease subtype-specific mortality in patients with systemic sclerosis : An updated meta-analysis.

Authors:
Y H Lee

Z Rheumatol 2018 Jun 4. Epub 2018 Jun 4.

Department of Rheumatology, Korea University Anam Hospital, Korea University College of Medicine, 73, Inchon-ro, Seongbuk-gu, 02841, Seoul, Korea (Republic of).

Objective: To assess the overall and sex- and disease subtype-specific standardized mortality ratios (SMRs) in patients with systemic sclerosis (SSc).

Methods: We surveyed studies examining overall and sex- and disease subtype-specific SMRs in patients with SSc compared with the general population using MEDLINE, EMBASE, and Cochrane databases and manual searches. A meta-analysis of the overall and sex- and disease subtype-specific SMRs in patients with SSc was then performed. Read More

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June 2018
3 Reads

Decreased CD22 expression and intracellular signaling aberrations in B cells of patients with systemic sclerosis.

Rheumatol Int 2018 07 4;38(7):1225-1234. Epub 2018 Jun 4.

Division of Internal Medicine, Department of Rheumatology, Patras University Hospital, University of Patras Medical School, 26504, Patras, Greece.

The objective of the study was to explore the phenotype and intracellular signaling events of B cells in patients with systemic sclerosis (SSc). Peripheral blood B cell surface markers CD19 and CD22 were evaluated by flow cytometry in 23 patients with SSc and seven healthy individuals. Levels of intracellular kinases Lyn, Syk and P-Y 348 Syk along with phosphatase SHP-1 were examined with Western immunoblotting in selected patients. Read More

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July 2018
2 Reads

A Feasibility Study of a Novel Low Level Light Therapy for Digital Ulcers in Systemic Sclerosis.

J Dermatolog Treat 2018 Jun 4:1-30. Epub 2018 Jun 4.

a Centre for Musculoskeletal Research, The University of Manchester, Salford Royal NHS Foundation Trust, Manchester Academic Health Science Centre , Manchester , UK.

Background: Locally acting, well-tolerated treatments for systemic sclerosis (SSc) digital ulcers (DUs) are needed.

Objectives: Our primary aim was to investigate the safety, feasibility and tolerability of a novel low level light therapy (LTTT). A secondary aim was to tentatively assess efficacy. Read More

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June 2018
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Hearing loss in patients with scleroderma: associations with clinical manifestations and capillaroscopy.

Clin Rheumatol 2018 Jun 2. Epub 2018 Jun 2.

Departments of Internal Medicine, Division of Rheumatology, Namazee Hospital, Shiraz University of Medical Sciences, Shiraz, 71345-1414, Iran.

Systemic sclerosis is a multi-systemic disease with widespread small-vessel vasculopathy and fibrosis. Involvement of the middle and inner ear and hearing loss has been reported as an uncommon manifestation of scleroderma in some studies. In this study, we evaluated hearing problems in scleroderma patients and determined its association with clinical manifestations and capillaroscopy. Read More

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June 2018
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Oxidative stress in autoimmune rheumatic diseases.

Free Radic Biol Med 2018 May 30. Epub 2018 May 30.

University of Exeter Medical School, St Luke's Campus, Exeter, Devon EX1 2LU, UK.

The management of patients with autoimmune rheumatic diseases such as rheumatoid arthritis (RA) remains a significant challenge. Often the rheumatologist is restricted to treating and relieving the symptoms and consequences and not the underlying cause of the disease. Oxidative stress occurs in many autoimmune diseases, with the excess production of reactive oxygen species (ROS) and reactive nitrogen species (RNS). Read More

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May 2018
3 Reads

Skin Gene Expression Is Prognostic for the Trajectory of Skin Disease in Patients With Diffuse Cutaneous Systemic Sclerosis.

Arthritis Rheumatol 2018 Jun;70(6):912-919

University of Pittsburgh Medical Center, Pittsburgh, Pennsylvania.

Objective: At present, there are no clinical or laboratory measures that accurately forecast the progression of skin fibrosis and organ involvement in patients with systemic sclerosis (SSc). The goal of this study was to identify skin biomarkers that could be prognostic for the progression of skin fibrosis in patients with early diffuse cutaneous SSc (dcSSc).

Methods: We analyzed clinical data and gene expression in skin biopsy samples from 38 placebo-treated patients, part of the Roche Safety and Efficacy of Subcutaneous Tocilizumab in Adults with Systemic Sclerosis (FASSCINATE) phase II study of tocilizumab in SSc. Read More

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June 2018
2 Reads

Response to: 'Not all drugs (and routes) are same' by Zanwar and Gandhi.

Ann Rheum Dis 2018 Jun 1. Epub 2018 Jun 1.

Internal Medicine, Referral Center for Rare Systemic and Autoimmune Diseases: Vasculitis and Scleroderma, Cochin Hospital, Paris Descartes University, Paris, France.

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June 2018
2 Reads

Should all digital ulcers be included in future clinical trials of systemic sclerosis-related digital vasculopathy?

Med Hypotheses 2018 Jul 2;116:101-104. Epub 2018 May 2.

Centre for Musculoskeletal Research, The University of Manchester, Salford Royal NHS Foundation Trust, Manchester Academic Health Science Centre, Manchester, UK; NIHR Manchester Biomedical Research Centre, Central Manchester NHS Foundation Trust, Manchester Academic Health Science Centre, UK.

Digital ulcers (DU) are a common manifestation of systemic sclerosis (SSc) and occur at a variety of locations including the fingertips and over the extensor aspects of the hands. However, most recent clinical trials have included only fingertip DUs as these are believed to be ischaemic in aetiology, and therefore likely to benefit from treatment with vasoactive drug therapies. There is an emerging evidence base to suggest that all DUs in SSc could share an ischaemic component which is potentially responsive to vascular therapy. Read More

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July 2018
3 Reads

Impact of systemic sclerosis oral manifestations on patients' health-related quality of life: a systematic review.

J Oral Pathol Med 2018 Jun 1. Epub 2018 Jun 1.

Unité de médecine bucco-dentaire, Hôpital Pellegrin, CHU de Bordeaux, place Amélie Raba-Léon, 33076, Bordeaux, France.

Background: Oropharyngeal features are frequent and often understated in the treatment clinical guidelines of systemic sclerosis in spite of important consequences on comfort, esthetics, nutrition and daily life. The aim of this systematic review was to assess a correlation between the oropharyngeal manifestations of systemic sclerosis and patients' health-related quality of life.

Methods: A systematic search was conducted using four databases [PubMed , Cochrane Database , Dentistry & Oral Sciences Source , and SCOPUS ] up to January 2018, according to the Preferred reporting items for systematic reviews and meta analyses. Read More

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June 2018
3 Reads

Refractory Hepatic Hydrothorax: A Rare Complication of Systemic Sclerosis and Presinusoidal Portal Hypertension.

Case Reports Hepatol 2018 30;2018:2704949. Epub 2018 Apr 30.

Department of Pathology, Greenville Health System, University of South Carolina School of Medicine Greenville, Greenville, SC, USA.

We report on a rare case of refractory hepatic hydrothorax in an individual with Scleroderma/CREST syndrome and noncirrhotic portal hypertension. Portal pressure measurements revealed a normal transjugular hepatic venous portal pressure gradient, mild pulmonary hypertension, and an unremarkable liver biopsy except for mild sinusoidal dilation. Pulmonary hypertension, cardiac diastolic dysfunction, and chronic kidney disease were determined to be the causes of his refractory pleural effusions and ascites. Read More

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April 2018
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Value of cardiac magnetic resonance imaging in systemic sclerosis.

Reumatologia 2018 9;56(2):92-98. Epub 2018 May 9.

Internal Medicine Department, Faculty of Medicine, Khon Kaen University, Thailand.

Objectives: To assess the prevalence and patterns of cardiac abnormalities as detected by cardiac magnetic resonance imaging (MRI) in systemic sclerosis.

Material And Methods: Twenty-six consecutive patients with systemic sclerosis underwent cardiac MRI to determine morphological, functional, perfusion at rest, and delayed enhancement abnormalities.

Results: At least one abnormality on cardiac MRI was observed in 19/26 (73%) patients. Read More

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Therapeutic interleukin-6 blockade reverses transforming growth factor-beta pathway activation in dermal fibroblasts: insights from the faSScinate clinical trial in systemic sclerosis.

Ann Rheum Dis 2018 May 31. Epub 2018 May 31.

Genentech, South San Francisco, California, USA.

Objectives: Skin fibrosis mediated by activated dermal fibroblasts is a hallmark of systemic sclerosis (SSc), especially in the subset of patients with diffuse disease. Transforming growth factor-beta (TGFβ) and interleukin-6 (IL-6) are key candidate mediators in SSc. Our aim was to elucidate the specific effect of IL-6 pathway blockade on the biology of SSc fibroblasts in vivo by using samples from a unique clinical experiment-the faSScinate study-in which patients with SSc were treated for 24 weeks with tocilizumab (TCZ), an IL-6 receptor-α inhibitor. Read More

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May 2018
4 Reads

Higher levels of serum interleukin-35 are associated with the severity of pulmonary fibrosis and Th2 responses in patients with systemic sclerosis.

Rheumatol Int 2018 May 30. Epub 2018 May 30.

Department of Rheumatology and Clinical Immunology, The First Affiliated Hospital of Guangxi Medical University, Nanning, Guangxi, China.

The aim of the study is to investigate the levels of serum interleukin-35 (IL-35) before and after treatment and its relationship with clinical parameters as well as the frequency of different subsets of CD4 T cells in patients with systemic sclerosis (SSc) to explore the role of IL-35 in the pathogenesis of SSc. The levels of serum IL-35, interferon-γ (IFN-γ), IL-4, IL-17A, and IL-10 in 49 patients with SSc and 50 age- and gender-matched healthy subjects were measured by enzyme-linked immunosorbent assay (ELISA). The percentages of peripheral blood Th1, Th2, Th17 cells and Tregs in 49 SSc patients and 20 healthy controls were characterized by flow cytometry. Read More

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Comorbidities in vitiligo: comprehensive review.

Int J Dermatol 2018 May 28. Epub 2018 May 28.

Department of Dermatology, Bispebjerg Hospital, Copenhagen, Denmark.

Vitiligo is a common skin disorder characterized by idiopathic, progressive cutaneous hypomelanosis. Vitiligo is associated with several comorbid autoimmune, systemic, and dermatological diseases, primarily thyroid disease, alopecia areata, diabetes mellitus, pernicious anemia, systemic lupus erythematosus, rheumatoid arthritis, Addison's disease, inflammatory bowel disease, Sjögren's syndrome, dermatomyositis, scleroderma, ocular and audiological abnormalities, psoriasis, and atopic dermatitis. It is essential to increase awareness of these comorbidities in order to improve the disease burden and quality of life of patients with vitiligo. Read More

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May 2018
2 Reads

Diffuse cardiac fibrosis quantification in early systemic sclerosis by magnetic resonance imaging and correlation with skin fibrosis.

J Scleroderma Relat Disord 2018 Jun 12;3(2):159-169. Epub 2018 Apr 12.

Feinberg Cardiovascular Research Institute, 303 E. Chicago Avenue, Tarry 14-725, Chicago, IL 60611.

Purpose: To evaluate the utility of cardiac magnetic resonance (CMR) T1 mapping in early systemic sclerosis (SSc) and its association with skin score.

Methods: Twenty-four consecutive patients with early SSc referred for cardiovascular evaluation and 12 controls without SSc were evaluated. All patients underwent cine, T1 mapping, and late gadolinium enhanced (LGE) CMR imaging. Read More

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June 2018
2 Reads

Asiatic acid prevents the development of interstitial lung disease in a hypochlorous acid-induced mouse model of scleroderma.

Oncol Lett 2018 Jun 2;15(6):8711-8716. Epub 2018 Apr 2.

Department of Pulmonary Medicine, The First Affiliated Hospital of Wenzhou Medical University, Wenzhou, Zhejiang 325000, P.R. China.

Interstitial lung disease is the most common complication of systemic sclerosis (SSc) and is associated with a high rate of mortality. Due to the complex pathogenesis of SSc, the therapies currently available remain limited. In the present study, the effect of asiatic acid (AA) on SSc-associated pulmonary fibrosis (PF) and its association with the transforming growth factor-β1 (TGF-β1)/Smad2/3 signaling pathway were evaluated. Read More

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June 2018
3 Reads

[Sclerodermatous changes revealing porphyria cutanea tarda].

Authors:
A Brunet E Hainaut

Ann Dermatol Venereol 2018 May 24. Epub 2018 May 24.

Service de dermatologie, CHU de Poitiers, 2, rue de la Milétrie, 86021 Poitiers, France.

Background: Porphyria cutanea tarda (PCT) is associated with cutaneous accumulation of porphyrins. This accumulation results from a deficiency of uroporphyrinogen decarboxylase occurring only in the liver. The classical presentation is blistering on sun-exposed areas. Read More

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May 2018
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Vascular complications in systemic sclerosis: a prospective cohort study.

Clin Rheumatol 2018 May 26. Epub 2018 May 26.

Division of Rheumatology, Johns Hopkins University School of Medicine, 5200 Eastern Ave, MFL Bldg, Center Tower, Suite 4100, Baltimore, MD, 21224, USA.

Two major complications in scleroderma patients that cause substantial morbidity and mortality are ischemic digital lesions (DL) and pulmonary hypertension (PH). The clinician's ability to predict which patients will develop these complications is imperfect. We conducted a prospective observational cohort study of 300 patients with scleroderma who were followed for at least a 5-year period. Read More

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May 2018
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Systemic scleroderma-related interstitial pneumonia associated with borderline pulmonary arterial hypertension.

BMJ Case Rep 2018 May 26;2018. Epub 2018 May 26.

Department of Respiratory Medicine, Toho University Omori Medical Center, Tokyo, Japan.

A 65-year-old woman with a 35-year history of limited cutaneous systemic scleroderma was admitted to our hospital complaining of a 3-month history of progressive dyspnoea on exertion. High-resolution CT images of the chest revealed diffuse reticular opacities and traction bronchiectasis predominantly in the bilateral lower lobes of the lung. Specimens obtained during video-assisted thoracic surgery were consistent with fibrocellular non-specific interstitial pneumonia and accompanied by accumulation of lymph follicles within areas of fibrosis. Read More

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May 2018
6 Reads

Systemic sclerosis-rheumatoid arthritis overlap syndrome complicated with Sweet's syndrome.

Clin Rheumatol 2018 May 25. Epub 2018 May 25.

Department of Dermatology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Shuai Fu Yuan No.1, Dongcheng District, Beijing, 100730, China.

Herein, we report a case of a 34-year-old woman with systemic sclerosis (SSc)-rheumatoid arthritis (RA) overlap syndrome (OS) complicated with Sweet's syndrome. OS has been defined as entities satisfying classification criteria of at least two connective tissue diseases (CTD) occurring at the same or at different times in the same patient. The CTD include RA, SSc, systemic lupus erythematosus (SLE), polymyositis, and dermatomyositis. Read More

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May 2018
1 Read

The impact of anti-U1-RNP positivity: systemic lupus erythematosus versus mixed connective tissue disease.

Rheumatol Int 2018 07 23;38(7):1169-1178. Epub 2018 May 23.

Internal Medicine Department, Carol Davila University of Medicine and Pharmacy, Dionisie Lupu Street 37, 020022, Bucharest S2, Romania.

Anti-U1-RNP positivity remains mandatory for the mixed connective tissue disease (MCTD) diagnosis, reason for which anti-U1-RNP occurrence in patients with lupus clinical features might determine diagnostic issues. Thus, the prevalence of 25-30% for anti-RNP was reported in John Hopkins and LUMINA lupus cohorts and also 13% prevalence for the anti-U1-RNP in Euro-Lupus cohort. Presence of anti-U1-RNP antibodies in patients fulfilling SLE criteria (but not the MCTD ones) was associated with manifestations such as Raynaud phenomenon, musculoskeletal and lung impairment or nail fold capillaroscopy changes, some clinical features frequently encountered in MCTD patients and only rarely described in lupus population. Read More

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July 2018
2 Reads

Existing and novel biomarkers for precision medicine in systemic sclerosis.

Nat Rev Rheumatol 2018 Jul;14(7):421-432

Jefferson Institute of Molecular Medicine, Thomas Jefferson University, Philadelphia, PA, USA.

The discovery and validation of biomarkers resulting from technological advances in the analysis of genomic, transcriptomic, lipidomic and metabolomic pathways involved in the pathogenesis of complex human diseases have led to the development of personalized and rationally designed approaches for the clinical management of such disorders. Although some of these approaches have been applied to systemic sclerosis (SSc), an unmet need remains for validated, non-invasive biomarkers to aid in the diagnosis of SSc, as well as in the assessment of disease progression and response to therapeutic interventions. Advances in global transcriptomic technology over the past 15 years have enabled the assessment of microRNAs that circulate in the blood of patients and the analysis of the macromolecular content of a diverse group of lipid bilayer membrane-enclosed extracellular vesicles, such as exosomes and other microvesicles, which are released by all cells into the extracellular space and circulation. Read More

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July 2018
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A novel CHCHD10 mutation implicates a Mia40-dependent mitochondrial import deficit in ALS.

EMBO Mol Med 2018 Jun;10(6)

German Center for Neurodegenerative Diseases (DZNE) Munich, Munich, Germany

mutations are linked to amyotrophic lateral sclerosis, but their mode of action is unclear. In a 29-year-old patient with rapid disease progression, we discovered a novel mutation (Q108P) in a conserved residue within the coiled-coil-helix-coiled-coil-helix (CHCH) domain. The aggressive clinical phenotype prompted us to probe its pathogenicity. Read More

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Parasympathetic activity increases with digital microvascular damage and vascular endothelial growth factor in systemic sclerosis.

Clin Exp Rheumatol 2018 May 11. Epub 2018 May 11.

Department of Clinical Medicine, Scleroderma Unit, Sapienza University of Rome, Italy.

Objectives: The imbalance between angiogenic and angiostatic factors with derangement of the microvasculature are hallmarks of systemic sclerosis (SSc). Raynaud's phenomenon in SSc probably is due to the impaired neuroendothelial control mechanisms between vasoconstriction and vasodilatation. The aim of this study is to evaluate autonomic nervous system function using heart rate variability (HRV) analysis and to correlate with vascular endothelial growth factor (VEGF). Read More

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May 2018
4 Reads

Diagnostic and Treatment Strategies of Dermatologists for Treating Morphea in Hungary.

Acta Dermatovenerol Croat 2018 Apr;26(1):21-24

Gabor Bali, MD, Department of Dermatology, Venereology and Dermatooncology, Faculty of Medicine, Semmelweis University, Mária u. 41. 1085 Budapest, Hungary;

Localized scleroderma is an uncommon disease, only infrequently encountered by dermatologists in private practices or even in larger academic centers. Because of its rarity, current treatment guidelines are mostly based on low-level clinical evidence and expert opinions. The aim of this study was to evaluate treatment strategies to treat localized scleroderma. Read More

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April 2018
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Smoking in Systemic Sclerosis: a Longitudinal European Scleroderma Trials and Research Group Study.

Arthritis Rheumatol 2018 May 21. Epub 2018 May 21.

Department of Rheumatology, University Hospital Basel, Basel, Switzerland.

Objectives: Data on the role of tobacco exposure in systemic sclerosis (SSc) severity and progression are scarce. We aimed to assess the effects of smoking on the evolution of pulmonary and skin manifestations in the EUSTAR database.

Methods: Adult SSc patients with data on smoking history and a 12-24 months follow-up visit were included. Read More

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May 2018
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Treatment algorithms for systemic sclerosis according to experts.

Arthritis Rheumatol 2018 May 21. Epub 2018 May 21.

Rheumatology Division, Department of Medicine, University of Western Ontario, London, ON, Canada.

Introduction: Treatment for many aspects of systemic sclerosis (SSc) lacks agreement.

Objectives: To generate SSc treatment algorithms endorsed by high percentage of SSc experts.

Methods: Experts from the Scleroderma Clinical Trials Consortium and the Canadian Scleroderma Research group (N=170) were asked whether they agreed with SSc algorithms (from 2012). Read More

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May 2018
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Ultrasonography for the assessment of skin in systemic sclerosis: a systematic review.

Arthritis Care Res (Hoboken) 2018 May 21. Epub 2018 May 21.

Department of Rheumatology, Centro Hospitalar e Universitário de Coimbra, Coimbra, Portugal.

Objective: To identify and synthesise the best available evidence on the use of ultrasound to assess skin involvement in systemic sclerosis (SSc).

Methods: We conducted a systemic review of the literature on Pubmed MEDLINE and Embase, using the vocabulary terms: ('systemic sclerosis OR scleroderma') AND ('ultrasonography' OR 'Elasticity Imaging Techniques') AND ('skin' OR 'dermis'). Two independent reviewers selected articles, collected data from studies and carried out a manual search of the references from the studies included. Read More

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May 2018
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Long-term Outcomes in Systemic Sclerosis Associated Pulmonary Arterial Hypertension from the Pulmonary Hypertension Assessment and Recognition of Outcomes in Scleroderma Registry (PHAROS).

Chest 2018 May 16. Epub 2018 May 16.

Division of Immunology and Rheumatology, Department of Medicine, Stanford University School of Medicine, Palo Alto, CA; Division of Rheumatology, Department of Medicine, Palo Alto VA Health Care System, Palo Alto, CA.

Background: Pulmonary arterial hypertension (PAH) is a leading cause of death in patients with systemic sclerosis (SSc). The purpose of this study was to assess long-term outcomes in patients with SSc-PAH.

Methods: Pulmonary Hypertension Assessment and Recognition of Outcomes in Scleroderma is a prospective registry of SSc patients at high risk for or with incident pulmonary hypertension based on right heart catheterization (RHC). Read More

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May 2018
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Dermoscopic patterns of filiform papillae of the tongue in patients with and without connective tissue autoimmune diseases.

Int J Dermatol 2018 May 18. Epub 2018 May 18.

Department of Stomatology, Institute of Biomedical Sciences, Autonomous University of Ciudad Juárez, Ciudad Juárez, Chihuahua, Mexico.

Background: Connective tissue autoimmune diseases (CTADs) constitute a group of conditions, including rheumatoid arthritis; systemic lupus erythematosus; mixed connective tissue disease; calcinosis, Raynaud's phenomenon, esophageal dysmotility, sclerodactyly, and telangiectasia (CREST) syndrome; scleroderma; dermatomyositis; and Sjögren syndrome. There are few studies on the alterations in filiform papillae in CTAD. Thus, the objective of this work was to determine whether there are changes in the macroscopic and dermoscopic patterns of filiform papillae. Read More

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May 2018
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Scleroderma in children.

Authors:
Francesco Zulian

Best Pract Res Clin Rheumatol 2017 Aug 27;31(4):576-595. Epub 2018 Mar 27.

Division of Pediatric Rheumatology, Department of Woman and Child Health, University of Padua, Padua, Italy. Electronic address:

Juvenile scleroderma with its two varieties, juvenile localized scleroderma and systemic sclerosis (SSc), represents the third most frequent rheumatic disease in childhood. In juvenile SSc, new developments have been recently reported in the fields of classification and monitoring. The introduction of new classification criteria for adult SSc has stimulated new ideas on how to improve the performance of the provisional 2007 PRES/ACR/EULAR pediatric criteria. Read More

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August 2017
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The Anti-fibrotic Effect of Adenosine A2B Receptor Antagonism in a Mouse Model of Dermal Fibrosis.

Arthritis Rheumatol 2018 May 17. Epub 2018 May 17.

Department of Biochemistry and Molecular Biology, McGovern Medical School, Houston, TX, USA.

Objective: Systemic sclerosis (SSc - scleroderma) is a chronic disease that affects the skin and various internal organs. Dermal fibrosis is a major component of this disease. The mechanisms that promote dermal fibrosis remain elusive. Read More

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May 2018
7 Reads