Research Laboratory and Academic Division of Clinical Rheumatology, Department of Internal Medicine, University of Genova, IRCCS Ospedale Policlinico San Martino, Genoa.
Laser speckle contrast analysis (LASCA) is a good tool to evaluate the variation in peripheral blood perfusion during long-term follow-up and is able to safely monitor digital ulcer evolution in scleroderma patients. It evaluates blood perfusion in different areas within the skin lesions and surrounding them during standard treatment. Read More
c Department of Rheumatology , University College London , London , UK.
Objective: The epidemiology of polyautoimmunity in Sjögren's syndrome (secondary Sjögren's syndrome - sSS) is not well defined and has not been investigated before using a systematic approach. We conducted a systematic review of the epidemiology of sSS associated with rheumatoid arthritis (RA), systemic lupus erythematosus (SLE), scleroderma, and myositis, assessing the prevalence rates (PRs) and clinical and serological features of sSS.
Method: A systematic literature search of PubMed and Embase databases (updated to March 2016) was performed to identify all published data on PR, demographic profile, clinical manifestations, laboratory features, and causes of death associated with sSS. Read More
Division of Rheumatology, Brigham and Women's Hospital, Harvard Medical School, Boston, MA 02115, United States. Electronic address:
Interstitial lung disease (ILD) in the connective tissue diseases (CTD) is amongst the most challenging aspect of care of patients with rheumatic diseases and is the source of significant morbidity and mortality. While there has been progress in our understanding of the natural history of these complications, we still suffer from a limited reservoir of data to confidently determine which patients are at highest risk for disease and those who are at highest risk for disease progression. Treatment options until recently have been limited to anti-inflammatory therapies but with the emerging availability of anti-fibrotic therapies, a shift in strategy is emerging to target therapies based on the specific radiographic, histopathologic features and biomarker profiles that are unique to patients with rheumatic diseases and ILD. Read More
Johns Hopkins University School of Medicine, 5200 Eastern Avenue, Mason F. Lord Bldg, Center Tower, Suite 4100, Baltimore MD 21224, United-States. Electronic address:
Background: The incidence of cancer is increased in patients with systemic sclerosis (SSc). Further, recent studies have also shown that the presence of anti-RNA polymerase III antibodies is associated with a higher incidence of cancer in this population.
Patients And Methods: Herein we present the cases of two men aged 56 and 23 years presenting SSc without anti-Scl70 or anti-centromere antibodies but with anti-RNA polymerase III antibodies. Read More
Skin is an organ that is susceptible to damage by external injury, chronic inflammation, and autoimmunity. Tissue damage causes alterations in both the configuration and type of cells in lesional skin. This phenomenon, called tissue remodeling, is a universal biological response elicited by programmed cell death, inflammation, immune disorders, and tumorigenic, tumor proliferative, and cytoreductive activity. Read More
Cardiac manifestations in juvenile scleroderma or systemic sclerosis (JSSc) have poor prognosis, begin in early stages of the disease, and remain clinically asymptomatic. New echocardiography modalities, such as 2D/3D speckle tracking (STE, strain analysis for regional and global ventricular functions), can detect cardiac involvement in early stages. We assessed 21 JSSc patients and 19 controls using 2D/3D STE. Read More
Background: The aim of our study was to evaluate the serum concentration of klotho in a cohort of systemic sclerosis (SSc) patients compared to that of healthy controls and to correlate its levels with the degree and the kind of organ involvement.
Methods: Blood samples obtained from both patients and controls were collected and analysed by an ELISA test for the determination of human soluble klotho. Scleroderma patients were evaluated for disease activity through clinical, laboratory, and instrumental assessment. Read More
Background: Systemic sclerosis (SSc), or systemic scleroderma, is a chronic multisystem autoimmune disease characterised by widespread vascular injury and progressive fibrosis of the skin and internal organs. Patients with SSc have decreased survival, with pulmonary involvement as the main cause of death. Current treatments for SSc manage a range of symptoms but not the cause of the disease. Read More
Objectives: To compare microvascular damages on nailfold capillaroscopy (NC) with macrovascular manifestations evaluated by hand power doppler ultrasonography (PDUS) in Systemic Sclerosis (SSc) patients, and to assess the associations of these damages with the main digital manifestations of the disease: digital ulcers (DU), acro-osteolysis and Calcinosis.
Methods: NC, hand X-Rays and PDUS were systematically performed in 64 unselected SSc patients. PDUS evaluation with assessment of ulnar artery occlusion (UAO) and finger pulp blood flow (FPBF) was performed blinded for the results of X-Rays and NC. Read More
Centre for Musculoskeletal Research, The University of Manchester, Salford Royal NHS Foundation Trust, Manchester Academic Health Science Centre, Manchester, UK.
To canvas opinion concerning the role of non-invasive techniques in the assessment of patients with Raynaud's phenomenon (Rp) in clinical and research settings: four nailfold capillaroscopy methods [videocapillaroscopy (NVC), dermoscopy, stereomicroscopy, digital USB microscopy], four laser Doppler methods (laser Doppler flowmetry, imaging, anemometry/velocimetry, laser speckle contrast analysis), thermographic imaging, and upper limb arterial Doppler ultrasound. Emails with a link to the survey were sent to physicians from the European Scleroderma Trials and Research group (EUSTAR), the EULAR Study Group on Microcirculation in Rheumatic Diseases (SG_MC/RD) and members of the pediatric rheumatology Email board. The main descriptive analysis related to physicians looking after adult patients, with some analysis also of opinions from paediatric rheumatologists. Read More
Objectives: Following results in open-label studies of rituximab in patients with systemic sclerosis, a Belgian three-centre initiative was launched to explore safety and efficacy of rituximab in early, diffuse cutaneous systemic sclerosis (dcSSc).
Methods: Open-label study of 17 patients with early dcSSc, treated with two courses of rituximab, at month 0 and 6. Clinical examination, lung function testing, echocardiography, disease activity score (DAS) and functional status were performed at baseline and over 24 months of follow-up. Read More
Graduate Research Assistant, University of San Francisco, San Francisco, Calif.
A technique for using silver diamine fluoride (SDF) as part of a regimen to help maintain dental prostheses in a patient with scleroderma and at high risk of caries is presented. Medically compromised, xerostomic, or elderly patients generally face greater risk of caries and specifically with prosthetic retainer teeth. SDF is a minimally invasive solution to this problem. Read More
We found for the first time that IL-4 and IL-13, signature type 2 cytokines, are able to induce periostin expression. We and others have subsequently shown that periostin is highly expressed in chronic inflammatory diseases-asthma, atopic dermatitis, eosinophilc chronic sinusitis/chronic rhinosinusitis with nasal polyp, and allergic conjunctivitis-and that periostin plays important roles in the pathogenesis of these diseases. The epithelial/mesenchymal interaction via periostin is important for the onset of allergic inflammation, in which periostin derived from fibroblasts acts on epithelial cells or fibroblasts, activating their NF-κB. Read More
Monitoring of perfusion is a cornerstone in surgery, phlebology and basic science to proof wound healing by interventions. In chronic wound management it is of utmost importance to realize and parametrize wound bed perfusion to verify actual, and plan further treatment by noninvasive diagnostics. Up to now monitoring is based on visual inspection of wounds as conventionally practiced over more than decades. Read More
Objective: Peripheral blood mononuclear cells taken from patients with scleroderma express increased levels of IL-13. Moreover, the expression of MMP-1 from involved scleroderma skin fibroblasts is refractory to stimulation by tumor necrosis factor alpha. To elucidate the mechanism(s) involved, we examined the effect of IL-13 on TNF-α-induced matrix metalloproteinase-1 expression in normal and scleroderma human dermal fibroblast lines and studied the involvement of serine/threonine kinase B/Akt in this response. Read More
The classic manometric findings in systemic sclerosis are aperistalsis of the esophageal body with hypotensive lower esophageal sphincter. These changes contribute to gastroesophageal reflux disease in these patients. With widespread use of high-resolution esophageal manometry, diverse abnormalities are seen. Read More
Aim Of The Work: This study aims to assess Growth differentiation factor-15 (GDF-15) level in Scleroderma patients and its relation to disease manifestations.
Patients And Methods: This study included 55 scleroderma patients and 40 age and sex matched healthy volunteers. All patients were subjected to full history taking, thorough clinical examination, and laboratory investigations. Read More
Systemic sclerosis (scleroderma, SSc) is a devastating fibrotic disease with few treatment options. Fumaric acid esters, including dimethyl fumarate (DMF, Tecfidera®) have shown therapeutic effects in several disease models, prompting us to determine whether DMF is effective as a treatment for SSc dermal fibrosis. We found that DMF blocks the pro-fibrotic effects of TGFβ in SSc skin fibroblasts. Read More
Clinica Medica, Department of Internal Medicine, Ospedali Riuniti University Hospital, Ancona; and Department of Clinical and Molecular Sciences, Section of Clinical Medicine, Università Politecnica delle Marche, School of Medicine, Ancona, Italy.
Objectives: The objective of the study is to evaluate the feasibility, safety and efficacy of home infusion of iloprost with the new portable syringe pump Infonde®, for the treatment of scleroderma-related Raynaud's phenomenon and digital ulcers.
Methods: 12 scleroderma patients were treated with iloprost at home, using the pump, with infusion cycles of 2 days per month (24 hours a day), for 6 months.
Results: The home treatment proved feasible since ten patients (83%) completed the entire infusion cycle, thus satisfying the feasibility target imposed by the protocol (75%). Read More
Objectives: Severe oesophageal disease in patients with systemic sclerosis (SSc), referred as scleroderma oesophagus, is characterised by ineffective or absent peristalsis along with hypotensive oesophagogastric junction (hEGJ). The associations between scleroderma oesophagus and different clinical and laboratory manifestations of SSc is still controversial. In this study we aimed to assess associations between scleroderma oesophagus, diagnosed by high resolution manometry (HRM), and other manifestations of disease. Read More
Front Med (Lausanne) 2017 18;4:120. Epub 2017 Aug 18.
Department of Dermatology, University of Cologne, Cologne, Germany.
Sclerosing skin diseases represent a large number of distinct disease entities, which include systemic sclerosis, localized scleroderma, and scleredema adultorum. These pathologies have a common clinical appearance and share histological features. However, the specific interplay between cytokines and growth factors, which activate different mesenchymal cell populations and production of different extracellular matrix components, determines the biomechanical properties of the skin and the clinical features of each disease. Read More
Department of Biology, Faculty of Mathematics and Natural Sciences, Bogor Agricultural University, Darmaga Campus, Bogor, West Java, 16880, Indonesia.
This research aimed to study the ectomycorrhiza formed by basidiospores attached to the outer surface of Shorea leprosula (Dipterocarpaceae) seed collected from a lowland tropical dipterocarp forest. Two groups of seeds were collected: control seeds collected from plastic net hanging 2 m above the ground and forest floor seeds collected on the forest floor. Before planting, 15 seeds from each group were observed for basidiospores attached to the seed. Read More
Bioorganic Chemistry Laboratory, Council of Scientific and Industrial Research (CSIR) - Central Leather Research Institute (CLRI), Adyar, Chennai 600020, India; Academy of Scientific and Innovative Research (AcSIR), CSIR-CLRI Campus, Chennai 600020, India. Electronic address:
Excess accumulation of collagen (fibrosis) undergoes self-aggregation, which leads to fibrillar collagen, on the extracellular matrix is the hallmark of a number of diseases such as keloids, hypertrophic scars, and systemic scleroderma. Direct inhibition or disintegration of collagen fibrils by small molecules offer a therapeutic approach to prevent or treat the diseases related to fibrosis. Herein, the anti-fibrotic property of Glucono-δ-lactone (GdL), known as acidifier, on the fibrillation and its disintegration of collagen was investigated. Read More
CRIIS (Interdisciplinary, Interdepartmental and Specialistic Reference Center for Early Diagnosis of Scleroderma, Treatment of Sclerodermic Ulcers and Videocapillaroscopy), Sandro Pertini Hospital, Rome, Italy.
Background: Werner syndrome is a progeroid disorder characterized by premature age-related phenotypes. Although it is well established that autosomal recessive mutations in the WRN gene is responsible for Werner syndrome, the molecular alterations that lead to disease phenotype remain still unidentified.
Results: To address whether epigenetic changes can be associated with Werner syndrome phenotype, we analysed genome-wide DNA methylation profile using the Infinium MethylationEPIC BeadChip in the whole blood from three patients affected by Werner syndrome compared with three age- and sex-matched healthy controls. Read More
The peripheral vascular disease in systemic sclerosis (scleroderma) is a challenging problem because it presents with both vasospasm and an underlying structural vascular disease that leads not only to severe Raynaud phenomenon but often critical digital ischemia with progressive tissue damage or acute ulceration. While current therapy with a calcium channel blocker alone or combined with another vasodilating agent provides some modest benefit, it does not provide adequate control of Raynaud's events in many patients with scleroderma. Vasodilating therapy alone also does not address the underlying biological process causing progressive vascular disease. Read More
Katherine Alexis Athanasiou practices rheumatology at the Arthritis Institute of Long Island in Hicksville, N.Y. At Northwell Health System in New Hyde Park, N.Y., Sonu Sahni is a clinical research physician, Amrinder Rana is a research volunteer, and Arunabh Talwar is a physician in the Department of Pulmonary, Critical Care, and Sleep Medicine and director of the Advanced Lung Disease Center. The authors have disclosed no potential conflicts of interest, financial or otherwise.
Scleroderma is an uncommon autoimmune disease of unknown cause that may affect any organ system in the body. Patients with scleroderma are prone to developing pulmonary complications, including pulmonary arterial hypertension (PAH), that are the leading cause of death in this population. This article describes scleroderma-related PAH and its diagnosis and management. Read More
EQ-5D is becoming the preferred instrument to measure health-state utilities involved in health technology assessment. The objective of this study is to assess the state of EQ-5D research in musculoskeletal disorders in 8 Central and Eastern European countries and to provide a meta-analysis of EQ-5D index scores. Original research articles published in any language between Jan 2000 and Sept 2016 were included, if they reported any EQ-5D outcome from at least two musculoskeletal patients from Austria, Bulgaria, the Czech Republic, Hungary, Poland, Romania, Slovakia, or Slovenia. Read More
Rheumatology Clinic, Iuliu Hatieganu University of Medicine and Pharmacy, Cluj-Napoca, Romania.
Background: In patients with systemic sclerosis, NT-proBNP is a useful diagnostic marker for pulmonary hypertension and ventricular dysfunction, with important prognostic significance. The aim of this study was to assess the relationship between the NT-proBNP levels and the presence and severity of ventricular arrhythmias in patients with scleroderma.
Methods: Forty consecutive patients with a diagnostic of systemic sclerosis according to the EULAR criteria admitted at the Rheumatology Clinic of Cluj-Napoca, Romania, from Jan 2014 to Apr 2014 were enrolled. Read More
Innate lymphoid cells (ILCs) are innate immune cells that do not possess B or T cell receptors but belong to the lymphoid lineage. While these cells have not yet been extensively investigated since their classification as a homogenous group, emerging evidence suggests that they exert significant regulatory roles in both tissue remodelling and inflammation, and are therefore, also involved in fibrotic regulation. The following review will serve to outline the transcription factors, surface markers, and cytokines that define each subgroup, and the process by which these cells differentiate. Read More
Arthritis Rheumatol 2017 Aug 25. Epub 2017 Aug 25.
Division of Rheumatology, Departments of Medicine, Microbiology and Immunology, Biochemistry and Molecular Biology, State University of New York, Upstate Medical University, College of Medicine, Syracuse, New York, 13210.
Metabolic pathways exert profound influence over the development of unicellular and multi-cellular organisms. Engagement of antigen receptors and co-stimulatory molecules, growth factors, hormones, cytokines, environmental factors, and other regulatory cues shape the development of the immune system by reprogramming of metabolic gene expression in a cell type-specific manner. In fact, the heterogeneity of cells both within the innate and adaptive immune systems depends on the supply of metabolites that allow for lineage-specific differentiation. Read More
a Department of Internal Medicine, Scleroderma Unit, Clinical Immunology Unit , University of Genova and IRCCS-Azienda Ospedaliera Universitaria San Martino , Genova , Italy.
TNF-α inhibitors have demonstrated efficacy both as monotherapy and in combination with disease-modifying anti-rheumatic drugs (DMARDs) in the treatment of chronic inflammatory immune-mediated diseases such as rheumatoid arthritis, Crohn's disease, ulcerative colitis, ankylosing spondylitis (AS), psoriasis (Ps) and/or psoriatic arthritis (PsA) and may be administered off-label to treat disseminated granuloma annulare, systemic lupus erythematosus and systemic sclerosis. There are several TNF-α inhibitors available for clinical use including infliximab, adalimumab, golimumab, certolizumab pegol and etanercept. In this article, we discuss the efficacy and safety of etanercept in the treatment of spondyloarthritis and juvenile idiopathic arthritis (JIA). Read More
Systemic sclerosis (scleroderma) is a fibrotic condition characterized by immunologic abnormalities, vascular injury, and increased accumulation of extracellular matrix proteins in the affected organs. Although the etiology of scleroderma has not yet been fully elucidated, a growing body of evidence suggests that extracellular matrix overproduction by activated fibroblasts results from a complex interaction among endothelial cells, immunocytes, and fibroblasts, involving a number of mediators such as cytokines, chemokines, growth factors, and their receptors. For a better understanding of the pathophysiology of scleroderma, animal models are important tools. Read More
Objectives: To determine the causes of death and risk factors in systemic sclerosis (SSc).
Methods: Between 2000 and 2011, we examined the death certificates of all French patients with SSc to determine causes of death. Then we examined causes of death and developed a score associated with all-cause mortality from the international European Scleroderma Trials and Research (EUSTAR) database. Read More
Considering the role of endothelin-1 (ET-1) in tissue remodeling and fibrosis during the development of scleroderma as well as the effect of α-Klotho in pathogenesis of calcinosis and/or endothelial cell injury and its correlation with severity of disease, this study aimed to evaluate serum ET-1, α-Klotho and 25(OH) vitamin D levels in patients with limited and diffuse scleroderma compared to healthy subjects. In this cross-sectional study, 60 scleroderma patients according to the ACR/EULAR 2013 criteria and 60 age- and sex-matched healthy controls were included. In patients, clinical examination was performed and Medsger severity scale was assessed. Read More
Juvenile systemic sclerosis (JSSc) is a rare disorder with paucity of information on its treatment and longterm outcome. Herein, we are sharing our experience with this rare entity. Case records of children, diagnosed to have systemic sclerosis attending Pediatric Rheumatology Clinic at All India Institute of Medical Sciences, New Delhi from January 1998 to June 2016 were reviewed. Read More
Laboratorio de Acuicultura Experimental, Departamento de Ciencias Biológicas, Centro Universitario de la Costa, Universidad de Guadalajara, Puerto Vallarta, Jalisco, México. Electronic address:
Background: The consumption of wild mushrooms has increased in recent years. However, not all mushrooms are edible and some of them may cause poisoning. Therefore, their toxicity needs to be studied. Read More
Background: Latex agglutination test for Kala-azar (KAtex) is an easy, inexpensive, and field-applicable antigen detection test. However, the main drawback of this method is the boiling step applied to remove false positivity of the test. This study was conducted to survey false positivity results of latex agglutination test for KAtex without boiling process in urine of some autoimmune patients. Read More
Department of Medicine, Cumming School of Medicine, University of Calgary, Calgary, Alberta, Canada; Department of Community Health Sciences, Cumming School of Medicine, University of Calgary, Calgary, Alberta, Canada. Electronic address:
Background: Indigenous populations of Canada, America, Australia, and New Zealand have increased rates and severity of rheumatic disease. Our objective was to summarize mortality outcomes and explore disease and social factors related to mortality.
Methods: A systematic search was performed in medical (Medline, EMBASE, and CINAHL), Indigenous and conference abstract databases (to June 2015) combining search terms for Indigenous populations and rheumatic diseases. Read More
Halofuginone (HF) is a derivative of dichroine which is the extract of traditional Chinese medicine. It is widely used as an efficient anticoccidial drug. Recent studies have found that HF has unique biological activities, showing great potential capacities in the treatment of autoimmune diseases. Read More
The aim of this study was to validate the 2013 ACR/EULAR classification criteria for systemic sclerosis (SSc) in patients with SSc, including patients with early SSc. Fifty-six consecutive patients with early SSc (2001 LeRoy and Medsger criteria), 122 patients with established SSc (1980 ACR classification criteria), and 141 patients with SSc-like disorders were included in this cross-sectional study. The diagnostic performance of the 2013 ACR/EULAR criteria was compared with the 1980 ACR criteria in several subsets of patients. Read More
Tetrachloroethene (perchloroethylene, PCE), an ubiquitous environmental contaminant, has been implicated in inducing autoimmunity/autoimmune diseases (ADs), including systemic lupus erythematosus (SLE) and scleroderma in humans. However, experimental evidence suggesting the potential of PCE in mediating autoimmunity is lacking. This study was, therefore, undertaken to explore PCE's potential in inducing/exacerbating an autoimmune response. Read More
Purpose: Systemic sclerosis or scleroderma is a chronic, rare connective tissue disease with negative physical and psychological implications. Coping strategies used by scleroderma patients have not been studied in-depth. The objective of the present study was to gain a greater understanding of the coping strategies employed by people living with scleroderma. Read More
Background: Nintedanib is an inhibitor targeting platelet-derived growth factor receptor, fibroblast growth factor receptor and vascular endothelial growth factor receptor tyrosine kinases that has recently been approved for the treatment of idiopathic pulmonary fibrosis. The aim of this study was to analyse the effects of nintedanib in the fos-related antigen-2 (Fra2) mouse model of systemic sclerosis (SSc).
Methods: The effects of nintedanib on pulmonary arterial hypertension with proliferation of pulmonary vascular smooth muscle cells (PVSMCs) and luminal occlusion, on microvascular disease with apoptosis of microvascular endothelial cells (MVECs) and on fibroblast activation with myofibroblast differentiation and accumulation of extracellular matrix were analysed. Read More
Background: Scurfy mice have a functional defect in regulatory T cells (Treg), which leads to lethal multi-organ inflammation. The missing Treg function results in uncontrolled autoimmune cellular and humoral inflammatory responses. We and others have previously shown that during the course of disease scurfy mice develop severe skin inflammation and autoantibodies including anti-nuclear autoantibodies (ANA). Read More