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    Changes in the pattern of death of 987 patients with systemic sclerosis from 1990 to 2009 from the nationwide Spanish Scleroderma Registry (RESCLE).
    Clin Exp Rheumatol 2017 Feb 6. Epub 2017 Feb 6.
    Vall d'Hebron University Hospital, Department of Internal Medicine, Barcelona, Spain.
    Objectives: To determine the changes in the pattern of death of patients with systemic sclerosis (SSc) throughout 20 years.

    Methods: Data were collected from the Spanish Scleroderma Registry (RESCLE), retrospective multicentre database from 1990 to 2009. SSc-related and SSc-non related causes of death were assessed. Read More

    Relationship between calcium channel blockers and skin fibrosis in patients with systemic sclerosis.
    Clin Exp Rheumatol 2017 Jan 31. Epub 2017 Jan 31.
    St. Joseph's Healthcare Hamilton, ON; and Division of Rheumatology, Department of Medicine, McMaster University, Hamilton, ON, Canada.
    Objectives: Recent experimental evidence suggests that calcium channel blockers (CCBs) may have anti-fibrotic effects on liver and pulmonary fibrosis. We aimed to investigate whether use of CCBs was associated with the skin fibrosis in patients with systemic sclerosis (SSc).

    Methods: Based on the 5-year follow-up data from the Canadian Scleroderma Research Group registry, we used the generalised estimating equations (GEE) model to assess the relationship between use of CCBs and the primary outcome of skin fibrosis measured by the modified Rodnan skin score (mRSS). Read More

    Multiplex assessment of serum cytokine and chemokine levels in idiopathic morphea and vitamin K1-induced morphea.
    Clin Rheumatol 2017 Feb 20. Epub 2017 Feb 20.
    Scleroderma Center and Jefferson Institute of Molecular Medicine, Jefferson Medical College, Thomas Jefferson University, Philadelphia, PA, USA.
    The levels of 63 cytokines, chemokines, and growth factors were measured in the serum of four patients with idiopathic morphea and of one patient with vitamin K1-induced morphea employing a multiplex assay to identify the role of inflammatory/immunologic events in their pathogenesis. Full-thickness skin biopsies of affected skin were analyzed by histopathology. Luminex assays for 63 cytokines, chemokines, and growth factors were performed in the sera from four patients with idiopathic morphea and in two different samples of serum obtained in two separate occasions from one patient with vitamin K1-induced morphea. Read More

    Anti-RNPC3 Antibodies as a Marker of Cancer-Associated Scleroderma.
    Arthritis Rheumatol 2017 Feb 19. Epub 2017 Feb 19.
    Division of Rheumatology, Johns Hopkins University School of Medicine, Baltimore, MD.
    Introduction: Prior studies have demonstrated an increased risk of cancer-associated scleroderma in patients with RNA polymerase III (POL) autoantibodies and in patients negative for anti-centromere (CENP), anti-topoisomerase-1 (TOPO), and anti-POL antibodies (referred to as CENP/TOPO/POL (CTP)-Negative). In a recent study of 16 CTP-negative scleroderma patients with coincident cancer, we found that 25% had autoantibodies to RNPC3, a member of the minor spliceosome complex. In this investigation, we validated the relationship between anti-RNPC3 antibodies and cancer and examined the associated clinical phenotype in a large sample of scleroderma patients. Read More

    Reasons for Not Participating in Scleroderma Patient Support Groups: A Cross-sectional Study.
    Arthritis Care Res (Hoboken) 2017 Feb 19. Epub 2017 Feb 19.
    Scleroderma Support Group Project Advisory Team Members.
    Objective: Peer-led support groups are an important resource for many people with scleroderma (systemic sclerosis; SSc). Little is known, however, about barriers to participation. The objective of this study was to identify reasons why some people with SSc do not participate in SSc support groups. Read More

    Development and validation of a patient-reported outcome instrument for skin involvement in patients with systemic sclerosis.
    Ann Rheum Dis 2017 Feb 17. Epub 2017 Feb 17.
    Division of Rheumatology and Clinical Immunology, University of Pittsburgh Medical Center, Pittsburgh, Pennsylvania, USA.
    Objectives: We developed a patient-reported outcome (PRO) instrument to assess the skin-related quality of life in patients with systemic sclerosis (SSc).

    Methods: Participants with SSc provided input on skin-related health effects through focus groups. We developed items for scleroderma skin PRO (SSPRO) to encompass these effects. Read More

    Towards developing criteria for scleroderma renal crisis: A scoping review.
    Autoimmun Rev 2017 Feb 14. Epub 2017 Feb 14.
    Faculty of Medicine, McGill University, Montreal, Quebec, Canada; Lady Davis Institute for Medical Research, Jewish General Hospital, Montreal, Quebec, Canada; Department of Medicine, Division of Rheumatology, Jewish General Hospital, Montreal, Quebec, Canada.
    Objective: The absence of a gold standard for scleroderma renal crisis (SRC) has hindered our understanding of this problem. The objective of this scoping review was to identify the criteria used to define SRC in order to guide the development of a consensus definition for SRC.

    Methods: We conducted a search in three databases: Medline, Embase and non-Ovid Pubmed. Read More

    Morphea "en coup de sabre": An unusual oral presentation.
    J Clin Exp Dent 2017 Feb 1;9(2):e315-e318. Epub 2017 Feb 1.
    DDS, MSc, Department of Oral Medicine and Oral Pathology, Universidad Andres Bello, Viña del Mar, Chile. Avenida Valparaíso 1560, Viña del Mar, Chile.
    Morphea, or localized scleroderma, is an inflammatory disease that leads to sclerosis of the skin and underlying tissues due to excessive collagen deposition. Oral involvement is unusual and it may produce white linear fibrotic areas with a scar-like appearance, atrophy of tongue papillae, gingival recession and alveolar bone resorption. We report a case of a 13-year-old girl who consulted for progressive recession on the attached gingiva of her upper left incisors. Read More

    Pulmonary hypertension in connective tissue diseases: an update.
    Int J Rheum Dis 2017 Feb 16. Epub 2017 Feb 16.
    Department ofClinical Immunology & Rheumatology, Christian Medical College, Vellore, Tamil Nadu, India.
    Pulmonary hypertension (PH) is a relatively commoner complication of systemic sclerosis (SSc) with estimated prevalence ranging between 8% and 12% as compared to much lower figures in other connective tissue diseases (CTD). It is a major cause of morbidity and mortality in CTDs. PH is classified into five major groups. Read More

    Treatment outcome in early diffuse cutaneous systemic sclerosis: the European Scleroderma Observational Study (ESOS).
    Ann Rheum Dis 2017 Feb 10. Epub 2017 Feb 10.
    UCL Division of Medicine, Centre for Rheumatology and Connective Tissue Diseases, London, UK.
    Objectives: The rarity of early diffuse cutaneous systemic sclerosis (dcSSc) makes randomised controlled trials very difficult. We aimed to use an observational approach to compare effectiveness of currently used treatment approaches.

    Methods: This was a prospective, observational cohort study of early dcSSc (within three years of onset of skin thickening). Read More

    Review article: pathogenesis and clinical manifestations of gastrointestinal involvement in systemic sclerosis.
    Aliment Pharmacol Ther 2017 Feb 9. Epub 2017 Feb 9.
    Jefferson Institute of Molecular Medicine and Scleroderma Center, Thomas Jefferson University, Sidney Kimmel Medical College, Philadelphia, PA, USA.
    Background: Gastrointestinal tract (GIT) involvement is a common cause of debilitating symptoms in patients with systemic sclerosis (SSc). There are no disease modifying therapies for this condition and the treatment remains symptomatic, largely owing to the lack of a clear understanding of its pathogenesis.

    Aims: To investigate novel aspects of the pathogenesis of gastrointestinal involvement in SSc. Read More

    Recent advances in managing systemic sclerosis.
    F1000Res 2017 30;6:88. Epub 2017 Jan 30.
    Division of Rheumatology, Medicine III, University Medical Center and Faculty of Medicine Carl Gustav Carus at the TU Dresden, Dresden, Germany.
    How the main components in systemic sclerosis-namely autoimmunity, vasculopathy, and fibrosis-fit together is still not sufficiently clear. However, vascular treatment options are well established, the body of evidence for the efficacy of immunomodulatory approaches is increasing, and now at least one hopeful substance that may directly interfere with fibrosis is being tested. Although we still wait for important breakthroughs, there is grounds for hope that better therapeutic options will be available in the near future. Read More

    Characteristics of human adipose derived stem cells in scleroderma in comparison to sex and age matched normal controls: implications for regenerative medicine.
    Stem Cell Res Ther 2017 Feb 7;8(1):23. Epub 2017 Feb 7.
    Charles Wolfson Center for Reconstructive Surgery, Royal Free Hospital, London, UK.
    Background: Adipose-derived stem cells (ADSCs) are emerging as an alternative stem cell source for cell-based therapies. Recent data suggest that autologous ADSC-enriched micrografting improves the effects of facial involvement in systemic sclerosis (SSc). We have extensively characterised ADSCs from SSc patients and compared their phenotype and function to healthy age- and sex-matched control ADSCs. Read More

    Phenotypic Alterations Involved in CD8+ Treg Impairment in Systemic Sclerosis.
    Front Immunol 2017 19;8:18. Epub 2017 Jan 19.
    Center of Excellence for Biomedical Research, University of Genoa, Genoa, Italy; Department of Internal Medicine, Clinical Immunology Unit, University of Genoa, Genoa, Italy.
    Systemic sclerosis (SSc) is a connective tissue disease characterized by tissue fibrosis, vasculopathy, and autoimmunity. Although the exact pathogenetic mechanisms behind SSc remain to be fully elucidated, a great deal of evidence suggests the existence of an unbalanced ratio between the effector and regulatory arms of the immune system. With regard to the T regulatory (Treg) compartment, we observed that CD8+ Treg subsets display functional defects in SSc-affected patients. Read More

    A Case of Eosinophilic Fasciitis Associated with Pyoderma Gangrenosum.
    J Eur Acad Dermatol Venereol 2017 Feb 1. Epub 2017 Feb 1.
    Department of Internal Medicine, Henry Ford Hospital, Detroit, MI, 48202.
    Eosinophilic fasciitis (EF) is a rare, systemic inflammatory disease that is characterized by symmetrical swelling and scleroderma-like indurations of the distal extremities and trunk, often accompanied by pain and eosinophilia. Rarely, muscle degeneration may occur. Histologically, EF is marked by thickened, fibrotic fascia and fascial inflammation with lymphocytes and eosinophils. Read More

    Biomechanical podiatric evaluation in an Italian cohort of patients with systemic sclerosis: A pilot study.
    Eur J Rheumatol 2016 Dec 1;3(4):169-174. Epub 2016 Dec 1.
    Division of Rheumatology, Department Of Experimental and Clinical Medicine, University of Florence, Florence, Italy.
    Objective: Foot problems are often present in Systemic Sclerosis (SSc) patients, however studies regarding podiatric problems related to SSc are lacking and there are no data evaluating the foot biomechanical changes. The aim of the present pilot study was to evaluate podiatric problems in an Italian cohort of SSc patients by assessing received podiatric services, foot pain and disability and biomechanical foot deformity.

    Material And Methods: 25 consecutive SSc patients were enrolled from the Division of Rheumatology, University of Florence. Read More

    A case report of coup de sabre patient with hair transplantation.
    J Dermatolog Treat 2017 Jan 31:1-6. Epub 2017 Jan 31.
    b Tongde Hospital of Zhejiang Province , Hangzhou , China.
    Background: Treatment of coup de sabre must remain conservative until the disease is no longer in an active state. When activity has ceased, some operative intervention is safe and effective for the correction of deformity. While hair transplantation showed high survival rates for the correction of cicatricial alopecia, it has rarely reported to be performed for the correction of coup de sabre. Read More

    Cell Transplant 2017 Jan 31. Epub 2017 Jan 31.
    Scleroderma Clinic, Rheumatology Department, ASST G. Pini-CTO, Milano, Italy.

    Mesenchymal stromal cells (MSCs) have received attention as an ideal source of regenerative cells because of their multi-potential abilities. Adipose tissue (AT) is an attractive source of MSCs. Recent studies have shown that autologous fat grafting may be effective in the treatment of systemic sclerosis (SSc), but no specific study exists aimed at investigating whether AT-MSCs from SSc patients maintain normal phenotypic and functional characteristics. Read More

    Quantitative nanohistological investigation of scleroderma: an atomic force microscopy-based approach to disease characterization.
    Int J Nanomedicine 2017 11;12:411-420. Epub 2017 Jan 11.
    Department of Biomaterials and Tissue Engineering, UCL Eastman Dental Institute.
    Scleroderma (or systemic sclerosis, SSc) is a disease caused by excess crosslinking of collagen. The skin stiffens and becomes painful, while internally, organ function can be compromised by the less elastic collagen. Diagnosis of SSc is often only possible in advanced cases by which treatment time is limited. Read More

    Dual endothelin receptor antagonists contrast the effects induced by endothelin-1 on cultured human microvascular endothelial cells.
    Clin Exp Rheumatol 2017 Jan 27. Epub 2017 Jan 27.
    Research Laboratory and Academic Division of Clinical Rheumatology, Department of Internal Medicine, University of Genova, IRCCS AOU San Martino Hospital, Genoa, Italy.
    Objectives: To evaluate the ability of dual endothelin (ET) receptor antagonists (ETA/ETB -ETA/BRAs) to contrast the ET-1-induced effects on cultured human microvascular endothelial cells (HMVECs).

    Methods: Some cultured HMVECs were untreated, or treated with ET-1 (100nM) or transforming growth factor β1 (TGFβ1, 10ng/mL) alone for 6 days, in order to induce the endothelial-to-mesenchymal transition (EndoMT). Other cultured HMVECs were pre-treated for 1hr with ETA/BRAs bosentan (10μM) or macitentan (1μM, 10μM) before the stimulation with ET-1 for 6 days. Read More

    Gastrointestinal Manifestations of Systemic Sclerosis.
    J Scleroderma Relat Disord 2016 18;1(3):247-256. Epub 2016 Oct 18.
    Division of Rheumatology, Department of Internal Medicine, University of Michigan Medical School, Ann Arbor, MI, USA.
    In patients with systemic sclerosis (SSc), gastrointestinal (GI) tract involvement is almost universal. Any segment of the GI tract from mouth to anus can be involved, and GI symptoms are a frequent cause of morbidity. In severe cases, GI tract involvement can progress to the point of malnutrition requiring parenteral nutrition. Read More

    Bone marrow-derived mesenchymal stem cells expressing thioredoxin 1 attenuate bleomycin-induced skin fibrosis and oxidative stress in scleroderma.
    J Invest Dermatol 2017 Jan 26. Epub 2017 Jan 26.
    Department of Dermatology, Tongji Hospital, Tongji University School of Medicine, 389 Xin-Cun Road, Shanghai 200065. Electronic address:
    Systemic sclerosis (SSc) is an autoimmune disorder that affects multiple organs. It is characterised by a thickening of the dermis and connective tissue caused by collagen accumulation, and vascular injuries that induce hypoxia. The present study investigated the therapeutic potential of bone marrow-derived mesenchymal stem cells (BMSCs) expressing thioredoxin 1 (Trx-1) in treating SSc-mediated skin disease following transplantation into a bleomycin-induced murine model. Read More

    Survival and outcomes after lung transplantation for non-scleroderma connective tissue-related interstitial lung disease.
    J Heart Lung Transplant 2016 Dec 31. Epub 2016 Dec 31.
    Division of Pulmonary and Critical Care, Hospital of the University of Pennsylvania, Philadelphia, Pennsylvania; Division of Rheumatology, Brigham and Women's Hospital, Boston, Massachusetts. Electronic address:
    Background: Patients with non-scleroderma connective tissue-related lung disease (NS-CTLD), including rheumatoid arthritis, idiopathic inflammatory myopathies, Sjögren syndrome, mixed connective tissue disease, and systemic lupus erythematosus, may be at risk for worse outcomes after lung transplantation because of immune dysregulation or extrapulmonary manifestations of their underlying disease. We compared survival, acute and chronic rejection, and extrapulmonary organ dysfunction after transplantation in patients with NS-CTLD and idiopathic pulmonary fibrosis (IPF).

    Methods: This was a retrospective cohort study of patients with NS-CTLD and IPF who were listed in the Scientific Registry of Transplant Recipients and underwent lung transplantation from May 5, 2005, to March 1, 2016. Read More

    Effects of UVA1 Phototherapy on Expression of Human Endogenous Retroviral Sequence (HERV)-K10 gag in Morphea: A Preliminary Study.
    Med Sci Monit 2017 Jan 28;23:505-512. Epub 2017 Jan 28.
    Department of Dermatology, Poznań University of Medical Sciences, Poznań, Poland.
    BACKGROUND Morphea, also known as localized scleroderma, is a rare autoimmune connective tissue disease characterized by skin fibrosis. UVA1 phototherapy is an important asset in the reduction of clinical manifestations in morphea. There are studies claiming that UV light modulates the expression of some human endogenous retroviral sequences. Read More

    Endothelial dysfunction, microvascular damage and ischemic peripheral vasculopathy in systemic sclerosis.
    Clin Hemorheol Microcirc 2017 Jan 27. Epub 2017 Jan 27.
    Clinical Immunology Unit, Centro Hospitalar do Porto (CHP), Instituto de Ciências Biomédicas Abel Salazar, University of Porto (ICBAS/UP), Multidisciplinar Unit of biomedical investigation (UMIB), Porto, Portugal.
    Objective: To evaluate endothelial dysfunction and microvascular damage in secondary Raynaud Phenomenon (SRP) and Systemic sclerosis (SSc)-associated patients as possible predictors of ischemic fingertip digital ulcers (DU) in a 3-year clinical follow-up.

    Methods: Flow-mediated dilatation (FMD), nailfold videocapillaroscopy (NVC), endothelin-1 (ET-1) and asymmetric dimethylarginine (ADMA) were analysed in a 3-year observational cohort study of 77 SRP patients with systemic sclerosis. The primary outcome was the occurrence of a new DU. Read More

    Human cytomegalovirus (HCMV) UL44 and UL57 specific antibody responses in anti-HCMV-positive patients with systemic sclerosis.
    Clin Rheumatol 2017 Jan 26. Epub 2017 Jan 26.
    Department of Rheumatology and Clinical Immunology, Faculty of Medicine, School of Health Sciences, University of Thessaly, Viopolis, 40500, Larissa, Thessaly, Greece.
    The role of human cytomegalovirus (HCMV) has been postulated as a trigger of systemic sclerosis (SSc). The aim of the study was to assess the prevalence of antibodies against HCMV UL44 and UL57 antigens not tested in the past. Sixty SSc patients, 40 multiple sclerosis and 17 normal controls (NCs), all anti-HCMV positive, were tested by immunoblotting. Read More

    Kidney transplantation in patients with systemic sclerosis: a nationwide multicenter study.
    Transpl Int 2017 Jan 25. Epub 2017 Jan 25.
    Centre Hospitalier Universitaire de Rouen, Nephrology Dialysis Transplantation, Rouen, Haute-Normandie, FR.
    Kidney transplantation is one of the therapeutic options for end-stage renal disease (ESRD) in systemic sclerosis (SS). Current evidence demonstrates poorer patient and graft survival after transplantation in SS than in other primary kidney diseases. All the patients presenting ESRD associated with SS who had received a kidney allograft between 1987 and 2013 were systematically included from 20 French kidney transplantation centres. Read More

    Fluoroscopy-guided intra-articular steroid injection for sternoclavicular joint arthritis secondary to limited cutaneous systemic sclerosis: a case report.
    Korean J Pain 2017 Jan 30;30(1):59-61. Epub 2016 Dec 30.
    Division of Rheumatology, Department of Physical Medicine and Rehabilitation, Erciyes University, School of Medicine, Kayseri, Turkey.
    We report a case of fluoroscopy-guided intraarticular steroid injection for sternoclavicular joint (SCJ) arthritis caused by limited cutaneous systemic sclerosis (SSc). A 50-year-old woman diagnosed with limited cutaneous SSc presented with swelling and pain in the right SCJ. MRI revealed signs of inflammation consistent with right-sided sternoclavicular joint arthritis. Read More

    Innate Immunity in Systemic Sclerosis.
    Curr Rheumatol Rep 2017 Jan;19(1)
    Department of Applied Sciences, Faculty of Health and Life Sciences, Northumbria University, Ellison Building, Newcastle Upon Tyne, NE2 8ST, UK.
    Purpose Of Review: Systemic sclerosis (SSc) is a heterogeneous autoimmune disease which has defined three hallmarks: Small vessel vasculopathy, production of autoantibodies and fibroblast dysfunction. The exact aetiology of the disease remains unknown, due to the complex nature of the cellular signalling pathways involved. However, there is strong and consistent evidence that the innate system, in particular toll-like receptor signalling, is contributing to the progression and perhaps onset of systemic sclerosis. Read More

    Calcinosis Cutis Circumscripta Of Knee-A Rare Presentation.
    J Orthop Case Rep 2016 Jul-Aug;6(3):78-79
    Department of Orthopaedics, ACPM Medical College, Dhule-424001, Maharashtra, India.
    Introduction: Calcinosis circumscripta is a form of ectopic mineralization characterized by single or multiple cutaneous nodules containing calcium salts. Calcinosis cutis circumscripta is a very rare form of idiopathic calcinosis cutis arising in the second half oflife. It typically involves the extremities and is associated with prior trauma and scleroderma. Read More

    Artifactual Hypoglycaemia in Systemic Sclerosis and Raynaud's Phenomenon: A Clinical Case Report and Short Review.
    Case Rep Endocrinol 2016 28;2016:7390927. Epub 2016 Dec 28.
    Department of Endocrinology and Metabolism, Concord Repatriation General Hospital, Concord, Sydney, NSW 2139, Australia; Sydney Medical School, University of Sydney, Sydney, NSW 2005, Australia.
    Background. Artifactual hypoglycaemia, defined as a discrepancy between glucometer (capillary) and plasma glucose levels, may lead to overtreatment and costly investigations. It is not infrequently observed in patients with Raynaud's phenomenon due to vascular capillary distortion, yet this is clinically underappreciated. Read More

    Self-care behaviors and trajectory management by people with scleroderma in northeastern Thailand.
    Int J Nurs Pract 2017 Jan 23. Epub 2017 Jan 23.
    Faculty of Medicine, Khon Kaen University, Khon Kaen, Thailand.
    Scleroderma, particularly systemic sclerosis, is a chronic illness that affects a person's physical, emotional, psychosocial, and spiritual dimensions. To investigate self-care deficits, health behaviors, and trajectory management of Thai people with systemic sclerosis, an exploratory case study method was used through interviews and patient records. Self-care deficit nursing theory and the chronic illness trajectory framework guided the research. Read More

    [Plasma exchange in nephrology: Indications and technique].
    Nephrol Ther 2017 Feb 16;13(1):43-55. Epub 2017 Jan 16.
    Service des urgences néphrologiques et transplantation rénale, hôpital Tenon, 4, rue de la Chine, 75020 Paris, France; Université Pierre-et-Marie-Curie, 4, place Jussieu, 75005 Paris, France.
    Plasma exchange is a non-selective apheresis technique that can be performed by filtration or centrifugation allowing rapid purification of high molecular weight pathogens. An immunosuppressive treatment is generally associated to reduce the rebound effect of the purified substance. Substitution solutes such as human albumin and macromolecules are needed to compensate for plasma extraction. Read More

    Adipose tissue-derived stem cells ameliorates dermal fibrosis in a mouse model of scleroderma.
    Asian Pac J Trop Med 2017 Jan 9;10(1):52-56. Epub 2016 Nov 9.
    Department of Dermatology, Department of Ultrasound, General Hospital of Beijing Military Command, Beijing, China. Electronic address:
    Objective: To investigate the therapeutic potential of adipose-derived stem cells (ADSCs) for limited cutaneous scleroderma (LS) in mouse models.

    Methods: ADSCs were isolated from pathogen-free female C57BL/6 mice and LS was induced in wild type (WT) C57BL/6 mice via daily injection of bleomycin (0.1 mL × 300 μg/mL) for 4 weeks; then the ADSCs were subcutaneously injected into the dorsal area in the model treatment group, and 100 μL of phosphate-buffered saline (PBS) solution was injected into the same site in the model control group. Read More

    Histopathological findings in systemic sclerosis-related myopathy: fibrosis and microangiopathy with lack of cellular inflammation.
    Ther Adv Musculoskelet Dis 2017 Jan 3;9(1):3-10. Epub 2016 Oct 3.
    Scleroderma Unit, Department of Medicine, Surgery and Neurosciences, University of Siena, Italy.
    Objectives: The objective of this study was to identify specific histopathological features of skeletal muscle involvement in systemic sclerosis (SSc) patients.

    Methods: A total of 35 out of 112 SSc-patients (32%, including 81% female and 68% diffuse scleroderma) presenting clinical, biological and electromyographic (EMG) features of muscle weakness, were included. Patients underwent vastus lateralis biopsy, assessed for individual pathologic features including fibrosis [type I collagen (Coll-I), transforming growth factor β (TGF-β)], microangiopathy [cluster of differentiation 31 (CD31), pro-angiogenic vascular endothelial growth factor A (VEGF-A), anti-angiogenic VEGF-A165b], immune/ inflammatory response [CD4, CD8, CD20, human leucocyte antigens ABC (HLA-ABC)], and membranolytic attack complex (MAC). Read More

    Exosomes isolated from serum of systemic sclerosis patients display alterations in their content of profibrotic and antifibrotic microRNA and induce a profibrotic phenotype in cultured normal dermal fibroblasts.
    Clin Exp Rheumatol 2017 Jan 13. Epub 2017 Jan 13.
    The Scleroderma Center and The Jefferson Institute of Molecular Medicine, Thomas Jefferson University, Philadelphia, PA, USA.
    Objectives: Exosomes are lipid bilayer-bound microvesicles containing various macromolecules including numerous microRNA (miRNA). Exosomes mediate intercellular communication by fusing and releasing their macromolecular content into target cells. Here, we analysed the content of profibrotic and antifibrotic miRNAs in exosomes isolated from the serum of systemic sclerosis (SSc) patients and tested their ability to induce a profibrotic phenotype in normal human dermal fibroblasts in vitro. Read More

    Occupational and environmental scleroderma. Systematic review and meta-analysis.
    Clin Rheumatol 2017 Mar 14;36(3):569-582. Epub 2017 Jan 14.
    Autoimmune Diseases Unit. Department of Internal Medicine, Bellvitge University Hospital-IDIBELL. L'Hospitalet de Llobregat, Feixa Llarga s/n, Hospitalet de Llobregat, Barcelona, Spain.
    The etiology of systemic sclerosis (SSc) remains unknown; however, several occupational and environmental factors have been implicated. Our objective was to perform a meta-analysis of all studies published on SSc associated with occupational and environmental exposure. The review was undertaken by means of MEDLINE and SCOPUS from 1960 to 2014 and using the terms: "systemic," "scleroderma," or "systemic sclerosis/chemically induced" [MesH]. Read More

    Musculoskeletal MRI findings of juvenile localized scleroderma.
    Pediatr Radiol 2017 Jan 14. Epub 2017 Jan 14.
    Nemours Children's Health System/Alfred I. duPont Hospital or Children, 1600 Rockland Road, Wilmington, DE, 19803, USA.
    Background: Juvenile localized scleroderma comprises a group of autoimmune conditions often characterized clinically by an area of skin hardening. In addition to superficial changes in the skin and subcutaneous tissues, juvenile localized scleroderma may involve the deep soft tissues, bones and joints, possibly resulting in functional impairment and pain in addition to cosmetic changes.

    Objective: There is literature documenting the spectrum of findings for deep involvement of localized scleroderma (fascia, muscles, tendons, bones and joints) in adults, but there is limited literature for the condition in children. Read More

    Hemodynamic response to treatment of iron deficiency anemia in pulmonary arterial hypertension: longitudinal insights from an implantable hemodynamic monitor.
    Pulm Circ 2016 Dec;6(4):616-618
    Cardiovascular Institute, Allegheny General Hospital, Pittsburgh, Pennsylvania, USA.
    Despite new therapeutic options, pulmonary arterial hypertension (PAH) remains a progressive disease associated with substantial morbidity and mortality. As such, additional strategies for monitoring and adjunctive management of this disease are important. A 59-year-old woman with scleroderma-associated PAH received an implantable hemodynamic monitor (IHM) as part of a research protocol at our institution. Read More

    Exhaled nitric oxide in pulmonary arterial hypertension associated with systemic sclerosis.
    Pulm Circ 2016 Dec;6(4):545-550
    Division of Pulmonary and Critical Care Medicine, Spectrum Health and Michigan State University College of Human Medicine, Grand Rapids, Michigan, USA.
    The fractional exhaled concentration of nitric oxide (FENO) has been shown to be reduced in idiopathic pulmonary arterial hypertension (PAH) but has not been adequately studied in PAH associated with systemic sclerosis (SSc). We measured FENO at an expiratory flow rate of 50 mL/s in 21 treatment-naive patients with SSc-associated PAH (SSc-PAH), 94 subjects with SSc without pulmonary involvement, and 84 healthy volunteers. Measurements of FENO at additional flow rates of 100, 150, and 250 mL/s were obtained to derive the flow-independent nitric oxide exchange parameters of maximal airway flux (J'awNO) and steady-state alveolar concentration (CANO). Read More

    Malignancies in Patients with Anti-RNA Polymerase III Antibodies and Systemic Sclerosis: Analysis of the EULAR Scleroderma Trials and Research Cohort and Possible Recommendations for Screening.
    J Rheumatol 2017 Jan 15. Epub 2017 Jan 15.
    From the Rheumatology and Clinical Immunology, University of Brescia and Spedali Civili of Brescia, Brescia; Rheumatology Unit, Azienda Ospedaliera Universitaria Integrata, Verona; Department of Internal Medicine and Medical Specialties, University Sapienza, Rome, Italy; Division of Rheumatology, University Hospital Zurich, Zurich, Switzerland; Department of Clinical Sciences, Section of Rheumatology, Lund University, Lund, Sweden; Department of Rheumatology and Immunology, Medical Center, University of Pecs, Pecs, Hungary; Rheumatology, Ghent University Hospital, Ghent University, Ghent, Belgium; Department of Internal Medicine, University Lille Nord-de-France, Lille; Department of Rheumatology, Strasbourg University Hospital, Strasbourg; Department of Rheumatology, University Paris Descartes and Cochin Hospital, Paris, France; B. Shine Rheumatology Unit, Rambam Health Care Campus, Rappaport Faculty of Medicine, Technion - Institute of Technology, Haifa, Israel; Department of Pathophysiology, Medical University of Warsaw and Department of Connective Tissue Diseases, Institute of Rheumatology, Warsaw, Poland; Unidade de Doenças Auto-Imunes, Serviço de Medicina 2, Hospital de Curry Cabral, Centro Hospitalar Lisboa Central, Lisbon, Portugal. M.G. Lazzaroni, MD, Rheumatology and Clinical Immunology, University of Brescia and Spedali Civili of Brescia; I. Cavazzana, MD, Rheumatology and Clinical Immunology, Spedali Civili of Brescia; E. Colombo, MD, Rheumatology and Clinical Immunology, University of Brescia and Spedali Civili of Brescia; R. Dobrota, MD, Division of Rheumatology, University Hospital Zurich; J. Hernandez, MD, Division of Rheumatology, University Hospital Zurich; R. Hesselstrand, MD, PhD, Department of Clinical Sciences, Section of Rheumatology, Lund University; C. Varju, MD, Department of Rheumatology and Immunology, Medical Center, University of Pecs; G. Nagy, MD, Department of Rheumatology and Immunology, Medical Center, University of Pecs; V. Smith, MD, PhD, Rheumatology, Ghent University Hospital, Ghent University; P. Caramaschi, MD, Rheumatology Unit, Azienda Ospedaliera Universitaria Integrata; V. Riccieri, MD, Department of Internal Medicine and Medical Specialties, University Sapienza; E. Hachulla, MD, PhD, Department of Internal Medicine, University Lille Nord-de-France; A. Balbir-Gurman, MD, PhD, B. Shine Rheumatology Unit, Rambam Health Care Campus, Rappaport Faculty of Medicine, Technion - Institute of Technology; E. Chatelus, MD, Department of Rheumatology, Strasbourg University Hospital; K. Romanowska-Próchnicka, MD, Department of Pathophysiology, Medical University of Warsaw and Department of Connective Tissue Diseases, Institute of Rheumatology; A.C. Araújo, MD, Unidade de Doenças Auto-Imunes, Serviço de Medicina 2, Hospital de Curry Cabral, Centro Hospitalar Lisboa Central; O. Distler, MD, PhD, Division of Rheumatology, University Hospital Zurich; Y. Allanore, MD, PhD, Department of Rheumatology, University Paris Descartes and Cochin Hospital; P. Airò, MD, Rheumatology and Clinical Immunology, Spedali Civili of Brescia. Address correspondence to Dr. M.G. Lazzaroni, Piazzale Spedali Civili 1, 25123 Brescia, Italy. E-mail: Accepted for publication November 16, 2016.
    Objective: To analyze the characteristics of anti-RNA polymerase III antibodies (anti-RNAP3)- positive patients with systemic sclerosis (SSc) in the European League Against Rheumatism Scleroderma Trials and Research group (EUSTAR) registry with a focus on the risk of cancer and the characteristics of malignancies, and the aim to provide guidelines about potential cancer screening in these patients.

    Methods: (1) Analysis of the EUSTAR database: 4986 patients with information on their anti-RNAP3 status were included. (2) Case-control study: additional retrospective data, including malignancy history, were queried in 13 participating EUSTAR centers; 158 anti-RNAP3+ cases were compared with 199 local anti-RNAP3- controls, matched for sex, cutaneous subset, disease duration, and age at SSc onset. Read More

    Evidence-based management of systemic sclerosis: Navigating recommendations and guidelines.
    Semin Arthritis Rheum 2016 Dec 9. Epub 2016 Dec 9.
    Department of Medicine, University of Western Ontario, London, ON, Canada; St Joseph Health Care, London, ON, Canada. Electronic address:
    Objectives: Systemic sclerosis (SSc) is a rare heterogeneous connective tissue disease. Recommendations addressing the major issues in the management of SSc including screening and treatment of organ complications are needed.

    Methods: The updated European League Against Rheumatism/European Scleroderma Trial and Research (EULAR/EUSTAR) and the British Society of Rheumatology (BSR) and British Health Professionals in Rheumatology (BHPR) guidelines were compared and contrasted. Read More

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