Search our Database of Scientific Publications and Authors

I’m looking for a

    24661 results match your criteria Scleroderma

    1 OF 494

    Autoantibodies and scleroderma phenotype define subgroups at high-risk and low-risk for cancer.
    Ann Rheum Dis 2018 Apr 20. Epub 2018 Apr 20.
    Division of Rheumatology, Johns Hopkins University School of Medicine, Baltimore, Maryland, USA.
    Objectives: Recent studies demonstrate autoantibodies are powerful tools to interrogate molecular events linking cancer and the development of autoimmunity in scleroderma. Investigating cancer risk in these biologically relevant subsets may provide an opportunity to develop personalised cancer screening guidelines. In this study, we examined cancer risk in distinct serologic and phenotypic scleroderma subsets and compared estimates with the general population. Read More

    Leonine facies presenting as scleromyxedema.
    Arthritis Rheumatol 2018 Apr 20. Epub 2018 Apr 20.
    University of Michigan, Scleroderma Program, University of Michigan, Ann Arbor, MI.
    A 61-year-old man previously in good health was referred to Scleroderma Clinic for a 2-year history of slowly progressive cutaneous eruption involving his dorsal hands, extremities, and central face. On physical examination, the patient had nodular, erythematous indurated lesions of his forehead and erythematous papular lesions on his nose with coalescence of firm erythematous papulonodules, resulting in a leonine facies. On the dorsal hands, arms, and legs were numerous, shiny, firm, closely set, 1-2 mm slightly translucent papules with background erythema (face and hand, shown in Panel A). Read More

    Musculoskeletal Manifestations of Non-RA Connective Tissue Diseases: Scleroderma, Systemic Lupus Erythematosus, Still's Disease, Dermatomyositis/Polymyositis, Sjögren's Syndrome, and Mixed Connective Tissue Disease.
    Semin Musculoskelet Radiol 2018 Apr 19;22(2):166-179. Epub 2018 Apr 19.
    Department of Musculoskeletal Radiology, University Hospital of Lille, Lille Cedex, France.
    The most common systemic rheumatologic conditions are connective tissue diseases (including rheumatoid arthritis [RA]) followed by spondyloarthropathy. With the advent of biotherapies and imaging biomarkers, development in the imaging of RA and spondyloarthropathies has received substantial attention in the literature. This article details the various musculoskeletal imaging features of the other connective tissue diseases such as scleroderma and progressive systemic sclerosis, systemic lupus erythematosus, Still's disease, dermatomyositis and polymyositis, Sjögren's syndrome, and mixed connective tissue disease. Read More

    Imaging Features of the Juvenile Inflammatory Arthropathies.
    Semin Musculoskelet Radiol 2018 Apr 19;22(2):147-165. Epub 2018 Apr 19.
    Department of Paediatric Imaging, Hospital Jeanne de Flandre, CHU Lille, France.
    We discuss the imaging of several juvenile inflammatory arthropathies including juvenile idiopathic arthritis, juvenile systemic lupus erythematosus, juvenile scleroderma, juvenile dermatomyositis, and chronic recurrent multifocal osteomyelitis. Juvenile idiopathic arthritis is the most common autoimmune chronic systemic disease of connective tissue in children. The remaining systemic juvenile connective tissue diseases are rare. Read More

    Characterization of inflammatory cell infiltrate of scleroderma skin: B cells and skin score progression.
    Arthritis Res Ther 2018 Apr 18;20(1):75. Epub 2018 Apr 18.
    Unità Operativa Complessa di Reumatologia, Istituto di Reumatologia e Scienze Affini, Università Cattolica del Sacro Cuore, Rome, Italy.
    Background: The purpose of this study was to investigate the frequency and the distribution of inflammatory cell infiltrate in two sets of cutaneous biopsies derived from clinically affected and unaffected skin in patients with systemic sclerosis (SSc) and to test correlation between the cell infiltrate and the progression of skin involvement.

    Methods: Skin was immunohistochemically assessed to identify CD68, CD3, CD20 and CD138-positive (+) cells in clinically affected and unaffected skin in 28 patients with SSc. Patients were followed for 6 months and the characteristics of the infiltrate were analyzed according to disease duration, clinical features and skin involvement progression. Read More

    From Localized Scleroderma to Systemic Sclerosis: Coexistence or Possible Evolution.
    Dermatol Res Pract 2018 30;2018:1284687. Epub 2018 Jan 30.
    Scleroderma Unit, Chair of Rheumatology, University of Modena and Reggio Emilia, Modena, Italy.
    Background: Systemic sclerosis (SSc) and localized scleroderma (LoS) are two different diseases that may share some features. We evaluated the relationship between SSc and LoS in our case series of SSc patients.

    Methods: We analysed the clinical records of 330 SSc patients, in order to find the eventual occurrence of both the two diseases. Read More

    Oxidative stress in the pathogenesis of systemic scleroderma: An overview.
    J Cell Mol Med 2018 Apr 17. Epub 2018 Apr 17.
    Center for Gender-Specific Medicine, Biomarkers Unit, Rome, Italy.
    Systemic sclerosis (SSc) is a rare disorder of the connective tissue characterized by fibrosis of the skin, skeletal muscles and visceral organs. Additional manifestations include activation of the immune system and vascular injury. SSc causes disability and death as the result of end-stage organ failure. Read More

    A Proposed Mechanism for Central Centrifugal Cicatricial Alopecia.
    Exp Dermatol 2018 Apr 16. Epub 2018 Apr 16.
    Wake Forest University, Department of Dermatology, Winston-Salem, NC, USA.
    Central centrifugal cicatricial alopecia (CCCA) has an unknown mechanism. Analyzing other scarring diseases (lichen planopilaris, fibrotic kidney disease, and scleroderma) may help clarify the mechanism of scarring in CCCA. These diseases were chosen for comparison due to either their location of disease (skin or scalp specifically), or prominence in patients of African descent. Read More

    Evaluation of membrane-bound and soluble forms of HLA-G in Systemic Sclerosis.
    Clin Exp Immunol 2018 Apr 16. Epub 2018 Apr 16.
    Department of Internal Medicine, Clinical Immunology Unit, University of Genoa and Ospedale Policlinico San Martino, Genoa (Italy).
    Systemic sclerosis (SSc) is a complex disease characterized by immune dysregulation, extensive vascular damage and wide-spread fibrosis. Human leukocyte antigen-G (HLA-G) is a non-classic class I major histocompatibility complex (MHC) molecule characterized by complex immuno-modulating properties. HLA-G is expressed on the membrane of different cell lineages in both physiological and pathological conditions. Read More

    Venous Thromboembolism in Systemic Sclerosis: Prevalence, Risk Factors, and Effect on Survival.
    J Rheumatol 2018 Apr 15. Epub 2018 Apr 15.
    From the Toronto Scleroderma Program, Mount Sinai Hospital, Toronto Western Hospital; Division of Rheumatology, Department of Medicine, University of Toronto; Institute of Health Policy, Management and Evaluation; Department of Anesthesia and Institute of Health Policy, Management and Evaluation, University of Toronto; Department of Anesthesia and Pain Management, Toronto General Hospital, Toronto, Ontario, Canada; King Fahad Central Hospital, Ministry of Health, Jizan, Saudi Arabia. Dr. Johnson is supported by a Canadian Institutes of Health Research Clinician Scientist Award, the Oscar and Eleanor Markovitz Fund for Scleroderma Research, and the Freda Fejer Fund of the Arthritis Research Foundation. Dr. Wijeysundera is supported in part by a New Investigator Award from the Canadian Institutes of Health Research and a Merit Award from the Department of Anesthesia at the University of Toronto. S.R. Johnson, MD, PhD, Toronto Scleroderma Program, Mount Sinai Hospital, Toronto Western Hospital, Division of Rheumatology, Department of Medicine, University of Toronto, and Institute of Health Policy, Management and Evaluation, University of Toronto; N. Hakami, MD, Toronto Scleroderma Program, Mount Sinai Hospital, Toronto Western Hospital, Division of Rheumatology, Department of Medicine, University of Toronto, and King Fahad Central Hospital, Ministry of Health; Z. Ahmad, MD, Toronto Scleroderma Program, Mount Sinai Hospital, Division of Rheumatology, Department of Medicine, University of Toronto; D.N. Wijeysundera, MD, PhD, Department of Anesthesia and Pain Management, Toronto General Hospital, and Department of Anesthesia and Institute of Health Policy, Management and Evaluation, University of Toronto. Address correspondence to Dr. S. Johnson, Division of Rheumatology, Ground Floor, East Wing, Toronto Western Hospital, 399 Bathurst St., Toronto, Ontario M5T 2S8, Canada. E-mail: Accepted for publication December 14, 2017.
    Objective: Whether systemic sclerosis (SSc) confers increased risk of venous thromboembolism (VTE) is uncertain. We evaluated the prevalence, risk factors, and effect of VTE on SSc survival.

    Methods: A cohort study was conducted of subjects with SSc who fulfilled the American College of Rheumatology/European League Against Rheumatism classification criteria between 1970 and 2017. Read More

    Comparing ultraviolet light A photo(chemo)therapy with Methotrexate protocol in childhood localized scleroderma: Evidence from systematic review and meta-analysis approach.
    Semin Arthritis Rheum 2018 Mar 14. Epub 2018 Mar 14.
    Department of Neurofarba, University of Studies of Florence, Italy; Paediatric Rheumatology, Hospital Anna Meyer, Florence, Italy.
    Objective: Localized scleroderma is a skin fibrosing disorder that, if untreated, may result in severe disability. The purpose of this systematic review is to compare the present evidence concerning the effectiveness of Methotrexate versus phototherapy, alone or associated with Psoralen, in childhood localized scleroderma.

    Method: A systematic search between January 1996 and May 2017 was performed to identify studies investigating the efficacy of Methotrexate (MTX) or phototherapy (UVA) for treating localized scleroderma with onset ≤18 years. Read More

    Articular Surgery of the Ischemic Hand in Systemic Scleroderma: A Vascular Basis for Arthrodesis and Arthroplasty.
    J Hand Surg Am 2018 Apr 9. Epub 2018 Apr 9.
    Division of Plastic and Reconstructive Surgery, Brigham & Women's Hospital/Harvard Medical School, Boston, MA.
    Hallmark deformities of systemic scleroderma are early onset and progressively disabling flexion contractures of the proximal interphalangeal (PIP) joints often in conjunction with extension or, less frequently, flexion contractures of the metacarpophalangeal (MCP) joints. Although surgical correction is generally recommended, a prevailing reluctance for operative treatment exists owing to the inherent ischemia of the disease with its potentially compromised healing capacity. Nonetheless, with recognition and preservation of the tenuous but well-defined and constant periarticular vascular networks of the PIP and MCP joints, articular reconstruction with uncomplicated wound healing can prove consistently successful for patients with scleroderma. Read More

    Oesophageal diameter is associated with severity but not progression of systemic sclerosis-associated interstitial lung disease.
    Respirology 2018 Apr 11. Epub 2018 Apr 11.
    Department of Medicine, University of British Columbia, Vancouver, BC, Canada.
    Background And Objective: It is unknown whether oesophageal disease is associated with systemic sclerosis-associated interstitial lung disease (SSc-ILD) severity, progression or mortality.

    Methods: High-resolution computed tomography (HRCT) scans from 145 SSc-ILD patients were scored for fibrosis score, oesophageal diameter and presence of hiatal hernia. Fibrosis asymmetry was calculated as: (most affected side - least affected side)/(most affected side + least affected side). Read More

    Detection of autoimmune antibodies in localized scleroderma by synthetic oligonucleotide antigens.
    PLoS One 2018 11;13(4):e0195381. Epub 2018 Apr 11.
    Department of Chemistry, Technical University of Denmark, Kongens Lyngby, Region Hovedstaden, Denmark.
    In this study, we developed a series of synthetic oligonucleotides that allowed us to investigate the details on the antigen recognition by autoimmune antibodies in localized scleroderma subjects. Besides dramatically improved analytical specificity of the assay, our data suggests a potential linking for antibodies to DNA to the biological status of disease state in localized scleroderma. Moreover, introducing chemical modifications into short synthetic deoxyribonucleic acid (DNA) and ribonucleic acid (RNA) molecules completely changed the binding titers of corresponding antibodies and their clinical relevance. Read More

    Lung transplantation for scleroderma lung disease: An international, multicenter, observational cohort study.
    J Heart Lung Transplant 2018 Mar 15. Epub 2018 Mar 15.
    Service de Chirurgie Thoracique, Vasculaire et Transplantation Cardio-pulmonaire, Hôpital Marie-Lannelongue, Le Plessis-Robinson, France; Université Paris-Sud, Faculté de Médecine, Université Paris-Saclay, Le Kremlin Bicêtre, France; UMR_S 999, Université Paris-Sud, INSERM, Hôpital Marie Lannelongue, Le Plessis Robinson, France. Electronic address:
    Background: Due to its multisystemic nature, scleroderma is considered a relative contraindication to lung transplantation at many centers. However, recent studies suggest similar post-transplant outcomes in patients with scleroderma compared to those with other causes of interstitial lung disease (ILD). Furthermore, it remains unknown whether scleroderma-associated pulmonary arterial hypertension (PAH) influences post-transplant outcomes. Read More

    Cutaneous lupus erythematosus, morphea profunda and psoriasis: A case report.
    Reumatol Clin 2018 Apr 3. Epub 2018 Apr 3.
    Servicio de Reumatología, Hospital General Universitario de Ciudad Real, Ciudad Real, España.
    Psoriasis is a common inflammatory dermatosis that may be associated with a number of diseases. Recent studies provide evidence that there is a greater frequency of autoimmune diseases, but association with autoimmune connective tissue diseases is uncommon. The coexistence of psoriasis and lupus erythematosus is rare. Read More

    Antiprotozoan sesterterpenes and triterpenes isolated from two Ghanaian mushrooms.
    Fitoterapia 2018 Apr 3. Epub 2018 Apr 3.
    Fundación MEDINA, Avda. del Conocimiento 34, 18016 Armilla, Granada, Spain.
    Bioassay-guided compound isolation led to the discovery of two new scalarane sesterterpenes (1 and 2) and two new triterpenes (3 and 4) from two mushroom species, Pleurotus ostreatus (edible) and Scleroderma areolatum, collected from Ghana. Their structures, including absolute stereochemistry, were established by spectroscopic methods, particularly (+)-ESI-TOF mass spectrometry and 1D and 2D NMR. The four compounds exhibited IC values of 1. Read More

    Autoantibodies in Serum of Systemic Scleroderma Patients: Peptide-Based Epitope Mapping Indicates Increased Binding to Cytoplasmic Domains of CXCR3.
    Front Immunol 2018 22;9:428. Epub 2018 Mar 22.
    Department of Rheumatology, University of Lübeck, Lübeck, Germany.
    Systemic sclerosis (SSc) is a severe chronic autoimmune disease with high morbidity and mortality. Sera of patients with SSc contain a large variety of autoantibody (aab) reactivities. Among these are functionally active aab that bind to G protein-coupled receptors (GPCR) such as C-X-C motif chemokine receptor 3 (CXCR3) and 4 (CXCR4). Read More

    Applied Bayesian Methods in the Rheumatic Diseases.
    Rheum Dis Clin North Am 2018 May;44(2):361-370
    Institute of Health Policy, Management and Evaluation, Dalla Lana School of Public Health, University of Toronto, Toronto, Ontario, Canada; Division of Rheumatology, Department of Paediatrics, Hospital for Sick Children, 555 University Avenue, Toronto, Ontario M5G 1X8, Canada.
    The use of applied Bayesian methods is increasing in rheumatology. Using the Bayes theorem, past evidence is updated with new data. Preexisting data are expressed as a prior probability distribution or prior. Read More

    Qualitative Methods to Advance Care, Diagnosis, and Therapy in Rheumatic Diseases.
    Rheum Dis Clin North Am 2018 May 21;44(2):267-284. Epub 2018 Feb 21.
    Royal National Hospital for Rheumatic Diseases, Royal United Hospitals, Upper Borough Walls, Bath BA1 1RL, UK; Department of Pharmacy and Pharmacology, University of Bath, Bath BA11RL, UK.
    This article provides an overview of the basis, usefulness, and validity of qualitative methods in research. It is aimed to enhance the understanding of a broad spectrum of readers, ranging from those mystified by such approaches, to those wanting a better critical knowledge to apply to literature review, and for health care providers considering developing an interest in the field. Qualitative research is crucial in augmentation of disease knowledge as well as the development of incremental care strategies and operational aspects of care that improves health outcomes. Read More

    Propensity Score Methods for Bias Reduction in Observational Studies of Treatment Effect.
    Rheum Dis Clin North Am 2018 May;44(2):203-213
    Institute of Health Policy, Management and Evaluation, University of Toronto, 155 College Street, Toronto, Ontario M5T 3M6, Canada; Dalla Lana School of Public Health, University of Toronto, 155 College Street, Toronto, Ontario M5T 3M7, Canada; Division of Rheumatology, Department of Paediatrics, The Hospital for Sick Children, 555 University Avenue, Toronto, Ontario M5G 1X8, Canada.
    A challenge to the use of observational data to study treatment effects is the issue of confounding. Noncomparability of exposed and nonexposed subjects can lead to biased estimation of the treatment effect. The propensity score is a balancing score that can be used to form matched groups, or pairs, that are not systematically different and enable nonbiased comparisons between groups. Read More

    Anatomical Variation of the Radial Artery Associated With Clinically Significant Ischemia.
    J Hand Surg Am 2018 Mar 27. Epub 2018 Mar 27.
    The Curtis National Hand Center, Baltimore, MD. Electronic address:
    Purpose: The purpose of this retrospective review was to investigate the incidence of radial artery anatomical variations in patients with clinically significant distal upper extremity (UE) ischemia. Available anatomical studies report that high takeoff of the radial artery occurs in up to 15% of the population. We hypothesized that there is a higher incidence of high origin of the radial artery in patients with clinically significant ischemia compared with the reported frequency in the general population. Read More

    Does nailfold capillaroscopy help predict future outcomes in systemic sclerosis? A systematic literature review.
    Semin Arthritis Rheum 2018 Feb 14. Epub 2018 Feb 14.
    Department of Pharmacy and Pharmacology, University of Bath, Bath, UK; Royal National Hospital for Rheumatic Diseases (at Royal United Hospitals), Upper Borough Walls, Bath, Bath BA1 1RL, UK. Electronic address:
    Background: Nailfold capillaroscopy (NC) is an important diagnostic tool in systemic sclerosis (SSc). Confirmation of NC as a prognostic factor could facilitate earlier intervention and slow disease progression in SSc. We undertook a systematic literature review to evaluate the prognostic value of NC in predicting SSc disease progression. Read More

    Different profile of cytokine production in patients with systemic sclerosis and association with clinical manifestations.
    Immunol Lett 2018 Mar 27;198:12-16. Epub 2018 Mar 27.
    Laboratório de Imunomodulação e Novas Abordagens Terapêuticas - Núcleo de Pesquisa em Inovação Terapêutica (LINAT-NUPIT) - UFPE, Av. Prof. Moraes Rego, 1235, Cidade Universitária, Recife, PE, CEP: 50670-901, Brazil.
    Immune dysregulation is a central process in the pathogenesis of systemic sclerosis (SSc). Cytokines produced by lymphocytes and monocytes are important mediators and induce tissue damage, recruit additional inflammatory cells, and promote extracellular matrix production and fibrosis. In the present research, we aimed to study the associations between levels of cytokines in serum and culture supernatants from peripheral blood mononuclear cells (PBMCs) and clinical manifestations in SSc patients. Read More

    Serum level of endostatin and digital ulcers in systemic sclerosis patients.
    Int Wound J 2018 Mar 30. Epub 2018 Mar 30.
    Department of Clinical Medicine, Sapienza University of Rome, Rome, Italy.
    Patients with systemic sclerosis (SSc) are at a high risk of the development of ischaemic digital ulcers (DUs) that can be complicated with infections, gangrene, and osteomyelitis. The aim of this study is to evaluate the role of endostatin in scleroderma DUs.In total, 90 SSc patients were enrolled in this study. Read More

    Exosomes as a novel pathway for regulating development and diseases of the skin.
    Biomed Rep 2018 Mar 31;8(3):207-214. Epub 2018 Jan 31.
    Department of Geriatrics, Tongji Hospital of Shanghai Affiliated to Tongji University, Shanghai 200065, P.R. China.
    Exosomes are one of the most potent intercellular communicators, which are able to communicate with adjacent or distant cells. Exosomes deliver various bioactive molecules, including membrane receptors, proteins, mRNA and microRNA, to target cells and serve roles. Recent studies have demonstrated that exosomes may regulate the functions and diseases of the skin, which is the largest organ of the human body. Read More

    The Influence of Connective Tissue Disease in Breast Reconstruction: A National Database Analysis.
    Ann Plast Surg 2018 Apr;80(4 Suppl 4):S182-S188
    From the Division of Plastic and Reconstructive Surgery, Beth Israel Deaconess Medical Center, Harvard Medical School, Boston, MA.
    Background: Patients with connective tissue diseases (CTD), or collagen vascular diseases, are at risk of potentially higher morbidity after surgical procedures. We aimed to investigate the complication profile in CTD versus non-CTD patients who underwent breast reconstruction on a national scale.

    Methods: A retrospective analysis of the Healthcare Cost and Utilization Project NIS Database between 2010 and 2014 was conducted for patients 18 years or older admitted for immediate autologous or implant breast reconstruction. Read More

    PAI1 mediates fibroblast-mast cell interactions in skin fibrosis.
    J Clin Invest 2018 Mar 26. Epub 2018 Mar 26.
    IFOM-inStem Joint Research Laboratory, Institute for Stem Cell Biology and Regenerative Medicine, Bangalore, Karnataka, India.
    Fibrosis is a prevalent pathological condition arising from the chronic activation of fibroblasts. This activation results from the extensive intercellular crosstalk mediated by both soluble factors and direct cell-cell connections. Prominent among these are the interactions of fibroblasts with immune cells, in which the fibroblast-mast cell connection, although acknowledged, is relatively unexplored. Read More

    Calcinosis Cutis and Negative Pressure Wound Therapy as Adjuncts to Surgical Management: Case Report and Review of the Literature.
    Wounds 2018 Mar;30(3):E32-E35
    Flushing Hospital Medical Center, Queens, NY.
    Calcinosis cutis (CC), or calcium deposition in soft tissue, can cause significant morbidity associated with arthralgias and ulceration. This condition has an elusive pathophysiology and is often associated with autoimmune disorders, significantly impacting the disease burden. The clinical presentation of CC varies, and there is no gold standard treatment modality. Read More

    ANA as an Entry Criterion in SLE Classification.
    Arthritis Care Res (Hoboken) 2018 Mar 26. Epub 2018 Mar 26.
    University Medical Center Carl Gustav Carus, TU Dresden, Dresden.
    Thoughtful and eloquent as always, Drs. Pisetsky and Lipsky in their letter (1) highlight a critical point in the current SLE classification criteria project jointly supported by EULAR and ACR: if ANA positivity is to be the entry criterion, the sensitivity of the assay to detect ANA and that of ANA positivity for SLE are both important. Indeed, this issue has been a matter constantly discussed by the classification criteria steering committee since the beginning of the project. Read More

    A Case of Hereditary Fibrosing Poikiloderma with Tendon Contractures, Myopathy, and Pulmonary Fibrosis (POIKTMP) with the Emphasis on Cutaneous Histopathological Findings.
    J Eur Acad Dermatol Venereol 2018 Mar 26. Epub 2018 Mar 26.
    Department of Dermatology, SUNY Downstate Medical Center, Brooklyn, NY, 11203.
    POIKTMP (POIKiloderma, Tendon contractures, Myopathy and Pulmonary fibrosis) is a recently described autosomal dominant genetic syndrome caused by mutation of FAM111B gene, encoding a protein with unknown function . The syndrome is confirmed in 4 families and nine sporadic cases . Skin lesions of POIKTMP are rarely biopcied . Read More

    Multidimensional fatigue in pulmonary hypertension: prevalence, severity and predictors.
    ERJ Open Res 2018 Jan 23;4(1). Epub 2018 Mar 23.
    New Orleans Scleroderma and Sarcoidosis Patient Care and Research Center, New Orleans, LA, USA.
    Pulmonary hypertension is a potentially fatal disease. Despite pharmacological advances in pulmonary hypertension, fatigue remains common in patients with pulmonary hypertension. A convenience sample of 120 participants at an international patient conference completed the Multidimensional Fatigue Inventory (MFI)-20 scale. Read More

    Improving life expectancy of patients with scleroderma. Results from the South Australian Scleroderma Register.
    Intern Med J 2018 Mar 23. Epub 2018 Mar 23.
    Department of Immunology, Flinders Medical Centre, Adelaide, Australia.
    Background: Scleroderma is a rare connective tissue disorder characterised by inflammation, vasculopathy and excessive fibrosis. Patients with scleroderma are known to have decreased life expectancy.

    Aim: To investigate changes in life expectancy in patients with scleroderma over a 30 year period. Read More

    Cd and Zn interactions and toxicity in ectomycorrhizal basidiomycetes in axenic culture.
    PeerJ 2018 7;6:e4478. Epub 2018 Mar 7.
    Centre for Agri-Environmental Research & Soil Research Centre, School of Agriculture, Policy and Development, University of Reading, Reading, Berkshire, United Kingdom.
    Background: Metal contamination in soils affects both above- and belowground communities, including soil microorganisms. Ectomycorrhizal (ECM) fungi are an important component in belowground community and tolerant strains have great potential in enhancing plant-based remediation techniques. We assessed cadmium and zinc toxicity in five ECM species in liquid media (; ; ; sp. Read More

    Effect of 6-min Walk Test on pro-BNP Levels in Patients with Pulmonary Arterial Hypertension.
    Lung 2018 Mar 21. Epub 2018 Mar 21.
    Division of Pulmonary and Critical Care Medicine, Department of Medicine, University of North Carolina, Chapel Hill, USA.
    Background: Plasma pro-BNP (brain natriuretic peptide) levels are often elevated in response to right ventricular (RV) volume and pressure overload, parameters potentially affected by exercise. Plasma pro-BNP levels change in association with long-term changes in pulmonary hemodynamics, thereby serving as a potential biomarker in pulmonary arterial hypertension (PAH). The 6-min Walk Test (6MWT) and pro-BNP level are often checked in a single office visit. Read More

    Scleroderma-related choroiditis.
    Oman J Ophthalmol 2018 Jan-Apr;11(1):75-77
    Department of Uvea and Ocular Pathology, Sankara Nethralaya, Chennai, Tamil Nadu, India.
    A 34-year-old female a known scleroderma patient presented to us with ocular manifestations in the form choroiditis along with optic nerve involvement. Blood investigations were done, and the ocular findings were confirmed by fundus fluorescein angiography. She was successfully managed with systemic steroid therapy. Read More

    Incidence of pulmonary hypertension and determining factors in patients with systemic sclerosis.
    Eur Respir J 2018 Apr 4;51(4). Epub 2018 Apr 4.
    Centre for Pulmonary Hypertension, Thorax Clinic at the University Hospital Heidelberg, Heidelberg, Germany.
    The objective of this study was to evaluate the incidence of pulmonary hypertension (PH) and determining factors in patients with systemic sclerosis (SSc) and a diffusing capacity of the lung for carbon monoxide () <60% predicted.In this bicentric, prospective cohort study, patients with SSc were clinically assessed at baseline and after 3 years, including right heart catheterisation (RHC). Analysis of determining factors for the development of PH was performed using univariate and multivariate analyses. Read More

    ASCA (Anti-Saccharomyces cerevisiae Antibody) in Patients With Scleroderma.
    J Clin Rheumatol 2018 Mar 21. Epub 2018 Mar 21.
    Objectives: The majority of scleroderma (SSc) patients present gastrointestinal involvement. Motility is usually compromised but few studies address permeability changes in the intestinal wall. ASCA (anti-Saccharomyces cerevisiae antibodies) positivity is associated with increased intestinal permeability. Read More

    Gene Profiling in Patients with Systemic Sclerosis Reveals the Presence of Oncogenic Gene Signatures.
    Front Immunol 2018 6;9:449. Epub 2018 Mar 6.
    Immunology Area, Pediatric Hospital Bambino Gesù, Rome, Italy.
    Systemic sclerosis (SSc) is a rare connective tissue disease characterized by three pathogenetic hallmarks: vasculopathy, dysregulation of the immune system, and fibrosis. A particular feature of SSc is the increased frequency of some types of malignancies, namely breast, lung, and hematological malignancies. Moreover, SSc may also be a paraneoplastic disease, again indicating a strong link between cancer and scleroderma. Read More

    Sirtuins and Accelerated Aging in Scleroderma.
    Curr Rheumatol Rep 2018 Mar 17;20(4):16. Epub 2018 Mar 17.
    Research Service, Baltimore VA Medical Center, Baltimore, MD, 21201, USA.
    Purpose Of Review: Premature activation of aging-associated molecular mechanisms is emerging as an important contributor to many diseases, including scleroderma. Among central regulators of the aging process are a group of histone deacetylases called sirtuins (SIRTs). Recent findings implicate these molecules as pathophysiological players in scleroderma skin and lung fibrosis. Read More

    Erasmus Syndrome: Silicosis and Systemic Sclerosis.
    Indian J Occup Environ Med 2017 May-Aug;21(2):94-96
    Department of Respiratory Medicine, Institute of Respiratory Diseases, SMS Medical College, Jaipur, Rajasthan, India.
    Several occupational hazards, especially exposure to silica, have been implicated as causal factors for the development of scleroderma-like disorders. Compared to other connective tissue disorders, silica-associated systemic sclerosis (SA-SS) is relatively rare. Silica-induced scleroderma is indistinguishable from idiopathic systemic sclerosis. Read More

    In systemic sclerosis, microvascular and hands digital arteries damage correlates with serum levels of endostatin.
    Microcirculation 2018 Mar 14:e12449. Epub 2018 Mar 14.
    Department of Clinical Medicine, Scleroderma Unit, Sapienza University of Rome, Rome, Italy.
    Objective: In SSc, vascular injury leads to endothelial dysfunction with reduced capillary blood flow and tissue hypoxia. In SSc, the angiogenesis is impaired and implicated in the microvascular damage. In severe vascular damage, VEGF is reduced and endostatin is increased. Read More

    Chronic cutaneous graft-versus-host disease in children: A report of 14 patients from a tertiary care pediatric dermatology clinic.
    Pediatr Dermatol 2018 Mar 14. Epub 2018 Mar 14.
    Dermatopathology Unit, As'ad Al-Hamad Dermatology Center, Kuwait.
    Background/objectives: Allogeneic hematopoietic stem cell transplantation (HSCT) is a treatment option for many life-threatening disorders in children. Chronic graft-versus-host disease (cGVHD) is a significant complication of HSCT, and its treatment is challenging. Skin is the most common organ affected in cGVHD, with protean manifestations posing a challenge in diagnosis and management. Read More

    The non-neuronal cyclin-dependent kinase 5 is a fibrotic mediator potentially implicated in systemic sclerosis and a novel therapeutic target.
    Oncotarget 2018 Feb 20;9(12):10294-10306. Epub 2017 Dec 20.
    Northwestern Scleroderma Program, Department of Medicine, Feinberg School of Medicine, Chicago, IL, USA.
    The mechanisms underlying persistent fibroblast activation and myofibroblast phenoconversion in underlying multi-organ fibrosis in systemic sclerosis (SSc) remain incompletely understood, hindering effective therapies to slow or reverse the process. Cyclin-dependent kinase 5 (CDK5) is a pleiotropic member of the CDK family originally identified in neuronal cells. In contrast to other CDKs, CDK5 activity depends on its CDK5R1 subunit p35. Read More

    A pantropically introduced tree is followed by specific ectomycorrhizal symbionts due to pseudo-vertical transmission.
    ISME J 2018 Mar 13. Epub 2018 Mar 13.
    Laboratoire de Biologie et Physiologie Végétales, Université des Antilles, BP 592, 97159, Pointe-à-Pitre, France.
    Global trade increases plant introductions, but joint introduction of associated microbes is overlooked. We analyzed the ectomycorrhizal fungi of a Caribbean beach tree, seagrape (Coccoloba uvifera, Polygonacaeae), introduced pantropically to stabilize coastal soils and produce edible fruits. Seagrape displays a limited symbiont diversity in the Caribbean. Read More

    1 OF 494