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    Gastrointestinal and Hepatic Disease in Systemic Sclerosis.
    Rheum Dis Clin North Am 2018 Feb;44(1):15-28
    Department of Internal Medicine, University of Colorado, Denver, 12631 East 17th Avenue, Aurora, CO 80045, USA.
    Although classification criteria for systemic sclerosis (SSc) do not incorporate gastrointestinal tract (GIT) manifestations often present in this disease, the GIT is the most common internal organ involved. Pathophysiology of GIT involvement is thought to be similar to other organs in SSc with fibroproliferative vascular lesions of small arteries and arterioles, increased production of profibrotic growth factors, and alterations of innate, humoral, and cellular immunity. These processes result in neuropathy progressing to myopathy with eventual fibrosis. Read More

    Defining a unified vascular phenotype in systemic sclerosis.
    Arthritis Rheumatol 2017 Nov 16. Epub 2017 Nov 16.
    UCL Division of Medicine, University College London, London, UK.
    Microcirculation impairment and related vasculopathy is a hallmark of systemic sclerosis. Digital ulcers are second only to Raynaud's phenomenon as a vascular complication occurring in scleroderma patients. Digital ulcers are painful and generate disability. Read More

    STAT3 controls COL1A2 enhancer activation cooperatively with JunB, regulates type I collagen synthesis post-transcriptionally and is essential for lung myofibroblast differentiation.
    Mol Biol Cell 2017 Nov 15. Epub 2017 Nov 15.
    Centre for Rheumatology and Connective Tissue Diseases, Division of Medicine, University College London, London, UK
    Fibroblast differentiation is key cellular process that underlies the process of fibrosis, a deadly complication of fibrotic diseases like Scleroderma (SSc). This transition coincides with the overproduction of Collagen type I (COL1) and other extracellular matrix proteins. High level expression of the collagen type 1α2 subunit (COL1A2), requires the engagement of a far upstream enhancer, whose activation is strongly dependent on the AP1 factor JunB. Read More

    The Activation of Human Dermal Microvascular Cells by Poly(I:C), Lipopolysaccharide, Imiquimod, and ODN2395 Is Mediated by the Fli1/FOXO3A Pathway.
    J Immunol 2017 Nov 15. Epub 2017 Nov 15.
    Section of Rheumatology, School of Medicine, Boston University, Boston, MA 02118
    Endothelial cell (EC) dysfunction has been associated with inflammatory and autoimmune diseases; however, the factors contributing to this dysfunction have not been fully explored. Because activation of TLRs has been implicated in autoimmune diseases, the goal of this study was to determine the effects of TLR ligands on EC function. Human dermal microvascular ECs (HDMECs) treated with TLR3 [Poly(I:C)], TLR4 (LPS), and TLR7 (imiquimod) agonists showed decreased proliferation and a reduced total number of branching tubules in three-dimensional human dermal organoid ex vivo culture. Read More

    Estimating autoantibody signatures to detect autoimmune disease patient subsets.
    Biostatistics 2017 Nov 13. Epub 2017 Nov 13.
    Department of Biostatistics, The Johns Hopkins University, 615 N Wolfe Street, Baltimore, MD 21205, USA.
    Autoimmune diseases are characterized by highly specific immune responses against molecules in self-tissues. Different autoimmune diseases are characterized by distinct immune responses, making autoantibodies useful for diagnosis and prediction. In many diseases, the targets of autoantibodies are incompletely defined. Read More

    Identification of novel systemic sclerosis biomarkers employing aptamer proteomic analysis.
    Rheumatology (Oxford) 2017 Nov 13. Epub 2017 Nov 13.
    Jefferson Institute of Molecular Medicine and The Scleroderma Center, Thomas Jefferson University, Philadelphia, PA, USA.
    There is an important unmet need for clinically validated non-invasive biomarkers for SSc diagnosis, assessment of disease activity, extent of internal organ involvement, therapeutic response and prognosis. There is also an unmet need for biomarkers to accurately differentiate primary RP from recent onset RP evolving into SSc. The lack of sensitive and specific biomarkers for SSc and SSc-associated RP is a limitation for the optimal clinical management of these patients. Read More

    Localized Scleroderma, Systemic Sclerosis and Cardiovascular Risk: A Danish Nationwide Cohort Study.
    Acta Derm Venereol 2017 Nov 14. Epub 2017 Nov 14.
    Department of Dermatology and Allergy, Herlev and Gentofte University Hospital, University of Copenhagen, Kildegaardsvej 28, DK-2900 Hellerup, Denmark.
    Recent findings indicate that patients with systemic sclerosis have an increased risk of cardiovascular disease. To determine whether patients with systemic sclerosis or localized scleroderma are at increased risk of cardiovascular disease, a cohort study of the entire Danish population aged ≥ 18 and ≤ 100 years was conducted, followed from 1997 to 2011 by individual-level linkage of nationwide registries. Multivariable adjusted Cox regression models were used to estimate the hazard ratios (HRs) for a composite cardiovascular disease endpoint. Read More

    [Investigation of parameters related to prognosis in diffuse parenchymal lung diseases prognosis in interstitial lung diseases].
    Tuberk Toraks 2017 Sep;65(3):210-219
    Department of Chest Diseases, Faculty of Istanbul Medicine, Istanbul University, Istanbul, Turkey.
    Introduction: Parameters related to prognosis in diffuse parenchymal lung disease (DPLD) have a decisive influence on treatment and follow-up processes. We aimed to define baseline characteristics and factors that effect the mortality of the group of patients with DPLD and to determine distinctions between subgroups.

    Materials And Methods: Demographic characteristics, complaints, comorbidity, treatment, pulmonary function tests, echocardiographic findings, six minute walking test (6MWT), arterial blood gases analysis, radiological findings and survival time were collected from outpatient clinics database. Read More

    Can hybrid hyaluronic acid represent a valid approach to treat rizoarthrosis? A retrospective comparative study.
    BMC Musculoskelet Disord 2017 Nov 13;18(1):444. Epub 2017 Nov 13.
    Rheumatology Unit, Department of Medicine, Surgery and Neurosciences, University of Siena, Viale Bracci 1, 53100, Siena, Italy.
    Background: Osteoarthritis (OA) of the trapeziometacarpal joint (TMJ) is a disabling condition with a significant impact on quality of life. The optimal management of hand OA requires a combination of non-pharmacological and pharmacological treatments that include intra-articular (i.a. Read More

    Novel lung imaging biomarkers and skin gene expression subsetting in dasatinib treatment of systemic sclerosis-associated interstitial lung disease.
    PLoS One 2017 9;12(11):e0187580. Epub 2017 Nov 9.
    Northwestern Scleroderma Program, Feinberg School of Medicine, Chicago, IL, United States of America.
    Background: There are no effective treatments or validated clinical response markers in systemic sclerosis (SSc). We assessed imaging biomarkers and performed gene expression profiling in a single-arm open-label clinical trial of tyrosine kinase inhibitor dasatinib in patients with SSc-associated interstitial lung disease (SSc-ILD).

    Methods: Primary objectives were safety and pharmacokinetics. Read More

    Bench to Bedside - and Back Again: Finding the Goldilocks Zone within the Scleroderma Universe.
    Arthritis Rheumatol 2017 Nov 9. Epub 2017 Nov 9.
    Royal Free Hospital and UCL Medical School, Centre of rRheumatology.
    Systemic sclerosis (SSc) is a complex inflammatory connective tissue disease characterized by skin thickening, organ fibrosis and vasculopathy [1, 2]. The underlying disease mechanisms and pathophysiology are not fully understood. However, it appears to be, at least in part, driven by autoimmunity and inflammation associated with microvascular dysfunction ultimately resulting in excess extracellular matrix deposition in target organs [2-4]. Read More

    The safety and efficacy of light emitting diodes-based ultraviolet A1 phototherapy in bleomycin-induced scleroderma in mice.
    Adv Med Sci 2017 Nov 6;63(1):152-159. Epub 2017 Nov 6.
    State Research Institute Centre for Innovative Medicine, Vilnius, Lithuania; The Clinic of Rheumatology, Traumatology Orthopaedics and Reconstructive Surgery, Institute of Clinical Medicine of the Faculty of Medicine of Vilnius University, Vilnius, Lithuania.
    Purpose: To define the efficacy and safety of narrowband ultraviolet A1 (UVA1) for the treatment of dermal fibrosis in bleomycin-induced mouse model of scleroderma.

    Materials And Methods: 42 DBA/2 strain mice were included in the study: healthy mice and mice with established scleroderma, treated with high or medium dose of UVA1. Non-treated groups served as control. Read More

    Imaging findings in systemic childhood diseases presenting with dermatologic manifestations.
    Clin Imaging 2017 Oct 31;49:17-36. Epub 2017 Oct 31.
    Department of Radiology, Division of Pediatric Radiology, Montefiore Medical Center, Bronx, NY, United States. Electronic address:
    Purpose: Many childhood diseases often present with skin abnormalities with which radiologists are largely unfamiliar. Knowledge of associated dermatologic manifestations may aid the radiologist in confirming the diagnosis and recommending targeted imaging of affected organs.

    Methods: We review the imaging findings in childhood diseases associated with dermatologic manifestations. Read More

    Orthopedic complications of linear morphea: Implications for early interdisciplinary care.
    Pediatr Dermatol 2017 Nov 9. Epub 2017 Nov 9.
    Department of Dermatology, Mayo Clinic, Rochester, MN, USA.
    Linear morphea of the limb primarily affects children, and extracutaneous manifestations are common. Orthopedic surgeons are often essential in the care of patients with linear morphea, yet there are few reports outlining specific orthopedic complications in this population. We sought to improve the understanding of orthopedic complications in linear morphea of the limb. Read More

    Determinants of mortality in systemic sclerosis: a focused review.
    Rheumatol Int 2017 Nov 7. Epub 2017 Nov 7.
    Division of Rheumatology, University of Pennsylvania School of Medicine, 3400 Spruce Street, Philadelphia, PA, 19104, USA.
    Scleroderma (systemic sclerosis) is an autoimmune rheumatic disorder that is characterized by fibrosis, vascular dysfunction, and autoantibody production that involves most visceral organs. It is characterized by a high morbidity and mortality rate, mainly due to disease-related complications. Epidemiological data describing mortality and survival in this population have been based on both population and observational studies. Read More

    Brachio-cervical inflammatory myopathy with associated scleroderma phenotype and lupus serology.
    Neurol Neuroimmunol Neuroinflamm 2018 Jan 27;5(1):e410. Epub 2017 Oct 27.
    Department of Laboratory Medicine and Pathobiology (A.F.G., D.G.M.), University of Toronto; Division of Neurology (P.A.S., C.D.K.) and Division of Rheumatology (O.V.), Department of Medicine, St. Michael's Hospital; and Department of Laboratory Medicine (A.F.G., D.G.M.), St. Michael's Hospital, Toronto, Ontario, Canada.

    Abnormal esophageal acid exposure on high-dose proton pump inhibitor therapy is common in systemic sclerosis patients.
    Neurogastroenterol Motil 2017 Nov 6. Epub 2017 Nov 6.
    Division of Gastroenterology and Hepatology, Department of Medicine, Northwestern University Feinberg School of Medicine, Chicago, IL, USA.
    Background: Esophageal dysfunction and gastro-esophageal reflux disease (GERD) are common among patients with systemic sclerosis (SSc). Although high-dose proton pump inhibitors (PPIs) typically normalize esophageal acid exposure, the effectiveness of PPI therapy has not been systematically studied in SSc patients. The aim of this study was to characterize reflux in SSc patients on high-dose PPI using esophageal pH-impedance testing. Read More

    Post-irradiation morphoea of the breast: a case report and review of the literature.
    Histopathology 2017 Aug 14. Epub 2017 Aug 14.
    Breast Cancer Research Program, Vanderbilt-Ingram Cancer Center, Vanderbilt University Medical Center, Nashville, TN, USA.
    We describe a 44-year-old female with triple-negative breast cancer who developed skin erythaema, sclerosis and contracture of her entire right breast 15 months after completion of post-lumpectomy chemotherapy and radiotherapy, consistent with post-irradiation morphoea (PIM). PIM is a rare complication of breast irradiation that impairs a patient's quality of life. PIM is located usually at the radiation port or in the surrounding tissue. Read More

    Mycobacterium intracellulare Infection Mimicking Progression of Scleroderma.
    Case Rep Rheumatol 2017 27;2017:4029271. Epub 2017 Sep 27.
    Center for Rheumatology and Spine Diseases, Rigshospitalet, 2100 Copenhagen, Denmark.
    This case report describes a patient with scleroderma who developed Mycobacterium intracellulare infection, which for more than a year mimicked worsening of her connective tissue disorder. The patient was diagnosed with scleroderma based on puffy fingers that developed into sclerodactyly, abnormal nail fold capillaries, interstitial lung disease, Raynaud's phenomenon, esophageal dysmotility, and positivity for rheumatoid factor and anti-SSA antibodies. She developed massive inflammatory changes of the cutis, the subcutis, and the muscle fasciae of the right leg, that after several failed attempts of immunosuppressive treatments were found to be caused by Mycobacterium intracellulare. Read More

    Reliability and minimal clinically important differences of forced vital capacity: Results from the Scleroderma Lung Studies (SLS-I and SLS-II).
    Am J Respir Crit Care Med 2017 Nov 3. Epub 2017 Nov 3.
    University of Michigan, Ann Arbor, Michigan, United States ;
    Objectives: To assess the reliability and the minimal clinically important differences (MCID) for FVC% predicted in the Scleroderma Lung Study I and II.

    Methods: Using data from SLS I and II (N=300), we evaluated the test-retest reliability for FVC% predicted (FVC%; screening vs. baseline) using intra-class correlation (ICC). Read More

    Pomalidomide in Patients with Interstitial Lung Disease due to Systemic Sclerosis: A Phase II, Multicenter, Randomized, Double-blind, Placebo-controlled, Parallel-group Study.
    J Rheumatol 2017 Nov 1. Epub 2017 Nov 1.
    From the Robert Wood Johnson (RWJ) Medical School Scleroderma Program, New Brunswick; Celgene Corp., Summit, New Jersey; Department of Rheumatology, University of Pittsburgh, Pittsburgh, Pennsylvania; David Geffen School of Medicine at the University of California at Los Angeles (UCLA), Los Angeles, California; Georgetown University Medical Center, Washington, DC, USA; Centre for Rheumatology and Connective Tissue Disease, London, UK; The Queen Elizabeth Hospital and University of Adelaide, Adelaide, Australia; Research Rheumatology Institute n.a. V.A. Nassonova, Moscow, Russia; Department of Internal Medicine 3, University of Erlangen-Nuremberg, Erlangen, Germany. Funded by Celgene Corp. V.M. Hsu, MD, RWJ Medical School Scleroderma Program; C.P. Denton, MBBS, MRCP, Centre for Rheumatology and Connective Tissue Disease; R.T. Domsic, MD, MPH, Department of Rheumatology, University of Pittsburgh; D.E. Furst, MD, David Geffen School of Medicine at UCLA; M. Rischmueller, MD, FRACP, The Queen Elizabeth Hospital and University of Adelaide; M. Stanislav, MD, PhD, DMSc, Research Rheumatology Institute n.a. V.A. Nassonova; V.D. Steen, MD, Georgetown University Medical Center; J.H. Distler, MD, Department of Internal Medicine 3, University of Erlangen-Nuremberg; S. Korish, MD, Celgene Corp.; A. Cooper, Celgene Corp.; S. Choi, PhD, Celgene Corp.; P.H. Schafer, PhD, Celgene Corp.; G. Horan, PhD, Celgene Corp.; D.R. Hough, MD, Celgene Corp. Address correspondence to Dr. V.M. Hsu, RWJ Medical School Scleroderma Program, 51 French St., P.O. Box 19, New Brunswick, New Jersey 08903, USA. E-mail: Accepted for publication August 9, 2017.
    Objective: To evaluate the safety and efficacy of pomalidomide (POM) on forced vital capacity (FVC), modified Rodnan skin score (mRSS), and gastrointestinal (GI) symptomatology over 52 weeks of treatment in patients with interstitial lung disease due to systemic sclerosis (SSc).

    Methods: Twenty-three adult patients diagnosed with SSc were randomized 1:1 POM:placebo (PBO).

    Results: Mean change at Week 52 from baseline in predicted FVC% -5. Read More

    Surgical Management of Localized Scleroderma.
    Arch Craniofac Surg 2017 Sep 26;18(3):166-171. Epub 2017 Sep 26.
    Department of Plastic and Reconstructive Surgery, Hanyang University College of Medicine, Seoul, Korea.
    Background: Localized scleroderma is characterized by a thickening of the skin from excessive collagen deposits. It is not a fatal disease, but quality of life can be adversely affected due to changes in skin appearance, joint contractures, and, rarely, serious deformities of the face and extremities. We present six cases of localized scleroderma in face from our surgical practice. Read More

    Fibrocytes in Asthma and COPD: Variations on the Same Theme.
    Am J Respir Cell Mol Biol 2017 Oct 31. Epub 2017 Oct 31.
    University of Bordeaux , Centre de Recherche Cardio-thoracique de Bordeaux, Bordeaux, France.
    Fibrocytes are circulating cells that have fibroblast properties. They are produced by the bone marrow stroma, and they move from the blood to injured organs using multiple chemokine pathways. They exhibit marked functional and phenotypic plasticity in response to the local tissue microenvironment to ensure a pro-inflammatory or a more resolving phenotype. Read More

    Association between cortisol levels and pain threshold in systemic sclerosis and major depression.
    Rheumatol Int 2017 Oct 30. Epub 2017 Oct 30.
    Division of Rheumatology, Department of Medicine, University of Louisville, Kentucky, USA.
    Pain perception and threshold show complex interactions with the inflammatory, psychiatric and neuroendocrine stimuli. This study aims to test whether lower serum cortisol levels are associated with lower pain thresholds and higher degree of depression in systemic sclerosis (SSc) and major depression with atypical features (MD-AF) patients compared to controls. 180 female subjects (SSc = 60, MD-AF = 60, healthy controls = 60) participated in this observational, cross-sectional, parallel group study. Read More

    Diagnostic specificity of autoantibodies to M-type phospholipase A2 receptor (PLA2R) in differentiating idiopathic membranous nephropathy (IMN) from secondary forms and other glomerular diseases.
    J Nephrol 2017 Oct 28. Epub 2017 Oct 28.
    Renal Unit, Department of Medicine and Surgery, University of Milano-Bicocca and ASST Monza, Monza, MB, Italy.
    Autoantibody against phospholipase A2 receptor (anti-PLA2R) is a sensitive and specific biomarker of idiopathic membranous nephropathy (iMN), being found in approximately 70% of iMN patients and only occasionally in other glomerular diseases. However, whereas its diagnostic specificity vs. normal controls and other glomerulonephritides (GN) has been firmly established, its specificity vs. Read More

    Body mass index (BMI) and body surface area (BSA) in scleroderma patients.
    J Eur Acad Dermatol Venereol 2017 Oct 28. Epub 2017 Oct 28.
    Department of Dermatology, School of Medicine in Katowice Medical University of Silesia, Poland.
    Dysfunctions of the digestive tract in the course of systemic sclerosis were observed in as many as 90% cases [1]. It causes fluctuations of body composition and consequently lead to body mass disorders. Among the methods of assessing the nutritional condition, the most traditional is BMI (Body Mass Index) technique. Read More

    Patient acceptable symptom state in scleroderma: results from the tocilizumab compared with placebo trial in active diffuse cutaneous systemic sclerosis.
    Rheumatology (Oxford) 2017 Oct 25. Epub 2017 Oct 25.
    Schulich School of Medicine and Dentistry, Western University, London, ON, Canada.
    Objectives: Patient acceptable symptom state (PASS) as an absolute state of well-being has shown promise as an outcome measure in many rheumatologic conditions. We aimed to assess whether PASS may be effective in active diffuse cutaneous SSc differentiating active from placebo.

    Methods: Data from the phase 2 Safety and Efficacy of Subcutaneous Tocilizumab in Adults with Systemic Sclerosis (faSScinate) trial were used, which compared tocilizumab (TCZ) vs placebo over 48 weeks followed by an open-label TCZ period to 96 weeks. Read More

    Successful Treatment of a Scleroderma-Associated Leg Ulcer With Endovenous Laser Ablation.
    Dermatol Surg 2017 Oct 26. Epub 2017 Oct 26.
    Division of Dermatology and Mohs Surgery, Scripps Clinic, La Jolla, California Vein Clinic, Scripps Clinic, San Diego, California Division of Dermatology and Mohs Surgery, Scripps Clinic, La Jolla, California Vein Clinic, Scripps Clinic, San Diego, California.

    A systematic review of the epidemiology, disease characteristics and management of systemic sclerosis in Australian adults.
    Int J Rheum Dis 2017 Oct 27. Epub 2017 Oct 27.
    Department of Medicine, The University of Melbourne at St Vincent's Hospital, Melbourne, Vic., Australia.
    Objective: Australia has one of the highest prevalence rates of systemic sclerosis (SSc) worldwide. In order to highlight management deficiencies and key areas for further research, it is essential to understand its local epidemiological patterns, natural history, prognosis and mortality trends over time.

    Methods: To identify Australian SSc-specific information through a systematic review focusing on areas of epidemiology, disease characteristics, treatment, functional ability, disease burden and health-related quality of life (HRQoL). Read More

    Toll-like receptor 9 in systemic sclerosis patients: relation to modified Rodnan skin score, disease severity, and functional status.
    Clin Rheumatol 2017 Oct 26. Epub 2017 Oct 26.
    Clinical and Chemical Pathology Department (Immunology), Faculty of Medicine, Helwan University, Helwan, Egypt.
    The objective of this study is to assess toll-like receptor-9 (TLR9) expression in CD3-positive T lymphocytes and CD19-positive B lymphocytes in systemic sclerosis (SSc) patients and to study their relation to the extent of skin fibrosis, disease characteristics, and severity as well as the functional status. Fifty-five female SSc patients and 30 matched controls were included. Skin thickness was scored according to the modified Rodnan skin score (mRss). Read More

    Safety and efficacy of subcutaneous tocilizumab in systemic sclerosis: results from the open-label period of a phase II randomised controlled trial (faSScinate).
    Ann Rheum Dis 2017 Oct 24. Epub 2017 Oct 24.
    University of California, Los Angeles, California, USA.
    Objectives: Assess the efficacy and safety of tocilizumab in patients with systemic sclerosis (SSc) in a phase II study.

    Methods: Patients with SSc were treated for 48 weeks in an open-label extension phase of the faSScinate study with weekly 162 mg subcutaneous tocilizumab. Exploratory end points included modified Rodnan Skin Score (mRSS) and per cent predicted forced vital capacity (%pFVC) through week 96. Read More

    Discovery and pharmacological characterization of a new class of prolyl-tRNA synthetase inhibitor for anti-fibrosis therapy.
    PLoS One 2017 24;12(10):e0186587. Epub 2017 Oct 24.
    Immunology Unit, Pharmaceutical Research Division, Takeda Pharmaceutical Company Limited, Fujisawa, Kanagawa, Japan.
    Scleroderma has clinical characteristics including skin and other tissue fibrosis, but there is an unmet need for anti-fibrotic therapy. Halofuginone (HF) is a well-known anti-fibrosis agent in preclinical and clinical studies which exerts its effect via inhibition of TGF-β/Smad3 signaling pathway. Recently, prolyl-tRNA synthetase (PRS) was elucidated as a target protein for HF that binds to the proline binding site of the catalytic domain of PRS. Read More

    Toll-Like Receptor-4 Signaling Drives Persistent Fibroblast Activation and Prevents Fibrosis Resolution in Scleroderma.
    Adv Wound Care (New Rochelle) 2017 Oct;6(10):356-369
    Northwestern Scleroderma Program, Feinberg School of Medicine, Chicago, Illinois.
    Significance: This review provides current overview of the emerging role of innate immunity in driving fibrosis, and preventing its resolution, in scleroderma (systemic sclerosis, SSc). Understanding the mechanisms of dysregulated innate immunity in fibrosis and SSc will provide opportunities for therapeutic interventions using novel agents and repurposed existing drugs. Recent Advances: New insights from genomic and genetic studies implicate components of innate immune signaling such as pattern recognition receptors (PRRs), downstream signaling intermediates, and endogenous inhibitors, in fibrosis in SSc. Read More

    Factors associated with disease progression in early-diagnosed pulmonary arterial hypertension associated with systemic sclerosis: longitudinal data from the DETECT cohort.
    Ann Rheum Dis 2017 Oct 23. Epub 2017 Oct 23.
    Department of Rheumatology, University Hospital Zurich, Zurich, Switzerland.
    Objective: Pulmonary arterial hypertension (PAH) is a severe complication of systemic sclerosis (SSc). In this longitudinal study, we aimed to identify factors associated with an unfavourable outcome in patients with SSc with early PAH (SSc-PAH) from the DETECT cohort.

    Methods: Patients with SSc-PAH enrolled in DETECT were observed for up to 3 years. Read More

    The involvement of galectin-3 in skin injury in systemic lupus erythematosus patients.
    Lupus 2017 Jan 1:961203317736144. Epub 2017 Jan 1.
    1 Department of Dermatology, 56713 Sun Yat-sen Memorial Hospital , Guangzhou, China.
    Objective Our previous research suggested that anti-galectin-3 antibody was highly associated with the development of lupus skin lesions in systemic lupus erythematosus (SLE). In this study we aimed to investigate the involvement of galectin-3 in SLE skin damage. Methods The study consisted of 49 patients with SLE, 16 with dermatomyositis and 11 with systemic scleroderma and 20 healthy controls. Read More

    Combined platelet-rich plasma and lipofilling treatment provides great improvement in facial skin-induced lesion regeneration for scleroderma patients.
    Stem Cell Res Ther 2017 Oct 23;8(1):236. Epub 2017 Oct 23.
    Cellular and Molecular Pathophysiology Laboratory, Department of Surgical Oncological and Stomatological Sciences, University of Palermo, Via del Vespro 131, Palermo, 90127, Italy.
    Background: The use of stem cells, including mesenchymal stem cells (MSCs), for regenerative medicine is gaining interest for the clinical benefits so far obtained in patients. This study investigates the use of adipose autologous tissue in combination with platelet-rich plasma (PRP) to improve the clinical outcome of patients affected by systemic sclerosis (SSc).

    Methods: Adipose-derived mesenchymal stem cells (AD-MSCs) and PRPs were purified from healthy donors and SSc patients. Read More

    PTP4A1 promotes TGFβ signaling and fibrosis in systemic sclerosis.
    Nat Commun 2017 Oct 20;8(1):1060. Epub 2017 Oct 20.
    Division of Rheumatology, Allergy and Immunology, Department of Medicine, University of California San Diego, 9500 Gilman Dr, La Jolla, CA, 92093, USA.
    Systemic sclerosis (SSc) is an autoimmune disease characterized by fibrosis of skin and internal organs. Protein tyrosine phosphatases have received little attention in the study of SSc or fibrosis. Here, we show that the tyrosine phosphatase PTP4A1 is highly expressed in fibroblasts from patients with SSc. Read More

    Assessment of hospitalization and mortality of scleroderma in-patients: a thirteen-year study.
    Reumatologia 2017 31;55(4):163-168. Epub 2017 Aug 31.
    Division of Rheumatology, Departments of Internal Medicine, Shiraz University of Medical Sciences, Iran.
    Objective: Systemic sclerosis (SSc) is an uncommon non-hereditary sporadic disease that increases the risk of premature death, especially in diffuse type. We determined the prevalence of SSc in the last 13 years in our rheumatologic hospitals as a referral center for southern Iranian patients, the causes of hospitalization, the average length of stay (LOS), the mortality rate, and the reason for their mortality.

    Material And Methods: A cross-sectional study was performed in Shiraz University of Medical Sciences, Iran. Read More

    Early-Life Gut Dysbiosis: A Driver of Later-Life Fibrosis?
    J Invest Dermatol 2017 Nov;137(11):2253-2255
    Department of Medicine, Northwestern University, Chicago, Illinois, USA. Electronic address:
    Using a novel mouse model of scleroderma induced by immunization with topoisomerase-I peptide-loaded dendritic cells, Mehta et al. found that early-life antibiotic exposure resulted in increased later-life fibrosis in the skin and lungs. These observations advance the novel concept that gut microbiome alterations caused by early-life exposures may contribute to scleroderma pathogenesis, and warrant in-depth characterization and validation in complementary disease models. Read More

    Scleroderma Renal Crisis in Mixed Connective Tissue Disease With Full Renal Recovery Within 3 Months: A Case Report With Expanding Treatment Modalities to Treat Each Clinical Sign as an Independent Entity.
    J Investig Med High Impact Case Rep 2017 Oct-Dec;5(4):2324709617734012. Epub 2017 Oct 10.
    Eastern Virginia Medical School, Norfolk, VA, USA.
    Mixed connective tissue disease (MCTD) is a rheumatologic overlap syndrome that can present with symptoms of systemic lupus erythematous, scleroderma, and polymyositis. A severe but rare complication that can occur in MCTD is scleroderma renal crisis. With multiple poor prognostic indicators, the renal outcome is usually poor. Read More

    Antioxidants and NOX1/NOX4 inhibition blocks TGFβ1-induced CCN2 and α-SMA expression in dermal and gingival fibroblasts.
    PLoS One 2017 19;12(10):e0186740. Epub 2017 Oct 19.
    Department of Dentistry, Schulich School of Medicine and Dentistry, University of Western Ontario, London, Ontario, Canada.
    TGFbeta induces fibrogenic responses in fibroblasts. Reactive oxygen species (ROS)/nicotinamide adenine dinucleotide phosphate (NADPH) oxidase (NOX) may contribute to fibrogenic responses. Here, we examine if the antioxidant N-acetylcysteine (NAC), the NOX inhibitor diphenyleneiodonium (DPI) and the selective NOX1/NOX4 inhibitor GKT-137831 impairs the ability of TGFbeta to induce profibrotic gene expression in human gingival (HGF) and dermal (HDF) fibroblasts. Read More

    Rheopheresis as a causal therapy option for systemic scleroderma (SSc).
    Clin Hemorheol Microcirc 2017 Oct 7. Epub 2017 Oct 7.
    Department of Dermatology, University Medicine Greifswald, Greifswald, Germany.
    A complex pathomechanism accounts systemic sclerosis as a form of collagenosis. A triad of vasculopathy, autoinflammation, and dysbalance of the fibroblast function can be seen as cause, as well as symptomatic appearance. Comparative with other collagenoses, e. Read More

    Swallowing difficulties with medication intake assessed with a novel self-report questionnaire in patients with systemic sclerosis - a cross-sectional population study.
    Patient Prefer Adherence 2017 28;11:1687-1699. Epub 2017 Sep 28.
    Pharmaceutical Care Research Group, Department of Pharmaceutical Sciences, University of Basel, Basel, Switzerland.
    Objectives: To assess subjective swallowing difficulties (SD) with medication intake and their practical consequences in patients suffering from systemic sclerosis (SSc) with a novel self-report questionnaire.

    Design And Setting: Based on a systematic literature review, we developed a self-report questionnaire and got it approved by an expert panel. Subsequently, we sent the questionnaire by post mail to SSc patients of the European Center for the Rehabilitation of Scleroderma Rheinfelden, Switzerland. Read More

    Agonistic antibodies in systemic sclerosis.
    Immunol Lett 2017 Oct 13. Epub 2017 Oct 13.
    Dipartimento di Scienze Cliniche e Molecolari, Università Politecnica delle Marche, Ancona, 60126, Italy. Electronic address:
    Systemic sclerosis (SSc) is characterized by microangiopathy, excessive fibrosis, and the presence of circulating autoantibodies to several cellular and extracellular components. The role of autoimmunity in generating the clinical and pathologic phenotypes in SSc has been long debated and is still matter of controversy. Distinct specificities of antinuclear antibodies (ANAs) are selectively detected in SSc patients and are associated with unique disease manifestations, but do not have a proven pathogenic role. Read More

    Spectrum of orocutaneous disease associations: Immune-mediated conditions.
    J Am Acad Dermatol 2017 Nov;77(5):795-806
    Division of Dermatology, Baylor University Medical Center, Dallas, Texas. Electronic address:
    There are a number of diseases that manifest both on the skin and the oral mucosa, and therefore the importance for dermatologists in clinical practice to be aware of these associations is paramount. In the following continuing medical education series, we outline orocutaneous disease associations with both immunologic and inflammatory etiologies. Read More

    Polymorphism of keratin 1 associates with systemic lupus erythematosus and systemic sclerosis in a south Chinese population.
    PLoS One 2017 13;12(10):e0186409. Epub 2017 Oct 13.
    Department of Immunology, Xiangya School of Medicine, Central South University, Changsha, Hunan, China.
    Both systemic lupus erythematosus (SLE) and systemic sclerosis (SSc) diseases are related to the genetic and environmental factors, causing damage to the skin. The mutations of keratin 1 gene (KRT1) were reported to associate with skin diseases. The single-nucleotide polymorphism (SNP, rs14024) and the indel polymorphism (cds-indel, rs267607656), consisting mostly of the common haplotypes and could be used for genotyping of KRT1. Read More

    Use of Lipotransfer in Scleroderma.
    Aesthet Surg J 2017 07;37(suppl_3):S33-S37
    Charles Wolfson Center for Reconstructive Surgery, Royal Free Hospital, London, UK.
    Lipotransfer for soft tissue filling is a well-established plastic and aesthetic surgical technique. Elective fat grafting is considered a safe and well-tolerated procedure. Coleman and others have reported that fat grafting may have tissue regenerative properties and not only serve as a soft tissue filler. Read More

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