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    Trichoscopic Features of Linear Morphea on the Scalp.
    Skin Appendage Disord 2018 Jan 12;4(1):31-33. Epub 2017 Jul 12.
    Department of Dermatology and Cutaneous Surgery, University of Miami, Miami, FL, USA.
    Linear scleroderma en coup de sabre (LSCS) is the most common form of morphea on the scalp and its trichoscopic features have not been described yet. We report 2 adult women with LSCS with distinct dermoscopic findings. They both had an atrophic well-defined linear patch of alopecia on the frontotemporal scalp. Read More

    S100A9 aggravates bleomycin-induced dermal fibrosis in mice via activation of ERK1/2 MAPK and NF-κB pathways.
    Iran J Basic Med Sci 2018 Feb;21(2):194-201
    Department of Rheumatology and Immunology, Nanjing Drum Tower Hospital, the Affiliated Hospital of Nanjing University Medical School, Nanjing 210008, Jiangsu, China.
    Objectives: This study aims to investigate the pathogenicity and possible mechanisms of S100A9 function in mice models of scleroderma.

    Materials And Methods: The content of S100A9 in the skin tissues of mice with scleroderma was determined. Different concentrations of bleomycin (BLM) and S100A9 were subcutaneously injected into the backs of mice simultaneously, and then pathological changes in the skin of these mice were monitored. Read More

    Guidelines for management of rheumatic diseases in developing countries from basics to real-world situation: relevance, need, and processes for development.
    Rheumatol Int 2018 Feb 14. Epub 2018 Feb 14.
    Department of Clinical Immunology, Sanjay Gandhi Postgraduate Institute of Medical Sciences (SGPGIMS), Lucknow, 226014, India.
    Guidelines or recommendations help to provide uniform standards in medical practice. The development of guidelines requires adherence to pre-defined norms prescribed by different international organizations such as the European League against Rheumatism (EULAR). We searched Pubmed and LILACS to identify published papers in five major rheumatic diseases (rheumatoid arthritis, systemic lupus erythematosus, spondyloarthropathies, osteoarthritis, and scleroderma) from different countries based on their economic prosperity and could find a lack of published literature from most economically weaker regions. Read More

    Progressive Hemifacial Atrophy and Linear Scleroderma En Coup de Sabre: A Spectrum of the Same Disease?
    Front Med (Lausanne) 2017 31;4:258. Epub 2018 Jan 31.
    Pirogov Russian National Research Medical University, Moscow, Russia.
    Similar clinical and histhopathological features in progressive hemifacial atrophy and linear scleroderma en coup de sabre are well known. Trauma may predispose to the development of both diseases. The lack of association with anti-Borrelia antibodies was shown in both cases as well. Read More

    Surgical management of gastroesophageal reflux disease in patients with systemic sclerosis.
    Surg Endosc 2018 Feb 12. Epub 2018 Feb 12.
    Department of General Surgery, Cleveland Clinic, 9500 Euclid Avenue, Desk A-100, Cleveland, OH, 44195, USA.
    Background: Systemic sclerosis (scleroderma) is frequently associated with both gastroesophageal reflux disease (GERD) and simultaneous esophageal dysmotility. Anti-reflux procedures in this patient population must account for the existing physiology of each patient and likely disease progression. We aim to compare perioperative and intermediate outcomes of fundoplication versus gastric bypass for the treatment of GERD. Read More

    A unique role for galectin-9 in angiogenesis and inflammatory arthritis.
    Arthritis Res Ther 2018 Feb 12;20(1):31. Epub 2018 Feb 12.
    Division of Rheumatology and Clinical Autoimmunity Center of Excellence, Department of Internal Medicine, University of Michigan Medical School, 4368 BSRB, 109 Zina Pitcher Drive, Ann Arbor, MI, 48109-2200, USA.
    Background: Galectin-9 (Gal-9) is a mammalian lectin secreted by endothelial cells that is highly expressed in rheumatoid arthritis synovial tissues and synovial fluid. Roles have been proposed for galectins in the regulation of inflammation and angiogenesis. Therefore, we examined the contribution of Gal-9 to angiogenesis and inflammation in arthritis. Read More

    Eosinophilic Fasciitis: A Single Center Experience of Seven Patients.
    Isr Med Assoc J 2018 Feb;20(2):95-99
    Department of Medicine, Hadassah-Hebrew University Medical Center, Ein Kerem Campus, Jerusalem, Israel.
    Background: Eosinophilic fasciitis (EF) is a rare disease characterized by scleroderma-like skin, inflammation of deep muscle fascia, hypergammaglobulinemia, peripheral eosinophilia, and elevated erythrocyte sedimentation rate.

    Objectives: To present our experience in diagnosis and treatment of seven biopsy-proven EF patients in a large tertiary medical center.

    Methods: We screened all patients who were admitted to our tertiary medical center and diagnosed with EF by tissue biopsies from January 2000 to January 2016. Read More

    [Etiological profile of secondary Raynaud's phenomenon in an internal medicine department. About 121 patients].
    J Med Vasc 2018 Feb 20;43(1):29-35. Epub 2017 Dec 20.
    Service de médecine interne, La Rabta, Tunis, Tunisie.
    Introduction: Raynaud's phenomenon is a reversible episodic vasospastic disorder triggered by cold or emotion. Two types of Raynaud's phenomenon were distinguished: Raynaud's disease and secondary Raynaud's phenomenon. The purpose of this study was to determine the etiologic profile of secondary Raynaud's phenomenon in an internal medicine department. Read More

    Characterization and Treatment of Unilateral Facial Muscle Spasm in Linear Scleroderma: A Case Report.
    Tremor Other Hyperkinet Mov (N Y) 2018 25;8:531. Epub 2018 Jan 25.
    Marist College, Poughkeepsie, NY, USA.
    Background: Linear scleroderma has been associated with muscle spasms ipsilateral to skin lesions. Typically, spasms are located in trigeminal innervated muscles, leading to hemimasticatory spasm (HMS).

    Case Report: We report a case of linear scleroderma associated with spasm of muscles innervated not only by the trigeminal but also by the facial nerve. Read More

    Factors influencing early referral, early diagnosis and management in patients with diffuse cutaneous systemic sclerosis.
    Rheumatology (Oxford) 2018 Feb 2. Epub 2018 Feb 2.
    Division of Rheumatology, University of Michigan, Ann Arbor, MI, USA.
    Objective: To gain insight into clinical practice regarding referral, early diagnosis and other aspects of the management of patients with dcSSc in Europe and the USA.

    Methods: Semi-structured interviews were conducted with 84 rheumatologists (or internal medicine physicians) and 40 dermatologists in different countries (the UK, France, Germany, Italy, Spain and the USA). Physicians were asked to identify key steps in the patient pathway relating to patient presentation, diagnosis and referral, in addition to other treatment and follow-up processes. Read More

    Pulmonary arterial hypertension in the setting of scleroderma is different than in the setting of lupus: A review.
    Respir Med 2018 Jan 2;134:42-46. Epub 2017 Dec 2.
    Yale University, School of Medicine, Section of Pulmonary, Critical Care & Sleep Medicine, New Haven, CT, USA. Electronic address:
    Pulmonary hypertension (PH) is a clinical syndrome that is subdivided into five groups per the World Health Organization (WHO) classification, based largely on hemodynamic and pathophysiologic criteria. WHO Group 1 PH, termed pulmonary arterial hypertension (PAH), is a clinically progressive disease that can eventually lead to right heart failure and death, and it is hemodynamically characterized by pre-capillary PH and increased pulmonary vascular resistance in the absence of elevated left ventricular filling pressures. PAH can be idiopathic, heritable, or associated with a variety of conditions. Read More

    Validation of the Brief Fear of Negative Evaluation Scale-II in patients with systemic sclerosis: A Scleroderma Patient-centered Intervention Network Cohort study.
    Arthritis Care Res (Hoboken) 2018 Feb 6. Epub 2018 Feb 6.
    San Diego State University/University of California, San Diego Joint Doctoral Program in Clinical Psychology, San Diego, California, United States.
    Objective: Fear of negative evaluation is a common concern among individuals with visible differences but has received limited attention in systemic sclerosis (SSc), which can involve substantial changes to appearance. The Brief Fear of Negative Evaluation Scale (BFNE) was specifically designed to evaluate fear of negative evaluation. There are currently three versions of the BFNE with strong demonstrated measurement properties: two eight-item versions (BFNE-S, BFNE-8) and one 12-item version (BFNE-II). Read More

    A Systematic Review and Meta-Analysis of the Effects of Topical Nitrates in the Treatment of Primary and Secondary Raynaud's Phenomenon.
    J Am Acad Dermatol 2018 Feb 2. Epub 2018 Feb 2.
    Skin Lupus & Autoimmune Connective Tissue Section, The Ronald O. Perelman Department of Dermatology, New York University School of Medicine, New York, New York. Electronic address:
    Background: Multiple placebo controlled trials have assessed locally applied topical nitrate preparations in treating Raynaud's phenomenon (RP).

    Objectives: The objective of this meta-analysis was to assess the effects of local topical nitrates in primary and secondary RP with respect to a combined endpoint integrating parameters of digital blood flow and clinical severity.

    Methods: A systematic review was performed using MEDLINE, Embase and the Cochrane library. Read More

    New developments on skin fibrosis - Essential signals emanating from the extracellular matrix for the control of myofibroblasts.
    Matrix Biol 2018 Feb 12. Epub 2018 Feb 12.
    Department of Dermatology, University of Cologne, Cologne, Germany; Translational Matrix Biology, University of Cologne, Medical Faculty, Cologne, Germany. Electronic address:
    Many different diseases are associated with fibrosis of the skin. The clinical symptoms can vary considerably with a broad range from isolated small areas to the involvement of the entire integument. Fibrosis is triggered by a multitude of different stimuli leading to activation of the immune and vascular system that then initiate fibroblast activation and formation of matrix depositing and remodeling myofibroblasts. Read More

    Parasitism and venom of ectoparasitoid Scleroderma guani impairs host cellular immunity.
    Arch Insect Biochem Physiol 2018 Feb 5. Epub 2018 Feb 5.
    Key Laboratory of Forest Disaster Warning and Control of Yunnan Province, Southwest Forestry University, Kunming, China.
    Venom is a prominently maternal virulent factor utilized by parasitoids to overcome hosts immune defense. With respect to roles of this toxic mixture involved in manipulating hosts immunity, great interest has been mostly restricted to Ichneumonoidea parasitoids associated with polydnavirus (PDV), of which venom is usually considered as a helper component to enhance the role of PDV, and limited Chalcidoidea species. In contrast, little information is available in other parasitoids, especially ectoparasitic species not carrying PDV. Read More

    Pyridostigmine for the treatment of gastrointestinal symptoms in systemic sclerosis.
    Semin Arthritis Rheum 2018 Jan 5. Epub 2018 Jan 5.
    Division of Rheumatology, Johns Hopkins University School of Medicine, Baltimore, MD.
    Background/purpose: Symptoms of gastrointestinal dysmotility are common among patients with systemic sclerosis (SSc), and the management of severe cases is often limited by a relative lack of effective interventions. The objective of this case series was to review our experience with pyridostigmine as a treatment for patients with SSc and symptomatic gastrointestinal disease.

    Methods: This study evaluated rates of symptom improvement, side effects, medication adherence, and dose ranges for SSc patients prescribed pyridostigmine for refractory gastrointestinal symptoms over a 10-year period at a quaternary referral center. Read More

    Long-Term Survival in Bilateral Lung Transplantation for Scleroderma-Related Lung Disease.
    Ann Thorac Surg 2018 Mar 1;105(3):893-900. Epub 2018 Feb 1.
    Houston Methodist DeBakey Heart and Vascular Center, Department of Cardiovascular Surgery, Houston Methodist Hospital, Houston, Texas; J. C. Walter Jr. Transplant Center, Department of Surgery, Weill Cornell Medical College, Houston Methodist Hospital, Houston, Texas.
    Background: Lung disease is the leading cause of morbidity and death in scleroderma patients, but scleroderma is often considered a contraindication to lung transplantation because of concerns for worse outcomes. We evaluated whether 5-year survival in scleroderma patients after lung transplantation differed from other patients with restrictive lung disease.

    Methods: This was a single-center, retrospective cohort study of all patients undergoing bilateral lung transplantation for scleroderma-related pulmonary disease between January 2006 and December 2014. Read More

    Diagnostic potential of tears in ophthalmology.
    Cesk Slov Oftalmol 2017 ;73(3):101-108
    In research circles, there is an increasing need to seek and identify new methods in the diagnosis of pathologies, monitoring the progression of the disease and response to treatment. The sensitivity of detection technologies has improved markedly, and enables the quantification of analyses in very small quantities. Tears represent a biological material with ever increasingly developing possibilities in the diagnosis of various pathologies. Read More

    Stem cell transplant in systemic sclerosis: An Indian experience.
    Int J Rheum Dis 2018 Feb 2. Epub 2018 Feb 2.
    Rheumatology & Clinical Immunology, Army Hospital R & R, New Delhi, India.
    Aim: To prospectively evaluate long term outcomes in a cohort of patients with Systemic sclerosis treated with Hematopoietic stem cell transplant (HSCT).

    Method: This is a prospective observational study of four SSc patients who underwent HSCT at a tertiary care center in India between 2008-2012. The selection criteria included young individuals with rapidly progressive disease and at least one major organ involvement. Read More

    Mosaic capillaroscopic findings in systemic sclerosis.
    Wien Med Wochenschr 2018 Feb 1. Epub 2018 Feb 1.
    Department of Rheumatology and Clinical Immunology, Justus-Liebig University Giessen, Giessen, Germany.
    A 67-year-old woman with triphasic Raynaud's phenomenon since 2 years was diagnosed as having limited cutaneous systemic sclerosis (SSc) 1 year ago based on puffy fingers, positive antinuclear and anti-Scl-70 autoantibodies, and initial pulmonary fibrosis. Capillaroscopic examination revealed "mosaic" capillaroscopic findings that included normal pattern; "scleroderma"-type capillaroscopic pattern "early" phase, with single giant capillaries and haemorrhages, preserved capillary distribution; and "scleroderma"-type capillaroscopic pattern "active" phase, with high number of giant capillary loops and haemorrhages, moderately disturbed distribution. Nailfold capillaroscopy is an established non-invasive imaging technique for morphologic evaluation of the capillaries in the nail-fold area that reveals diagnostic changes in the majority of SSc patients. Read More

    Mycophenolate mofetil treatment of systemic sclerosis reduces myeloid cell numbers and attenuates the inflammatory gene signature in skin.
    J Invest Dermatol 2018 Jan 29. Epub 2018 Jan 29.
    Department of Molecular and Systems Biology, Hanover, NH 03755. Electronic address:
    Fewer than half of patients with systemic sclerosis (SSc) demonstrate modified Rodnan skin score (mRSS) improvement during mycophenolate mofetil (MMF) treatment. To understand the molecular basis for this observation, we extended our prior studies and characterized molecular and cellular changes in skin biopsies from subjects with SSc treated with MMF. Eleven subjects completed ≥24 months of MMF therapy. Read More

    Clinical and serological features of systemic sclerosis in a multicenter African American cohort: Analysis of the genome research in African American scleroderma patients clinical database.
    Medicine (Baltimore) 2017 Dec;96(51):e8980
    Division of Rheumatology, University of California San Francisco, CA.
    Racial differences exist in the severity of systemic sclerosis (SSc). To enhance our knowledge about SSc in African Americans, we established a comprehensive clinical database from the largest multicenter cohort of African American SSc patients assembled to date (the Genome Research in African American Scleroderma Patients (GRASP) cohort).African American SSc patients were enrolled retrospectively and prospectively over a 30-year period (1987-2016), from 18 academic centers throughout the United States. Read More

    Case report: One case of primary AL amyloidosis repeatedly misdiagnosed as scleroderma.
    Medicine (Baltimore) 2017 Dec;96(50):e8771
    Department of Rheumatology and Clinical Immunology, The First Affiliated Hospital of Zhengzhou University, Zhengzhou, China.
    Introduction: Amyloid light chain (AL) results from the deposition of immunoglobulin light chain fragments, and can affect multiple organs/systems. Our patient was diagnosed as scleroderma repeatedly because of extensive skin thickening and hardening, but the treatment was not effective. We did extensive laboratory examinations including serum/urine protein electrophoresis and flow cytometry assay of bone marrow aspiration. Read More

    Imaging molecular signatures for clinical detection of scleroderma in the hand by multispectral photoacoustic elastic tomography.
    J Biophotonics 2018 Feb 1. Epub 2018 Feb 1.
    Bioimaging Core, Faculty of Health Sciences, University of Macau, Macau, SAR, China.
    Scleroderma (SD) is a rare autoimmune disease, which is divided into two categories: the localized SD and systemic SD. The localized SD mainly causes skin thickening of the fingers, whereas the systemic SD can further affect the blood vessels and internal organs. In this pilot study, the multispectral photoacoustic elastic tomography (PAET) imaging technique was used to recover the quantitative physiological and elastic properties of biological tissues for the diagnosis of SD. Read More

    Emphysematous change with scleroderma-associated interstitial lung disease: the potential contribution of vasculopathy?
    BMC Pulm Med 2018 Jan 30;18(1):25. Epub 2018 Jan 30.
    Department of Respiratory Medicine, Kanagawa Cardiovascular and Respiratory Center, 6-16-1 Tomioka-higashi, Kanazawa-ku, Yokohama, 236-0051, Japan.
    Background: Pulmonary emphysema combined with systemic sclerosis (SSc)-associated interstitial lung disease (ILD) occurs more often in smokers but also in never-smokers. This study aimed to describe a new finding characterized by peculiar emphysematous change with SSc-associated ILD (SSc-ILD).

    Methods: We conducted a retrospective review of 21 consecutive patients with SSc-ILD diagnosed by surgical lung biopsy and focused on the radio-pathological correlation of the emphysematous change. Read More

    Decreased global myocardial perfusion at adenosine stress as a potential new biomarker for microvascular disease in systemic sclerosis: a magnetic resonance study.
    BMC Cardiovasc Disord 2018 01 30;18(1):16. Epub 2018 Jan 30.
    Skane University Hospital, Department of Clinical Physiology, Lund University, Lund, Sweden.
    Background: Patients with systemic sclerosis (SSc) have high cardiovascular mortality even though there is no or little increase in prevalence of epicardial coronary stenosis. First-pass perfusion on cardiovascular magnetic resonance (CMR) have detected perfusion defects indicative of microvascular disease, but the quantitative extent of hypoperfusion is not known. Therefore, we aimed to determine if patients with SSc have lower global myocardial perfusion (MP) at rest or during adenosine stress, compared to healthy controls, quantified with CMR. Read More

    An overlap case of Parry-Romberg syndrome and en coup de sabre with striking ocular involvement and anti-double-stranded DNA positivity.
    Indian J Ophthalmol 2018 Feb;66(2):336-338
    Department of Ophthalmology, Ministry of Health, Diskapi Yildirim Beyazit Training and Research Hospital, Ankara, Turkey.
    Parry-Romberg syndrome (PRS) may overlap localized scleroderma (morphea) lesions with linear depression (en coup de sabre [ECDS]). Overlap case with PRS and ECDS was presented. Enophthalmos, uveitis, ocular torticollis, keratic linear precipitates, and anti-double-stranded DNA positivity were identified. Read More

    Development of PBC/SSc overlap syndrome in chronic GVHD patient: immunological implications in the presence of mitochondrial, nucleolar and spindle midzone autoantigens.
    Gastroenterol Hepatol Bed Bench 2017 ;10(4):323-331
    Clinical Investigation Laboratory, Diagnostics Department, Spedali Civili of Brescia, Brescia, Italy.
    Chronic Graft versus Host Disease (cGVHD) is a complex disease resulting from donor T-cell recognition of a genetically disparate recipient that is unable to reject donor cells after allogeneic Stem Cell Transplantation (HSCT). cGVHD has some features resembling to autoimmune diseases (AD) such as Sjögren syndrome, primary biliary cirrhosis (PBC) and scleroderma (SSc). Also patients with cGVHD could develop extensive cGVHD with scleroderma-like skin manifestations and other clinical signs similar to those of patients with scleroderma. Read More

    Serum microRNA screening and functional studies reveal miR-483-5p as a potential driver of fibrosis in systemic sclerosis.
    J Autoimmun 2018 Jan 19. Epub 2018 Jan 19.
    Department of Rheumatology & Clinical Immunology, University Medical Center Utrecht, Utrecht University, Utrecht, The Netherlands; Laboratory of Translational Immunology, University Medical Center Utrecht, Utrecht University, Utrecht, The Netherlands. Electronic address:
    Objective: MicroRNAs (miRNAs) are regulatory molecules, which have been addressed as potential biomarkers and therapeutic targets in rheumatic diseases. Here, we investigated the miRNA signature in the serum of systemic sclerosis (SSc) patients and we further assessed their expression in early stages of the disease.

    Methods: The levels of 758 miRNAs were evaluated in the serum of 26 SSc patients as compared to 9 healthy controls by using an Openarray platform. Read More

    Bicaudal D2 is a novel autoantibody target in systemic sclerosis that shares a key epitope with CENP-A but has a distinct clinical phenotype.
    Autoimmun Rev 2018 Jan 31. Epub 2018 Jan 31.
    Department of Medicine, McGill University, Montréal, Quebec, Canada; Division of Rheumatology, Jewish General Hospital, Montréal, Quebec, Canada. Electronic address:
    We studied the clinical correlations and epitopes of autoantibodies directed to a novel autoantigen, Bicaudal D (BICD2), in systemic sclerosis (SSc) and reviewed its relationship to centromere protein A (CENP-A). 451 SSc sera were tested for anti-BICD2 using a paramagnetic bead immunoassay and then univariate and multivariate logistic regression was used to study the association between anti-BICD2 and demographic and clinical parameters as well as other SSc-related autoantibodies. Epitope mapping was performed on solid phase matrices. Read More

    Impact of a new simplified disability scoring system for adult patients with localized scleroderma.
    J Dermatol 2018 Jan 25. Epub 2018 Jan 25.
    Department of Dermatology and Plastic Surgery, Faculty of Life Sciences, Kumamoto University, Kumamoto, Japan.
    Localized scleroderma (LoS) involves dermal but not internal inflammation and fibrosis. Cosmetic changes often impact quality of life (QOL), however, impairment of activities of daily living (ADL) in LoS patients has not been investigated. To determine what factor(s) are associated with ADL in adult patients with LoS, we performed a retrospective observational study in 177 Japanese adult LoS patients using a novel LoS disability score based on Barthel's indices of ADL: feeding, bathing, grooming, dressing, bowels, bladder, toilet use, transfers, mobility and stairs. Read More

    Scleroderma with an update about clinico-pathological correlation.
    G Ital Dermatol Venereol 2018 Jan 24. Epub 2018 Jan 24.
    Dipartimento di Medicina Molecolare "Sapienza" Università di Roma, Rome, Italy.
    Scleroderma is divided into a systemic form called systemic sclerosis and a localized form also called morphea. According to 2013 ACR/EULAR Classification Criteria for Systemic Sclerosis, developed by the American College of Rheumatology (ACR) and the European League Against Rheumatism (EULAR), skin thickening of the fingers extending proximal to the metacarpophalangeal joints is sufficient for a patient to be classified as having scleroderma. Histological examination is not included in the diagnostic criteria and is not routinely performed. Read More

    Ruxolitinib: a steroid sparing agent in chronic graft-versus-host disease.
    Bone Marrow Transplant 2018 Jan 24. Epub 2018 Jan 24.
    Division of Medical Oncology, Washington University School of Medicine, St. Louis, MO, USA.
    Inhibition of the Janus-associated kinases (JAK) with ruxolitinib (RUX) reduces graft-versus-host disease (GVHD) in preclinical and clinical models. In total 19 allograft recipients with moderate/severe steroid-dependent chronic GVHD received RUX as ≥2nd line salvage. RUX was well tolerated, and led to complete/partial resolution of oral (92/7%), cutaneous (82/0%), hepatic (71/28%), gastro-intestinal (75/17%), musculoskeletal (33/67%), pulmonary (0/80%), scleroderma (0/75%), vaginal (0/75%), and ocular (0/100%) chronic GVHD. Read More

    The role of Nailfold Capillary Dropout on Mortality in Systemic Sclerosis.
    Intern Med J 2018 Jan 24. Epub 2018 Jan 24.
    Immunology Department, Flinders Medical Centre and Flinders University, Bedford Park, South Australia, Australia.
    Aim: Semi-quantitative wide-field nailfold capillaroscopy (NFC) is a simple technique with proven utility in the early diagnosis of systemic sclerosis (SSc). Its role in prognosis, however, remains uncertain. This studyseeks to investigate the possible utility of NFC in predicting survival. Read More

    [Renal Involvement in Connective Tissue Diseases].
    Dtsch Med Wochenschr 2018 Jan 22;143(2):89-100. Epub 2018 Jan 22.
    Renal involvement is common and heterogenous in connective tissue diseases and has a main influence on prognosis and mortality. In systemic lupus erythematosus proliferative glomerulonephritis is the most common manifestation, while in primary Sjogren's syndrome interstitial nephritis with tubular dysfunction is the predominant pathological feature. In systemic sclerosis the most serious renal manifestation is scleroderma renal crisis characterized by abrupt onset of hypertension and acute kidney injury associated with an increase in plasma renin activity. Read More

    Clinical Characteristics and Associated Systemic Diseases in Patients With Esophageal "Absent Contractility"-A Clinical Algorithm.
    J Clin Gastroenterol 2018 Jan 19. Epub 2018 Jan 19.
    Digestive Disease Institute, Cleveland Clinic.
    Goals: This study was carried out to assess the clinical characteristics and associated systemic diseases seen in patients diagnosed with absent contractility as per the Chicago Classification version 3.0, allowing us to propose a diagnostic algorithm for their etiologic testing.

    Background: The Chicago Classification version 3. Read More

    Optical coherence tomography angiography of normal skin and inflammatory dermatologic conditions.
    Lasers Surg Med 2018 Jan 22. Epub 2018 Jan 22.
    Department of Bioengineering, University of Washington, 3720 15th Ave. N.E., Seattle, Washington, 98195.
    Background: In clinical dermatology, the identification of subsurface vascular and structural features known to be associated with numerous cutaneous pathologies remains challenging without the use of invasive diagnostic tools.

    Objective: To present an advanced optical coherence tomography angiography (OCTA) method to directly visualize capillary-level vascular and structural features within skin in vivo.

    Methods: An advanced OCTA system with a 1310 nm wavelength was used to image the microvascular and structural features of various skin conditions. Read More

    Right Ventricular Myofilament Functional Differences in Humans with Systemic Sclerosis-associated versus Idiopathic Pulmonary Arterial Hypertension.
    Circulation 2018 Jan 19. Epub 2018 Jan 19.
    Division of Cardiology, Johns Hopkins University School of Medicine, Baltimore, MD & Department of Biomedical Engineering, Johns Hopkins University School of Medicine, Baltimore, MD.
    -Patients with systemic sclerosis-associated pulmonary arterial hypertension (SSc-PAH) have a far worse prognosis than those with idiopathic PAH (IPAH). In the intact heart, SSc-PAH exhibits depressed rest and reserve right ventricular (RV) contractility as compared to IPAH. We tested whether this disparity involves underlying differences in myofilament function. Read More

    Matrix Stiffness: the Conductor of Organ Fibrosis.
    Curr Rheumatol Rep 2018 Jan 19;20(1). Epub 2018 Jan 19.
    Fibrosis Research Center, Massachusetts General Hospital, Boston, MA, USA.
    Purpose Of Review: Organ fibrosis is a lethal component of scleroderma. The hallmark of scleroderma fibrosis is extensive extracellular matrix (ECM) deposition by activated myofibroblasts, specialized hyper-contractile cells that promote ECM remodeling and matrix stiffening. The purpose of this review is to discuss novel mechanistic insight into myofibroblast activation in scleroderma. Read More

    Scleroderma renal crisis and cancer.
    QJM 2018 Jan 15. Epub 2018 Jan 15.
    Internal Medicine Department, Hôtel-Dieu, CHU Nantes, France.
    We report two cases of elderly patients with diffuse systemic sclerosis (SSc) and inaugural scleroderma renal crisis (SRC). Initial exhaustive search for an underlying cancer was negative, but aggressive metastatic neoplasia was diagnosed after 1 year of follow-up. We emphasize the role of 18-FDG PET-scan to detect neoplasia in the following months after inaugural SRC in elderly. Read More

    Preeclampsia and scleroderma: a prospective nationwide analysis.
    Acta Obstet Gynecol Scand 2018 Jan 18. Epub 2018 Jan 18.
    Clinical Research Division, Fred Hutchinson Cancer Research Center, Seattle, WA, USA.
    Introduction: In a preliminary case-control study, women with scleroderma more frequently reported having had hypertensive complications during pregnancy compared with healthy women.

    Material And Methods: To prospectively investigate this possible association, we conducted a nation-wide cohort analysis of a major hypertensive complication during pregnancy, namely preeclampsia, and later scleroderma. Analyses were based on Danish register-based birth and hospital contact data on preeclampsia and scleroderma. Read More

    Validation of the Social Appearance Anxiety Scale in Patients with Systemic Sclerosis: A Scleroderma Patient-centered Intervention Network Cohort Study.
    Arthritis Care Res (Hoboken) 2018 Jan 17. Epub 2018 Jan 17.
    SDSU/UC San Diego Joint Doctoral Program in Clinical Psychology, San Diego, California, United States.
    Objective: Systemic sclerosis (SSc) is an autoimmune disease that can cause disfiguring changes in appearance. This study examined the structural validity, internal consistency reliability, convergent validity, and measurement equivalence of the Social Appearance Anxiety Scale (SAAS) across SSc disease subtypes.

    Methods: Patients enrolled in the Scleroderma Patient-centered Intervention Network Cohort completed the SAAS and measures of appearance-related concerns and psychological distress. Read More

    The molecular basis for dysregulated activation of NKX2-5 in vascular remodelling of systemic sclerosis.
    Arthritis Rheumatol 2018 Jan 17. Epub 2018 Jan 17.
    Centre for Rheumatology and Connective Tissue Diseases, Division of Medicine, University College London, London, UK.
    Objective: NKX2-5 is a homeobox transcription factor required for the formation of the heart and vessels during development, with significant postnatal downregulation and reactivation in disease states characterised by vascular remodelling. In this study, we sought to investigate mechanisms that activate NKX2-5 expression in diseased vessels, such as scleroderma associated pulmonary hypertension (SSc-PH), and identify genetic variability that potentially underlies susceptibility to specific vascular complications.

    Methods: We explored NKX2-5 expression in biopsies of patients with SSc-PH and in pulmonary artery smooth muscle cells (PASMC) from scleroderma patients. Read More

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