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    Fibrosing myopathy in systemic sclerosis associates with higher mortality.
    Arthritis Care Res (Hoboken) 2017 May 23. Epub 2017 May 23.
    Division of Rheumatology, Johns Hopkins University School of Medicine, Baltimore, Maryland, USA.
    Objective: To determine if a unique subtype of scleroderma muscle disease exists by comparing the clinical features of systemic sclerosis (SSc) patients with predominant fibrosis on muscle biopsy to those with inflammatory muscle histopathology.

    Methods: This retrospective, cross-sectional study included SSc patients with muscle weakness and an available muscle biopsy. Biopsies with fibrosis but without inflammation/necrosis were designated as "fibrosing myopathy" and those with inflammation and/or necrosis were assigned a category of "inflammatory myopathy". Read More

    Performance of Anti-nuclear Antibodies for Classifying Systemic Lupus Erythematosus: a Systematic Literature Review and Meta-regression of Diagnostic Data.
    Arthritis Care Res (Hoboken) 2017 May 23. Epub 2017 May 23.
    Rheumatology, Clinical Immunology and Allergy, University of Crete Medical School, Heraklion, Greece.
    Objective: To review the published literature on the performance of indirect immunofluorescence (IIF)-HEp-2 ANA testing for classification of SLE.

    Methods: A systematic literature search was conducted in the MEDLINE, EMBASE and COCHRANE databases for articles published between January 1990 and October 2015. The research question was structured according to PICO (Population, Intervention, Comparator, Outcome) format rules, and PRISMA recommendations were followed where appropriate. Read More

    Efficacy of mycophenolate mofetil and oral cyclophosphamide on skin thickness: Post-hoc analyses from the Scleroderma Lung Study I and II.
    Arthritis Care Res (Hoboken) 2017 May 23. Epub 2017 May 23.
    Division of Rheumatology, University of Michigan Scleroderma Program, Ann Arbor, MI, USA.
    Objectives: To assess the efficacy of mycophenolate mofetil (MMF) and cyclophosphamide (CYC) on the modified Rodnan skin score (mRSS) in participants enrolled in the Scleroderma Lung Study (SLS)-I and II.

    Methods: SLS-I participants received daily oral CYC or matching placebo for one year, whereas SLS-II participants received daily MMF for 2 years or daily oral CYC for 1 year followed by placebo for second year. We assessed the impact of MMF and CYC on the mRSS in SLS-II over 24-month period. Read More

    Thrombocytopenia Associated with Localized Scleroderma: Report of Four Pediatric Cases and Review of the Literature.
    Pediatr Dermatol 2017 May 23. Epub 2017 May 23.
    Department of Paediatrics, Advanced Pediatrics Centre, Chandigarh, India.
    We report on four children with localized scleroderma (morphea) and thrombocytopenia. All four had the en coup de sabre subtype of morphea and had varying degrees of thrombocytopenia (8 × 10(9) /L to 120 × 10(9) /L). None of them had major bleeding manifestations, and thrombocytopenia resolved with treatment of morphea. Read More

    Bullous morphoea: a retrospective study.
    Clin Exp Dermatol 2017 May 22. Epub 2017 May 22.
    Section of Dermatology, Mario Aresu Department of Medical Science, University of Cagliari, Cagliari, Italy.
    Bullous morphoea is a rare variant of localized scleroderma whose pathogenesis has been widely discussed. We retrospectively reviewed the records of all histopathologically confirmed cases of morphoea followed from 2005 to 2015 at the Dermatology Clinic and Pathology Institute of the University of Cagliari, Sardinia, Italy. Among 137 patients with morphoea, 2 cases of the bullous variant were identified, which were successfully treated with methotrexate. Read More

    Mycophenolate mofetil for scleroderma-related interstitial lung disease: A real world experience.
    PLoS One 2017 25;12(5):e0177107. Epub 2017 May 25.
    Division of Pulmonary and Critical Care Medicine, Department of Internal Medicine, Mayo Clinic, Rochester, Minnesota, United States of America.
    Background And Objective: Interstitial lung disease (ILD) remains the number one cause of mortality in scleroderma (SSc). Our goal was to determine the effectiveness of mycophenolate mofetil (MMF) in treating SSc-ILD in a retrospective study.

    Methods: A retrospective, computer-assisted search was performed to identify patients with SSc-ILD treated with MMF from 1997 through 2014. Read More

    FGF-23, Klotho and Vitamin D Levels in Scleroderma.
    Iran J Public Health 2017 Apr;46(4):530-536
    Dept. of Clinical Biochemistry and Laboratory Medicine, Tabriz University of Medical Sciences, Tabriz, Iran.
    Background: Scleroderma is a chronic connective tissue disease of unknown etiology. Vitamin D and parathyroid hormone (PTH) that play particular functions in calcium and phosphate homeostasis may be involved in the etiology of this disorder. Klotho, the co-receptor of the fibroblast growth factor 23 (FGF-23), can interfere with calcium and phosphate metabolism. Read More

    Coexistence of diabetes mellitus type 1 with diffuse systemic sclerosis - case report and literature review.
    Reumatologia 2017 28;55(2):104-107. Epub 2017 Apr 28.
    Department of Rheumatology and Connective Tissue Diseases, Medical University, Lublin, Poland.
    Diabetic sclerodactyly is a frequently recognized skin finding that may occur in patients with diabetes mellitus but coexistence of diabetes and systemic sclerosis is rare. We describe a case of coexistence of type 1 diabetes mellitus and systemic sclerosis in 42-year-old man with the history of Raynaud's phenomenon, progressive diffuse hardening of the skin and sclerodactyly, slowly worsening with time. The medical history included type 1 diabetes since childhood with microvascular complications. Read More

    Do you know this syndrome? Werner syndrome.
    An Bras Dermatol 2017 Mar-Apr;92(2):271-272
    School of Medicine, Selcuk University - Konya, Turkey.
    Werner syndrome is a rare autosomal recessive disorder, caused by mutations in the WRN gene. Clinical findings include: senile appearance, short stature, grey hair, alopecia, bird-like face, scleroderma-like skin changes, skin ulcers, voice abnormalities, cataracts, osteoporosis, type 2 diabetes mellitus, ischemic heart disease and hypogonadism. The syndrome begins to become apparent in adolescence but it is usually diagnosed in the third or fourth decade of life. Read More

    Serum prolidase activity in systemic sclerosis.
    Clin Rheumatol 2017 May 22. Epub 2017 May 22.
    Medical Faculty, Department of Medical Biochemistry, Sutcu Imam University, Kahramanmaras, Turkey.
    Systemic sclerosis, also known as scleroderma, is a complex systemic inflammatory autoimmune disease that targets the vasculature and connective tissue-producing cells and components of the innate and adaptive immune systems. The disease is characterized by a hardening of the skin and an increased synthesis of collagen . Prolidase is a specific imidodipeptidase involved in collagen degradation. Read More

    Echocardiographic Predictors for Worsening of Six-Minute Walk Distances in Patients With Systemic Sclerosis (Scleroderma).
    Am J Cardiol 2017 Apr 26. Epub 2017 Apr 26.
    Department of Cardiovascular Medicine, Tokushima University Hospital, Tokushima, Japan.
    Change in 6-minute walk distance (6MWD) has been used as a clinical marker in pulmonary hypertension. Determinants and worsening of 6MWD remain a matter of debate because nonpulmonary factors have an impact on the 6MWD. We hypothesized that future reduction of 6MWD in patients with systemic sclerosis (SSc) was more closely associated with cardiac dysfunction. Read More

    Sirt1 ameliorates systemic sclerosis by targeting the mTOR pathway.
    J Dermatol Sci 2017 May 3. Epub 2017 May 3.
    Division of Rheumatology, Huashan Hospital, Fudan University, China; Institute of Rheumatology, Immunology and Allergy, Fudan University, China. Electronic address:
    Background: Systemic sclerosis (SSc) is a chronic autoimmune disease characterized by inflammation and fibrosis. Our previous research has indicated that Sirtuin1 (Sirt1) plays a role in the regulation of TNF-α-induced inflammation; however, whether Sirt1 may inhibit the progress of SSc by blocking inflammation remains unknown.

    Objective: We aimed to investigate the function of Sirt1 in SSc. Read More

    Cardiopulmonary assessment of patients with systemic sclerosis for hematopoietic stem cell transplantation: recommendations from the European Society for Blood and Marrow Transplantation Autoimmune Diseases Working Party and collaborating partners.
    Bone Marrow Transplant 2017 May 22. Epub 2017 May 22.
    Department of Haematology, Sheffield Teaching Hospitals NHS Foundation Trust, University of Sheffield, Sheffield, UK.
    Systemic sclerosis (SSc) is a rare disabling autoimmune disease with a similar mortality to many cancers. Two randomized controlled trials of autologous hematopoietic stem cell transplantation (AHSCT) for SSc have shown significant improvement in organ function, quality of life and long-term survival compared to standard therapy. However, transplant-related mortality (TRM) ranged from 3-10% in patients undergoing HSCT. Read More

    Craniofacial linear scleroderma associated with retinal telangiectasia and exudative retinal detachment.
    J AAPOS 2017 May 18. Epub 2017 May 18.
    Manchester Royal Eye Hospital, Central Manchester University Hospitals NHS Foundation Trust, Manchester Academic Health Science Centre, Manchester, United Kingdom; Centre for Ophthalmology & Vision Sciences, Institute of Human Development, Faculty of Medical & Human Sciences, University of Manchester, Manchester. Electronic address:

    Unfolding the pathogenesis of scleroderma through genomics and epigenomics.
    J Autoimmun 2017 May 16. Epub 2017 May 16.
    Division of Rheumatology, Department of Internal Medicine, University of Michigan, Ann Arbor, MI, USA; Center for Computational Medicine and Bioinformatics, University of Michigan, Ann Arbor, MI, USA. Electronic address:
    With unknown etiology, scleroderma (SSc) is a multifaceted disease characterized by immune activation, vascular complications, and excessive fibrosis in internal organs. Genetic studies, including candidate gene association studies, genome-wide association studies, and whole-exome sequencing have supported the notion that while genetic susceptibility to SSc appears to be modest, SSc patients are genetically predisposed to this disease. The strongest genetic association for SSc lies within the MHC region, with loci in HLA-DRB1, HLA-DQB1, HLA-DPB1, and HLA-DOA1 being the most replicated. Read More

    Validity of the Workers Productivity and Activity Impairment Questionnaire: Specific Health Problem (WPAI:SHP) in patients with systemic sclerosis.
    Clin Exp Rheumatol 2017 May 8. Epub 2017 May 8.
    Department of Medicine, The University of Melbourne at St. Vincent's Hospital; and Department of Rheumatology, St. Vincent's Hospital, Melbourne, Australia.
    Objectives: To evaluate the construct validity of the Workers Productivity and Impairment Activity Index: Specific Health Problem (WPAI:SHP) in Australian systemic sclerosis (SSc) patients.

    Methods: SSc patients, identified through the Australian Scleroderma Cohort Study database, completed the WPAI:SHP and a quality of life instrument (PROMIS-29) cross-sectionally. The construct validity of the WPAI:SHP was assessed by the correlations between the WPAI:SHP and a range of SSc health states. Read More

    Standardization of the modified Rodnan skin score for use in clinical trials of systemic sclerosis.
    J Scleroderma Relat Disord 2017 Jan-Apr;2(1):11-18
    Centre for Rheumatology, Royal Free and University College London Medical School, London, UK.
    The modified Rodnan skin score (mRSS) is a measure of skin thickness and is used as a primary or secondary outcome measure in clinical trials of systemic sclerosis (scleroderma). This state-of-art review provides a historical perspective of the development of the mRSS, summarizes the performance of mRSS as an outcome measure, provides guidance on assessing mRSS, and makes recommendations for incorporation of the mRSS into clinical trials. Read More

    Circulating Anti-Nuclear Antibodies in Systemic Sclerosis: Utility in Diagnosis and Disease Subsetting.
    J Nippon Med Sch 2017 ;84(2):56-63
    Department of Allergy and Rheumatology, Nippon Medical School.
    The presence of circulating anti-nuclear antibodies (ANAs) is a hallmark of immune dysregulation in patients with systemic sclerosis (SSc). Currently, a variety of SSc-specific ANAs, including anticentromere, anti-topoisomerase I, and anti-RNA polymerase III antibodies, have been well characterized, and their commercial kits are available worldwide. Since these autoantibodies are specifically detected in SSc patients and are associated with unique sets of disease manifestations, they are widely used in routine clinical practice for diagnosis, clinical subgrouping, and prediction of future organ involvements and prognosis. Read More

    IL-10-producing regulatory B cells (B10 cells), IL-17+ T cells and autoantibodies in systemic sclerosis.
    Clin Immunol 2017 May 11. Epub 2017 May 11.
    Department of Rheumatology and Clinical Immunology, Faculty of Medicine, School of Health Sciences, University of Thessaly, Larissa 40500, Greece. Electronic address:
    We aimed to analyze IL-10+ Breg (B10) cells, found to be reduced in systemic sclerosis (SSc), in relation to SSc-specific autoAbs and IL-17+ and IFNγ+ T cells in SSc. Peripheral blood B10 cells from 26 patients with SSc positive for anti-Topo I or anti-Cen autoAbs, and 12 healthy controls (HC) were studied by flow cytometry. IL-17+ and IFNγ+ T cells were also studied. Read More

    Clinical characteristics and survival of systemic sclerosis patients with pulmonary hypertension and elevated wedge pressure: Observations from the PHAROS cohort.
    Respirology 2017 May 12. Epub 2017 May 12.
    Division of Rheumatology, Georgetown University, Washington, DC, USA.
    Background And Objective: Systemic sclerosis (SSc) is a complex autoimmune disease commonly associated with pulmonary hypertension (PH). When associated with elevated pulmonary artery wedge pressure (PAWP), pulmonary artery pressure (PAP) is either in-proportion (post-capillary PH) or higher than expected (combined PH) relative to the increased PAWP.

    Methods: Patients from the PHAROS registry (a prospective observational cohort of SSc-PH patients) who had mean PAP ≥ 25 and PAWP > 15 on right heart catheterization were stratified based on diastolic pressure gradient (DPG). Read More

    Collagenous Gastritis in a Young Female With IgA Deficiency.
    Gastroenterology Res 2017 Apr 19;10(2):126-127. Epub 2017 Apr 19.
    UHS Wilson Medical Center, Binghamton, NY, USA.
    Collagenous gastritis, without colonic involvement, is exceptionally rare. It is not known to be associated with IgA deficiency and scleroderma. This is the first report of this type of association. Read More

    Systemic sclerosis associated with colliquative necrosis in the cerebellum.
    Surg Neurol Int 2017 5;8:44. Epub 2017 Apr 5.
    Department of Life, Health & Environmental Sciences (MESVA), University of L'Aquila, Italy.
    Background: The scleroderma is a complex autoimmune collagen disorder that can affect many organs simultaneously, as it occurs in the systemic sclerosis (SS), or only the skin, as it occurs in the localized scleroderma (LS). The neurological presentation is extremely uncommon, and even more uncommon are the symptoms of the scleroderma in the cerebellum.

    Case Description: We report the case of a 56-year-old male with cerebellar lesions mimicking a brain abscess. Read More

    Premature Ovarian Failure - An Unusual Manifestation of Systemic Sclerosis.
    J Hum Reprod Sci 2017 Jan-Mar;10(1):58-60
    Department of Medicine, Lady Hardinge Medical College and SSK Hospital, New Delhi, Delhi, India.
    A 31-year-old woman presented with secondary amenorrhoea and inability to conceive, which was of 3 years duration. She had Raynaud's phenomenon for 16 years, primary hypothyroidism for 5 years, digital ulcers for 4 years and skin tightening for 2 years. She had an expressionless face, with loss of wrinkles and restriction of the mouth opening along with flexion contractures of the hands and the terminal digit resorptions. Read More

    Exercise habits and factors associated with exercise in systemic sclerosis: a Scleroderma Patient-centered Intervention Network (SPIN) cohort study.
    Disabil Rehabil 2017 May 8:1-7. Epub 2017 May 8.
    a Lady Davis Institute for Medical Research , Jewish General Hospital , Montréal , Québec , Canada.
    Objective: Exercise is associated with improved health in many medical conditions. Little is known about the exercise habits of people with systemic sclerosis (SSc, or scleroderma). This study assessed the proportion of individuals with SSc who exercise and associations of demographic and disease variables with exercise. Read More

    Comprehensive metabolic and morphologic disease characterization in systemic sclerosis: initial results using combined positron emission tomography and magnetic resonance imaging.
    Q J Nucl Med Mol Imaging 2017 May 3. Epub 2017 May 3.
    Eberhard Karls University, Department of Radiology, Diagnostic and Interventional Radiology, Tuebingen, Germany.
    Background: To evaluate the role of metabolic and morphologic parameters derived from simultaneous hybrid PET/MRI in correlation to clinical criteria for an image-based characterization of musculoskeletal, esophagus and lymph node involvement in systemic sclerosis (SSc).

    Methods: Between November 2013 and May 2015, simultaneous whole-body hybrid PET/MRI was performed in 13 prospectively recruited patients with SSc. A mean dose of 241. Read More

    Histopathologic Spectrum of Connective Tissue Diseases Commonly Affecting the Skin.
    Surg Pathol Clin 2017 Jun 14;10(2):477-503. Epub 2017 Mar 14.
    Department of Pathology, Brigham and Women's Hospital, Harvard Medical School, Amory-3, 75 Francis Street, Boston, MA 02115, USA.
    Connective tissue disorders (CTDs), also known as collagen vascular diseases, are a heterogeneous group of diseases with a common pathogenic mechanism: autoimmunity. Precise classification of CTDs requires clinical, serologic, and pathologic correlation and may be difficult because of overlapping clinical and histologic features. The main contribution of histopathology in the diagnosis of these disorders is to confirm, rule out, or alert clinicians to the possibility of CTD as a disease category, rather than producing definitive diagnoses of specific entities. Read More

    Distinctive metabolomic fingerprint in scleroderma patients with pulmonary arterial hypertension.
    Int J Cardiol 2017 Apr 19. Epub 2017 Apr 19.
    Department of Medical Sciences and Public Health, University of Cagliari, 09042 Monserrato, Cagliari, Italy. Electronic address:
    Background: Pulmonary arterial hypertension (PAH) in systemic sclerosis (SS) identifies a poor prognosis subset of patients. Recent studies suggested a "metabolic theory" on the development of pulmonary arterial hypertension. On this basis we performed a metabolomic study in order to evaluate whether differences in pulmonary arterial blood metabolites were identifiable in SS patients with increased pulmonary vascular resistance (PVR). Read More

    Annular Lichenoid Dermatitis (of Youth): Report of a Case With Lichen Planus-Like Features.
    Am J Dermatopathol 2017 Apr 25. Epub 2017 Apr 25.
    Department of Anatomic Pathology, Azienda Ospedaliero-Universitaria Policlinico, Modena, Italy.
    Annular lichenoid dermatitis of youth (ALDY), a dermatosis with peculiar clinical and pathological features, represents still a debated entity, given its similarity, among others, with mycosis fungoides. A case of ALDY in a 50-year-old male patient is reported. Clinically, the patient presented an oval scleroderma-like plaque on the right flank. Read More

    Nodular Sclerodermatous Chronic Cutaneous Graft-Versus-Host Disease (GvHD): A New Clinicopathological Variant of Cutaneous Sclerodermatous GvHD Resembling Nodular/Keloidal Scleroderma.
    Am J Dermatopathol 2017 Apr 25. Epub 2017 Apr 25.
    *Department of Dermatology, Hospital Clínico Universitario Lozano Blesa, Zaragoza, Spain;†Dermatopathology Research Unit, Department of Dermatology, Medical University of Graz, Graz, Austria; and‡School of Pathology, University of Milan, Fondazione IRCCS Ca' Granda-Ospedale Maggiore Policlinico, Milan, Italy.
    Cutaneous chronic graft-versus-host disease (GvHD) has a broad spectrum of clinicopathological presentations, the most common ones being poikiloderma, lichen planus-like eruptions, lichen sclerosus-like lesions, morphea-like plaques, and deep sclerosis. New forms of chronic cutaneous GvHD with different clinicopathological characteristics have been described, most of them mimicking cutaneous manifestations of autoimmune diseases. We report the case of a 35-year-old man who underwent allogenic stem cell transplantation for a therapy-associated acute myeloid leukemia and developed an acute GvHD with involvement of skin and gastrointestinal tract. Read More

    Risk of hemorrhagic transformation after ischemic stroke in patients with antiphospholipid antibody syndrome.
    Neurol Res 2017 Jun 5;39(6):477-483. Epub 2017 May 5.
    d Department of Neurology , McGovern Medical School, University of Texas Health Science Center , Houston , TX , USA.
    Background And Purpose: Several rheumatologic conditions including systemic lupus erythematosus, antiphospholipid antibody (APS) syndrome, rheumatoid arthritis, and scleroderma are known risk factors for stroke. The risk of hemorrhagic transformation after an acute ischemic stroke (AIS) in these patients is not known.

    Methods: We queried the Nationwide Inpatient Sample (NIS) data between 2010 and 2012 with ICD 9 diagnostic codes for AIS. Read More

    Could Lymphocyte Profiling be Useful to Diagnose Systemic Autoimmune Diseases?
    Clin Rev Allergy Immunol 2017 May 4. Epub 2017 May 4.
    Service de Rhumatologie, Hôpital de la Cavale Blanche, CHRU Brest, BP 824, 29609, Brest cedex, France.
    Considering the implications of B, T, and natural killer (NK) cells in the pathophysiology of systemic autoimmune diseases, the assessment of their distribution in the blood could be helpful for physicians in the complex process of determining a precise diagnosis. In primary Sjögren's syndrome, transitional and active naive B cells are increased and memory B cells are decreased compared to healthy controls and other systemic diseases. However, their utility to improve the accuracy of classification criteria has not been proven. Read More

    Shell extracts of the edible mussel and oyster induce an enhancement of the catabolic pathway of human skin fibroblasts, in vitro.
    Cytotechnology 2017 May 4. Epub 2017 May 4.
    Normandie Université, CS 14032, Caen, 14032, France.
    Mollusc shells are composed of more than 95% calcium carbonate and less than 5% organic matrix consisting mostly of proteins, glycoproteins and polysaccharides. In this study, we investigated the effects of matrix macromolecular components extracted from the shells of two edible molluscs of economic interest, i.e. Read More

    Scleroderma Mimickers.
    Curr Treatm Opt Rheumatol 2016 Mar 5;2(1):69-84. Epub 2016 Feb 5.
    Division of Rheumatology, Johns Hopkins University School of Medicine, Baltimore, MD, USA.
    Cutaneous fibrosing disorders encompass a diverse array of diseases united by the presence of varying degrees of dermal sclerosis. The quality and distribution of skin involvement, presence or absence of systemic complications and unique associated laboratory abnormalities often help to distinguish between these diseases. It is imperative that an effort is made to accurately differentiate between scleroderma and its mimics, in order to guide long-term management and facilitate implementation of the appropriate treatment modality where indicated. Read More

    Photopheresis: Advances and Use in Systemic Sclerosis.
    Curr Rheumatol Rep 2017 Jun;19(6):31
    Department of Dermatology, Northwestern University Feinberg School of Medicine, Chicago, IL, USA.
    Purpose Of Review: Extracorporeal photochemotherapy (photopheresis, ECP) is a cell-based immunomodulatory treatment that separates leukocytes from peripheral blood, exposes them to a photosensitizing agent followed by ultraviolet A light, and then reinfuses them back into the patient. ECP has been found to be effective for graft versus host disease, transplant rejection, and various autoimmune diseases. The mechanism is not well understood but studies have shown clinical benefit in the treatment of systemic sclerosis (SSc). Read More

    Women with silicone breast implants and autoimmune inflammatory syndrome induced by adjuvants: description of three patients and a critical review of the literature.
    Rheumatol Int 2017 May 2. Epub 2017 May 2.
    Institute of Rheumatology, School of Medicine, University of Belgrade, Resavska 69, 11000, Belgrade, Serbia.
    Silicone has been widely used in the manufacture of medical implants. It is well tolerated in most cases. However, in this paper we report the cases of three women who developed autoimmune/inflammatory syndrome induced by adjuvants (ASIA syndrome), namely with silicone breast implants. Read More

    Quantitative Assessment of Skin Stiffness in Localized Scleroderma Using Ultrasound Shear-Wave Elastography.
    Ultrasound Med Biol 2017 Jul 28;43(7):1339-1347. Epub 2017 Apr 28.
    Department of Ultrasound, West China Hospital of Sichuan University, Chengdu, China. Electronic address:
    The purpose of this study was to evaluate the usefulness of ultrasound shear-wave elastography (US-SWE) in characterization of localized scleroderma (LS), as well as in the disease staging. A total of 21 patients with 37 LS lesions were enrolled in this study. The pathologic stage (edema, sclerosis or atrophy) of the lesions was characterized by pathologic examination. Read More

    Pulmonary Artery Dimensions as a Prognosticator of Transplant-Free Survival in Scleroderma Interstitial Lung Disease.
    Lung 2017 Apr 29. Epub 2017 Apr 29.
    Department of Pulmonary and Critical Care Medicine, Cleveand Clinic Florida, 2950 Cleveland Clinic Boulevard, Weston, FL, 33331, USA.
    Background: Systemic sclerosis is a chronic debilitating autoimmune disease characterized by endothelial dysfunction and multi-organ fibrosis. Interstitial lung disease, a common manifestation of SSc, is termed scleroderma-related interstitial lung disease (SSc-ILD) and along with pulmonary hypertension contributes to a majority of deaths in SSc. SSc-ILD patients frequently develop pulmonary hypertension, which prognosticates a poorer outcome. Read More

    Clinical algorithms for the diagnosis and prognosis of interstitial lung disease in systemic sclerosis.
    Semin Arthritis Rheum 2017 Apr 1. Epub 2017 Apr 1.
    Department of Internal Medicine, Federal University of Rio Grande do Sul (UFRGS), Porto Alegre, Brazil; Division of Rheumatology, Hospital de Clínicas de Porto Alegre, Porto Alegre, Brazil.
    Introduction: Interstitial lung disease (ILD) is currently the primary cause of death in systemic sclerosis (SSc). Thoracic high-resolution computed tomography (HRCT) is considered the gold standard for diagnosis. Recent studies have proposed several clinical algorithms to predict the diagnosis and prognosis of SSc-ILD. Read More

    Detection of early endothelial damage in patients with Raynaud's phenomenon.
    Microvasc Res 2017 Apr 25;113:22-28. Epub 2017 Apr 25.
    Medicina Interna, Dipartimento di Fisiopatologia Medico-Chirurgica e dei Trapianti, Università degli Studi di Milano, Ospedale Maggiore Policlinico, Fondazione IRCCS Ca' Granda, Milan, Italy. Electronic address:
    Objectives: Raynaud's phenomenon (RP) can be the first manifestation of systemic sclerosis (SSc) or other connective tissue diseases (CTDs), often preceding an overt disease by years. It is not known if markers of endothelial damage are detectable in those RP patients who subsequently develop a CTD.

    Methods: We studied 82 RP patients at their first evaluation to correlate the levels of endothelial markers with the subsequent development of an overt disease 36months later. Read More

    Transcriptional and cytokine profiles identify CXCL9 as a biomarker of disease activity in morphea.
    J Invest Dermatol 2017 Apr 24. Epub 2017 Apr 24.
    University of Texas Southwestern Medical Center, Department of Dermatology, Dallas, TX. Electronic address:
    Interferon-related pathways have not been studied in morphea and biomarkers are needed. We sought to characterize morphea serum cytokine imbalance and interferon-related gene expression in blood and skin to address this gap by performing a case-control study of 87 participants with morphea and 26 healthy controls. We used multiplexed immunoassays to determine serum cytokine concentrations, performed transcriptional profiling of whole blood and lesional morphea skin, and employed double-staining immunohistochemistry to determine the cutaneous cellular source of CXCL9. Read More

    Diffuse parenchymal lung disease.
    Eur Respir Rev 2017 Jun 26;26(144). Epub 2017 Apr 26.
    Dept of Diseases of the Thorax, GB Morgagni Hospital, Forlì, Italy.
    Between September 2015 and August 2016 there were >1500 publications in the field of diffuse parenchymal lung diseases (DPLDs). For the Clinical Year in Review session at the European Respiratory Society Congress that was held in London, UK, in September 2016, we selected only five articles. This selection, made from the enormous number of published papers, does not include all the relevant studies that will significantly impact our knowledge in the field of DPLDs in the near future. Read More

    The Gastrointestinal dysmotility and infections in Systemic Sclerosis- A real world scenario.
    Curr Rheumatol Rev 2017 Apr 25. Epub 2017 Apr 25.
    Postgraduate Institute of Medical Education and Research, Chandigarh. India.
    Introduction: Systemic Sclerosis is known to involve the gastrointestinal system and can lead to multitude of problems predominantly affecting the GI motility.

    Methods: It was a prospective, observational, single centre study of fifty consecutive patients with SSc who presented to rheumatology clinic. Gut score was assessed using UCLA SCTC GIT 2. Read More

    Anterior ST-elevation myocardial infarction induced by rituximab infusion: A case report and review of the literature.
    J Clin Pharm Ther 2017 Jun;42(3):356-362
    Department of Medicine "B", Sheba Medical Center, Tel-Hashomer, Israel.
    What Is Known And Objectives: Rituximab is a chimeric monoclonal anti-CD20 antibody approved for the treatment of some lymphoid malignancies as well as for autoimmune diseases including rheumatoid arthritis (RA), idiopathic thrombocytopenic purpura (ITP) and vasculitis. Generally, rituximab is well tolerated; nevertheless, some patients develop adverse effects including infusion reactions. Albeit rare, these reactions may in some cases be life-threatening conditions. Read More

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