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    The Childhood Arthritis & Rheumatology Research Alliance Consensus Treatment Plans: Towards Comparative Effectiveness in the Pediatric Rheumatic Diseases.
    Arthritis Rheumatol 2018 Jan 15. Epub 2018 Jan 15.
    Pediatric Rheumatology, Hackensack University Medical Center, Hackensack, United States.
    The pediatric rheumatic diseases are a heterogeneous group of rare diseases, posing a number of challenges for the use of traditional clinical and translational research approaches. Innovative comparative effectiveness approaches are needed to efficiently study treatment approaches and disease outcomes. The Childhood Arthritis and Rheumatology Research Alliance (CARRA) developed the consensus treatment plan (CTP) approach as a comparative effectiveness tool for research in pediatric rheumatology. Read More

    Do scleroderma patients look young?: Evaluation by using facial imaging system.
    Drug Discov Ther 2017 ;11(6):342-345
    Department of Dermatology and Plastic Surgery, Faculty of life Sciences, Kumamoto University.
    These days various collagen supplements have widely been marketed. However, it has not been scientifically proved whether increasing collagen can actually prevent skin aging. Systemic sclerosis (SSc) is an autoimmune disease that is characterized by thickening of the skin caused by accumulation of collagen. Read More

    Epigenetics and Pathogenesis of Systemic Sclerosis; the ins and outs.
    Hum Immunol 2018 Jan 9. Epub 2018 Jan 9.
    Rheumatology Research Center, Tehran University of Medical Sciences, Tehran, Iran. Electronic address:
    The pathogenesis of many diseases is influenced by environmental factors which can affect human genome and be inherited from generation to generation. Adverse environmental stimuli are recognized through the epigenetic regulatory complex, leading to gene expression alteration, which in turn culminates in disease outcomes. Three epigenetic regulatory mechanisms modulate the manifestation of a gene, namely DNA methylation, histone changes, and microRNAs. Read More

    The central vein sign differentiates MS from CNS inflammatory vasculopathies.
    Ann Neurol 2018 Jan 12. Epub 2018 Jan 12.
    Department of Neuroscience, Drug and Child Health, University of Florence, Italy.
    Objectives: In multiple sclerosis (MS), MRI is a sensitive tool for detecting white matter lesions, but its diagnostic specificity is still suboptimal; ambiguous cases are frequent in clinical practice. Detection of perivenular lesions in the brain (the "central vein sign") improves the pathological specificity of MS diagnosis, but comprehensive evaluation of this MRI biomarker in MS-mimicking inflammatory and/or autoimmune diseases, such as CNS inflammatory vasculopathies, is missing. In a multicenter study, we assessed the frequency of perivenular lesions in MS vs. Read More

    Regulation of Fn14 Receptor and NF-κB Underlies Inflammation in Meniere's Disease.
    Front Immunol 2017 13;8:1739. Epub 2017 Dec 13.
    Otology and Neurotology Group CTS495, Department of Genomic Medicine - Centre for Genomics and Oncological Research - Pfizer/Universidad de Granada/Junta de Andalucía (GENYO), Granada, Spain.
    Meniere's disease (MD) is a rare disorder characterized by episodic vertigo, sensorineural hearing loss, tinnitus, and aural fullness. It is associated with a fluid imbalance between the secretion of endolymph in the cochlear duct and its reabsorption into the subarachnoid space, leading to an accumulation of endolymph in the inner ear. Epidemiological evidence, including familial aggregation, indicates a genetic contribution and a consistent association with autoimmune diseases (AD). Read More

    Mycophenolate mofetil as a therapeutic agent for interstitial lung diseases in systemic sclerosis.
    Respir Investig 2018 Jan 6;56(1):14-20. Epub 2017 Dec 6.
    Uonuma Institute of Community Medicine, Niigata University Medical and Dental Hospital, Niigata, Japan. Electronic address:
    Systemic sclerosis (SSc) is an intractable disease that causes fibrosis in all organs. Approximately 40% of patients with SSc have some degree of interstitial lung disease (ILD). One third of patients with SSc and ILD, approximately 15% of all patients, have pulmonary lesions, which slowly progress to respiratory failure resistant to corticosteroid and other treatments. Read More

    Immunogenicity and safety of pneumococcal vaccination in patients with systemic sclerosis.
    Rheumatology (Oxford) 2018 Jan 8. Epub 2018 Jan 8.
    Department of Clinical Sciences, Lund, Section for Rheumatology, Lund University and Skåne University Hospital.
    Objective: To study the impact of disease and treatment with DMARDs on antibody response elicited by either pneumococcal conjugate vaccine (PCV13) or pneumococcal polysaccharide vaccine (PPV23) in patients with SSc.

    Methods: Forty-four SSc patients and 49 controls received a dose of either PCV13 or PPV23. Twelve patients were treated with DMARDs. Read More

    Stem cells therapy: the future in the management of systemic sclerosis? A case report.
    Hell J Nucl Med 2017 Sep-Dec;20 Suppl:164
    Clinic of Plastic, Hand Surgery and Burn Care Unit, University Hospital Schleswig-Holstein, Campus Lübeck, Ratzeburger Allee 160, 23538 Lübeck, Germany.
    Objective: Systemic sclerosis (SSc) is a connective tissue disorder of unknown etiology, with heterogeneous clinical manifestations and chronic and often progressive course. The diffuse cutaneous form of SSc (dcSSc) is characterized by thickening of the skin (scleroderma) and distinctive involvement of multiple internal organs. Patients with limited cutaneous SSc (lcSSc) generally have long-standing Raynaud's phenomenon before other manifestations of SSc appear. Read More

    Impaired resolution of DSS-induced colitis in mice lacking the glucocorticoid receptor in myeloid cells.
    PLoS One 2018 11;13(1):e0190846. Epub 2018 Jan 11.
    Institute for Cellular and Molecular Immunology, University Medical Center Goettingen, Göttingen, Germany.
    Inflammatory bowel disease (IBD) is a highly prevalent intestinal disorder for which no cure exists. Currently, the standard first-line treatment of IBD consists of systemic glucocorticoid (GC) application, even though therapy can be complicated by unresponsiveness or adverse effects. In view of the importance of macrophages and neutrophils for the pathogenesis of IBD we set out to define the relevance of these cell types as targets of GC using the mouse model of DSS-induced colitis. Read More

    Microvascular involvement in Systemic Sclerosis and Systemic Lupus Erythematosus.
    Microcirculation 2018 Jan 11. Epub 2018 Jan 11.
    Department of Pulmonary, Allergy and Critical Care Medicine, Respiratory Institute, Cleveland Clinic, Cleveland, Ohio, USA.
    Microvascular changes play central roles in the pathophysiology of systemic sclerosis (SSc) and systemic lupus erythematosus (SLE), and represent major causes of morbidity and mortality in these patients. Therefore, clinical tools that can assess the microvasculature are of great importance both at the time of diagnosis and follow up of these cases. These tools include capillaroscopy, laser imaging techniques, infrared thermography and iontophoresis. Read More

    Echocardiographic assessment of regional right ventricular systolic function using two-dimensional strain echocardiography and evaluation of the predictive ability of longitudinal 2D-strain imaging for pulmonary arterial hypertension in systemic sclerosis patients.
    Int J Cardiovasc Imaging 2018 Jan 10. Epub 2018 Jan 10.
    Department of Cardiology, Hacettepe University Faculty of Medicine, Ankara, Turkey.
    Systemic sclerosis (SSc) is a generalized connective tissue disorder, and SSc patients are at risk of developing pulmonary arterial hypertension (PAH). The aims of this study are to evaluate the right ventricular regional systolic function using two-dimensional speckle-tracking echocardiography (2D STE) and to determine the predictive ability of peak longitudinal systolic strain (PLSS) at the RV lateral wall for PAH in SSc patients. 80 SSc patients (mean age 51 ± 12 years) were included in the study. Read More

    Interstitial lung disease in systemic sclerosis: data from the spanish scleroderma study group.
    Rheumatol Int 2018 Jan 10. Epub 2018 Jan 10.
    Internal Medicine Department, Hospital VallD'Hebron, 08035, Barcelona, Spain.
    Objectives: To evaluate the clinical characteristics of patients with interstitial lung disease (ILD) in the setting of a large cohort of systemic sclerosis (SSc) patients, and to analyse the differences according to the SSc subtype (following the modification of classification criteria of the American College of Rheumatology for SSc proposed by LeRoy and Medsger), factors are associated with moderate-to-severe impairment of lung function, as well as mortality and causes of death.

    Methods: A descriptive study was performed, using the available data from the Spanish Scleroderma Study Group.

    Results: Twenty-one referral centers participated in the registry. Read More

    Plasmacytoid dendritic cells promote systemic sclerosis with a key role for TLR8.
    Sci Transl Med 2018 Jan;10(423)
    Autoimmunity and Inflammation Program, HSS Research Institute, Hospital for Special Surgery, New York, NY 10021, USA.
    Systemic sclerosis (SSc) is a multisystem life-threatening fibrosing disorder that lacks effective treatment. The link between the inflammation observed in organs such as the skin and profibrotic mechanisms is not well understood. The plasmacytoid dendritic cell (pDC) is a key cell type mediating Toll-like receptor (TLR)-induced inflammation in autoimmune disease patients, including lupus and skin diseases with interface dermatitis. Read More

    The role of klotho in systemic sclerosis.
    Reumatismo 2017 Dec 21;69(4):156-163. Epub 2017 Dec 21.
    Department of Rheumatology, Luigi Sacco University-Hospital, Milan.
    The aim was to evaluate the role of klotho in the pathogenesis of systemic sclerosis (SSc), through the measurement of its serum concentration in SSc patients compared to healthy controls, and to assess the association with cutaneous and visceral involvement. Blood samples obtained from both SSc patients and healthy controls were analysed by an ELISA assay for the detection of human klotho. SSc patients were globally evaluated for disease activity and assessed through the modified Rodnan's Skin Score, Medsger's scale, pulmonary function tests, 2D-echocardiography, nailfold capillaroscopy and laboratory tests. Read More

    Microvascular damage evaluation in systemic sclerosis: the role of nailfold videocapillaroscopy and laser techniques.
    Reumatismo 2017 Dec 21;69(4):147-155. Epub 2017 Dec 21.
    Research Laboratory and Academic Division of Clinical Rheumatology, Department of Internal Medicine, University of Genova, IRCCS San Martino-IST, Genova.
    Microvascular damage and a decrease in peripheral blood perfusion are typical features of systemic sclerosis (SSc) with serious clinical implications, not only for a very early diagnosis, but also for disease progression. Nailfold videocapillaroscopy is a validated and safe imaging technique able to detect peripheral capillary morphology, as well as to classify and to score any nailfold abnormalities into different microangiopathy patterns. Capillaroscopic analysis is now included in the ACR/EULAR classification criteria for SSc. Read More

    Disease staging and sub setting of interstitial lung disease associated with systemic sclerosis: impact on therapy.
    Expert Rev Clin Immunol 2018 Jan 10. Epub 2018 Jan 10.
    a Interstitial Lung Disease Unit , Royal Brompton Hospital , London SW3 6NP, UK.
    Introduction: Interstitial lung disease (ILD) is the most serious complication of systemic sclerosis (SSc). There is no accepted guidance as to which clinical, radiological or physiological thresholds should prompt initiation or changes in treatment. Furthermore, some patients with extensive disease remain stable without the need for intervention whilst others with limited disease at the outset, experience a precipitous decline. Read More

    ANCA-Positive pauci-immune crescentic glomerulonephritis in a patient with systemic lupus erythematosus.
    J Bras Nefrol 2017 Oct-Dec;39(4):454-457
    Laboratorios Integrados S.A, Lima, Perú.
    The pauci-immune crescentic glomerulonephritis (PICGN) is generally associated with small-vessel vasculitis with a few reported cases associated with other autoimmune diseases such as Systemic Lupus Erythematosus (SLE). We present the case of a female 34-year-old patient with acute kidney injury symptoms with indication for renal replacement therapy in the context of clinical SLE diagnosis. A kidney biopsy was conducted and it was found that most glomeruli showed some segmental sclerosis with synechia to the Bowman's capsule. Read More

    Increased Whole Blood Viscosity Is Associated with the Presence of Digital Ulcers in Systemic Sclerosis: Results from a Cross-Sectional Pilot Study.
    Autoimmune Dis 2017 29;2017:3529214. Epub 2017 Nov 29.
    Division of Rheumatology, University of Illinois at Chicago, Chicago, IL, USA.
    Objective: To investigate the role of whole blood viscosity in digital ulcer (DU) development in patients with diffuse and limited Systemic sclerosis.

    Methods: A convenience sample of patients with Systemic sclerosis (SSc) was selected from the adult Rheumatology clinic at the University of Chicago. The study group consisted of patients with SSc (with ulcers present, a history of ulcers, and no ulcers); the control group consisted of matched healthy Rheumatology clinic staff. Read More

    Anti-fibrotic effects of pirfenidone by interference with the hedgehog signalling pathway in patients with systemic sclerosis-associated interstitial lung disease.
    Int J Rheum Dis 2018 Jan 7. Epub 2018 Jan 7.
    Southern Medical University, Guangzhou, China.
    Aim: To determine whether pirfenidone attenuates lung fibrosis by interfering with the hedgehog (Hh) signalling pathway in patients with systemic sclerosis-associated interstitial lung disease (SSc-ILD).

    Methods: Twenty-five SSc-ILD patients (20 first visit, five who underwent pirfenidone treatment for 6 months) and 10 healthy controls were recruited. Lung tissues were obtained by open-chest surgery, and primary lung fibroblasts were isolated, cultured and stimulated with pirfenidone. Read More

    Chronicity index, especially glomerular sclerosis, is the most powerful predictor of renal response following immunosuppressive treatment in patients with lupus nephritis.
    Int J Rheum Dis 2018 Jan 5. Epub 2018 Jan 5.
    Departments of Rheumatology, Chonnam National University Medical School & Hospital, Gwangju, Korea.
    Aim: Renal responses to immunosuppressive agents in patients with lupus nephritis (LN) differ depending on ethnicity, follow-up duration, disease severity and treatment. Thus, we evaluated predictors of complete remission during the first year following immunosuppressive treatment in patients with LN.

    Methods: We retrospectively reviewed 79 patients who underwent kidney biopsy prior to the start of induction treatment and who were subsequently treated with immunosuppressive drugs for at least 6 months and followed-up for more than a year. Read More

    Erasmus syndrome associated with an immune thrombocytopenic purpura.
    J Eur Acad Dermatol Venereol 2018 Jan 5. Epub 2018 Jan 5.
    Department of Dermatology, University Hospital, Brest, France.
    We report the observation of a 57-year-old patient followed for an Erasmus syndrome (a systemic sclerosis developing secondary to silica exposure) (1) diagnosed in 2002 after being exposed as a dental technician, for whom a severe thrombocytopenia was discovered. He suffered from a pulmonary fibrosis, polyarthralgia, esophageal dysphagia and cutaneous involvement such as finger ulcerations and sclerodactylia. The treatment was made of nifedipine 30mg/day, esomeprazole 20mg/day and bosentan 25 mg twice a day. Read More

    Scleroderma in hospital settings in Lomé: 50 cases.
    Med Sante Trop 2017 Nov;27(4):446-448
    Service dermatologie du CHU Campus, Université de Lomé, BP 81056 Lomé, Togo.
    The aim of this study was to document the epidemiological and clinical profile, treatment used, and outcome of patients with scleroderma in hospital settings in Lomé. This descriptive study examined the records of all patients seen as outpatients or admitted for scleroderma in hospital dermatology and rheumatology departments in Lomé during the 20-year period of 1993-2012. During the study period, 50 (0. Read More

    Sarcoidosis and Systemic Sclerosis: Strange Bedfellows.
    Case Rep Rheumatol 2017 24;2017:7851652. Epub 2017 Aug 24.
    Division of Rheumatology, Department of Medicine, Loma Linda University, Loma Linda, CA, USA.
    Coexistence of systemic sclerosis and sarcoidosis is rare. Both have predominant lung manifestations, each with distinctive features on computed tomography (CT) of the chest. We present herein a 52-year-old male with limited systemic sclerosis manifested primarily by sclerodactyly and subsequently by shortness of breath. Read More

    The shared and specific mechanism of four autoimmune diseases.
    Oncotarget 2017 Dec 19;8(65):108355-108374. Epub 2017 Jul 19.
    College of Bioinformatics Science and Technology, Harbin Medical University, Harbin, China.
    Interaction between genetic and epigenetic mechanisms may lead to autoimmune diseases. The features of these diseases show familial aggregation. The generality and specificity are keys to studying pathogenesis and etiology of them. Read More

    Protein kinases G are essential downstream mediators of the antifibrotic effects of sGC stimulators.
    Ann Rheum Dis 2018 Jan 8. Epub 2018 Jan 8.
    Department of Internal Medicine III - Rheumatology and Immunology, Friedrich-Alexander-University Erlangen-Nürnberg (FAU), Universitätsklinikum Erlangen, Erlangen, Germany.
    Objectives: Stimulators of soluble guanylate cyclase (sGC) are currently investigated in clinical trials for the treatment of fibrosis in systemic sclerosis (SSc). In this study, we aim to investigate the role of protein kinases G (PKG) as downstream mediators of sGC-cyclic guanosine monophosphate (cGMP) in SSc.

    Methods: Mice with combined knockout of PKG1 and 2 were challenged with bleomycin and treated with the sGC stimulator BAY 41-2272. Read More

    Quantifying the direct public health care cost of systemic sclerosis: A comprehensive data linkage study.
    Medicine (Baltimore) 2017 Dec;96(48):e8503
    Department of Medicine, The University of Melbourne at St Vincent's Hospital (Melbourne).
    To quantify the direct healthcare cost of systemic sclerosis (SSc) and identify its determinants. Healthcare use was captured through data linkage, wherein clinical and medication data for SSc patients from the state of Victoria enrolled in the Australian Scleroderma Cohort Study were linked with the Victorian hospital admissions and emergency presentations data sets, and the Medicare Benefits Schedule which contains all government subsidized ambulatory care services, for the period 2011-2015. Medication cost was determined from the Pharmaceutical Benefits Scheme. Read More

    A Mini Review of Clinical and Research Applications of the Retinal Function Imager.
    Curr Eye Res 2018 Jan 8:1-16. Epub 2018 Jan 8.
    b Department of Ophthalmology, Bascom Palmer Eye Institute , University of Miami , Miami , FL , USA.
    Purpose: To review the clinical applications and diagnostic value of the retinal function imager (RFI), briefly compare RFI to other optical imaging devices, and to describe recent developments.

    Methods: The search words "Retinal Functional Imager," "optical imaging," "retina angiography," "avascular zone," "foveal avascular zone," and other closely related terms were used in PubMed to review current literature involving the RFI.

    Results: The functions of the RFI were utilized in over 44 microvascular studies, which reported that the microvasculature may alter in velocity, morphology, and oximetry when affected by a number of ocular, neurological, or systemic diseases. Read More

    Pathogenic roles of B lymphocytes in systemic sclerosis.
    Immunol Lett 2018 Jan 5. Epub 2018 Jan 5.
    Department of Dermatology, The University of Tokyo Graduate School of Medicine, 7-3-1 Hongo, Bunkyo-ku, 113-8655, Japan. Electronic address:
    Systemic sclerosis (SSc) is a collagen disease characterized by autoimmunity and excessive extracellular matrix deposition in the skin and visceral organs. Although the pathogenic relationship between systemic autoimmunity and the clinical manifestations of SSc remains unknown, SSc patients show a variety of abnormal immune activation including the production of disease-specific autoantibodies and cytokine production. Many recent studies have demonstrated that immune cells, including T cells, B cells, and macrophages, have a variety of immunological abnormalities in SSc. Read More

    An introduction to the microbiome and MS.
    Mult Scler 2018 Jan;24(1):53-57
    Department of Microbiology and Immunology, Microbiome and Disease Tolerance Centre, McGill University, Montreal, QC, Canada.
    The human microbiota is composed of diverse forms of microorganisms that live on or in us and plays a crucial role in the health and development. Commensal species that reside in the intestine particularly influence host physiology at local and systemic levels. Multiple sclerosis (MS) is a debilitating autoimmune disorder of the central nervous system for which there is currently no cure. Read More

    Screening for connective tissue disease-associated antibodies by automated immunoassay.
    Clin Chem Lab Med 2018 Jan 8. Epub 2018 Jan 8.
    Department of Microbiology and Immunology, KU Leuven, Leuven, Belgium.
    Background: Antinuclear antibodies (ANAs) are useful for the diagnosis of ANA-associated systemic rheumatic disease (AASRD). The objective of this study was the evaluation of an immunoassay that detects antibodies to a mixture of 17 antigens as an alternative to indirect immunofluorescence (IIF).

    Methods: Nine thousand eight hundred and fifty-six consecutive patients tested for ANAs were tested by IIF and EliA connective tissue disease screen (Thermo-Fisher). Read More

    Patterns and predictors of skin score change in early diffuse systemic sclerosis from the European Scleroderma Observational Study.
    Ann Rheum Dis 2018 Jan 6. Epub 2018 Jan 6.
    UCL Division of Medicine, Centre for Rheumatology and Connective Tissue Diseases, London, UK.
    Objectives: Our aim was to use the opportunity provided by the European Scleroderma Observational Study to (1) identify and describe those patients with early diffuse cutaneous systemic sclerosis (dcSSc) with progressive skin thickness, and (2) derive prediction models for progression over 12 months, to inform future randomised controlled trials (RCTs).

    Methods: The modified Rodnan skin score (mRSS) was recorded every 3 months in 326 patients. 'Progressors' were defined as those experiencing a 5-unit and 25% increase in mRSS score over 12 months (±3 months). Read More

    Vertebrobasilar Artery Stroke as the Heralding Sign of Systemic Lupus Erythematosus.
    J Stroke Cerebrovasc Dis 2018 Jan 3. Epub 2018 Jan 3.
    Department of Anesthesiology, SUNY-Buffalo, Buffalo, New York. Electronic address:
    Cerebral ischemia because of vertebrobasilar insufficiency (VBI) rarely presents as an initial sign within the systemic lupus erythematosus (SLE) population, and there are very few case reports supporting this manifestation. This report details 3 different patients with SLE who experienced VBI as an initial manifestation. Patient 1 was a 24-year-old female who developed a bilateral pontine lesion as a consequence of basilar artery stenosis. Read More

    Plasma urotensin II levels in primary Raynaud's phenomenon and systemic sclerosis
    Turk J Med Sci 2017 Dec 19;47(6):1687-1692. Epub 2017 Dec 19.
    Background/aim: The pathogenesis of Raynaud's phenomenon (RP) has not yet been fully elucidated. RP is characterized by exaggerated cold-induced vasoconstriction. Urotensin II (UII) is a potent vasoconstrictor. Read More

    Blue toe syndrome as a first sign of systemic sclerosis.
    BMJ Case Rep 2018 Jan 5;2018. Epub 2018 Jan 5.
    Department of Internal Medicine, Ikazia Hospital, Rotterdam, The Netherlands.
    We describe an unusual case of blue toe syndrome as the primary and solitary manifestation of systemic sclerosis. The possible cause was long-term occupational exposure in construction work. Blue toe syndrome is a small vessel disease, characterised by the sudden development of painful, blue discolouration in one or more toes. Read More

    Fatigue in systemic sclerosis: a systematic review.
    Clin Exp Rheumatol 2017 Dec 15. Epub 2017 Dec 15.
    Department of Clinical Medicine and Rheumatology, Campus Bio-Medico, University of Rome, Italy.
    Objectives: To systematically review fatigue in systemic sclerosis (SSc) in terms of prevalence, features, correlates, predictors and management.

    Methods: We performed a literature search in PubMed (Medline), EBSCO and COCHRANE databases up to June 2017 selecting articles regarding fatigue in SSc. The articles finally selected fulfilled the following eligibility criteria: written in English, referred to fatigue in SSc, reporting original data, including validated questionnaires measuring fatigue. Read More

    Antibodies to watch in 2018.
    MAbs 2018 Jan 4. Epub 2018 Jan 4.
    b The Antibody Society , 247 Prospect Street, Framingham , MA , USA.
    The pace of antibody therapeutics development accelerated in 2017, and this faster pace is projected to continue through 2018. Notably, the annual number of antibody therapeutics granted a first approval in either the European Union (EU) or United States (US) reached double-digits (total of 10) for the first time in 2017. The 10 antibodies granted approvals are: brodalumab, dupilumab, sarilumab, guselkumab, benralizumab, ocrelizumab, inotuzumab ozogamicin, avelumab, duvalumab, and emicizumab. Read More

    Myeloablative Autologous Stem-Cell Transplantation for Severe Scleroderma.
    N Engl J Med 2018 01;378(1):35-47
    From the Duke University Medical Center (K.M.S., O.C., E.W.S.C.) and RTI International (D.W.), Durham, and Rho Federal Systems Division, Chapel Hill (L.K.-E., A.P., B.E., S.C.) - all in North Carolina; National Institute of Allergy and Infectious Diseases, Bethesda, MD (E.A.G., B.W., L.M.G., J.S.G.); Colorado Blood Cancer Institute, Denver (P.A.M., R.A.N.); University of Texas McGovern Medical School (M.D.M.) and M.D. Anderson Cancer Center (C.H.) - both in Houston; Vanderbilt University, Nashville (L.J.C., K.P.); University of Michigan, Ann Arbor (D.K., J.R.S.); Case Western Reserve University and University Hospitals, Cleveland (R.J.F.); University of Alabama, Birmingham (S.M.); Boston University, Boston (R.W.S.); University of Virginia, Charlottesville (K.B.); University of Washington (M.H.W., D.E.F.) and the Fred Hutchinson Cancer Research Center (G.E.G., S.H.) - both in Seattle; University of California, Los Angeles, Los Angeles (J.G., S.K., D.E.F.); City of Hope National Medical Center, Duarte, CA (S.F.); Medical University of South Carolina, Charleston (R.M.S.); Mayo Clinic, Scottsdale, AZ (L.G.); University of Calgary, Calgary, AB, Canada (J.S., S.L.); Washington University, St. Louis (R.B.); Medical College of Wisconsin, Milwaukee (M.E.C., C.K.-T.); Ottawa Hospital Research Institute, Ottawa (C.B.); University of Pittsburgh, Pittsburgh (T.M., R.T.D.); and University of Toledo Medical Center, Toledo, OH (M.B.K.).
    Background: Despite current therapies, diffuse cutaneous systemic sclerosis (scleroderma) often has a devastating outcome. We compared myeloablative CD34+ selected autologous hematopoietic stem-cell transplantation with immunosuppression by means of 12 monthly infusions of cyclophosphamide in patients with scleroderma.

    Methods: We randomly assigned adults (18 to 69 years of age) with severe scleroderma to undergo myeloablative autologous stem-cell transplantation (36 participants) or to receive cyclophosphamide (39 participants). Read More

    Cyclophosphamide for connective tissue disease-associated interstitial lung disease.
    Cochrane Database Syst Rev 2018 Jan 3;1:CD010908. Epub 2018 Jan 3.
    Department of Allergy, Immunology and Respiratory Medicine, The Alfred Hospital, Commercial Rd, Melbourne, Australia, 3004.
    Background: Approximately one-third of individuals with interstitial lung disease (ILD) have associated connective tissue disease (CTD). The connective tissue disorders most commonly associated with ILD include scleroderma/systemic sclerosis (SSc), rheumatoid arthritis, polymyositis/dermatomyositis, and Sjögren's syndrome. Although many people with CTD-ILD do not develop progressive lung disease, a significant proportion do progress, leading to reduced physical function, decreased quality of life, and death. Read More

    Evolution, trends, outcomes, and economics of hematopoietic stem cell transplantation in severe autoimmune diseases.
    Blood Adv 2017 Dec 20;1(27):2742-2755. Epub 2017 Dec 20.
    Unité Clinique de Médecine Interne, Maladies Auto-immunes et Pathologie Vasculaire, Unité Fonctionnelle 04, Hôpital Saint-Louis, AP-HP, INSERM Unité Mixte de Recherche Scientifique 1160, Paris Denis Diderot University, Paris, France; and.
    Hematopoietic stem cell transplantation (HSCT) has evolved for >20 years as a specific treatment of patients with autoimmune disease (AD). Using European Society for Blood and Marrow Transplantation registry data, we summarized trends and identified factors influencing activity and outcomes in patients with AD undergoing first autologous HSCT (n = 1951; median age, 37 years [3-76]) and allogeneic HSCT (n = 105; median age, 12 years [<1-62]) in 247 centers in 40 countries from 1994 to 2015. Predominant countries of activity were Italy, Germany, Sweden, the United Kingdom, The Netherlands, Spain, France, and Australia. Read More

    Tuberous sclerosis presenting as neonatal cyanosis because of rhabdomyoma causing tricuspid valve obstruction needing a Blalock-Taussig shunt.
    J Saudi Heart Assoc 2018 Jan 21;30(1):66-68. Epub 2017 Apr 21.
    Prince Rashid Hospital, Pediatrics, Royal Medical Services, Irbid, Jordan.
    We report a newborn female baby who presented at 6 hours of age with cyanosis without any signs of respiratory distress. Cardiovascular and systemic examination was unremarkable apart from cyanosis (saturation 75%). An echocardiogram showed multiple echogenic and homogeneous masses in the interventricular septum, one of which was big and protruding through the tricuspid valve causing right ventricular inflow obstruction. Read More

    Multiple Immune-Inflammatory and Oxidative and Nitrosative Stress Pathways Explain the Frequent Presence of Depression in Multiple Sclerosis.
    Mol Neurobiol 2018 Jan 2. Epub 2018 Jan 2.
    Department of Medicine, Imperial College London, Hammersmith Hospital, London, UK.
    Patients with a diagnosis of multiple sclerosis (MS) or major depressive disorder (MDD) share a wide array of biological abnormalities which are increasingly considered to play a contributory role in the pathogenesis and pathophysiology of both illnesses. Shared abnormalities include peripheral inflammation, neuroinflammation, chronic oxidative and nitrosative stress, mitochondrial dysfunction, gut dysbiosis, increased intestinal barrier permeability with bacterial translocation into the systemic circulation, neuroendocrine abnormalities and microglial pathology. Patients with MS and MDD also display a wide range of neuroimaging abnormalities and patients with MS who display symptoms of depression present with different neuroimaging profiles compared with MS patients who are depression-free. Read More

    Presence of anti-eukaryotic initiation factor-2B, anti-RuvBL1/2 and anti-synthetase antibodies in patients with anti-nuclear antibody negative systemic sclerosis.
    Rheumatology (Oxford) 2017 Dec 22. Epub 2017 Dec 22.
    Royal National Hospital for Rheumatic Diseases, Royal United Hospitals NHS Foundation Trust.
    Objectives: Autoantibodies targeting ubiquitously expressed nuclear antigens can be identified in most patients with SSc. Cytoplasmic autoantibodies (in otherwise ANA-negative sera) targeting eukaryotic initiation factor-2B (anti-eIF2B) have recently been identified in SSc with clinical associations to dcSSc disease and interstitial lung disease (ILD), although the majority of samples originated from a tertiary SSc-ILD centre. We investigated the prevalence and clinical associations of recently described SSc-specific (including anti-eIF2B) and other cytoplasmic autoantibodies in ANA-negative sera obtained from a large representative SSc cohort. Read More

    Gene-level association analysis of systemic sclerosis: A comparison of African-Americans and White populations.
    PLoS One 2018 2;13(1):e0189498. Epub 2018 Jan 2.
    Department of Internal Medicine, Division of Rheumatology, University of Texas McGovern Medical School, Houston, TX, United States of America.
    Gene-level analysis of ImmunoChip or genome-wide association studies (GWAS) data has not been previously reported for systemic sclerosis (SSc, scleroderma). The objective of this study was to analyze genetic susceptibility loci in SSc at the gene level and to determine if the detected associations were shared in African-American and White populations, using data from ImmunoChip and GWAS genotyping studies. The White sample included 1833 cases and 3466 controls (956 cases and 2741 controls from the US and 877 cases and 725 controls from Spain) and the African American sample, 291 cases and 260 controls. Read More

    Resilience in women with autoimmune rheumatic diseases.
    Joint Bone Spine 2017 Dec 28. Epub 2017 Dec 28.
    Center for Autoimmune Diseases Research (CREA), School of Medicine and Health Sciences, Universidad del Rosario, Bogotá, Colombia. Electronic address:
    Objective: To evaluate the relationship between resilience and clinical outcomes in patients with autoimmune rheumatic diseases.

    Methods: Focus groups, individual interviews, and chart reviews were done to collect data on 188 women with autoimmune rheumatic diseases, namely rheumatoid arthritis (n=51), systemic lupus erythematosus (n=70), systemic sclerosis (n=35), and Sjögren's syndrome (n=32). Demographic, clinical, and laboratory variables were assessed including disease activity by patient reported outcomes. Read More

    A Review of Women's Neurology.
    Am J Med 2017 Dec 27. Epub 2017 Dec 27.
    Brigham and Women's Hospital, Department of Neurology. 60 Fenwood Road; Boston, MA 02115,. Electronic address:
    The concern about what is best practice when caring for women with neurologic disease is a common clinical scenario. Therefore, knowledge about women's health issues and their intersection with neurologic disorders is imperative. This review will discuss the appropriate gender based considerations in epilepsy, multiple sclerosis, migraine, autoimmune disease, sleep, stroke and paraneoplastic disorders. Read More

    Could conservative iron chelation lead to neuroprotection in amyotrophic lateral sclerosis?
    Antioxid Redox Signal 2017 Dec 29. Epub 2017 Dec 29.
    University of Lille Nord de France Faculty of Medicine, Medical Pharmacology , 1 place de Verdun , Lille, France , 59000 ;
    Iron accumulation has been observed in mouse models and both sporadic and familial forms of Amyotrophic lateral sclerosis. Iron chelation could reduce iron accumulation and the related excess of oxidative stress in the motor pathways. However, classical iron chelation would induce systemic iron depletion. Read More

    INVISIBLE, HONEYCOMB-LIKE, CAVITARY RETINAL ASTROCYTIC HAMARTOMA.
    Retin Cases Brief Rep 2017 Dec 28. Epub 2017 Dec 28.
    Ocular Oncology Service, Wills Eye Hospital, Thomas Jefferson University, Philadelphia, Pennsylvania.
    Purpose: To report a patient with clinically invisible retinal astrocytic hamartomas (RAHs) detected on optical coherence tomography (OCT) and studied with OCT angiography (OCTA).

    Method: Case report.

    Results: An 11-year-old healthy girl was evaluated for reduced visual acuity in the right eye. Read More

    Pulmonary arterial hypertension associated with connective tissue diseases: A review focusing on distinctive clinical aspects.
    Eur J Clin Invest 2017 Dec 15. Epub 2017 Dec 15.
    Department of Rheumatology, Endocrinology and Nephrology, Faculty of Medicine and Graduate School of Medicine, Hokkaido University, Sapporo, Japan.
    Recent studies have clarified that pulmonary arterial hypertension associated with connective tissue diseases (CTD-PAH) has some distinctive clinical aspects from other PAH, such as high prevalence, venous and cardiac involvement, less favourable outcome, helpfulness of detection algorithm, response to immunosuppression, pre-PAH conditions in borderline pulmonary arterial pressure and coexistence of interstitial lung disease. In this review, by focusing on these distinctive aspects, we discuss how to provide an efficacious and safe management of CTD-PAH and garner attention to areas where further evidence is desired. Read More

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