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    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

    Increased proportions of functionally impaired regulatory T cell subsets in systemic sclerosis.
    Clin Immunol 2017 May 15. Epub 2017 May 15.
    Department of Immunology and Biotechnology, University of Pécs, Clinical Center, Szigeti út 12, 7624 Pécs, Hungary. Electronic address:
    Treg abnormalities have been implicated in the pathogenesis of systemic sclerosis (SSc). Treg subpopulations and their cytokines, IL-10 and TGF-β in the peripheral blood of early stage SSc patients were investigated. We hypothesized that epigenetically regulated methylation of the FOXP3 promoter and enhancer regions are altered in Tregs of SSc patients, which might be involved in the T cell imbalance. 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

    Different aggregation states of a nuclear localization signal-tagged 25-kDa C-terminal fragment of TAR RNA/DNA-binding protein 43 kDa.
    Genes Cells 2017 May 12. Epub 2017 May 12.
    Laboratory of Molecular Cell Dynamics, Faculty of Advanced Life Science, Hokkaido University, Sapporo, Japan.
    The mechanism and cause of motor neuronal cell death in amyotrophic lateral sclerosis (ALS), a devastating neurodegenerative disorder, are unknown; gain of function of oligomers and aggregation of misfolded proteins, including carboxyl-terminal fragments (CTFs) of TAR RNA/DNA-binding protein 43 kDa (TDP-43), have been proposed as important causative factors in the onset of ALS. We recently reported that a nuclear localization signal (NLS)-tagged 25-kDa CTF of TDP-43 (TDP25) could decrease the cell-death proportion compared with that promoted by TDP25. Here, we show oligomeric states of NLS-TDP25 and its detailed localization property using super-resolution fluorescence microscopy, FRET, fluorescence recovery after photobleaching, and fluorescence correlation spectroscopy analysis. Read More

    Disturbed microcirculation in the hands of patients with systemic sclerosis detected by fluorescence optical imaging: a pilot study.
    Arthritis Res Ther 2017 May 8;19(1):87. Epub 2017 May 8.
    Department of Rheumatology and Clinical Immunology, Charité University Hospital, Berlin, Germany.
    Background: Utilising fluorescence optical imaging (FOI), the distribution of an intravenously applied colouring agent indocyanine green (ICG) can be analysed with the potential to identify malperfusion by little to no tissue enhancement. Systemic sclerosis (SSc) is characterised by the presence of digital ulcers reflecting progressive vasculopathy. The objective was to investigate the potential of FOI in the detection of disturbed microcirculation in the hands and fingers of patients with SSc and to link FOI findings to clinical signs of ischemia such as digital ulcers and pitting scars. 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

    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

    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

    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

    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

    Thin-section CT findings of thoracolithiasis.
    Jpn J Radiol 2017 Apr 27. Epub 2017 Apr 27.
    Division of Diagnostic Radiology, Department of Diagnostic and Therapeutic Radiology, Faculty of Medicine, Ramathibodi Hospital, Mahidol University, 270 Rama VI Road, Ratchathewi, Bangkok, 10400, Thailand.
    Purpose: To review thin-section CT findings of thoracolithiasis.

    Materials And Methods: Thirty-three thin-section CT scans of 9 patients with thoracolithiasis diagnosed between 2008 and 2016 were reviewed for the location, shape, longest diameter, and calcification of each freely mobile nodule (thoracolith) and for the presence of coexisting abnormalities.

    Results: The mean age of 9 patients (5 women) was 65. 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

    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

    Performance of the Patient-Reported Outcomes Measurement Information System-29 in scleroderma: a Scleroderma Patient-centered Intervention Network Cohort Study.
    Rheumatology (Oxford) 2017 Apr 18. Epub 2017 Apr 18.
    Department of Medicine, McGill UniversityMontréal, Québec, Canada.
    Objective.: The Patient-Reported Outcomes Measurement Information System (PROMIS)-29 assesses seven health-related quality of life domains plus pain intensity. The objective was to examine PROMIS-29v2 validity and explore clinical associations in patients with SSc. Read More

    Gray matter and white matter changes in non-demented amyotrophic lateral sclerosis patients with or without cognitive impairment: A combined voxel-based morphometry and tract-based spatial statistics whole-brain analysis.
    Brain Imaging Behav 2017 Apr 19. Epub 2017 Apr 19.
    First Department of Neurology, Aeginition Hospital, Medical School, National & Kapodistrian University, Athens, Greece.
    The phenotypic heterogeneity in amyotrophic lateral sclerosis (ALS) implies that patients show structural changes within but also beyond the motor cortex and corticospinal tract and furthermore outside the frontal lobes, even if frank dementia is not detected. The aim of the present study was to investigate both gray matter (GM) and white matter (WM) changes in non-demented amyotrophic lateral sclerosis (ALS) patients with or without cognitive impairment (ALS-motor and ALS-plus, respectively). Nineteen ALS-motor, 31 ALS-plus and 25 healthy controls (HC) underwent 3D-T1-weighted and 30-directional diffusion-weighted imaging on a 3 T MRI scanner. Read More

    Unifying mechanism for different fibrotic diseases.
    Proc Natl Acad Sci U S A 2017 May 19;114(18):4757-4762. Epub 2017 Apr 19.
    Institute for Stem Cell Biology and Regenerative Medicine, Stanford University School of Medicine, Stanford, CA 94305;
    Fibrotic diseases are not well-understood. They represent a number of different diseases that are characterized by the development of severe organ fibrosis without any obvious cause, such as the devastating diseases idiopathic pulmonary fibrosis (IPF) and scleroderma. These diseases have a poor prognosis comparable with endstage cancer and are uncurable. Read More

    Deficient Adipogenesis of Scleroderma Patient and Healthy African American Monocytes.
    Front Pharmacol 2017 3;8:174. Epub 2017 Apr 3.
    Division of Rheumatology and Immunology, Department of Medicine, Medical University of South CarolinaCharleston, SC, USA.
    Monocytes from systemic sclerosis (SSc, scleroderma) patients and healthy African Americans (AA) are deficient in the regulatory protein caveolin-1 leading to enhanced migration toward chemokines and fibrogenic differentiation. While dermal fibrosis is the hallmark of SSc, loss of subcutaneous adipose tissue is a lesser-known feature. To better understand the etiology of SSc and the predisposition of AA to SSc, we studied the adipogenic potential of SSc and healthy AA monocytes. Read More

    Primary Sjögren's syndrome with diffuse cystic lung changes developed systemic lupus erythematosus: a case report and literature review.
    Oncotarget 2017 Mar 8. Epub 2017 Mar 8.
    Department of Respiratory Medicine, Qilu Hospital of Shandong University, Jinan, China.
    Sjögren's syndrome (SS) is a chronic inflammatory autoimmune disease that can occur as a unique existence (primary Sjögren's syndrome) or merge with other systemic diseases like systemic lupus erythematosus (SLE), rheumatoid arthritis or systemic sclerosis (secondary Sjögren's syndrome). Data on the two diseases occurrence order are inadequate. Primary Sjögren's syndrome (pSS) may relatively uncommonly lead to diffuse cystic lung changes. Read More

    Systemic sclerosis.
    Lancet 2017 Apr 13. Epub 2017 Apr 13.
    University of Michigan, Ann Arbor, MI, USA.
    Systemic sclerosis, also called scleroderma, is an immune-mediated rheumatic disease that is characterised by fibrosis of the skin and internal organs and vasculopathy. Although systemic sclerosis is uncommon, it has a high morbidity and mortality. Improved understanding of systemic sclerosis has allowed better management of the disease, including improved classification and more systematic assessment and follow-up. Read More

    Structural Validity of the Rheumatology Attitudes Index in Systemic Sclerosis: Analysis from the UCLA Scleroderma Quality of Life Study.
    J Rheumatol 2017 Apr 15. Epub 2017 Apr 15.
    From the San Diego State University (SDSU)/University of California (UC) San Diego Joint Doctoral Program in Clinical Psychology, Department of Psychology, San Diego; Department of Psychology, California State University, Dominguez Hills; SDSU, Department of Psychology, San Diego; David Geffen School of Medicine, University of California, Los Angeles (UCLA) School of Medicine, Los Angeles, California; University of Michigan Health System, Ann Arbor, Michigan, USA. Supported by the grant, Evaluation of Health-Related Quality of Life in Systemic Sclerosis from the Scleroderma Foundation Inc. Dr. Khanna has been funded by the US National Institutes of Health (NIH)/US National Institute of Arthritis and Musculoskeletal and Skin Diseases) grants K24 AR063120 and K23 AR053858. Dr. Khanna has served as a consultant for Bayer and Genentech. S. Gholizadeh, Doctoral Student, MS, MSc, MPH, SDSU/UC San Diego Joint Doctoral Program in Clinical Psychology, Department of Psychology; S.D. Mills, Doctoral Student, MS, MPH, SDSU/UC San Diego Joint Doctoral Program in Clinical Psychology, Department of Psychology; R.S. Fox, Postdoctoral Fellow, PhD, MPH, SDSU/UC San Diego Joint Doctoral Program in Clinical Psychology, Department of Psychology; E.L. Merz, Assistant Professor, PhD, MPH, California State University, Dominguez Hills, Department of Psychology; S.C. Roesch, Professor, PhD, SDSU, Department of Psychology; P.J. Clements, MD, Professor Emeritus, David Geffen School of Medicine, UCLA; S. Kafaja, MD, Clinical Instructor, David Geffen School of Medicine, UCLA; D.E. Furst, MD, Professor, David Geffen School of Medicine, UCLA; D. Khanna, MD, Professor, University of Michigan Health System; V.L. Malcarne, PhD, Professor, SDSU/UC San Diego Joint Doctoral Program in Clinical Psychology, and Department of Psychology, SDSU. Address correspondence to Professor V.L. Malcarne, Doctoral Training Facility, 6363 Alvarado Court, Suite 103, San Diego, California 92120- 4913, USA. E-mail: Accepted for publication February 7, 2017.
    Objective: To evaluate the structural validity of the Rheumatology Attitudes Index (RAI), a widely used measure of rheumatic disease-related helplessness in patients with systemic sclerosis (SSc).

    Methods: Patients with physician-confirmed SSc from the University of California, Los Angeles (UCLA) Scleroderma Quality of Life Study (n = 208) received clinical examinations and completed self-report questionnaires. The structural validity of the RAI was examined through confirmatory and exploratory factor analysis (CFA/EFA). Read More

    Varicella vaccination elicits a humoral and cellular response in children with rheumatic diseases using immune suppressive treatment.
    Vaccine 2017 May 12;35(21):2818-2822. Epub 2017 Apr 12.
    Laboratory for Translational Immunology, Paediatric Immunology, University Medical Center Utrecht, Utrecht, The Netherlands; Department of Paediatric Rheumatology, Wilhelmina Children's Hospital, University Medical Center Utrecht, Utrecht, The Netherlands.
    Objective: To assess humoral and cellular responses to live-attenuated varicella zoster virus (VZV) vaccination of patients with juvenile idiopathic arthritis (JIA), juvenile dermatomyositis (JDM) or juvenile scleroderma (JScle) compared to those of healthy controls (HC).

    Methods: Before, 4-6weeks and one year after VZV vaccination, blood samples of patients and HC were collected. VZV-specific antibody concentrations were measured by ELISA and multiplex immune-assay. Read More

    Lung transplantation in systemic sclerosis: A single center cohort study.
    Joint Bone Spine 2017 Apr 11. Epub 2017 Apr 11.
    Lung Transplant Unit, Hospital Universitari Vall d'Hebron, Universitat Autònoma de Barcelona, Passeig de la Vall d'Hebron 119-129, 08035 Barcelona, Spain.
    Objective: Lung transplantation (LT) has been proposed as a treatment for advanced interstitial lung disease (ILD) and/or pulmonary hypertension (PH) associated to systemic sclerosis (SSc) but few studies have been reported. The aim of this study was to describe the clinical features, complications and survival of a single-center cohort of patients with SSc that underwent LT and to compare their survival with a group of non-SSc transplanted patients.

    Methods: Fifteen patients with SSc were transplanted between May 2005 and April 2015. Read More

    Maintenance treatment of ANCA-associated vasculitides.
    Clin Exp Rheumatol 2017 Mar-Apr;35 Suppl 103(1):199-201. Epub 2017 Apr 5.
    Department of Internal Medicine, Referral Centre for Rare Systemic and Autoimmune Diseases, Vasculitis and Scleroderma, Hôpital Cochin, APHP, Université Paris Descartes, Paris, France.
    ANCA-associated vasculitis(AAV) covers a group of systemic necrotising vasculitides characterised by inflammation of small vessels, sometimes with granuloma, and associated with autoantibodies to neutrophil cytoplasmic proteases (proteinase-3 or myeloperoxidase). Potentially lethal if not promptly diagnosed and treated, AAV in most patients can be induced into remission with the current treatment modalities. However, the risk of relapse remains high, necessitating prolonged immunosuppressive or immunomodulating maintenance therapy, whose optimal duration remains undetermined. Read More

    Elevated levels of TL1A are associated with disease activity in patients with systemic sclerosis.
    Clin Rheumatol 2017 Apr 10. Epub 2017 Apr 10.
    Department of Rheumatology and Immunology, West China Hospital, Sichuan University, Chengdu, Sichuan, China.
    TL1A is a member of the TNF superfamily. It performs significantly in the pathogenesis of rheumatic and autoimmune diseases partly through regulating the Th17 pathway. The clinical implication of circulating TL1A in patients with systemic sclerosis (SSc) remains unclear, and correlation between TL1A and Th17-related cytokines in the pathogenesis of SSc needs to be discussed. Read More

    Reaction time impairments in decision-making networks as a diagnostic marker for traumatic brain injuries and neurological diseases.
    J Comput Neurosci 2017 Jun 10;42(3):323-347. Epub 2017 Apr 10.
    Department of Applied Mathematics, University of Washington, Seattle, WA, 98195-3925, USA.
    The presence of diffuse Focal Axonal Swellings (FAS) is a hallmark cellular feature in many neurological diseases and traumatic brain injury. Among other things, the FAS have a significant impact on spike-train encodings that propagate through the affected neurons, leading to compromised signal processing on a neuronal network level. This work merges, for the first time, three fields of study: (i) signal processing in excitatory-inhibitory (EI) networks of neurons via population codes, (ii) decision-making theory driven by the production of evidence from stimulus, and (iii) compromised spike-train propagation through FAS. Read More

    Traumatic multiple cervical spine injuries in a patient with osteopetrosis and its management.
    Eur Spine J 2017 Apr 9. Epub 2017 Apr 9.
    Spine Fellow, Lilavati Hospital and Research Centre, Bandra reclamation, Bandra west, Mumbai, Maharashtra, 400050, India.
    Study Design: Single case report.

    Objective/purpose: To report multiple level fractures of cervical spine in a patient with osteopetrosis and its management. Osteopetrosis is a rare inherited condition characterized by defective remodeling resulting in hard and brittle bones with diffuse osteosclerosis. Read More

    Genetics of hereditary nephrotic syndrome: a clinical review.
    Korean J Pediatr 2017 Mar 27;60(3):55-63. Epub 2017 Mar 27.
    Department of Pediatrics, College of Medicine and Medical Research Institute, Chungbuk National University, Cheongju, Korea.
    Advances in podocytology and genetic techniques have expanded our understanding of the pathogenesis of hereditary steroid-resistant nephrotic syndrome (SRNS). In the past 20 years, over 45 genetic mutations have been identified in patients with hereditary SRNS. Genetic mutations on structural and functional molecules in podocytes can lead to serious injury in the podocytes themselves and in adjacent structures, causing sclerotic lesions such as focal segmental glomerulosclerosis or diffuse mesangial sclerosis. Read More

    Graves' disease in a 3 year-old patient with agranulocytosis due to anti-thyroid drugs: Radioiodine ablation therapy as an effective alternative.
    Rev Esp Med Nucl Imagen Mol 2017 Apr 6. Epub 2017 Apr 6.
    Unidad de Gestión Clínica de Medicina Nuclear, Hospital Regional Universitario de Málaga, Málaga, España.
    The case is presented of a 3 year-old girl with mitochondrial disease (subacute necrotizing encephalomyelopathy of Leigh syndrome), v-stage chronic kidney disease of a diffuse mesangial sclerosis, as well as developmental disorders, and diagnosed with hyperthyroidism Graves-Basedow disease. Six weeks after starting the treatment with neo-carbimazole, the patient reported a serious case of agranulocytosis. This led to stopping the anti-thyroid drugs, and was treated successfully with (131)I ablation therapy. Read More

    A genetic algorithm-based model for longitudinal changes detection in white matter fiber-bundles of patient with multiple sclerosis.
    Comput Biol Med 2017 May 31;84:182-188. Epub 2017 Mar 31.
    CREATIS CNRS UMR5220 & INSERM U1044, Université de Lyon, Université Claude Bernard-Lyon 1, INSA-Lyon, Villeurbanne, France; CERMEP - Imagerie du Vivant, Université de Lyon, Bron, France. Electronic address:
    Analysis of white matter (WM) tissue is essential to understand the mechanisms of neurodegenerative pathologies like multiple sclerosis (MS). Recently longitudinal studies started to show how the temporal component is important to investigate temporal diffuse effects of neurodegenerative pathologies. Diffusion tensor imaging (DTI) constitutes one of the most sensitive techniques for the detection and characterization of brain related pathological processes and allows also the reconstruction of WM fibers. Read More

    Importance of alternative-site blood glucose testing in the diagnosis of artifactual hypoglycaemia in systemic scleroderma.
    Diabetes Metab 2017 Apr 4. Epub 2017 Apr 4.
    Inserm UMRS 1166, diabetology-metabolism department, Sorbonne université, Pierre-et-Marie-Curie university (UPMC), Paris 6, institute of cardiometabolism and nutrition (ICAN), Pitié-Salpêtrière-Charles-Foix hospital, AP-HP, Paris, France. Electronic address:

    Lung Transplantation in Patients With Systemic Sclerosis.
    Curr Rheumatol Rep 2017 May;19(5):23
    Division of Rheumatology, University of California, San Francisco, 513 Parnassus Ave, Medical Science Building, S-847, San Francisco, CA, 94143, USA.
    Purpose Of Review: The purpose of this study was to highlight recent findings on evaluation for lung transplantation and outcomes after transplantation in patients with systemic sclerosis (scleroderma).

    Recent Findings: The recognition that extra-pulmonary disease manifestations can seriously compromise post-transplant outcomes has changed the way patients are screened for lung transplant candidacy. Reluctance to transplant subjects affected by scleroderma has been driven by the complexity and multisystem nature of this disease. Read More

    Geniposide inhibited endothelial-mesenchymal transition via the mTOR signaling pathway in a bleomycin-induced scleroderma mouse model.
    Am J Transl Res 2017 15;9(3):1025-1036. Epub 2017 Mar 15.
    Division of Rheumatology and Clinical Immunogenetics, Department of Internal Medicine, University of Texas Health Science Center at Houston Houston 77030, Texas, USA.
    Aim: Geniposide is an iridoid glycoside isolated from the gardenia plant. It has multiple biological activities. The roles of geniposide in systemic sclerosis (SSc) and in endothelial-to-mesenchymal transition (EndMT) are unclear. Read More

    Nailfold digital capillaroscopic findings in patients with diffuse and limited cutaneous systemic sclerosis.
    Reumatologia 2017 22;55(1):15-23. Epub 2017 Mar 22.
    Shiraz Geriatric Research Center, Shiraz University of Medical Sciences, Iran.
    Background: Systemic sclerosis (SSc) is a chronic disease with microvascular damage. Nailfold capillaroscopy is a non-invasive method used for evaluating capillaries in SSc. Its findings could be related to the internal organ involvement and SSc course. Read More

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