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    Free light chains of immunoglobulins in patients with systemic sclerosis: correlations with lung involvement and inflammatory milieu.
    J Clin Pathol 2018 Jan 31. Epub 2018 Jan 31.
    Institute of Rheumatology and Affine Sciences, Department of Rheumatology, Fondazione Policlinico Universitario Agostino Gemelli, School of Medicine, Catholic University of the Sacred Heart, Rome, Italy.
    Aim: Humoral immunity and B cells are thought to play an important role in the pathophysiology of the systemic sclerosis (SSc). The production of free light chains (FLC) of immunoglobulins is abnormally high in several pathological autoimmune conditions and reflects B cell activation. Furthermore, FLCs demonstrated different biological activities including their capability to modulate the immune system, proteolytic activity and complement cascade activation. Read More

    Heart involvement in systemic sclerosis.
    Tunis Med 2017 Mar;95(3):215-220
    Introduction: Primary and secondary heart involvement in systemic sclerosis are important mortality predictors. Aim of this study was to investigate by standard echocardiography associated to pulsed-tissue Doppler imaging, six-minute walk test (6MWT) and BNP level cardiac manifestation in 30 patients with ScS and to establish a strategy to detect and to evaluate this complication.

    Methods: This was a cross-sectional study conducted over a period of 16 months: a total of 30 consecutive patients with ScS diagnosed as proposed by the American college of Rheumatology and the European League Against Rheumatism 2013 and who were hospitalized in Internal Medicine department of Habib Thameur hospital underwent cardiac assessment. Read More

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

    Biopsy-proven multiple sclerosis in an adult patient with atypical craniometaphyseal dysplasia.
    BMJ Case Rep 2018 Feb 14;2018. Epub 2018 Feb 14.
    Unit of Genetics of Neurodegenerative and Metabolic Diseases, IRCCS Fondazione "Istituto Neurologico C Besta", Milano, Italy.
    Craniometaphyseal dysplasia (CMD) is a rare condition characterised by progressive, diffuse hyperostosis of cranial and long bones, with compression of cranial nerves, linked to mutations inorgenes. Here we describe an adult case with clinical features of CMD, who developed cerebral expansive lesion of undetermined nature. Brain biopsy revealed active demyelinating lesions, consistent with multiple sclerosis. Read More

    Tuberous Sclerosis Complex and Diffuse Lipomatosis: Case Report of a Rare Association.
    Indian Dermatol Online J 2018 Jan-Feb;9(1):37-39
    Department of Radiodiagnosis, Postgraduate Institute of Medical Education and Research, Chandigarh, India.
    Lipomatosis is characterized by diffuse infiltration of adipocytes in a tissue. A young male patient presented for evaluation of unilateral limb swelling. On evaluation, he was found to have tuberous sclerosis complex with diffuse lipomatosis of the right leg. Read More

    Bryostatin-1 alleviates experimental multiple sclerosis.
    Proc Natl Acad Sci U S A 2018 Feb 12. Epub 2018 Feb 12.
    Department of Psychiatry and Behavioral Sciences, Johns Hopkins University School of Medicine, Baltimore, MD 21287;
    Multiple sclerosis (MS) is an inflammatory disorder targeting the central nervous system (CNS). The relapsing-remitting phase of MS is largely driven by peripheral activation of autoreactive T-helper (Th) 1 and Th17 lymphocytes. In contrast, compartmentalized inflammation within the CNS, including diffuse activation of innate myeloid cells, characterizes the progressive phase of MS, the most debilitating phase that currently lacks satisfactory treatments. Read More

    Symptom-associated change of motor-related neuromagnetic fields in a patient with multiple sclerosis: A case report.
    J Clin Neurosci 2018 Feb 10. Epub 2018 Feb 10.
    Department of Neurosurgery, Brain Research Institute, Yonsei University College of Medicine, Seoul, Republic of Korea. Electronic address:
    The objective of this study was to investigate functional abnormalities of the brain in a patient with multiple sclerosis (MS) by using magnetoencephalography (MEG) and a finger-tapping task. A 46-year-old woman that presented with motor weakness of left hand and was diagnosed with MS. Conventional magnetic resonance imaging demonstrated a white matter lesion with hyperintensity on T2-weighted images in the right motor area. Read More

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

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

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

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

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

    Changes in structural network are associated with cortical demyelination in early multiple sclerosis.
    Hum Brain Mapp 2018 Feb 6. Epub 2018 Feb 6.
    Institute of Biomedical Engineering, Polytechnique Montreal, Montreal, Quebec, Canada.
    The aim of this study was to investigate the interplay between structural connectivity and cortical demyelination in early multiple sclerosis. About 27 multiple sclerosis patients and 18 age-matched controls underwent two MRI scanning sessions. The first was done at 7T and involved acquiring quantitative Tand T* high-resolution maps to estimate cortical myelination. Read More

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

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

    High Bone Mass in Adults.
    Joint Bone Spine 2018 Jan 30. Epub 2018 Jan 30.
    Service de Rhumatologie, CHU de Rennes, Hôpital Sud, 16, boulevard de Bulgarie, BP 90347, 35203 Rennes cedex 2, France; INSERM UMR 991, 35043 Rennes, France; Université Rennes 1, Faculté de médecine, 35043 Rennes, France.
    A finding of high bone mineral density (BMD) from routine dual-energy X-ray absorptiometry (DXA) screening is not uncommon. No consensus exists about the definition of high BMD, and T-score and/or Z-score cutoffs of ≥+2.5 or ≥+4 have been suggested. Read More

    Potential neuroprotective effect of Fingolimod in multiple sclerosis and its association with clinical variables.
    Expert Opin Pharmacother 2018 Feb 3:1-9. Epub 2018 Feb 3.
    a Neurology Section, Department of Neurosciences, Biomedicine and Movement Sciences , University of Verona , Verona , Italy.
    Introduction: Multiple sclerosis (MS) is a chronic inflammatory, demyelinating disease of the central nervous system affecting both white matter and grey matter in the earliest phases of its course. The crucial role of neurodegeneration in disability progression in MS, regardless of white matter damage, has been confirmed by several imaging and neuropathological studies. Fingolimod is an effective immunomodulator of the sphingosine 1-phosphate receptor, approved in relapsing remitting MS and able to cross the blood-brain barrier and to slow disability progression and brain volume loss. Read More

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

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

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

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

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

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

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

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

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

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

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

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

    Occupational Therapy Treatment to Improve Upper Extremity Function in Individuals with Early Systemic Sclerosis: A Pilot Study.
    Arthritis Care Res (Hoboken) 2018 Jan 30. Epub 2018 Jan 30.
    Department of Physical Medicine and Rehabilitation and Research Health Science Specialist, VA Ann Arbor Health Care System.
    Objective: To determine feasibility and preliminary effects of an occupational therapy treatment to improve upper extremity (UE) function in patients with early systemic sclerosis (SSc) who have UE contractures.

    Methods: A one-arm pilot clinical rehabilitation trial was conducted at a university health system. Participants with SSc and ≥ 1 UE contracture (n = 21) participated in a total of 8 weekly in-person occupational therapy sessions. Read More

    Immunohistochemical assessment of the diagnostic utility of PD-L1: a preliminary analysis of anti-PD-L1 antibody (SP142) for lymphoproliferative diseases with tumour and nonmalignant Hodgkin/Reed-Sternberg (HRS)-like cells.
    Histopathology 2018 Jan 30. Epub 2018 Jan 30.
    Department of Surgical Pathology, Aichi Medical University Hospital, Nagakute, Japan.
    Aims: The programmed death 1 (PD1)/PD1 ligand (PD-L1) axis plays an important role in tumour cells escape from immune control. PD-L1 immunohistochemistry is a useful predictor of immunotherapy response, but is still not widely used in the diagnostic setting. Here we describe results using PD-L1 immunohistochemistry during routine diagnostics in lymphoma. Read More

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

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

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

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

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

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

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

    Multiple Sclerosis Pathology.
    Cold Spring Harb Perspect Med 2018 Jan 22. Epub 2018 Jan 22.
    Center for Brain Research, Medical University of Vienna, A-1090 Wien, Austria.
    Multiple sclerosis (MS) is a chronic inflammatory demyelinating disease of the central nervous system (CNS), which gives rise to focal lesions in the gray and white matter and to diffuse neurodegeneration in the entire brain. In this review, the spectrum of MS lesions and their relation to the inflammatory process is described. Pathology suggests that inflammation drives tissue injury at all stages of the disease. Read More

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

    Background: The Chicago Classification version 3. Read More

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

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

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

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

    Diffuse Colour Discrimination as Marker of Afferent Visual System Dysfunction in Amyotrophic Lateral Sclerosis.
    Neuroophthalmology 2017 Dec 1;41(6):310-314. Epub 2017 Jun 1.
    Departments of Ophthalmology and Visual Sciences and Neurology and Rehabilitation, University of Illinois at Chicago, Chicago, Illinois, USA.
    Abnormalities of the inner and intermediate retinal structures in patients with amyotrophic lateral sclerosis (ALS) have been described using optical coherence tomography and histopathology. Colour vision is a potential marker of these structural changes. The purpose of this study is to test the hypothesis that colour vision impairment is associated with ALS. Read More

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

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

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

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

    Regulatory mechanisms of collagen expression by interleukin-22 signaling in scleroderma fibroblasts.
    J Dermatol Sci 2017 Dec 29. Epub 2017 Dec 29.
    Department of Dermatology and Plastic Surgery, Faculty of Life Sciences, Kumamoto University, 1-1-1 Honjo, Kumamoto, Japan.
    Background: Various cytokines have been indicated to be involved in the pathogenesis of systemic sclerosis (SSc). IL-22 is one of the member of IL-10 cytokine family, and several studies have implicated IL-22 signaling in the pathogenesis of autoimmune diseases.

    Objectives: To clarify the role of IL-22 in the regulatory mechanism of ECM expression and to determine the contribution of IL-22 to the phenotype of SSc. Read More

    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

    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

    Disability in progressive MS is associated with T2 lesion changes.
    Mult Scler Relat Disord 2017 Dec 18;20:73-77. Epub 2017 Dec 18.
    Danish Multiple Sclerosis Center, University of Copenhagen, Rigshospitalet, Copenhagen, Denmark.
    Background: Progressive multiple sclerosis (MS) is characterised by diffuse changes on brain magnetic resonance imaging (MRI), which complicates the use of MRI as a diagnostic and prognostic marker. The relationship between MRI measures (conventional and non-conventional) and clinical disability in progressive MS therefore warrants further investigation.

    Objective: To investigate the relationship between clinical disability and MRI measures in patients with progressive MS. 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

    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

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