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    Microglia and C9orf72 in neuroinflammation and ALS and frontotemporal dementia.
    J Clin Invest 2017 Jul 24. Epub 2017 Jul 24.
    Board of Governors Regenerative Medicine Institute and.
    Amyotrophic lateral sclerosis (ALS) is a degenerative disorder that is characterized by loss of motor neurons and shows clinical, pathological, and genetic overlap with frontotemporal dementia (FTD). Activated microglia are a universal feature of ALS/FTD pathology; however, their role in disease pathogenesis remains incompletely understood. The recent discovery that ORF 72 on chromosome 9 (C9orf72), the gene most commonly mutated in ALS/FTD, has an important role in myeloid cells opened the possibility that altered microglial function plays an active role in disease. Read More

    Blinded Sample Size Recalculation in clinical trials incorporating historical data.
    Contemp Clin Trials 2017 Jul 19. Epub 2017 Jul 19.
    Institute for Medical Biometry and Informatics, University of Heidelberg. Electronic address:
    Recruiting sufficient patients within an acceptable time horizon is an issue for most clinical trials and is especially challenging in the field of rare diseases. It is therefore an attractive option to include historical data from previous (pilot) trials in the current study thus reducing the recruitment burden. In clinical trials with binary endpoint, the required sample size does not only depend on the type I error rate, the power, and the treatment group difference but additionally on the overall event rate. Read More

    Incidence, Prevalence, Mortality and Causes of Death in Systemic Sclerosis in Korea: A Nationwide Population-Based Study.
    Br J Dermatol 2017 Jul 22. Epub 2017 Jul 22.
    Department of Preventive Medicine, College of Medicine, Korea University.
    While the epidemiology of systemic sclerosis (SSc) is known to vary by geographical region and race, studies from the national level as well as reports from Asian countries are scarce and the majority of existing studies were limited to cases from small geographic areas.(1-3) This article is protected by copyright. All rights reserved. Read More

    Severity and features of frailty in systemic sclerosis-associated interstitial lung disease.
    Respir Med 2017 Aug 25;129:1-7. Epub 2017 May 25.
    Department of Medicine, University of British Columbia, Vancouver, Canada; Centre for Heart Lung Innovation, University of British Columbia, Vancouver, Canada. Electronic address:
    Background: Systemic sclerosis-associated interstitial lung disease (SSc-ILD) is characterized by multiple symptoms and comorbidities. The cumulative impact of these deficits can be summarized using the concept of frailty; however, frailty has not been characterized in patients with SSc-ILD.

    Methods: Patients with SSc-ILD and non-CTD fibrotic ILD were recruited from specialized clinics. Read More

    Downregulation of Aquaporin3 in Systemic Sclerosis Dermal Fibroblasts.
    Iran J Allergy Asthma Immunol 2017 Jun;16(3):228-234
    Rheumatology Research Center, Tehran University of Medical Sciences, Tehran, Iran.
    Skin dryness and thickening are hallmarks of systemic sclerosis (SSc) disease. Aquaporins (AQPs) are plasma membrane proteins that transport glycerol and water, resulting in water retention and skin hydration. Expression of AQPs has been evaluated in human normal skin. Read More

    An update on biomarker discovery and use in systemic sclerosis.
    Expert Rev Mol Diagn 2017 Jul 21. Epub 2017 Jul 21.
    a Department of Dermatology, Faculty of Medicine, Institute of Medical, Pharmaceutical and Health Sciences , Kanazawa University , Kanazawa 920-8641 , Japan.
    Introduction: Systemic sclerosis (SSc) is an autoimmune disease characterized by excessive extracellular matrix deposition in the skin and internal organs. Three major abnormalities, autoimmunity, vasculopathy, and fibrosis, are considered to play important roles in the pathophysiology of SSc. SSc is a heterogeneous disease with clinical features, disease progress, therapeutic response, and prognosis. Read More

    Mucosal-associated invariant (MAIT) cells are deficient in systemic sclerosis.
    Scand J Immunol 2017 Jul 18. Epub 2017 Jul 18.
    Sorbonne Universités, UPMC Université Paris 06, INSERM, Centre de Recherche Saint-Antoine (CRSA), F-75012, Paris, France.
    Objectives: Systemic sclerosis (SSc) is an autoimmune disease, characterized by fibrosis of the skin and other organs, vascular impairment and deficient immune responses. Mucosal associated invariant T cells (MAIT) cells have been involved in various inflammatory and autoimmune diseases. The aim of this study was to determine the frequencies of MAIT cells in the blood of patients with systemic sclerosis (SSc), and to compare their distribution in the different types of SSc. Read More

    The discovery of the Flammer syndrome: a historical and personal perspective.
    EPMA J 2017 Jun 22;8(2):75-97. Epub 2017 May 22.
    Department of Ophthalmology, University of Basel, Mittlere Strasse 91, CH-4031 Basel, Switzerland.
    This review describes the clinical and basic research that led to the description of Flammer syndrome. It is narrated from a personal perspective. This research was initiated by the observation of an increased long-term fluctuation of visual fields in a subgroup of glaucoma patients. Read More

    Sustained benefit from combined plasmapheresis and allogeneic mesenchymal stem cells transplantation therapy in systemic sclerosis.
    Arthritis Res Ther 2017 Jul 19;19(1):165. Epub 2017 Jul 19.
    Department of Rheumatology and Immunology, The Affiliated Drum Tower Hospital of Nanjing University Medical School, 321 Zhongshan Road, Nanjing, 210008, China.
    Background: Systemic sclerosis (SSc) is an autoimmune disease involving the skin and several internal organs. Most therapies available for this disease are symptomatic. Given the difficulty in treating SSc, we conducted this study to investigate the effect of combined plasmapheresis (PE) and allogeneic mesenchymal stem cells transplantation (MSCT) therapy on SSc. Read More

    Treatment of digital ulcers in systemic sclerosis: Case series study of thirteen patients and discussion on outcome.
    Rev Assoc Med Bras (1992) 2017 May;63(5):422-426
    Clinic of autoimmune diseases, Department of Internal Medicine - III, Hospital Distrital de Santarém, Santarém, Portugal.
    Introduction:: In systemic sclerosis (SSc), digital ulcers (DU) are debilitating and recurrent. They are markers of prognosis and are associated with disability and mortality. Treatment strategies have been developed to block the proposed mechanisms of this complication. Read More

    Epidemiology of autoimmune and inflammatory diseases in a French Nationwide HIV cohort.
    AIDS 2017 Jul 18. Epub 2017 Jul 18.
    aDepartment of Internal Medicine, Clinical Immunology and Infectious Diseases, Robert Debré Hospital, University Hospital, Reims, France bDepartment of Internal Medicine and Infectious Diseases, Manchester Hospital, Charleville-Mézières, France cINSERM, UMR 1027, Toulouse III University, CHU Toulouse, COREVIH Toulouse, Toulouse, France dLe Trait d'Union, Centre de Soins de l'Infection par le VIH, Nouvel Hôpital Civil, University Hospital, Strasbourg, France eDepartment of Infectious Diseases, Bichat-Claude Bernard Hospital, APHP, Paris, France fDepartment of Infectious Diseases, Croix-Rousse Hospital, Hospices Civils de Lyon, and INSERM U1052, Lyon, France gDepartment of Infectious Diseases, Hôtel Dieu, Nantes, France hDepartment of Infectious Diseases, University Hospital, Archet Hospital, Nice, France iReims Champagne-Ardenne University, EA-4684 / SFR CAP-SANTE, Reims, F-51095.
    HIV infection and inflammatory and autoimmune diseases (IAD) are both related to immune dysfunction. Epidemiological data on IAD in patients living with HIV (PLHIV) are scarce. The aim of this study was thus to estimate the prevalence of 26 IAD among PLHIV followed in a large French multicenter cohort in the cART era (from January 2000 to July 2013), and to describe their occurrence according to cART onset, the immuno-virological status of patients and HCV and/or HBV co-infection. Read More

    Are there risk factors for scleroderma-related calcinosis?
    Mod Rheumatol 2017 Jul 19:1-5. Epub 2017 Jul 19.
    b Department of Medicine, Division of Rheumatology and Connective Tissue Research , Rutgers-Robert Wood Johnson Medical School , New Brunswick , NJ , USA.
    Objectives: Pathogenesis and risk factors of scleroderma associated calcinosis is poorly understood and there is no effective treatment. This study was performed to better understand the prevalence and clinical features associated with calcinosis in a cohort of SSc outpatients.

    Methods: In this cross-sectional study, we compared clinical characteristics of SSc patients with (SSc-calcinosis) and without calcinosis (SSc-control) seen in the outpatient Rutgers-RWJ Scleroderma Program between 2012 and 2015. Read More

    Intestinal microbiome in scleroderma: recent progress.
    Curr Opin Rheumatol 2017 Jul 15. Epub 2017 Jul 15.
    Department of Medicine, University of California, David Geffen School of Medicine, California, Los Angeles, USA.
    Purpose Of Review: Our evolving understanding of how gut microbiota affects immune function and homeostasis has led many investigators to explore the potentially pathologic role of gut microbiota in autoimmune diseases. This review will discuss the rapidly advancing field of microbiome research in systemic sclerosis (SSc), an incurable autoimmune disease with significant gastrointestinal morbidity and mortality.

    Recent Findings: Recent reports have identified common perturbations in gut microbiota across different SSc cohorts. Read More

    The potential role of adult stem cells in the management of the rheumatic diseases.
    Ther Adv Musculoskelet Dis 2017 Jul 20;9(7):165-179. Epub 2017 Apr 20.
    Arthritis & Regenerative Medicine Laboratory, Aberdeen Centre for Arthritis and Musculoskeletal Health, Institute of Medical Sciences, University of Aberdeen, Aberdeen, UK.
    Adult stem cells are considered as appealing therapeutic candidates for inflammatory and degenerative musculoskeletal diseases. A large body of preclinical research has contributed to describing their immune-modulating properties and regenerative potential. Additionally, increasing evidence suggests that stem cell differentiation and function are disrupted in the pathogenesis of rheumatic diseases. Read More

    Chronic Urinary Retention in Multiple Sclerosis Patients: Physiology, Systematic Review of Urodynamic Data, and Recommendations for Care.
    Urol Clin North Am 2017 Aug;44(3):429-439
    Department of Urology, University of Michigan Hospital, University of Michigan, 3875 Taubman Center, 1500 East Medical Center Drive, Ann Arbor, MI 48109, USA. Electronic address:
    Many multiple sclerosis (MS) patients are affected by urinary retention. Common causes include neurogenic underactive bladder and/or bladder outlet obstruction from detrusor sphincter dyssynergia. Systemic review of contemporary MS urodynamic studies demonstrates that 53% of MS patients have detrusor overactivity, 43% have detrusor sphincter dyssynergia, and 12% have atonic bladder (12 studies, 1524 patients). Read More

    New genetic causes for complex hereditary spastic paraplegia.
    J Neurol Sci 2017 Aug 15;379:283-292. Epub 2017 Jun 15.
    Division of Neuromuscular Diseases, Department of Neurology and Neurosurgery, Federal University of São Paulo (UNIFESP), São Paulo, SP, Brazil.
    Introduction: Hereditary Spastic Paraplegia (HSP) represents a complex and heterogeneous group of rare neurodegenerative disorders that share a common clinical feature of weakness and lower limb spasticity that can occur alone or in combination with a constellation of other neurological or systemic signs and symptoms. Although the core clinical feature of weakness and lower limb spasticity is virtually universal, the genetic heterogeneity is almost uncountable with more than 70 genetic forms described so far. We performed review of medical records from twenty-one patients from seventeen Brazilian families with complex phenotype of HSP. Read More

    Silent myocarditis in systemic sclerosis detected by cardiovascular magnetic resonance using Lake Louise criteria.
    BMC Cardiovasc Disord 2017 Jul 17;17(1):187. Epub 2017 Jul 17.
    Onassis Cardiac Surgery Center, 50 Esperou Street, 175-61 P.Faliro, Athens, Greece.
    Background: Systemic sclerosis (SSc) is an autoimmune disease characterized by microvascular abnormalities, inflammation and fibrosis. We hypothesized that myocarditis may be diagnosed in asymptomatic SSc, undergoing routine cardio-vascular magnetic resonance (CMR) for fibrosis assessment, using the Lake Louise criteria: T2 ratio, early (EGE) and late gadolinium enhanced (LGE) images.

    Methods: Eighty-two asymptomatic SSc, diagnosed according to American College of Rheumatology criteria, aged 43 ± 5 yrs. Read More

    Cutaneous Manifestations of Scleroderma and Scleroderma-Like Disorders: a Comprehensive Review.
    Clin Rev Allergy Immunol 2017 Jul 16. Epub 2017 Jul 16.
    Section of Dermatology, Department of Medical Sciences and Public Health, University of Cagliari, Cagliari, Italy.
    Scleroderma refers to an autoimmune connective tissue fibrosing disease, including three different subsets: localized scleroderma, limited cutaneous systemic sclerosis, and diffuse cutaneous systemic sclerosis with divergent patterns of organ involvement, autoantibody profiles, management, and prognostic implications. Although systemic sclerosis is considered the disease prototype that causes cutaneous sclerosis, there are many other conditions that can mimic and be confused with SSc. They can be classified into immune-mediated/inflammatory, immune-mediated/inflammatory with abnormal deposit (mucinoses), genetic, drug-induced and toxic, metabolic, panniculitis/vascular, and (para)neoplastic disorders according to clinico-pathological and pathogenetic correlations. Read More

    Assessment of English-French differential item functioning of the Satisfaction with Appearance Scale (SWAP) in systemic sclerosis.
    Body Image 2017 Jul 13;22:97-102. Epub 2017 Jul 13.
    Departments of Educational and Counselling Psychology; Psychiatry; Medicine; Epidemiology, Biostatistics, and Occupational Health; and Psychology, McGill University and Lady Davis Institute for Medical Research, Jewish General Hospital, Montréal, Québec, Canada.
    The Satisfaction with Appearance Scale (SWAP) has been used to assess body image distress among people with the rare and disfiguring disease systemic sclerosis (SSc); however, it has not been validated across different languages groups. The objective was to examine differential item functioning of the SWAP among 856 Canadian English- or French-speaking SSc patients. Confirmatory factor analysis was used to evaluate the SWAP two-factor structure (Dissatisfaction with Appearance and Social Discomfort). Read More

    Clinical and Biological Insights from the University of California San Francisco Prospective and Longitudinal Cohort.
    Lung 2017 Jul 13. Epub 2017 Jul 13.
    Division of Pulmonary and Critical Care, University of California, San Francisco, San Francisco, CA, 94143, USA.
    Introduction: Sarcoidosis is a systemic inflammatory disease characterized by non-necrotizing granulomas in involved organs, most commonly the lung. Description of patient characteristics in the Western United States is limited. Furthermore, blood-based measures that relate to clinical sarcoidosis phenotypes are lacking. Read More

    Incidence of Oral Lichen Planus in Perimenopausal Women: A Cross-sectional Study in Western Uttar Pradesh Population.
    J Midlife Health 2017 Apr-Jun;8(2):70-74
    Department of Oral Medicine and Radiology, Subharti Dental College, Meerut, Uttar Pradesh, India.
    Background: Hormonal fluctuations during menopause lead to endocrine changes in women, especially in their sex steroid hormone production. Studies have documented the role of estrogen and progesterone (Pg) on autoimmune disorders such as multiple sclerosis, systemic lupus erythematosus, and rheumatoid arthritis. Lichen planus (LP), an autoimmune disorder, seen frequently in perimenopausal women, may also get affected by sex steroid hormones, but no direct relationship has been established yet. Read More

    MicroRNAs in the skin: role in development, homoeostasis and regeneration.
    Clin Sci (Lond) 2017 Aug 13;131(15):1923-1940. Epub 2017 Jul 13.
    Department of Applied Sciences, Northumbria University, Newcastle upon Tyne, NE1 8ST, U.K.
    The skin is the largest organ of the integumentary system and possesses a vast number of functions. Due to the distinct layers of the skin and the variety of cells which populate each, a tightly regulated network of molecular signals control development and regeneration, whether due to programmed cell termination or injury. MicroRNAs (miRs) are a relatively recent discovery; they are a class of small non-coding RNAs which possess a multitude of biological functions due to their ability to regulate gene expression via post-transcriptional gene silencing. Read More

    Changes in pulmonary exercise haemodynamics in scleroderma: a 4-year prospective study.
    Eur Respir J 2017 Jul 13;50(1). Epub 2017 Jul 13.
    Dept of Internal Medicine, Division of Pulmonology, Medical University of Graz, Graz, Austria.
    Pulmonary arterial hypertension (PAH) is a feared complication of systemic sclerosis. In this prospective cohort study, we monitored the changes in resting and exercise pulmonary haemodynamics of scleroderma patients without initial PAH over a mean follow-up period of ∼4 years.All patients underwent exercise echocardiography and cardiopulmonary exercise testing at baseline and follow-up. Read More

    International consensus: what else can we do to improve diagnosis and therapeutic strategies in patients affected by autoimmune rheumatic diseases (rheumatoid arthritis, spondyloarthritides, systemic sclerosis, systemic lupus erythematosus, antiphospholipid syndrome and Sjogren's syndrome)?: The unmet needs and the clinical grey zone in autoimmune disease management.
    Autoimmun Rev 2017 Jul 10. Epub 2017 Jul 10.
    Department of Pathophysiology, University of Athens, Athens, Greece.
    Autoimmune diseases are a complex set of diseases characterized by immune system activation and, although many progresses have been done in the last 15 years, several unmet needs in the management of these patients may be still identified. Recently, a panel of international Experts, divided in different working groups according to their clinical and scientific expertise, were asked to identify, debate and formulate a list of key unmet needs within the field of rheumatology, serving as a roadmap for research as well as support for clinicians. After a systematic review of the literature, the results and the discussions from each working group were summarised in different statements. Read More

    [Systemic sclerosis and occupational difficulties: Results of a prospective study].
    Rev Med Interne 2017 Jul 10. Epub 2017 Jul 10.
    Service de pathologies professionnelles et maintien en emploi, CHRU de Lille, Lille, France; Laboratoire de recherche, EA4487 axe droit/santé-travail, Lille, France. Electronic address:
    Between 9000 and 14000 people have systemic sclerosis (ScS) in France. The work is often affected. Our study aims to assess the frequency of professional difficulties (DP) of scleroderma patients, identify these DP, the symptoms involved and the solutions used. Read More

    Treatment of connective tissue disease-associated interstitial lung disease: the pulmonologist's point of view.
    Korean J Intern Med 2017 Jul 30;32(4):600-610. Epub 2017 Jun 30.
    Division of Allergy and Respiratory Medicine, Department of Internal Medicine, Soon Chun Hyang University Seoul Hospital, Seoul, Korea.
    Interstitial lung disease (ILD) occurs in 15% of patients with collagen vascular disease (CVD), referred to as connective tissue disease (CTD). Despite advances in management strategies, ILD continues to be a significant cause of mortality in patients with CVD-associated ILD (CTD-ILD). There is a lack of randomized, clinical trials assessing pharmacological agents for CTD-ILD, except in cases of ILD-associated systemic sclerosis (SSc). Read More

    IL-17 and Th17 cells in systemic sclerosis: a comprehensive review.
    Rom J Intern Med 2017 Jul 8. Epub 2017 Jul 8.
    Internal Medicine Chair.
    T cells (especially T helper cells) seem to be strongly associated with systemic sclerosis pathogenesis. Th17-IL-17 axis was proved to be involved in the pathogenesis of multiple autoimmune diseases. By performing a comprehensive research of the literature indexed in PubMed database, the current review summarizes current knowledge related to Th17 and IL-17 in systemic sclerosis. Read More

    Phototherapy in systemic sclerosis: review.
    Photodermatol Photoimmunol Photomed 2017 Jul 13. Epub 2017 Jul 13.
    Division of Dermatology, Department of Medicine, Faculty of Medicine, Khon Kaen University, Khon Kaen, 40002, Thailand.
    Systemic scleroderma-also known as systemic sclerosis (SSc)-is a chronic systemic connective tissue disease characterized by collagen deposition in cutaneous and internal organs, leading to skin sclerosis and multiple organ fibrosis. The pathogenesis is complex and remains poorly understood. Treatment is based on organ involvement and requires a multidisciplinary approach. Read More

    Possible role of Helicobacter pylori in diseases of dermatological interest.
    J Biol Regul Homeost Agents 2017 07 13;31(2 Suppl. 2). Epub 2017 Jul 13.
    Department of Clinical and Experimental Medicine, University of Messina, Messina, Italy.
    Helicobacter pylori is a gram-negative, flagellate, microaerophilic bacterium identified for the first time about 30 years ago, as a pathogenic factor of gastritis and peptic ulcer. Soon after, it was linked to several gastrointestinal and extra-gastrointestinal diseases (hematological, cardiovascular, neurological, pulmonary and ocular diseases, obesity, diabetes mellitus, growth retardation and extragastric MALT lymphoma). Association and possible cause-effect correlation with H. Read More

    Personalized medicine in rheumatology: the paradigm of serum autoantibodies.
    Auto Immun Highlights 2017 Dec 12;8(1):10. Epub 2017 Jul 12.
    Division of Rheumatology and Clinical Immunology, Humanitas Research Hospital, Via A. Manzoni 56, Rozzano, 20089, Milan, Italy.
    The sequencing of the human genome is now well recognized as the starting point of personalized medicine. Nonetheless, everyone is unique and can develop different phenotypes of the same disease, despite identical genotypes, as well illustrated by discordant monozygotic twins. To recognize these differences, one of the easiest and most familiar examples of biomarkers capable of identifying and predicting the outcome of patients is represented by serum autoantibodies. Read More

    Role of Sex Hormone Levels and Psychological Stress in the Pathogenesis of Autoimmune Diseases.
    Cureus 2017 Jun 5;9(6):e1315. Epub 2017 Jun 5.
    Department of Medicine, Shifa International Hospital, Islamabad, Pakistan.
    The aim of this review article is to assess the connection between psychological stress and sex hormones and their effect on the development of autoimmune diseases. Psychological stress describes what people feel when they are under mental, physical, or emotional pressure. We searched for online articles using MEDLINE®, Embase, Cochrane Library and Google Scholar. Read More

    Autoamputation and Polyneuropathy in Mixed Connective Tissue Disorder: A Case Report.
    Cureus 2017 Jun 5;9(6):e1313. Epub 2017 Jun 5.
    Department of Medicine, Jinnah Postgraduate Medical Center Karachi Pakistan.
    Mixed connective tissue disorder (MCTD) is a multisystem disease with overlapping features of other autoimmune diseases, such as systemic lupus erythematosus (SLE), myositis, rheumatoid arthritis, and scleroderma. MCTD presents with a distinctive antibody in serum known as U1-ribonucleoprotein (RNP). MCTD is quite rare as compared to other connective tissue disorders like SLE, systemic sclerosis, dermatomyositis, and polymyositis. Read More

    An Unusual Case of Aortic Stenosis in Systemic Sclerosis.
    Cureus 2017 Jun 1;9(6):e1303. Epub 2017 Jun 1.
    Rheumatology, Mount Sinai Saint Luke's Hospital Center.
    Systemic sclerosis is a connective tissue disorder that frequently involves the heart. All cardiac structures can be involved but aortic valve involvement is rare. We report a case of an 83-year-old man with a history of systemic sclerosis presenting with shortness of breath. Read More

    Corrigendum to "Docetaxel-Induced Systemic Sclerosis with Internal Organ Involvement Masquerading as Congestive Heart Failure".
    Case Reports Immunol 2017 29;2017:4809124. Epub 2017 Jun 29.
    Department of Family Medicine, Henry Ford Hospital, Wayne State University School of Medicine, Detroit, MI 48202, USA.
    [This corrects the article DOI: 10.1155/2017/4249157.]. Read More

    Chromosome 21-Encoded microRNAs (mRNAs): Impact on Down's Syndrome and Trisomy-21 Linked Disease.
    Cell Mol Neurobiol 2017 Jul 7. Epub 2017 Jul 7.
    LSU Neuroscience Center, Louisiana State University Health Science Center, 2020 Gravier Street, Suite 904, New Orleans, LA, 70112-2272, USA.
    Down's syndrome (DS; also known as trisomy 21; T21) is caused by a triplication of all or part of human chromosome 21 (chr21). DS is the most common genetic cause of intellectual disability attributable to a naturally-occurring imbalance in gene dosage. DS incurs huge medical, healthcare, and socioeconomic costs, and there are as yet no effective treatments for this incapacitating human neurogenetic disorder. Read More

    Prevalence of anti-NT5C1A antibodies in Japanese patients with autoimmune rheumatic diseases in comparison with other patient cohorts.
    Clin Chim Acta 2017 Jul 4;472:1-4. Epub 2017 Jul 4.
    Department of Dermatology, Nagoya University Graduate School of Medicine, Nagoya, Japan.
    Background: Sporadic inclusion body myositis (sIBM) is usually classified as an idiopathic inflammatory myopathies. Although the diagnosis of sIBM is sometimes challenging, recent studies have shown that the autoantibodies against cytosolic 5'-nucleotidase 1A (NT5C1A) are the possible diagnostic biomarker for sIBM. Few reports have shown the frequencies of anti-NT5C1A antibodies in systemic autoimmune rheumatic diseases (SARDs) using large cohorts of SARDs. Read More

    Management of hydrocephalus associated with autoimmune diseases: a series of 19 cases.
    Autoimmunity 2017 Jul 7:1-6. Epub 2017 Jul 7.
    a The Department of Neurosurgery , Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College , Beijing , P.R. China.
    Objectives: To analyze the diagnosis and treatment of hydrocephalus associated with autoimmune diseases and to explore the possible mechanism of hydrocephalus in these patients.

    Methods: A retrospective case series study was conducted at Peking Union Medical College Hospital, Beijing, China. Files were retrieved from the hospital archives by screening records from Jan 1990 to Jan 2016. Read More

    Coexistence of multiple sclerosis and ankylosing spondylitis: Report of four cases from Russia and review of the literature.
    J Clin Neurosci 2017 Jul 3. Epub 2017 Jul 3.
    Bujanov Moscow City Clinical Hospital, Multiple Sclerosis Center № 1, Moscow, Russia.
    Multiple sclerosis is a chronic demyelinating disorder of the central nervous system. There are many cases of multiple sclerosis - like syndrome and demyelinating disorders in systemic lupus erythematosus, Sjogren disease, Behcet disease and other autoimmune conditions. Coexistence of ankylosing spondylitis and multiple sclerosis usually is rare but in this article we report 4 Russian patients with concomitant multiple sclerosis and ankylosing spondylitis diseases. Read More

    The "myth" of loss of angiogenesis in systemic sclerosis: a pivotal early pathogenetic process or just a late unavoidable event?
    Arthritis Res Ther 2017 Jul 6;19(1):162. Epub 2017 Jul 6.
    Department of Experimental and Clinical Medicine, Division of Rheumatology and Scleroderma Unit, Azienda Ospedaliera Universitaria Careggi, University of Florence, Viale Pieraccini 18, 50139, Florence, Italy.
    Systemic sclerosis is considered a disease dominated by a "loss of angiogenesis", although in its early phases evidence indicates a disturbed angiogenic response only. In fact, microvascular changes are primarily due to endothelial cell injury, triggering downstream significant enlargement of the capillary in an inflammatory environment, followed by capillary rupture (microhemorrhages). Subsequent pro-angiogenic efforts lead to an aberrant angiogenesis and, eventually, to a total loss of vessel repair and regeneration (loss of angiogenesis). Read More

    Nephron development and extrarenal features in a child with congenital nephrotic syndrome caused by null LAMB2 mutations.
    BMC Nephrol 2017 Jul 6;18(1):220. Epub 2017 Jul 6.
    Department of Pediatrics, Kansai Medical University, 2-5-1 Shimachi, Hirakata, Osaka, 573-1010, Japan.
    Background: Congenital nephrotic syndrome (CNS) is a rare disorder caused by various structural and developmental defects of glomeruli. It occurs typically as an isolated kidney disorder but associates sometimes with other systemic, extrarenal manifestations.

    Case Presentations: An infant presented with severe CNS, which progressed rapidly to renal failure at age of 3 months and death at 27 months. Read More

    IL-9 over-expression and Th9 polarization in Systemic Sclerosis patients.
    Clin Exp Immunol 2017 Jul 6. Epub 2017 Jul 6.
    Dipartimento Biomedico di Medicina Interna e Specialistica, Sezione di Reumatologia, Università di Palermo, Palermo.
    Th9 cells and IL-9 are involved in the pathogenesis of several autoimmune diseases. The exact role of IL-9 and Th9 cells in patients with Systemic Sclerosis (SSc) has not been yet adequately studied. IL-9, IL-9R, transcription factor PU. Read More

    A potential contribution of psoriasin to vascular and epithelial abnormalities and inflammation in systemic sclerosis.
    J Eur Acad Dermatol Venereol 2017 Jul 6. Epub 2017 Jul 6.
    Department of Dermatology, University of Tokyo Graduate School of Medicine, Tokyo, Japan.
    Background: Antimicrobial peptides have attracted much attention as a member of disease-associated molecules in systemic sclerosis (SSc), which is pathologically characterized by immune abnormalities, vasculopathy, and tissue fibrosis.

    Objective: To investigate the potential contribution of one of the anitimicrobial peptides psoriasin to the development of SSc.

    Methods: Psoriasin expression in the skin samples and sera derived from SSc patients and its correlation with clinical parameters were analyzed. Read More

    A high-yield isolation and enrichment strategy for human lung microvascular endothelial cells.
    Pulm Circ 2017 Mar 27;7(1):108-116. Epub 2017 Mar 27.
    Department of Medicine, Division of Allergy, Pulmonary and Critical Care Medicine, Vanderbilt University, Nashville, TN, USA.
    Vasculopathies, characterized by the formation of fragile and abnormal microvessels, are associated with the severity of many chronic lung diseases, including pulmonary fibrosis, emphysema/chronic obstructive pulmonary disease, systemic sclerosis, and hypertension. However, the study of human lung vasculature has been limited by the ability to isolate generous quantities of microvascular endothelial cells (MVEC) free from mesenchymal contamination. Expansion and passaging of primary human MVEC in vitro typically results in loss of a traditional phenotype in favor of an intermediate mesenchymal one, as early as passage five. Read More

    Association between CD40 rs1883832 and immune-related diseases susceptibility: a meta-analysis.
    Oncotarget 2017 Jun 28. Epub 2017 Jun 28.
    Department of Urology Surgery, The First Affliated Hospital of Xiamen University, Center of Diagnosis and Treatment of Urinary System Diseases, The First Affliated Hospital of Xiamen University, The Key Laboratory of Urinary Tract Tumors and Calculi of Xiamen City, The First Affliated Hospital of Xiamen University, Xiamen, Fujian, 361003, China.
    Background/objective: It has been reported that CD40 rs1883832 might be associated with immune-related diseases susceptibility. Owing to mixed and inconclusive results, we conducted a meta-analysis of case-control studies to summarize and clarify this association.Methods/main results: A systematic search of studies on the association between CD40 rs1883832 and immune-related diseases susceptibility was conducted in databases. Read More

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