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Clin Rheumatol 2019 Apr 19. Epub 2019 Apr 19.

Division of Diagnostic Radiology, Faculty of Medicine, Chiang Mai University, Chiang Mai, Thailand.

Objectives: To determine the prevalence of clinical manifestations and incidence rate of cardiopulmonary complications in a comparison between men and women with early SSc.

Methods: An inception cohort of early-SSc patients at the Rheumatology Clinic, Maharaj Nakorn Chiang Mai Hospital, between January 2010 and June 2016, was used. All patients were assessed for clinical manifestations and underwent ECG, echocardiography, and HRCT at the study entry and then annually. Read More

Clin Neuropsychol 2019 Apr 19:1-26. Epub 2019 Apr 19.

a University of Konstanz , Konstanz , Germany.

Objective: Limb apraxia is a motor cognitive disorder that has been mainly studied in patients with dementia or left hemisphere stroke (LHS). However, limb apraxia has also been reported in patients with right hemisphere stroke (RHS), multiple sclerosis (MS) or traumatic brain injury (TBI). This study's aim was to report detailed praxis performance profiles in samples suffering from these different neurological disorders by use of the Diagnostic Instrument for Limb Apraxia (DILA-S). Read More

Front Neurol 2019 3;10:291. Epub 2019 Apr 3.

Department of Neuroscience, Sheffield Institute for Translational Neuroscience (SITraN), University of Sheffield, Sheffield, United Kingdom.

Motor neuron disease can be viewed as an umbrella term describing a heterogeneous group of conditions, all of which are relentlessly progressive and ultimately fatal. The average life expectancy is 2 years, but with a broad range of months to decades. Biomarker research deepens disease understanding through exploration of pathophysiological mechanisms which, in turn, highlights targets for novel therapies. Read More

Arthritis Res Ther 2019 Apr 18;21(1):103. Epub 2019 Apr 18.

Department of Rheumatology, Tokyo Women's Medical University School of Medicine, 8-1, Kawada-cho, Shinjuku-ku, Tokyo, 162-8666, Japan.

Background: Cyclic phosphatidic acid (cPA) has an inhibitory effect on the autotaxin (ATX)/lysophosphatidic acid (LPA) axis, which has been implicated to play an important role in the progression of fibrosis in systemic sclerosis (SSc). The purpose of this study is to assess the antifibrotic activity of cPA for the treatment of SSc using SSc skin fibroblasts and an animal model of bleomycin-induced skin fibrosis.

Methods: We used a chemically stable derivative of cPA (2ccPA). Read More

Prog Retin Eye Res 2019 Apr 15. Epub 2019 Apr 15.

University of Iceland, Reykjavik, Iceland.

Retinal oximetry imaging of retinal blood vessels measures oxygen saturation of hemoglobin. The imaging technology is non-invasive and reproducible with remarkably low variability on test-retest studies and in healthy cohorts. Pathophysiological principles and novel biomarkers in several retinal diseases have been discovered, as well as possible applications for systemic and brain disease. Read More

RMD Open 2019 4;5(1):e000826. Epub 2019 Mar 4.

Rheumatology A Department, Universite Paris Descartes, Paris, France.

Background: Systemic sclerosis (SSc) is a severe, progressive multiorgan disease but to date, there are no established standardised international guidelines for follow-up of patients with SSc. The goal of this project was to develop an expert consensus for annual systematic investigations in patients with SSc to enhance their standard-of-care.

Material And Methods: The Delphi method was applied. Read More

Clin Transl Immunology 2019 6;8(4):e1045. Epub 2019 Apr 6.

Rheumatology Group Centre for Inflammatory Diseases School of Clinical Sciences at Monash Health Monash University Clayton VIC Australia.

Objectives: Systemic sclerosis (SSc) is an autoimmune disease characterised by fibrosis, vascular dysfunction and immune dysregulation. The pathogenesis of SSc remains poorly understood, although studies have indicated a role for the innate immune response.

Methods: Here, we measured serum interleukin (IL)-1α, IL-1β and IL-18 levels in 105 SSc patients and 47 healthy controls (HC) and analysed them with respect to multiple clinical parameters. Read More

JCI Insight 2019 Apr 18;4(8). Epub 2019 Apr 18.

Department of Physiology & Pharmacology and Program in Chemical Biology, Oregon Health & Science University, Portland, Oregon, USA.

Oligodendrocyte processes wrap axons to form neuroprotective myelin sheaths, and damage to myelin in disorders, such as multiple sclerosis (MS), leads to neurodegeneration and disability. There are currently no approved treatments for MS that stimulate myelin repair. During development, thyroid hormone (TH) promotes myelination through enhancing oligodendrocyte differentiation; however, TH itself is unsuitable as a remyelination therapy due to adverse systemic effects. Read More

J Clin Med 2019 Apr 16;8(4). Epub 2019 Apr 16.

Department of Medicine B and Zabludowicz Center for Autoimmune Diseases, Sheba Medical Center, Tel-Hashomer, Ramat-Gan 5265601, Israel.

Although skin manifestations are quite common in systemic sclerosis (SSc), a link between SSc and psoriasis (PsO) has been poorly investigated. We assessed the Clalit medical database in a cohort study to compare the prevalence of PsO between SSc-patients and SSc-free controls. We also evaluated the SSc-related autoantibodies' role in the co-existence of the two conditions. Read More

J Agric Food Chem 2019 Apr 17. Epub 2019 Apr 17.

Neuroinflammation is intensively demonstrated to be related with various neurodegenerative diseases including Parkinson disease (PD), amyotrophic lateral sclerosis (ALS), and Alzheimer disease (AD). As a natural polymethoxylated flavone, Nobiletin (NOB) is reported to alleviate oxidative stress, insulin resistance, and obesity. In this study, we evaluated the protection effects of NOB on neuroinflammation and memory deficit. Read More

Arthritis Rheumatol 2019 Apr 17. Epub 2019 Apr 17.

Department of Rheumatology, Tareev Clinic of Internal Diseases, Sechenov First Moscow State Medical University.

Interstitial lung disease (ILD) is a common manifestation of systemic sclerosis (SSc) accounting for every third SSc-related death (1). Patients with SSc-ILD have a variable clinical course and can present both with a relatively stable/slowly declining lung function or severe/rapidly progressive pulmonary fibrosis. Therefore, not all patients will require aggressive immunosuppression that can provide more harm than benefit in a proportion of cases. Read More

Int J Rheum Dis 2019 Apr 15. Epub 2019 Apr 15.

Department of Clinical Medicine, Clinical Immunology Unit-Scleroderma Center, Sapienza University of Rome, Rome, Italy.

Aim: Autonomic dysfunction (AD) is an early feature of systemic sclerosis (SSc). A regular endothelial function is a prerequisite for normal response of the myocardial blood flow (MBF) to cold pressure test (CPT). The aim of the study was to evaluate the relation between MBF and AD at rest and after CPT in asymptomatic SSc patients. Read More

Nutr Clin Pract 2019 Apr 15. Epub 2019 Apr 15.

University of Sydney, School of Life and Environmental Sciences, Sydney, Australia.

Background: The aim of this study was to systematically review effects of nutrition interventions on outcomes in patients with chronic gastrointestinal (GI) motility disorders. There is currently a lack of evidence-based guidelines for nutrition management in this group, likely a result of the rarity of the conditions.

Methods: A systematic review of all study types to evaluate current evidence-based nutrition interventions was performed using Medline, Embase, and CINAHL databases. Read More

Clin Exp Med 2019 Apr 15. Epub 2019 Apr 15.

Fondazione IRCCS Ca' Granda, Ospedale Maggiore Policlinico di Milano, Milan, Italy.

Vasculopathy is a crucial feature of systemic sclerosis (SSc), and Raynaud's phenomenon (RP) and digital ulcers (DU) have a deep impact on the quality of patients' life. The management of vascular disease can be challenging for the clinician because of the suboptimal tolerability of the treatments and lack of consensus on the best therapeutic approach. Intravenous iloprost, a synthetic analogue of prostacyclin, is broadly used for the treatment of RP and ischemic ulcers secondary to SSc. Read More

Open Access Rheumatol 2019 26;11:61-65. Epub 2019 Mar 26.

Division of Rheumatology and Allergology, St. Marianna University School of Medicine, Kawasaki City, Kanagawa, Japan,

Purpose: IL-10 is a cytokine known to inhibit inflammatory cytokines. To determine its role in the pathogenesis of systemic lupus erythematosus (SLE), the presence of anti-IL-10 antibody is required to be examined. Although antibodies against cytokines are known to be present in SLE, no studies have determined the role of IL-10, particularly in Japanese patients. Read More

Brain Nerve 2019 Apr;71(4):374-379

Dermatology, Department of Integrated Medicine, Graduate School of Medicine, Osaka University.

Tuberous sclerosis complex (TSC) is an autosomal dominant inherited disorders that is characterized by the systemic hamartomas, along with epilepsy, cognitive impairment and hypopigmented macules. It is caused by genetic mutations in either TSC1 or TSC2 gene which encodes hamartin and tuberin, respectively. As the hamartin-tuberin-complex downregulates the mechanistic/mammalian target of the rapamycin complex1 (mTORC1), dysfunction in either hamartin or tuberin induces the constitutive activation of mTORC1. Read More

Arthritis Res Ther 2019 Apr 15;21(1):95. Epub 2019 Apr 15.

Division of Rheumatology, University of California in Los Angeles (Emeritus), Los Angeles, USA.

Background/objectives: Systemic sclerosis (SSc) is an autoimmune disease associated with immune abnormalities and widespread vascular lesions, including increased intimal and medial thickness. These changes may be reflected in early atherosclerosis and cardiovascular risks. We aimed in this study to examine the carotid artery intima-media thickness and MRI brain findings in SSc patients and compared them to a group of normal controls. Read More

Arthritis Res Ther 2019 Apr 15;21(1):96. Epub 2019 Apr 15.

Department of Rheumatology and Clinical Immunology, Charité - Universitätsmedizin Berlin, Berlin, Germany.

Objective: Systemic sclerosis (SSc) can lead to ischemic complications such as digital ulcers (DUs). The aim of the study was to find predictors of DUs by clinical and new imaging methods.

Patients And Methods: All 79 SSc patients included in the study received a clinical, colour Doppler ultrasound (CDUS), fluorescence optical imaging (FOI) and capillaroscopy examination at baseline, and their capacity to predict new DU development was analysed in 76 patients at 12 months follow-up. Read More

Eur J Rheumatol 2019 Apr 9. Epub 2019 Apr 9.

Department of General Internal Medicine, Akashi Medical Center, Akashi, Japan.

In posterior reversible encephalopathy syndrome (PRES) triggered by scleroderma renal crisis (SRC), modulation and adherence in immunosuppressive therapy are key for avoiding recurrence, complications, and death. A patient with polymyositis (PM)/systemic sclerosis (SSc) overlap syndrome developed PRES triggered by SRC. To our knowledge, this is the first report of a case with PRES associated with PM/SSc overlap syndrome. Read More

GMS Ophthalmol Cases 2019 1;9:Doc07. Epub 2019 Mar 1.

Department of Internal Medicine, East Jeddah Hospital, Jeddah, Saudi Arabia.

We report a unique case of a 37-year-old patient, a known case of scleroderma, complaining of bilateral acute visual disturbance, which was diagnosed later on as Purtscher's retinopathy. After systemic assessment, she was diagnosed with bilateral kidney disease, consequently requiring further management accordingly. Read More

PeerJ 2019 5;7:e6711. Epub 2019 Apr 5.

Department of Life Sciences, Imperial College London, London, United Kingdom.

Since their discovery in the 1960s, further investigation into endogenous retroviruses (ERVs) has challenged the conventional view of viral sequences as exclusively parasitic elements. Once presumed to be a group of passive genetic relics, it is becoming increasingly clear that this view of ERVs, while generally accurate, is incorrect in many specific cases. Research has identified ERV genes that appear to be co-opted by their mammalian hosts, but the biological function of ERV elements in humans remains a controversial subject. Read More

F1000Res 2019 25;8. Epub 2019 Mar 25.

Department of Clinical and Experimental medicine, Brighton and Sussex Medical School, Brighton, Sussex, BN1 9PX, UK.

Immunosenescence is thought to contribute to the increase of autoimmune diseases in older people. Immunosenescence is often associated with the presence of an expanded population of CD4 T cells lacking expression of CD28 (CD28 ). These highly cytotoxic CD4 T cells were isolated from disease-affected tissues in patients with rheumatoid arthritis, systemic lupus erythematosus, multiple sclerosis, or other chronic inflammatory diseases and their numbers appeared to be linked to disease severity. Read More

Rev Med Interne 2019 Apr 10. Epub 2019 Apr 10.

Service de médecine interne, hôpital Joseph Ducuing, 15 rue de Varsovie, 31076 Toulouse, France.

Classification criteria for systemic sclerosis evolved over the last three decades, allowing an earlier classification. In the late 2000s, the EULAR Scleroderma Trials and Research Group validated very early and early systemic sclerosis criteria. Raynaud phenomenon, anti-nuclear antibody positivity and the puffy fingers are "Red flags" that must lead to refer the patient to a specialist and benefit from a capillaroscopy and the specific autoantibodies. Read More

Semin Arthritis Rheum 2019 Mar 9. Epub 2019 Mar 9.

Centre for Musculoskeletal Research, The University of Manchester, Salford Royal NHS Foundation Trust, Manchester Academic Health Science Centre, Manchester, UK; NIHR Manchester Biomedical Research Centre, Manchester University NHS Foundation Trust, Manchester Academic Health Science Centre, UK.

Introduction: Objective outcome measures are needed to facilitate clinical trials of much needed treatments for calcinosis in systemic sclerosis (SSc). Our primary aim was to compare radiography, computed tomography (CT) and magnetic resonance imaging (MRI) to measure calcinotic lesions. Secondary objectives included to examine reproducibility of radiography and MRI, and inter-rater reliability of MRI. Read More

J Cosmet Dermatol 2019 Apr 13. Epub 2019 Apr 13.

Department of Dermatology, Renmin Hospital of Wuhan University, Wuhan, China.

Background: Limited cutaneous systemic sclerosis is one subtype of systemic sclerosis which is characterized by a prototypic multisystem fibrotic disorder.

Objective: This study aimed to further investigate the pathological mechanism of limited cutaneous systemic sclerosis (lcSSc).

Methods: The dataset GSE76807 generated from 10 lcSSc patients and five healthy controls was used. Read More

Transplant Proc 2019 Apr 9;51(3):865-870. Epub 2019 Jan 9.

Institute for Laboratory and Transfusion Medicine, Herz- und Diabeteszentrum Nordrhein-Westfalen, Ruhr University Bochum, Bad Oeynhausen, Germany.

Background: Although low (but increasing) rates of lung/lung-heart transplantations of scleroderma (systemic sclerosis [SSc]) patients have been reported, exclusive heart transplantation is a rare approach for treatment of heart failure due to SSc.

Cases: We report on 2 cases of SSc patients receiving a heart transplantation (HTx) due to severe and progressive right heart failure without pulmonary artery hypertension. One patient received a hepatitis C virus (HCV)-positive donor heart and recovered excellently from viral transmission after administration of a direct-acting antiviral (DAA) regimen. Read More

J Clin Med 2019 Apr 11;8(4). Epub 2019 Apr 11.

Department of Medicine, Vanderbilt University School of Medicine, Nashville, TN 37232, USA.

Healthcare expenditures in the United States are growing at an alarming level with the Centers for Medicare and Medicaid Services (CMS) projecting that they will reach $5.7 trillion per year by 2026. Inflammatory diseases and related syndromes are growing in prevalence among Western societies. Read More

J Invest Dermatol 2019 Apr 9. Epub 2019 Apr 9.

Rheumatology Unit, Medical School, University of Modena and Reggio Emilia, University-Hospital Policlinico of Modena, Modena, Italy.

Parvovirus B19 (B19V) has been proposed as triggering agent of some autoimmune diseases including systemic sclerosis (SSc). In this study, we investigated whether B19V infection in vitro differently activates inflammatory pathways, including those dependent on caspase-1 activation, in monocytes from SSc patients and healthy controls. We showed that B19V can infect both THP-1 cells and primary monocytes but is not able to replicate in these cells. Read More

Arthritis Res Ther 2019 Apr 11;21(1):93. Epub 2019 Apr 11.

Department of Ultrasound, Peking University Shenzhen Hospital, Lianhua Road 1120, FuTian District, Shenzhen, 518036, Guangdong, China.

Background: Interstitial lung disease (ILD) is a common complication of connective tissue disease (CTD) and a leading cause of morbidity and mortality. There are various lung ultrasound (LUS) scoring systems with different lung intercostal spaces (LIS). The purpose of this meta-analysis was to find a simplified LUS method for the assessment of CTD-ILD. Read More

Adv Med Sci 2019 Apr 8;64(2):309-314. Epub 2019 Apr 8.

Department of Internal Medicine and Cardiology, Medical University of Warsaw, Warsaw, Poland.

Purpose: Pulmonary hypertension (PH) is an ominous complication in systemic sclerosis patients (SSc) and echocardiography is a screening tool for its detection. The goal of this study was to assess the reliability of resting and exercise echo Doppler parameters with data obtained by right heart catheterization (RHC).

Material And Methods: We included 91 patients (84 F, 53. Read More

Microvasc Res 2019 Apr 8. Epub 2019 Apr 8.

Research Laboratory and Academic Division of Clinical Rheumatology, Department of Internal Medicine (Di.M.I.), IRCCS San Martino Polyclinic Hospital, University of Genova, Genoa, Italy.

Objective: The aim of this study was to identify any correlations between microvascular damage, assessed by nailfold videocapillaroscopy and skin impairment, evaluated by three different methods, the modified Rodnan skin score (mRSS), skin high-frequency ultrasound (US) and the plicometer skin test (PST) in systemic sclerosis (SSc) patients.

Methods: Sixty-three SSc patients and 63 healthy subjects were enrolled. Nailfold videocapillaroscopy (NVC) was used to assess the nailfold capillaroscopy pattern ("Early", "Active" or "Late"), according to the Cutolo classification. Read More

Arthritis Rheumatol 2019 Apr 10. Epub 2019 Apr 10.

Univ. Lille, U995 - LIRIC - Lille Inflammation Research International Center, F-59000, Lille, France.

Objectives: Systemic sclerosis (SSc) is a heterogeneous connective tissue disease. The usual subclassification divides patients into limited (lc) and diffuse cutaneous (dc) subsets relying on the skin extension but may not capture the entire variability of clinical phenotypes. The European Scleroderma Trials and Research (EUSTAR) database is a prospective cohort providing data from 137 European referral centers. Read More

Int J Rheum Dis 2019 Apr 10. Epub 2019 Apr 10.

Servicio de Reumatología, Hospital General Universitario Gregorio Marañón, Madrid, Spain.

Background: Inflammatory idiopathic myositis (IIM) comprises a heterogeneous group of systemic muscular diseases that can occur together with other connective tissue diseases (CTD), named overlap myositis (OM). The question of whether OM is a distinct entity still remains controversial.

Aim: The present study was conducted to assess the clinical and prognostic differences between patients diagnosed with OM, primary polymyositis (PM) and primary dermatomyositis (DM). Read More

Ann Rheum Dis 2019 Apr 9. Epub 2019 Apr 9.

Cell Therapy Department, Hopital de la Conception, Marseille, France

Ann Rheum Dis 2019 Apr 9. Epub 2019 Apr 9.

Rheumatology A Department, Cochin Hospital, APHP, Paris Descartes University, Paris, France.

Objective: To assess the safety and efficacy of rituximab in systemic sclerosis (SSc) in clinical practice.

Methods: We performed a prospective study including patients with SSc from the European Scleroderma Trials and Research (EUSTAR) network treated with rituximab and matched with untreated patients with SSc. The main outcomes measures were adverse events, skin fibrosis improvement, lung fibrosis worsening and steroids use among propensity score-matched patients treated or not with rituximab. Read More

Br J Clin Pharmacol 2019 Apr 9. Epub 2019 Apr 9.

College of Pharmacy and Division of Life & Pharmaceutical Sciences, Ewha Womans University, Seoul, Korea.

Aims: To evaluate the real-world effect of DPP-4 inhibitor (DPP4i) on the incidence of autoimmune diseases (AD), including rheumatoid arthritis (RA), inflammatory bowel diseases (IBD), multiple sclerosis (MS), and systemic lupus erythematosus (SLE).

Methods: We identified new users of DPP4i (N=497 619) or non-DPP4i (N=643 165) oral combination therapy between 1 January 2011 and 30 June 2015 among patients with type 2 diabetes mellitus in the Korean national health insurance claims database. Patients were followed from the date of initiation of combination therapy until AD outcome, censoring for treatment discontinuation or switching, death or end of study (31 August 2016). Read More

Respir Med 2019 Apr 16;150:131-135. Epub 2019 Mar 16.

Comprehensive Pulmonary Hypertension Center-University Medical Center, New Orleans, LA, USA; Louisiana State University Health Sciences Center, Section of Pulmonary/Critical Care and Allergy/Immunology, New Orleans, LA, USA. Electronic address:

Background: Pulmonary hypertension (PH) often presents with non-specific symptoms making early diagnosis difficult. Red cell distribution width (RDW) is a parameter routinely reported on an automated complete blood cell count that has been associated with numerous disease states. The purpose of this study was to further evaluate RDW as a biomarker for PH in at-risk populations. Read More

Ann Med 2019 Apr 9:1-15. Epub 2019 Apr 9.

a Departments of Dermatology , Peking Union Medical College Hospital, Chinese Academy of Medical Sciences , Beijing , China , 100730.

Exosomes have generated significant interest in the last few decades owing to their important roles in a diverse range of biological pathways. They are nano-sized lipid bilayer membrane vesicles of endosomal origin, and are produced by a vast number of cell types. They are released into the extracellular environment and are found in most biological fluids. Read More

Autoimmun Rev 2019 Apr 5. Epub 2019 Apr 5.

Department of Endocrinology, Shanghai University of Medicine & Health Sciences Affiliated Zhoupu Hospital, No. 1500 Zhouyuan Road, Pudong District, Shanghai 201318, China. Electronic address:

Autoimmun Rev 2019 Apr 5. Epub 2019 Apr 5.

Department of Epidemiology and Biostatistics, School of Public Health, Anhui Medical University, 81 Meishan Road, Hefei, Anhui, China; Anhui Province Key Laboratory of Major Autoimmune Diseases, 81 Meishan Road, Hefei, Anhui, China. Electronic address:

Autoimmune diseases (ADs) are a broad spectrum of disorders featured by the body's immune responses being directed against its own tissues, resulting in prolonged inflammation and subsequent tissue damage. Recently, the exposure to ambient air pollution has been implicated in the occurrence and development of ADs. Mechanisms linking air pollution exposures and ADs mainly include systemic inflammation, increased oxidative stress, epigenetic modifications induced by exposures and immune response caused by airway damage. Read More

Orv Hetil 2019 Apr;160(15):563-572

Belgyógyászati Intézet, Klinikai Immunológiai Tanszék, Debreceni Egyetem, Általános Orvostudományi Kar Debrecen, Móricz Zs. krt. 22., 4032.

MicroRNAs (miRNAs) are 18-25 nucleotide long, single stranded, endogenous, non-coding small RNAs playing an important role in regulating gene expression at posttranscriptional level. miRNAs control approximately 90% of protein-coding genes, and play a central role in various biological processes including immune cell lineage commitment, differentiation, proliferation, apoptosis and maintenance of immune homeostasis. Changes in the expression of certain miRNAs may lead to the development of many diseases, including systemic autoimmune diseases. Read More

J Intern Med 2019 Apr 7. Epub 2019 Apr 7.

Molecular Psychoimmunology, Institute for Genetic Medicine, Graduate School of Medicine, Hokkaido University, Sapporo, 060-0815, Hokkaido, Japan.

The systemic regulation of immune reactions by the nervous system is well studied and depends on the release of hormones. Some regional regulations of immune reactions, on the other hand, depend on specific neural pathways. Better understanding of these regulations will expand therapeutic applications for neuroimmune and organ-to-organ functional interactions. Read More

Rheumatol Int 2019 Apr 6. Epub 2019 Apr 6.

Department of Epidemiology and Biostatistics, School of Public Health, Anhui Medical University, 81 Meishan Road, Hefei, Anhui, China.

This study aims to investigate the global public interest in rheumatoid arthritis by evaluating search term popularity changes of the disease over a decade. Google Trends was applied to retrieve search popularity scores for the term 'rheumatoid arthritis' between January 2004 and December 2017, utilizing the category of "health". Overall, relative searches volume for rheumatoid arthritis steadily decreased from January 2004 to December 2010, and then slowly rose from January 2011 to December 2017. Read More

J Dermatol Sci 2019 Mar 12. Epub 2019 Mar 12.

Department of Dermatology, Kagawa University, Kagawa, Japan.

Background: Systemic sclerosis (SSc) is characterized by fibrosis of the skin and internal organs. Although transforming growth factor (TGF)-β1-induced connective tissue growth factor (CTGF/CCN2) expression has been presented in SSc fibrosis, the therapeutic potential of targeting CTGF in SSc has not been fully explored. COA-Cl is a novel nucleic acid analog, which is reported to have pleiotropic beneficial biologic effects. Read More

Nat Rev Rheumatol 2019 Apr 5. Epub 2019 Apr 5.

Department of Rheumatology, Center of Experimental Rheumatology, University Hospital Zurich, Zurich, Switzerland.

Systemic sclerosis (SSc) is an autoimmune fibrotic disease of unknown aetiology that is characterized by vascular changes in the skin and visceral organs. Autologous haematopoietic stem cell transplantation can improve skin and organ fibrosis in patients with progressive disease and a high risk of organ failure, indicating that cells originating in the bone marrow are important contributors to the pathogenesis of SSc. Animal studies also indicate a pivotal function of myeloid cells in the development of fibrosis leading to changes in the tissue architecture and dysfunction in multiple organs such as the heart, lungs, liver and kidney. Read More

Ann Rheum Dis 2019 Apr 5. Epub 2019 Apr 5.

Division of Rheumatology, Department of Internal Medicine, St. Vincent's Hospital, College of Medicine, The Catholic University of Korea, Seoul, Republic of Korea

Objectives: Treatment of patients with systemic sclerosis (SSc) can be challenging because of clinical heterogeneity. Integration of genome-scale transcriptomic profiling for patients with SSc can provide insights on patient categorisation and novel drug targets.

Methods: A normalised compendium was created from 344 skin samples of 173 patients with SSc, covering an intersection of 17 424 genes from eight data sets. Read More

Neurol Clin 2019 May 16;37(2):345-357. Epub 2019 Mar 16.

Vasculitides and Scleroderma, Department of Internal Medicine, Referral Center for Rare Autoimmune and Systemic Diseases, Hôpital Cochin, Université Paris Descartes, 27, rue Fg Saint-Jacques, Paris 75679 Cedex 14, France. Electronic address:

Polyarteritis nodosa (PAN) is a necrotizing vasculitis affecting medium-sized vessels whose main manifestations are weight loss, fever, peripheral neuropathy, renal, musculoskeletal, gastrointestinal tract and/or cutaneous involvement(s), hypertension and/or cardiac failure. Peripheral neuropathy is one of the most frequent and earliest symptoms, affecting 50% to 75% of PAN patients. Central nervous system involvement affects only 2% to 10% of PAN patients, often late during the disease course. Read More

Front Immunol 2019 20;10:519. Epub 2019 Mar 20.

HSS Research Institute, Hospital for Special Surgery, New York, NY, United States.

Lymphatic vessels are critical for clearing fluid and inflammatory cells from inflamed tissues and also have roles in immune tolerance. Given the functional association of the lymphatics with the immune system, lymphatic dysfunction may contribute to the pathophysiology of rheumatic autoimmune diseases. Here we review the current understanding of the role of lymphatics in the autoimmune diseases rheumatoid arthritis, scleroderma, lupus, and dermatomyositis and consider the possibility that manual therapies such as massage and acupuncture may be useful in improving lymphatic function in autoimmune diseases. Read More

Clin Liver Dis 2019 May 8;23(2):247-261. Epub 2019 Mar 8.

Division of Gastroenterology and Hepatology, Department of Internal Medicine, Howard University Hospital and College of Medicine, 2041 Georgia Avenue Northwest, Suite 5C02, Washington, DC 20060, USA. Electronic address:

A variety of rheumatologic disorders may affect the liver. There is a significant epidemiologic, genetic, and immunologic overlap between immune-mediated rheumatologic disorders and autoimmune liver diseases. There is an increased frequency of autoimmune liver diseases, such as primary biliary cholangitis, autoimmune hepatitis, primary sclerosing cholangitis, or overlap syndrome, in patients with systemic lupus erythematosus, rheumatoid arthritis, Sjögren syndrome, systemic sclerosis, vasculitis, and other immune-related diseases. Read More

Clin Epigenetics 2019 Apr 4;11(1):58. Epub 2019 Apr 4.

Division of Rheumatology and Immunology, Department of Medicine, Medical University of South Carolina, Charleston, SC, USA.

Background: Systemic sclerosis (SSc) is a rare autoimmune fibrosing disease with an incompletely understood genetic and non-genetic etiology. Defining its etiology is important to allow the development of effective predictive, preventative, and therapeutic strategies. We conducted this epigenomic study to investigate the contributions of DNA methylation to the etiology of SSc while minimizing confounding due to genetic heterogeneity. Read More