27,002 results match your criteria Scleroderma


Propensity Score Methods in Rare Disease. A Demonstration Using Observational Data in Systemic Lupus Erythematosus.

J Rheumatol 2020 Jul 1. Epub 2020 Jul 1.

From the Division of Rheumatology, Department of Medicine, University of Toronto, Toronto, Canada; Centre for Prognosis in Rheumatic Diseases, University Health Network, Toronto, Canada; Rheumatology Unit, Department of Medicine, King Saud University, Saudi Arabia; College of Medicine Research Center, King Saud University, Saudi Arabia; Institute of Health Policy, Management and Evaluation, University of Toronto, Toronto, Ontario, Canada; The Krembil Research Institute, University Health Network, Toronto, Canada; Division of Biostatistics, Dalla Lana School of Public Health, University of Toronto, Toronto, Ontario, Canada; Program in Child Health Evaluative Sciences, SickKids Research Institute, Toronto, Ontario, Canada. Dr. Johnson is a site investigator for Scleroderma clinical trials supported by Boehringer Ingelheim, Bayer, Corbus and GlaxoSmithKline. Address correspondence to Sindhu Johnson MD PhD, Toronto Western Hospital, 399 Bathurst Street, Toronto, Ontario, Canada.

Observational studies allow researchers to understand the natural history of rheumatic conditions, risk factors for disease development, factors affecting important disease-related outcomes and estimate treatment effect from real world data. However, this design carries a risk of confounding bias. A propensity score is a balancing score that aims to minimize the difference between study groups and consequently potential confounding effects. Read More

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http://dx.doi.org/10.3899/jrheum.200254DOI Listing

Sine Scleroderma.

J Assoc Physicians India 2020 Apr;68(4):68-70

Junior Resident, RNT Medical College Udaipur, Rajasthan.

Systemic sclerosis (SSc) is a connective tissue disorder of unknown aetiology. A small subset (10%) of patients with limited systemic sclerosis have all other features of the disease without any skin involvement and is known as systemic sclerosis sine scleroderma (ssSSc). Severe Critical Limb Ischaemia is rare in sine scleroderma. Read More

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The AP1 Transcription Factor Fosl2 Promotes Systemic Autoimmunity and Inflammation by Repressing Treg Development.

Cell Rep 2020 Jun;31(13):107826

Center of Experimental Rheumatology, Department of Rheumatology, University Hospital Zurich, Zurich, Switzerland; Faculty of Medicine, University of Zurich, Zurich, Switzerland. Electronic address:

Regulatory T cells (Tregs) represent a major population in the control of immune homeostasis and autoimmunity. Here we show that Fos-like 2 (Fosl2), a TCR-induced AP1 transcription factor, represses Treg development and controls autoimmunity. Mice overexpressing Fosl2 (Fosl2) indeed show a systemic inflammatory phenotype, with immune infiltrates in multiple organs. Read More

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http://dx.doi.org/10.1016/j.celrep.2020.107826DOI Listing

Parry–Romberg syndrome

Orv Hetil 2020 07;161(28):1181-1185

Fül-Orr-Gégészeti és Fej-Nyaksebészeti Osztály,Borsod-Abaúj-Zemplén Megyei Központi Kórház és Egyetemi Oktatókórház, Miskolc, Szentpéteri kapu 72-76., 3526.

Parry-Romberg syndrome is a rare, slowly progressive, autolimitated disease, characterized by unilateral facial atrophy. It is usually manifested in childhood and young adulthood. Because of the degree of atrophy and the variety of symptoms, the diagnosis, prognosis, and treatment of Parry-Romberg syndrome are a major challenge. Read More

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http://dx.doi.org/10.1556/650.2020.31755DOI Listing

Monitoring the microcirculation in the diagnosis and follow-up of systemic sclerosis patients: focus on pulmonary and peripheral vascular manifestations.

Microcirculation 2020 Jun 30. Epub 2020 Jun 30.

Department of Experimental and Clinical Medicine, Division of Rheumatology, University of Firenze, Florence, Italy.

Systemic sclerosis (SSc) is a connective tissue disease, characterized by vascular damage and progressive fibrosis, affecting the skin and internal organs. The vascular changes include functional and structural abnormalities in the microcirculation, which play a central role not only in diagnosis, but also in the prognosis and follow-up of systemic sclerosis patients. Nailfold videocapillaroscopy (NVC) is a safe, validated, non-invasive, inexpensive, reliable and reproducible method that allows for the evaluation of structural changes in scleroderma microangiopathy. Read More

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http://dx.doi.org/10.1111/micc.12647DOI Listing

Bioactive Plasma Mitochondrial DNA is Associated with Disease Progression in Scleroderma Associated Interstitial Lung Disease.

Arthritis Rheumatol 2020 Jun 30. Epub 2020 Jun 30.

Yale University School of Medicine, Section of Pulmonary, Critical Care, and Sleep Medicine, USA.

Objective: SSc-ILD is characterized by variable clinical outcomes, activation of innate immune pattern recognition receptors (PRRs), and accumulation of αSMA expressing myofibroblasts. The association of these entities with mitochondrial DNA (mtDNA), an endogenous ligand for the intracellular DNA-sensing PRRs TLR9 and cGAS-STING, has yet to be determined.

Methods: Human lung fibroblasts from normal donors (NHLFs) and SSc-ILD explants were treated with synthetic CpG DNA and assayed for αSMA expression and extracellular mtDNA using qPCR for the human MT-ATP6 gene. Read More

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http://dx.doi.org/10.1002/art.41418DOI Listing

[Thrombotic microangiopathy in rheumatology: a link between thrombosis and autoimmunity].

Ter Arkh 2020 Jun 5;92(5):4-14. Epub 2020 Jun 5.

Uncontrolled hypercoagulation and inflammation (thromboinflammation), which are both independent and closely related and amplifying each other pathological processes, form the basis for pathogenesis of a wide range of diseases and complications, including immuno-inflammatory (autoimmune) rheumatic diseases, with the development of potentially fatal injuries of internal organs. Thrombotic microangiopathy is one of the most prominent prototypes of thromboinflammatory pathological conditions. The close link between environmental factors, hemostasis genetic defects and the complement system, inflammation and autoimmunity as pathogenetic mechanisms of microthrombosis draws particular attention to studying thrombotic microangiopathy in immuno-inflammatory rheumatic diseases, primarily systemic lupus erythematosus, antiphospholipid syndrome and scleroderma renal crisis. Read More

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http://dx.doi.org/10.26442/00403660.2020.05.000697DOI Listing

[New directions of pharmacotherapy of immune - inflammatory rheumatic diseases].

Authors:
E L Nasonov

Ter Arkh 2019 Aug 15;91(8):98-107. Epub 2019 Aug 15.

Nasonova Research Institute of Rheumatology.

Deciphering immunopathogenesis, expanding the scope of diagnostics and developing new methods for treating human autoimmune diseases are among the priority areas of XXI century medicine. Particularly widely autoimmune pathology is presented in immunoinflammatory rheumatic diseases (IIRD), such as rheumatoid arthritis, systemic lupus erythematosus, systemic scleroderma, systemic vasculitis associated with the synthesis of antineutrophilic cytoplasmic antibodies, Sjogren's syndrome, idiopathic inflammatory myopathies and other other types of others. Deciphering the pathogenesis mechanisms of IIRD created the prerequisites for improving pharmacotherapy, which in the future should lead to a dramatic improvement in the prognosis for these diseases. Read More

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http://dx.doi.org/10.26442/00403660.2019.08.000406DOI Listing

Biological effects of a new ultravioletA -prototype based on light emitting diodeson the treatment of localized scleroderma.

Exp Dermatol 2020 Jun 27. Epub 2020 Jun 27.

Department of Dermatology, University Medical Center Regensburg, Franz-Josef-Strauss Allee 11, D-93053, Regensburg, Germany.

Ultraviolet A (UVA )-phototherapy (spectral range 340-400 nm) is a well-established treatment option for various skin diseases such as localized scleroderma. Recent improvements ofconventional UVA -light sources (metal-halide or fluorescent lamps) have brought attention to a new Light Emitting Diodes (LED)-technology with remarkable advantages in handling and clinical routine. This study providesa preclinical histological and molecular evaluation of an LED-basedUVA -prototype with a narrower spectral range (360-400 nm) for treating localized scleroderma. Read More

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http://dx.doi.org/10.1111/exd.14135DOI Listing

Treatment in Juvenile Scleroderma.

Curr Rheumatol Rep 2020 Jun 26;22(8):45. Epub 2020 Jun 26.

Department of Woman's and Child's Health, University of Padua, Via Giustiniani 3, 35128, Padua, Italy.

Purpose Of Review: Treatment of scleroderma in children is challenging since little is known about its pathogenesis. Herein, we review the most recent evidence regarding the treatment of juvenile scleroderma.

Recent Findings: According to the recent recommendations for Pediatric Rheumatology in Europe (SHARE), systemic treatment in localized scleroderma is needed when there is a risk for disability, such as in generalized or pansclerotic morphea and progressive linear scleroderma. Read More

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http://dx.doi.org/10.1007/s11926-020-00910-xDOI Listing

Interaction between the promoter polymorphism and mucin expression: is there a difference according to ILD subtype?

Thorax 2020 Jun 24. Epub 2020 Jun 24.

Interstitial Lung Disease Unit, Royal Brompton and Harefield NHS Foundation Trust/ National Heart and Lung Institute, Imperial College, London, UK.

The promoter variant rs35705950 is associated with idiopathic pulmonary fibrosis (IPF). MUC5B glycoprotein is overexpressed in IPF lungs. We examined immunohistochemical expression of MUC5B in different interstitial lung disease patterns according to rs35705950 T-allele carriage. Read More

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http://dx.doi.org/10.1136/thoraxjnl-2020-214579DOI Listing

Pulmonary Hypertension Phenotypes in Systemic Sclerosis: The Right Diagnosis for the Right Treatment.

Int J Mol Sci 2020 Jun 22;21(12). Epub 2020 Jun 22.

Department of Translational Medical Sciences. Federico II University, 80131 Naples, Italy.

Systemic sclerosis is an auto-immune disease characterized by skin involvement that often affects multiple organ systems. Pulmonary hypertension is a common finding that can significantly impact prognosis. Molecular pathophysiological mechanisms underlying pulmonary hypertension in systemic sclerosis can be extremely heterogeneous, leading to distinct clinical phenotypes. Read More

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http://dx.doi.org/10.3390/ijms21124430DOI Listing

A Pediatrician's Alert: Misdiagnosis of Mixed Localized Scleroderma in a Child.

Pediatr Int 2020 Jun;62(6):759-761

Department of Dermatology, University General Hospital of Patras, Rio, Greece.

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http://dx.doi.org/10.1111/ped.14170DOI Listing

Evaluation of renal resistive index in different autoimmune diseases.

Clin Exp Rheumatol 2020 Jun 4. Epub 2020 Jun 4.

Lupus Clinic, Reumatologia, Dipartimento di Scienze Cliniche Internistiche, Anestesiologiche e Cardiovascolari, Sapienza University of Rome, Italy.

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Early detection of ventricular dysfunction in juvenile systemic sclerosis by speckle tracking echocardiography.

Rheumatology (Oxford) 2020 Jun 23. Epub 2020 Jun 23.

Department of Woman's and Child's Health, University of Padova, Padua, Italy.

Objective: Cardiac involvement is the most important cause of mortality in juvenile systemic sclerosis (JSSc). Recent reports in adult patients underline that traditional techniques of imaging are inadequate to assess the subclinical cardiac involvement, while speckle tracking echocardiography (STE) is able to identify ventricular dysfunctions in the early stages. The aim of our study was to assess the role of STE in JSSc. Read More

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http://dx.doi.org/10.1093/rheumatology/keaa208DOI Listing

Isolated Exercise-Induced Pulmonary Hypertension Associates with Higher Cardiovascular Risk in Scleroderma Patients.

J Clin Med 2020 Jun 18;9(6). Epub 2020 Jun 18.

Institute of Cardiology, University of Pisa, 56124 Pisa, Italy.

Background And Aim: Isolated exercise-induced pulmonary hypertension (ExPH) associates with cardiovascular (CV) events in patients with left heart disease. We investigated its prognostic significance in scleroderma patients at risk for pulmonary arterial hypertension (PAH).

Methods: In 26 consecutive scleroderma female patients with either low ( = 13) or intermediate probability ( = 13) of pulmonary hypertension (PH) at rest, we evaluated, both at time 0 and 1 year, prognostic determinants of CV risk: onset or progression of heart failure/syncope; worsening of functional class; functional performance at the 6-minute walking test and at cardiopulmonary exercise test; right atrial area; and pericardial effusion. Read More

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http://dx.doi.org/10.3390/jcm9061910DOI Listing

Reasons for Initiation of Areca Nut and Related Products in Patients with Oral Submucous Fibrosis within an Endemic Area in Gujarat, India.

Subst Use Misuse 2020 ;55(9):1413-1421

Menzies Health Institute Queensland, Griffith University, Queensland, Australia.

: While the patterns of consumption of areca nut and its by-products (ANBP) vary across South Asia and the rest of the world, all users share the probable severe outcome of oral submucous fibrosis (OSMF), and this is well reported. The role of psychosocial drivers of habit initiation and addiction is reported less. : To reveal the burden of ANBP abuse, exploring the psychosocial relationship between sex, age at first exposure (AFE), the reason for initiation (RFI) with the type of habit (TOH). Read More

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http://dx.doi.org/10.1080/10826084.2019.1660678DOI Listing
January 2020

Sleep Disturbance in Scleroderma.

J Clin Rheumatol 2020 Jun 19. Epub 2020 Jun 19.

From the Mackenzie Evangelical School of Medicine of Paraná, Curitiba, Brazil.

Introduction: Sleep quality may be impaired in systemic scleroderma (SSc) patients and contribute to loss of life quality.

Aim: The aims of this study were to study the prevalence of sleep disturbance in SSc patients and its possible association with epidemiological, clinical and laboratory data, treatment used, and depression, and to verify the association of sleep disturbance and quality of life in this group of patients.

Methods: This is a cross-sectional study including 60 SSc patients. Read More

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http://dx.doi.org/10.1097/RHU.0000000000001437DOI Listing

Diffuse Endobronchial Telangiectasia.

Int J Appl Basic Med Res 2020 Apr-Jun;10(2):137-139. Epub 2020 Apr 2.

Department of Medicine, Mercy Hospital, Joplin, Missouri, USA.

Hemoptysis is one of the most common reasons for seeking emergency care. Infections and malignancy are the leading causes of hemoptysis although caused by various other pulmonary and extrapulmonary conditions. Most causes are self-limiting and do not warrant any aggressive investigation. Read More

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http://dx.doi.org/10.4103/ijabmr.IJABMR_343_18DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7289210PMC

Network pharmacology strategy for predicting the correlation of Systemic Scleroderma with Vitamin D deficiency.

Int Immunopharmacol 2020 Jun 18;86:106702. Epub 2020 Jun 18.

Institute of Chemical and Industrial Bioengineering, Jilin Engineering Normal University, Changchun 130052, PR China.

The deficiency of Vitamin D (V) is a common symptom of systemic scleroderma (SSc), but the correlation of V deficiency and SSc is not completely clear. Therefore, a strategy based on network pharmacology was designed to explore the correlation of V deficiency and SSc. After a series of network construction and analysis, 5 integrins were predicated as the kernel targets in the correlation of V deficiency and SSc, including ITGA5, ITGA4, ITGB3, ITGB1 and ITGAV. Read More

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http://dx.doi.org/10.1016/j.intimp.2020.106702DOI Listing

Vertebroplasty with posterior spinal fusion for osteoporotic vertebral fracture using computer-assisted rod contouring system: A new minimally invasive technique.

Int J Surg Case Rep 2020 Jun 12;72:301-305. Epub 2020 Jun 12.

Department of Orthopaedic Surgery, School of Medicine, International University of Health and Welfare, Japan; Spine and Spinal Cord Center, International University of Health and Welfare Mita Hospital, Japan.

Introduction: Surgical treatment of osteoporotic vertebral fracture (OVF) has been challenging for spine surgeons, because there are potential risks of instrumentation failure; such as screw loosening, loss of correction, or pseudarthrosis, due to bone fragility in elderly patients with several comorbidities.

Presentation Of Case: A 68-year-old female presented with a severe low back pain and bilateral thigh pain. She had a history of systemic scleroderma, which was complicated by interstitial lung disease. Read More

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http://dx.doi.org/10.1016/j.ijscr.2020.06.009DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7305354PMC

Combined anti-fibrotic and anti-inflammatory properties of JAK-inhibitors on macrophages in vitro and in vivo: Perspectives for scleroderma-associated interstitial lung disease.

Biochem Pharmacol 2020 Jun 17;178:114103. Epub 2020 Jun 17.

Univ Rennes, CHU Rennes, Inserm, EHESP, Irset (Institut de Recherche en Santé, Environnement et Travail) - UMR_S 1085, F-35000 Rennes, France. Electronic address:

Janus kinase (JAK) inhibitors (also termed Jakinibs) constitute a family of small drugs that target various isoforms of JAKs (JAK1, JAK2, JAK3 and/or tyrosine kinase 2 (Tyk2)). They exert anti-inflammatory properties linked, in part, to the modulation of the activation state of pro-inflammatory M1 macrophages. The exact impact of JAK inhibitors on a wider spectrum of activation states of macrophages is however still to be determined, especially in the context of disorders involving concomitant activation of pro-inflammatory M1 macrophages and profibrotic M2 macrophages. Read More

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http://dx.doi.org/10.1016/j.bcp.2020.114103DOI Listing

Expert Perspective: Challenges in Scleroderma.

Authors:
Robert W Simms

Arthritis Rheumatol 2020 Jun 20. Epub 2020 Jun 20.

Professor of Medicine, Geisel School of Medicine at Dartmouth, Emeritus Professor of Medicine, Boston University School of Medicine, One Medical Center Drive, Lebanon, NH, 03756, United States.

You are consulted to evaluate a 56-year-old woman with known Raynaud's phenomenon, swollen fingers for several months and new hypertension with a blood pressure of 160/100. She also complains of progressive shortness of breath. Her examination shows telangiectasias, sclerodactly and proximal skin sclerosis (thick shiny skin on chest and upper arms), and bibasilar crackles on chest exam. Read More

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http://dx.doi.org/10.1002/art.41406DOI Listing

Mitochondria, Aging, and Cellular Senescence: Implications for Scleroderma.

Curr Rheumatol Rep 2020 Jun 19;22(8):37. Epub 2020 Jun 19.

Department of Medicine, Aging Institute, University of Pittsburgh, 560 Bridgeside Point 1 Bldg.100 Technology Drive, Pittsburgh, PA, 15219, USA.

Purpose Of Review: The etiology of systemic sclerosis (SSc), which is a rare immune-mediated inflammatory disease characterized by vascular damage and fibrosis, is still unknown. However, different intrinsic (genetics) and extrinsic (environmental) factors play a part in the progression of the disease. This review focuses on the role of aging, mitochondrial dysfunction, and senescence in SSc. Read More

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http://dx.doi.org/10.1007/s11926-020-00920-9DOI Listing

Genome-wide whole blood transcriptome profiling in a large European cohort of systemic sclerosis patients.

Ann Rheum Dis 2020 Jun 19. Epub 2020 Jun 19.

Instituto de Parasitologia y Biomedicina Lopez-Neyra, Granada, Spain.

Objectives: The analysis of annotated transcripts from genome-wide expression studies may help to understand the pathogenesis of complex diseases, such as systemic sclerosis (SSc). We performed a whole blood (WB) transcriptome analysis on RNA collected in the context of the European PRECISESADS project, aiming at characterising the pathways that differentiate SSc from controls and that are reproducible in geographically diverse populations.

Methods: Samples from 162 patients and 252 controls were collected in RNA stabilisers. Read More

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http://dx.doi.org/10.1136/annrheumdis-2020-217116DOI Listing

Autoantibodies to stratify systemic sclerosis patients into clinically actionable subsets.

Autoimmun Rev 2020 Jun 14:102583. Epub 2020 Jun 14.

Department of Medicine, Cumming School of Medicine, University of Calgary, Calgary, Alberta, Canada.

Systemic sclerosis (SSc) is a rare chronic disease of unknown etiology characterized by vascular abnormalities and fibrosis involving the skin and internal organs, especially the gastrointestinal tract, lung, heart and kidneys. Although the disease was historically stratified according to the extent of skin involvement, more recent approaches place more emphasis on patterns and extent of internal organ involvement. Despite numerous clinical trials in SSc, disease-modifying treatment options are still limited resulting in persistent poor quality of life and high mortality. Read More

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http://dx.doi.org/10.1016/j.autrev.2020.102583DOI Listing

Complement and Renal Thrombotic Microangiopathy Associated With Hypertension and Scleroderma.

Adv Chronic Kidney Dis 2020 Mar;27(2):149-154

Department of Pathology, University of Chicago Medicine, Chicago, IL. Electronic address:

Thrombotic microangiopathy is characterized by the presence of thrombocytopenia and microangiopathic hemolytic anemia and can occur in up to 50% of patients with hypertensive emergency and 10-15% with scleroderma. This review discusses the emerging role of complement in these 2 clinical entities. Specifically, we evaluate the evidence linking complement dysregulation with the manifestation of thrombotic microangiopathy and its clinical course in these settings. Read More

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http://dx.doi.org/10.1053/j.ackd.2019.11.002DOI Listing

Initial predictors of skin thickness progression in patients with diffuse cutaneous systemic sclerosis: results from a multicentre prospective cohort in Japan.

Mod Rheumatol 2020 Jun 18:1-18. Epub 2020 Jun 18.

Department of Molecular Pathology of Skin, Faculty of Medicine, Institute of Medical, Pharmaceutical and Health Sciences, Kanazawa University, Kanazawa, Japan.

To identify initial parameters that predict worsening of skin thickening in patients with diffuse cutaneous systemic sclerosis (dcSSc) using a multicentre, prospective, observational cohort in Japan. A total of 171 patients with dcSSc were selected from a prospective cohort database based on the following criteria: dcSSc, modified Rodnan total skin thickness score (mRSS) ≥7, disease duration <60 months, and valid mRSS data at one year. Worsening of skin thickness was defined as an increase in mRSS ≥3 points and an increase ≥25% from baseline to one year. Read More

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http://dx.doi.org/10.1080/14397595.2020.1784548DOI Listing

Microvascular Damage in a Young Female Archer Assessed by Nailfold Videocapillaroscopy: A Case Report.

Int J Environ Res Public Health 2020 Jun 12;17(12). Epub 2020 Jun 12.

Department of Life, Health and Environmental Sciences, University of L'Aquila, 67100 L'Aquila, Italy.

Archers are known to be exposed to the risk of developing various injuries, including less described microvascular damages, which can however heavily affect the performance of athletes. Nailfold videocapillaroscopy is a safe, proven and non-invasive method that allows us to examine the nail capillaries and diagnose vascular anomalies in athletes suffering from the consequences of microtrauma caused by repeated use of fingertips. The detection of defined capillaroscopic pictures is the basis for the follow-up and suggests carrying out further clinical investigations to exclude underlying pathologies. Read More

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http://dx.doi.org/10.3390/ijerph17124218DOI Listing

A Case of Extensive Debilitating Generalized Morphea.

Cureus 2020 May 14;12(5):e8117. Epub 2020 May 14.

Family Medicine, Southern Illinois University School of Medicine, Springfield, USA.

Morphea, also known as localized scleroderma, is an uncommon idiopathic inflammatory disorder leading to the development of sclerotic plaques in the skin. The disorder preferentially affects females. The pathogenesis of morphea is not well-understood. Read More

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http://dx.doi.org/10.7759/cureus.8117DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7292686PMC

Morphea (Localized Scleroderma) Presenting With Recurrent Skin and Soft Tissue Infections: A Diagnostic Dilemma.

Cureus 2020 May 12;12(5):e8067. Epub 2020 May 12.

Infectious Diseases, Northwell Health, Manhasset, USA.

Morphea or localized scleroderma is reported to be triggered through diverse stimuli. We present a case of morphea that presented as a non-healing wound with superimposed methicillin-sensitive (MSSA) infection. In our case, morphea was thought to have been potentially triggered by a post-surgical infection. Read More

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http://dx.doi.org/10.7759/cureus.8067DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7290118PMC

Cerebellar Atrophy in Craniofacial Morphea: A Report of 2 Cases with 1-year Neuroimaging Followup.

J Rheumatol 2020 Jun 15. Epub 2020 Jun 15.

From the Division of Dermatology, Department of Medicine, Section of Community Pediatrics, Section of Pediatric Rheumatology, Department of Pediatrics, Dermatology Research Institute, University of Calgary, Calgary, Alberta, Canada. Address correspondence to Dr. N.J. Luca, ACH C4-348, Alberta Children's Hospital, 28 Oki Drive, Calgary, Alberta T3B 6A8, Canada. E-mail:

Brain magnetic resonance imaging (MRI) findings in craniofacial morphea including en coup de sabre (ECDS) localized scleroderma and Parry-Romberg syndrome (PRS; progressive facial hemiatrophy) are not unusual, but the relevance of rare abnormalities is unknown. We report 2 cases in which distant cerebellar lesions on brain MRI occurred with varying clinical neurologic manifestations. Read More

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http://dx.doi.org/10.3899/jrheum.190808DOI Listing

Risk of scleroderma according to the type of immune checkpoint inhibitors.

Autoimmun Rev 2020 Jun 12:102596. Epub 2020 Jun 12.

Regional Center of Pharmacovigilance, Department of Pharmacology, Cochin Hospital, Paris, France.

Introduction: Immune checkpoint inhibitors (ICIs) are associated with immune-related adverse events (irAEs). Among them, ICIs-induced systemic sclerosis (SSc) is poorly known.

Methods: To better characterize this irAE, our comprehensive approach combined the description of ICIs-induced scleroderma cases, the systematic review of the literature and the analysis of VigiBase, the WHO pharmacovigilance database. Read More

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http://dx.doi.org/10.1016/j.autrev.2020.102596DOI Listing

The first composite score predicting Digital Ulcers in systemic sclerosis patients using Clinical data, Imaging and Patient history-CIP-DUS.

Arthritis Res Ther 2020 06 15;22(1):144. Epub 2020 Jun 15.

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

Background: Digital ulcers (DU) present a challenging complication in systemic sclerosis (SSc). The aim of this study was to combine clinical characteristics and imaging methods to a composite score for the prediction of DU in SSc patients.

Methods: Seventy-nine SSc patients received clinical examination, their patient history was taken and nailfold capillaroscopy (NC), colour Doppler ultrasonography (CDUS) and fluorescence optical imaging (FOI) of the hands were performed at baseline. Read More

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http://dx.doi.org/10.1186/s13075-020-02235-7DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7294661PMC

Drug-induced rheumatic syndromes: the need to be aware.

J R Coll Physicians Edinb 2020 03;50(1):8-9

Department of Clinical Immunology and Rheumatology, King George's Medical University, Lucknow, India.

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http://dx.doi.org/10.4997/JRCPE.2020.103DOI Listing

Immune checkpoint inhibitor-induced myositis, the earliest and most lethal complication among rheumatic and musculoskeletal toxicities.

Autoimmun Rev 2020 Jun 11:102586. Epub 2020 Jun 11.

Cardio-Oncology Program, Department of Medicine, Vanderbilt University Medical Center, Nashville, TN, USA; Regional Pharmacovigilance Centre, Department of Pharmacology, Sorbonne Université, INSERM CIC Paris-Est, Assistance Publique Hôpitaux de Paris, Pitié-Salpêtrière Hospital, Paris, France. Electronic address:

Background: In addition to restoring anti-tumor immune responses, immune checkpoint inhibitors (ICI) may also induce immune-related adverse events (irAE) that can affect any organ. We aim to determine the spectrum, timing, clinical features, and fatalities of rheumatic and musculoskeletal Immune-related adverse events (RMS-irAE) associated with ICI.

Patients Methods: We performed an observational, retrospective, pharmacovigilance study using the World Health Organization international pharmacovigilance database, VigiBase, from inception to January 2019. Read More

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http://dx.doi.org/10.1016/j.autrev.2020.102586DOI Listing

[Linear scleroderma "en coup de sabre" causing severe neurological and ocular symptoms].

Hautarzt 2020 Jun 12. Epub 2020 Jun 12.

Hautklinik, Universitätsklinikum Heidelberg, Heidelberg, Deutschland.

We present a rare case of a 4-year-old girl suffering from a sclerotic lesion on the forehead as well as uveitis and epileptic seizures. The patient was diagnosed with linear scleroderma "en coup de sabre" (LSECDS). Faced with no gold standard for treatment of LSECDS with severe extracutaneous manifestations, the case was intensively discussed within an interdisciplinary team setting and successfully treated. Read More

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http://dx.doi.org/10.1007/s00105-020-04621-3DOI Listing

What narrative devices do people with systemic sclerosis use to describe the experience of pain from digital ulcers: a multicentre focus group study at UK scleroderma centres.

BMJ Open 2020 Jun 11;10(6):e037568. Epub 2020 Jun 11.

Musculoskeletal Research Unit, Translational Health Sciences, Bristol Medical School, University of Bristol, Bristol, UK

Objectives: Digital ulcers (DUs) are a common complication in systemic sclerosis (SSc). No existing studies have specifically reported on the qualitative patient experience of DU pain, and our current patient-reported outcome measure (PROM) does not capture the multifaceted painful experience of SSc-DU. Our aim was to examine the patient experience of SSc-DU pain. Read More

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http://dx.doi.org/10.1136/bmjopen-2020-037568DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7295424PMC

Validity and reliability of the Mouth Handicap of Systemic Sclerosis questionnaire in a UK population.

J Oral Pathol Med 2020 Jun 12. Epub 2020 Jun 12.

Oral Medicine, UCL Eastman Dental Institute, London, United Kingdom.

Background: The objective of this study was to explore the psychometric properties of the Mouth Handicap in Systemic Sclerosis (MHISS) specifically exploring aspects of validity and reliability of MHISS in UK SSc patients.

Methods: 150 questionnaires were distributed in this study at the Oral Medicine Department of UCLHT Eastman Dental Hospital, the Outpatient Rheumatology Clinic of the Royal Free Hospital and Scleroderma Family Day. Participants were asked to complete three questionnaires (SF-36, OHIP-14 and MHISS) along with a proforma of demographic and disease-specific questions. Read More

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http://dx.doi.org/10.1111/jop.13060DOI Listing

Renal disorders in rheumatologic diseases: the spectrum is changing (Part 1: connective tissue diseases).

J Nephrol 2020 Jun 11. Epub 2020 Jun 11.

Division of Nephrology, Fondazione Ca' Granda IRCCS Ospedale Maggiore Policlinico Milano, Milano, Italy.

The kidney is frequently involved by autoimmune rheumatic diseases. The renal manifestations may be variable, ranging from asymptomatic proteinuria and microscopic haematuria to nephrotic syndrome and rapidly progressive glomerulonephritis or vasculitis. In a number of cases the kidney involvement is related to the treatment of the original disease and may represent a major cause of morbidity and mortality. Read More

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http://dx.doi.org/10.1007/s40620-020-00772-7DOI Listing

The potential for early diagnosis of pulmonary arterial hypertension using lung iodine-123-metaiodobenzylguanidine (I-MIBG) uptake: A case report.

Radiol Case Rep 2020 Aug 5;15(8):1164-1167. Epub 2020 Jun 5.

Department of Cardiovascular Medicine and Hypertension, Graduate School of Medical and Dental Sciences, Kagoshima University, 8-35-1 Sakuragaoka, Kagoshima 890-8520, Japan.

Previous reports have found evidence that the lung uptake of iodine-123-metaiodobenzylguanidine (I-MIBG) represents pulmonary vascular endothelial function. Therefore, it was believed that the reduced lung uptake of I-MIBG in patients with pulmonary artery hypertension may indicate poor pulmonary vascular endothelial function in those patients. In our previous report, we analyzed the lung uptake of I-MIBG in patients with pulmonary hypertension, and demonstrated that it is lower in patients with pulmonary arterial hypertension (PAH) than in those with chronic thromboembolic pulmonary hypertension and controls, suggesting that reduced uptake of I-MIBG in patients with PAH indicates endothelial dysfunction of the pulmonary artery. Read More

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http://dx.doi.org/10.1016/j.radcr.2020.05.036DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7281806PMC

Unilateral Neuroimaging Findings in Pediatric Craniofacial Scleroderma: Parry-Romberg Syndrome and En Coup de Sabre.

J Child Neurol 2020 Jun 12:883073820931253. Epub 2020 Jun 12.

Division of Pediatric Rheumatology, Department of Pediatrics, UPMC Children's Hospital of Pittsburgh, Pittsburgh, PA, USA.

Objective: Parry-Romberg syndrome (PRS) and en coup de sabre (ECDS) are subtypes of craniofacial localized scleroderma. Systematic analyses of central nervous system imaging findings and their clinical associations in children are lacking. Here, we aim to characterize neuroimaging findings and associated neurological symptoms in these conditions. Read More

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http://dx.doi.org/10.1177/0883073820931253DOI Listing

Effects of Polyurethane Foam Dressings as an Add-on Therapy in the Management of Digital Ulcers in Scleroderma Patients.

Transl Med UniSa 2020 May 31;22:10-14. Epub 2020 May 31.

Department of Translational Medical Sciences and Center for Basic and Clinical Immunology Research (CISI), University of Naples Federico II, Naples, Italy.

Digital ulcers (DUs) represent a severe and common complication occurring in patients affected by Systemic Sclerosis (SSc), with a consistent impact on the quality of life and often resulting in longer hospitalization than unaffected patients. Conventional treatment of SSc ulcers consists of both topical and systemic (oral or intravenous) pharmacological therapies. Several surgical options are also available, but there is overall a lack of official guidelines or recommendations. Read More

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http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7265916PMC

The contribution of epigenetics to the pathogenesis and gender dimorphism of systemic sclerosis: a comprehensive overview.

Ther Adv Musculoskelet Dis 2020 6;12:1759720X20918456. Epub 2020 May 6.

Department of Experimental and Clinical Medicine, Division of Rheumatology, University of Florence and Scleroderma Unit, Azienda Ospedaliero-Universitaria Careggi (AOUC), Florence, Italy.

Systemic sclerosis (SSc) is a life-threatening connective tissue disorder of unknown etiology characterized by widespread vascular injury and dysfunction, impaired angiogenesis, immune dysregulation and progressive fibrosis of the skin and internal organs. Over the past few years, a new trend of investigations is increasingly reporting aberrant epigenetic modifications in genes related to the pathogenesis of SSc, suggesting that, besides genetics, epigenetics may play a pivotal role in disease development and clinical manifestations. Like many other autoimmune diseases, SSc presents a striking female predominance, and even if the reason for this gender imbalance has yet to be completely understood, it appears that the X chromosome, which contains many gender and immune-related genes, could play a role in such gender-biased prevalence. Read More

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http://dx.doi.org/10.1177/1759720X20918456DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7236401PMC

Renal Involvement in Systemic Sclerosis: An Update.

Kidney Blood Press Res 2020 Jun 10:1-17. Epub 2020 Jun 10.

Department of Dermatology, Medical University of Warsaw, Warsaw, Poland.

Background: Systemic sclerosis is an immune-mediated rheumatic disease characterized by vascular abnormalities, tissue fibrosis and autoimmune phenomena.

Summary: Renal disease occurring in patients with systemic sclerosis may have a variable clinicopathological picture. The most specific renal condition associated with systemic sclerosis is scleroderma renal crisis, characterized by acute onset of renal failure and severe hypertension. Read More

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http://dx.doi.org/10.1159/000507886DOI Listing

Protocol for a partially nested randomised controlled trial to evaluate the effectiveness of the scleroderma patient-centered intervention network COVID-19 home-isolation activities together (SPIN-CHAT) program to reduce anxiety among at-risk scleroderma patients.

J Psychosom Res 2020 May 14;135:110132. Epub 2020 May 14.

Northwestern Scleroderma Program, Feinberg School of Medicine, Northwestern University, Chicago, IL, USA.

Objective: Contagious disease outbreaks and related restrictions can lead to negative psychological outcomes, particularly in vulnerable populations at risk due to pre-existing medical conditions. No randomised controlled trials (RCTs) have tested interventions to reduce mental health consequences of contagious disease outbreaks. The primary objective of the Scleroderma Patient-centered Intervention Network COVID-19 Home-isolation Activities Together (SPIN-CHAT) Trial is to evaluate the effect of a videoconference-based program on symptoms of anxiety. Read More

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http://dx.doi.org/10.1016/j.jpsychores.2020.110132DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7224675PMC

Anti-Ku antibody-positive systemic sclerosis-polymyositis overlap syndrome in an adolescent.

Pediatr Dermatol 2020 Jun 10. Epub 2020 Jun 10.

Departments of Dermatology (Pediatric Dermatology) and Pediatrics, Medical College of Wisconsin, Milwaukee, Wisconsin, USA.

Systemic sclerosis-polymyositis overlap syndrome is rare in children. Anti-PM/Scl is the most common autoantibody associated with this syndrome. We present a case of systemic sclerosis-polymyositis overlap syndrome in a child with isolated anti-Ku antibodies, an uncommon antibody associated with this rare syndrome. Read More

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http://dx.doi.org/10.1111/pde.14243DOI Listing

Outcome of pulse oral cyclophosphamide therapy in scleroderma interstitial lung disease.

Clin Rheumatol 2020 Jun 9. Epub 2020 Jun 9.

Department of Medicine, Faculty of Medicine, Khon Kaen University, Khon Kaen, 40002, Thailand.

Introduction: Standard regimens for scleroderma interstitial lung diseases (SSc-ILDs) are pulse intravenous (IV) and oral daily cyclophosphamide (CYC). However, IV CYC has limited access to diffuse cutaneous SSc, and oral daily CYC is associated with febrile neutropenia and hemorrhagic cystitis. Pulse oral CYC regimen has never been studied. Read More

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http://dx.doi.org/10.1007/s10067-020-05217-xDOI Listing

The systemic sclerosis patient in the COVID-19 era: the challenging crossroad between immunosuppression, differential diagnosis and long-term psychological distress.

Clin Rheumatol 2020 Jul 8;39(7):2043-2047. Epub 2020 Jun 8.

Department of Experimental and Clinical Medicine, Department of Geriatric Medicine, Division of Rheumatology AOUC & Scleroderma Unit, University of Florence, Florence, Italy.

COVID-19 is a world health emergency which may inevitably affect the management of a complex autoimmune disease such as systemic sclerosis (SSc). Several SSc patients are frail and, in this pandemic, need a careful protection. The COVID-19 infection might complicate the clinical scenario of interstitial lung disease (ILD) in SSc because it determines a severe pneumonia characterized by radiological features similar to SSc-ILD. Read More

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http://dx.doi.org/10.1007/s10067-020-05193-2DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7276334PMC