26,277 results match your criteria Diffuse Sclerosis


Juvenile polyautoimmunity in a rheumatology setting.

Autoimmun Rev 2019 Feb 14. Epub 2019 Feb 14.

Center for Autoimmune Diseases Research (CREA), School of Medicine and Health Sciences, Universidad del Rosario, Bogota, Colombia.. Electronic address:

Overt polyautoimmunity (PolyA) corresponds to the presence of more than one well-defined autoimmune disease (AD) manifested clinically in a single patient. The current study aimed to describe the main characteristics of juvenile PolyA in a pediatric rheumatology setting and analyze the chronological aspects, index cases, familial autoimmunity, and clustering pattern. This was a cross-sectional and multicenter study in which 313 children with overt PolyA were included. Read More

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http://dx.doi.org/10.1016/j.autrev.2018.11.006DOI Listing
February 2019
1 Read

Elevated kynurenine levels in diffuse cutaneous and anti-RNA polymerase III positive systemic sclerosis.

Clin Immunol 2019 Feb 13. Epub 2019 Feb 13.

Royal Free Hospital, Centre for Rheumatology and Connective Tissue Diseases, UCL, London, UK. Electronic address:

Systemic sclerosis (SSc) is a systemic disease characterized by vasculopathy, progressive fibrosis and autoimmune activation. Tryptophan (Trp) metabolism has been linked to altered immune cell function and to malignancy. We have investigated the role of Trp metabolic pathway in SSc measuring serum Trp, Kynurenine (Kyn) and Trp/Kyn ratio in a cohort of 97 SSc patients and 10 healthy controls. Read More

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http://dx.doi.org/10.1016/j.clim.2018.12.009DOI Listing
February 2019
2 Reads

Lysyl oxidase-a possible role in systemic sclerosis-associated pulmonary hypertension: a multicentre study.

Rheumatology (Oxford) 2019 Feb 15. Epub 2019 Feb 15.

Rheumatology Unit, Bnai-Zion Medical Center, Haifa, Israel.

Objective: Lysyl oxidase (LOX) is an extracellular enzyme that cross-links collagen fibrils. LOX was found to be increased in serum of SSc patients and was suggested to be related to skin fibrosis, yet a vascular source of LOX has been demonstrated in idiopathic pulmonary arterial hypertension (iPAH). We aimed to validate elevated LOX serum levels in SSc and to study its correlation with clinical characteristics and investigate its main source at the tissue level. Read More

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http://dx.doi.org/10.1093/rheumatology/kez035DOI Listing
February 2019
3 Reads

Cyclophosphamide for Systemic Sclerosis-related Interstitial Lung Disease: A Comparison of Scleroderma Lung Study I and II.

J Rheumatol 2019 Feb 15. Epub 2019 Feb 15.

From the Department of Medicine and Department of Radiology, University of California, Los Angeles, David Geffen School of Medicine, Los Angeles, California; Department of Biomathematics, University of California, Los Angeles, California; Department of Medicine, University of Michigan Medical School, Ann Arbor, Michigan, USA; Department of Rheumatology, Oslo University Hospital, Oslo, Norway. This work was supported by grants from the US National Heart, Lung, and Blood Institute/ National Institutes of Health (NIH): R01 HL089758 (DPT) and R01 HL089901 (RME), NIH/National Center for Advancing Translational Science, University of California, Los Angeles CTSI Grant Number UL1TR000124 (NL), the Scleroderma Foundation (ERV), and the Rheumatology Research Foundation (ERV). E.R. Volkmann, MD, MS, Assistant Professor, Department of Medicine, University of California, Los Angeles, David Geffen School of Medicine; D.P. Tashkin, MD, Emeritus Professor, Department of Medicine, University of California, Los Angeles, David Geffen School of Medicine; M. Sim, PhD, Associate Professor, Department of Medicine, University of California, Los Angeles, David Geffen School of Medicine; N. Li, PhD, Associate Professor, Department of Biomathematics, University of California, Los Angeles; D. Khanna, MD, MS, Department of Medicine, Professor, University of Michigan Medical School; M.D. Roth, MD, Professor, Department of Medicine, University of California, Los Angeles, David Geffen School of Medicine; P.J. Clements, MD, MPH, Professor, Department of Medicine, University of California, Los Angeles, David Geffen School of Medicine; A.M. Hoffmann-Vold, MD, PhD, Postdoctoral Candidate, Department of Rheumatology, Oslo University Hospital; D.E. Furst, MD, Emeritus Professor, Department of Medicine, University of California, Los Angeles, David Geffen School of Medicine; G. Kim, PhD, Associate Professor, Department of Radiology, University of California, Los Angeles, David Geffen School of Medicine; J. Goldin, MD, PhD, Professor, Department of Radiology, University of California, Los Angeles, David Geffen School of Medicine; R.M. Elashoff, PhD, Distinguished Professor, Department of Biomathematics, University of California, Los Angeles. Address correspondence to Dr. E.R. Volkmann, 1000 Veteran Ave., Ste. 32-59, Los Angeles, California 90095, USA. E-mail: Accepted for publication October 18, 2018.

Objective: To compare safety and efficacy outcomes between the cyclophosphamide (CYC) arms of Scleroderma Lung Study (SLS) I and II.

Methods: Participants enrolled in the CYC arms of SLS I (n = 79) and II (n = 69) were included. SLS I and II randomized participants to oral CYC for 1 year and followed patients for an additional year off therapy (in SLS II, patients received placebo in Year 2). Read More

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http://dx.doi.org/10.3899/jrheum.180441DOI Listing
February 2019
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Long-term Efficacy and Safety of Monotherapy Versus Combination Therapy in Systemic Sclerosis-associated Pulmonary Arterial Hypertension: A Retrospective Cohort Study from the Nationwide Spanish Scleroderma Registry (RESCLE).

J Rheumatol 2019 Feb 15. Epub 2019 Feb 15.

From the Autoimmune Diseases Unit. Department of Internal Medicine. Bellvitge University Hospital. L'Hospitalet de Llobregat, Barcelona, Spain; Department of Internal Medicine. Corporación Sanitaria Universitaria Parc Taulí. Sabadell, Barcelona, Spain; Unit of Autoimmune Diseases, Department of Internal Medicine. Hospital Universitario Vall d'Hebron. Barcelona, Spain; Unit of Systemic Autoimmune Diseases and Thrombosis. Department of Internal Medicine. Complejo Hospitalario Universitario de Vigo. Vigo, Pontevedra, Spain; Department of Internal Medicine. Hospital Universitario Virgen de las Nieves. Granada, Spain; Department of Internal Medicine. Hospital Universitario y Politécnico La Fe. Valencia, Spain; Department of Internal Medicine. Hospital Universitario Mútua Terrassa. Terrassa, Barcelona, Spain; Unit of Autoimmune Diseases, Department of Internal Medicine. Hospital Clínico Universitario Lozano Blesa. Zaragoza, Spain; Department of Systemic Autoimmune Diseases, Clinical Institute of Medicine and Dermatology. Hospital Universitario Clinic. Barcelona, Spain; Department of Internal Medicine. Hospital Universitario Central de Asturias. Oviedo, Asturias, Spain; Unit of Systemic Autoimmune Diseases, Department of Internal Medicine. Hospital Campus de la Salud, Complejo Universitario de Granada. Granada, Spain; Unit of Systemic Autoimmune Diseases, Department of Internal Medicine. Hospital de Cabueñes. Gijón, Asturias, Spain; Department of Internal Medicine, Complejo Asistencial Universitario de Salamanca, Spain; Unit of Systemic Autoimmune Diseases, Department of Internal Medicine. Consorci Hospitalari de Vic. Vic, Barcelona, Spain; Department of Internal Medicine. Hospital Universitario Rey Juan Carlos. Móstoles, Madrid, Spain; Faculty of Medicine and Health Sciences. Universitat Internacional de Catalunya,Barcelona,Spain. Address correspondence to: Melani Pestaña-Fernández MD; Bellvitge University Hospital, Department of Internal Medicine, Feixa Llarga s/n 08907 L'Hospitalet de Llobregat Barcelona (Spain). Email:

Objective: Monotherapy is an option as first line therapy for pulmonary arterial hypertension (PAH). However, combination therapy is a beneficial alternative. Our objective was to evaluate efficacy of monotherapy vs. Read More

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http://dx.doi.org/10.3899/jrheum.180595DOI Listing
February 2019
3.187 Impact Factor

Clinical features of systemic sclerosis patients with anti-RNA polymerase III antibody in a single centre in Spain.

Clin Exp Rheumatol 2019 Feb 11. Epub 2019 Feb 11.

Unit of Systemic Autoimmune Diseases, Institut de Recerca Vall d'Hebron, Hospital Universitari Vall d'Hebron, Universitat Autònoma de Barcelona, Spain.

Objectives: To evaluate the clinical features and survival of patients with positive anti-RNA polymerase III (anti-RNAP III) in a Spanish single centre.

Methods: We analysed 221 patients with SSc according to LeRoy and Medsger criteria. Twenty-six patients with positivity for anti-RNAP III antibodies were compared with 195 negative patients. Read More

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February 2019
2 Reads

Significance of anti-neutrophil cytoplasmic antibodies in systemic sclerosis.

Arthritis Res Ther 2019 Feb 14;21(1):57. Epub 2019 Feb 14.

The University of Melbourne, 41 Victoria Parade Fitzroy, Melbourne, Victoria, 3065, Australia.

Background: Up to 12% of patients with systemic sclerosis (SSc) have anti-neutrophil cytoplasmic antibodies (ANCA). However, the majority of these patients do not manifest ANCA-associated vasculitis (AAV) and the significance of ANCA in these patients is unclear. The aim of this study is to determine the prevalence of ANCA in a well-characterised SSc cohort and to examine the association between ANCA and SSc clinical characteristics, other autoantibodies, treatments and mortality. Read More

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https://arthritis-research.biomedcentral.com/articles/10.118
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http://dx.doi.org/10.1186/s13075-019-1839-5DOI Listing
February 2019
2 Reads

Prevalence, Treatment and Outcomes of Coexistent Pulmonary Hypertension and Interstitial Lung Disease in Systemic Sclerosis.

Arthritis Rheumatol 2019 Feb 14. Epub 2019 Feb 14.

Division of Rheumatology, Department of Internal Medicine, University of Michigan.

Objective: Systemic sclerosis (SSc) is associated with interstitial lung disease (ILD) and pulmonary hypertension (PH). We sought to determine prevalence, characteristics, treatment, and outcomes for subjects with PH in a SSc-associated ILD (SSc-ILD) cohort.

Methods: Subjects with SSc-ILD on high resolution computed tomography (HRCT) were included in a prospective observational cohort. Read More

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http://dx.doi.org/10.1002/art.40862DOI Listing
February 2019
1 Read

Scleroderma patient perspectives on social support from close social relationships.

Disabil Rehabil 2019 Feb 14:1-11. Epub 2019 Feb 14.

a Lady Davis Institute for Medical Research, Jewish General Hospital , Montreal , Canada.

Purpose: People with rare diseases must cope with many physical and psychological challenges; an endeavor that can be difficult to carry out without external support. The purpose of this study was to understand how patients with scleroderma perceive the social support they need and receive from close relationships to better manage their disease.

Method: Four focus groups with patients (N = 19) were conducted. Read More

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http://dx.doi.org/10.1080/09638288.2018.1531151DOI Listing
February 2019
1 Read

Axonal water fraction as marker of white matter injury in primary-progressive multiple sclerosis: a longitudinal study.

Eur J Neurol 2019 Feb 13. Epub 2019 Feb 13.

Department of Neurology, Icahn School of Medicine at Mount Sinai, New York, NY, USA.

Background: Diffuse white matter (WM) injury is prominent in primary progressive multiple sclerosis (PP-MS) pathology and is a potential biomarker of disease progression. Diffusion Kurtosis Imaging (DKI) allows the quantification of non-Gaussian water diffusion, providing metrics with high WM pathological specificity.

Objective: To characterize the pathological changes occurring in the normal appearing white matter (NAWM) of patients with PP-MS at baseline and at 1-year follow-up and to assess their impact on disability and short-term disease progression. Read More

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http://dx.doi.org/10.1111/ene.13937DOI Listing
February 2019
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Overexpression of apoptosis-related protein, survivin, in fibroblasts from patients with systemic sclerosis.

Ir J Med Sci 2019 Feb 13. Epub 2019 Feb 13.

Rheumatology Research Center, Shariati Hospital, Tehran University of Medical Sciences, Tehran, Iran.

Background/objectives: Recent studies suggest that, in addition to activation and hypersecretion of matrix components, fibroblasts from patients with systemic sclerosis (SSc) are resistant to apoptosis. Previous studies have shown that survivin, a member of inhibition of apoptosis (IAP) family, plays an important role in apoptosis resistance. Accordingly, we decided to study the expression of the most important members of IAP family in SSc fibroblasts, which can block apoptosis either by binding and inhibiting caspases or through caspase-independent mechanisms. Read More

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http://dx.doi.org/10.1007/s11845-019-01978-wDOI Listing
February 2019

Circulating follicular helper T cells are increased in systemic sclerosis and promote plasmablast differentiation through the IL-21 pathway which can be inhibited by ruxolitinib.

Ann Rheum Dis 2019 Feb 13. Epub 2019 Feb 13.

Centre de Recherche Saint-Antoine (CRSA), Sorbonne Université, INSERM U938, Paris, France

Objectives: Systemic sclerosis (SSc) is an autoimmune disease characterised by widespread fibrosis, microangiopathy and autoantibodies. Follicular helper T (Tfh) cells CD4CXCR5PD-1 cooperate with B lymphocytes to induce the differentiation of plasmocytes secreting immunoglobulins (Ig). Circulating Tfh (cTfh) cells are increased in several autoimmune diseases. Read More

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http://dx.doi.org/10.1136/annrheumdis-2018-214382DOI Listing
February 2019

Systemic Sclerosis and Serum Content of Transforming Growth Factor.

Adv Exp Med Biol 2019 Feb 14. Epub 2019 Feb 14.

Department of Rheumatology and Internal Diseases, Poznan University of Medical Sciences, Poznan, Poland.

Systemic sclerosis is a connective tissue disease characterized by tissue fibrosis leading to interstitial lung disease. Transforming growth factor-β (TGF-β) has been of interest as a potential diagnostic marker and also as a drug target in systemic sclerosis. The aim of this study was to assess the serum content of TGF-β1 in patients with systemic sclerosis and to assess its potential role in tissue fibrosis. Read More

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http://link.springer.com/10.1007/5584_2019_341
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http://dx.doi.org/10.1007/5584_2019_341DOI Listing
February 2019
3 Reads

Surgical management of pulmonary aspergillosis in pediatric population.

J Pediatr Surg 2019 Jan 11. Epub 2019 Jan 11.

Introduction: Aspergillosis is an invasive infection that affects mainly immunocompromised patients, and the lung is the most common site of infection. We reviewed our experience with the surgical management of pulmonary aspergillosis (PA) in pediatric population at a tertiary care institution.

Methods: A retrospective chart review was conducted for pediatric patients (<16 years) who were managed for PA over the period from January 2001 to December 2016. Read More

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http://dx.doi.org/10.1016/j.jpedsurg.2019.01.003DOI Listing
January 2019
1 Read

Altered human oligodendrocyte heterogeneity in multiple sclerosis.

Nature 2019 Jan 23. Epub 2019 Jan 23.

Laboratory of Molecular Neurobiology, Department of Biochemistry and Biophysics, Karolinska Institutet, Stockholm, Sweden.

Oligodendrocyte pathology is increasingly implicated in neurodegenerative diseases as oligodendrocytes both myelinate and provide metabolic support to axons. In multiple sclerosis (MS), demyelination in the central nervous system thus leads to neurodegeneration, but the severity of MS between patients is very variable. Disability does not correlate well with the extent of demyelination, which suggests that other factors contribute to this variability. Read More

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http://dx.doi.org/10.1038/s41586-019-0903-2DOI Listing
January 2019

Microparticles in systemic sclerosis: Potential pro-inflammatory mediators and pulmonary hypertension biomarkers.

Respirology 2019 Feb 12. Epub 2019 Feb 12.

Stanley S. Scott Cancer Center, Louisiana State University Health Sciences Center, New Orleans, LA, USA.

Background And Objective: Endothelial microparticles (EMP) are submicron vesicles released from endothelial cells. We aimed to determine the utility of EMP as biomarkers of pulmonary arterial hypertension (PAH) in systemic sclerosis (SSc) patients and the pathogenic role of microparticles (MP) in vascular inflammation.

Methods: Levels of EMP (CD144+, CD31+, CD62E+ and CD143+) were compared between three groups (10 SSc patients with PAH, 10 SSc patients without pulmonary hypertension (no-PH) and 10 healthy age- and sex-matched controls). Read More

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http://dx.doi.org/10.1111/resp.13500DOI Listing
February 2019
2 Reads

Efficacy of BAFF in Monitoring Treatment Response in Early Vietnamese Systemic Sclerosis Patients.

Open Access Maced J Med Sci 2019 Jan 28;7(2):264-268. Epub 2019 Jan 28.

University of Rome G. Marconi, Rome, Italy.

Background: B-cell activating factor (BAFF) is considered to have a role in the pathogenesis of systemic sclerosis (SSc).

Aim: We conducted a longitudinal study on early SSc patients to determine the change in BAFF serum level after treatment and its association with organ involvements.

Methods: A total of 46 patients (32 diffuse, 14 limited) were recruited, among which 35 patients (24 diffuse, 11 limited) completed 12-month follow-up. Read More

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http://dx.doi.org/10.3889/oamjms.2019.070DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6364724PMC
January 2019

A potential contribution of decreased galectin-7 expression in stratified epithelia to the development of cutaneous and esophageal manifestations in systemic sclerosis.

Exp Dermatol 2019 Feb 9. Epub 2019 Feb 9.

Department of Dermatology, University of Tokyo Graduate School of Medicine, Tokyo, 113-8655, Japan.

Backgrounds: Stratified epithelia have caught much attention as potential contributors to the development of dermal and esophageal fibrosis in systemic sclerosis (SSc). Galectin-7 is a marker of all types of stratified epithelia, which is involved in the maintenance of epidermal homeostasis. So far, the role of galectin-7 has not been studied in SSc. Read More

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http://dx.doi.org/10.1111/exd.13900DOI Listing
February 2019
1 Read

Prevalence and associated factors of small intestinal bacterial overgrowth among systemic sclerosis patients.

Int J Rheum Dis 2019 Feb 6. Epub 2019 Feb 6.

Faculty of Medicine, Division of Allergy-Immunology-Rheumatology, Department of Medicine, Khon Kaen University, Khon Kaen, Thailand.

Background: Small intestinal bacterial overgrowth (SIBO) results in nutrient malabsorption and malnutrition, thereby increasing the morbidity and mortality in systemic sclerosis (SSc) patients.

Objectives: To evaluate the prevalence and associated factors of SIBO in SSc patients.

Method: A cross-sectional study was conducted between July 2015 and January 2016 in SSc patients over 18, using the glucose H /CH breath test to evaluate SIBO. Read More

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http://dx.doi.org/10.1111/1756-185X.13495DOI Listing
February 2019
1 Read

Microbiome dysbiosis is associated with disease duration and increased inflammatory gene expression in systemic sclerosis skin.

Arthritis Res Ther 2019 Feb 6;21(1):49. Epub 2019 Feb 6.

Department of Molecular and Systems Biology, Geisel School of Medicine at Dartmouth, Hanover, NH, USA.

Background: Infectious agents have long been postulated to be disease triggers for systemic sclerosis (SSc), but a definitive link has not been found. Metagenomic analyses of high-throughput data allows for the unbiased identification of potential microbiome pathogens in skin biopsies of SSc patients and allows insight into the relationship with host gene expression.

Methods: We examined skin biopsies from a diverse cohort of 23 SSc patients (including lesional forearm and non-lesional back samples) by RNA-seq. Read More

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http://dx.doi.org/10.1186/s13075-019-1816-zDOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6366065PMC
February 2019
1 Read
3.753 Impact Factor

Increased expression levels of FcγRIIB on naïve and double-negative memory B cells in patients with systemic sclerosis.

Clin Exp Rheumatol 2019 Jan 17. Epub 2019 Jan 17.

Department of Dermatology, Faculty of Medicine, Institute of Medical, Pharmaceutical and Health Sciences, Kanazawa University, Kanazawa, Japan.

Objectives: In systemic sclerosis (SSc), B cell hyperactivation and abnormality are considered to play an important role in the disease pathophysiology. We aimed to clarify if the abnormal activation of B cells involves inhibitory FcγRIIB on B cells in SSc patients.

Methods: Blood samples were collected from 76 SSc patients (38 limited cutaneous SSc and 38 diffuse cutaneous SSc) and 59 healthy controls. Read More

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January 2019
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Successful treatment of mixed depth flame burns using enzymatic debridement with Nexobrid™ in a patient with aggressive systemic sclerosis (scleroderma).

Scars Burn Heal 2019 Jan-Dec;5:2059513118821563. Epub 2019 Jan 14.

Nottingham University Hospitals NHS Trust, Nottingham, UK.

Introduction: Enzymatic debridement for mixed depth and full thickness burns is gaining recognition as an important technique available to the burns surgeon. Reductions in blood loss, the need for autologous skin grafting and the number of wounds requiring surgical excision are among the benefits of this evolving treatment modality. We present a case of successful treatment using enzymatic debridement of mixed depth flame burns in a young patient with systemic sclerosis (scleroderma). Read More

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http://dx.doi.org/10.1177/2059513118821563DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6348547PMC
January 2019
1 Read

The impact of gastroesophageal reflux disease symptoms in scleroderma: effects on sleep quality.

Dis Esophagus 2019 Jan 31. Epub 2019 Jan 31.

Division of Gastroenterology and Hepatology.

Systemic scleroderma/sclerosis (SSc) is an autoimmune connective tissue disease, which can lead to esophageal motor dysfunction and gastroesophageal reflux disease (GERD). Nocturnal GERD symptoms may be associated with sleep disturbances, which in turn can drastically affect well-being and fatigue levels. We hypothesized that GERD symptoms would be associated with poorer sleep in patients with SSc. Read More

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https://academic.oup.com/dote/advance-article/doi/10.1093/do
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http://dx.doi.org/10.1093/dote/doy136DOI Listing
January 2019
6 Reads

Patient-reported outcome instruments for assessing Raynaud's phenomenon in systemic sclerosis: A SCTC Vascular Working Group Report.

J Scleroderma Relat Disord 2018 Oct 24;3(3):249-252. Epub 2018 May 24.

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

The episodic nature of Raynaud's phenomenon (RP) in systemic sclerosis (SSc) has led to a reliance on patient-reported outcome (PRO) instruments such as the Raynaud's Condition Score (RCS) diary. Little is known about the utilisation in routine clinical practice and health professional attitudes towards existing PRO instruments for assessing SSc-RP. Members of the Scleroderma Clinical Trials Consortium Vascular Working Group (SCTC-VWG, n=28) were invited to participate in a survey gauging attitudes towards the RCS diary and the perceived need for novel PRO instruments for assessing SSc-RP. Read More

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http://dx.doi.org/10.1177/2397198318774307DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6350902PMC
October 2018

Challenges and support service preferences of informal caregivers of people with systemic sclerosis: a cross-sectional survey.

Disabil Rehabil 2019 Jan 29:1-7. Epub 2019 Jan 29.

a Lady Davis Institute , Jewish General Hospital , Montreal , QC , Canada.

Purpose: To evaluate the importance of different challenges experienced by informal caregivers to persons with systemic sclerosis (SSc) and identify priorities for support services that could be developed.

Materials And Methods: Caregivers of people with SSc from three continents completed an online questionnaire to rate the importance of possible caregiver challenges and likelihood of using different forms of support services. Importance of challenges and likelihood of using support services were rated from 1 (not important; not likely to use) to 4 (very important; very likely to use). Read More

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http://dx.doi.org/10.1080/09638288.2018.1557268DOI Listing
January 2019
2 Reads

Clinicopathological analysis of clinically occult extrapulmonary lymphangioleiomyomatosis in intra-pelvic and para-aortic lymph nodes associated with pelvic malignant tumors: A study of nine patients.

Pathol Int 2019 Jan;69(1):29-36

Department of Pathology, Kobe City Medical Center General Hospital, Kobe, Japan.

The clinicopathological and immunohistochemical characteristics of clinically occult extrapulmonary lymphangioleiomyomatosis in lymph nodes (LN-LAM) being dissected during surgical staging of pelvic malignancy have not been well investigated. We assessed samples from nine female patients (median age, 61). None had past or familial history of tuberous sclerosis and had LAM lesions other than LN such as lung. Read More

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http://dx.doi.org/10.1111/pin.12749DOI Listing
January 2019
1 Read

Pathogenic Mechanisms Associated With Different Clinical Courses of Multiple Sclerosis.

Authors:
Hans Lassmann

Front Immunol 2018 10;9:3116. Epub 2019 Jan 10.

Center for Brain Research, Medical University of Vienna, Vienna, Austria.

In the majority of patients multiple sclerosis starts with a relapsing remitting course (RRMS), which may at later times transform into secondary progressive disease (SPMS). In a minority of patients the relapsing remitting disease is skipped and the patients show progression from the onset (primary progressive MS, PPMS). Evidence obtained so far indicate major differences between RRMS and progressive MS, but no essential differences between SPMS and PPMS, with the exception of a lower incidence in the global load of focal white matter lesions and in particular in the presence of classical active plaques in PPMS. Read More

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http://dx.doi.org/10.3389/fimmu.2018.03116DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6335289PMC
January 2019

Intriguing Relationships Between Cancer and Systemic Sclerosis: Role of the Immune System and Other Contributors.

Front Immunol 2018 10;9:3112. Epub 2019 Jan 10.

Medical School, Montpellier University, Montpellier, France.

Systemic sclerosis (SSc) is an autoimmune connective tissue disorder, characterized by multisystem involvement, vasculopathy, and fibrosis. An increased risk of malignancy is observed in SSc (including breast and lung cancers), and in a subgroup of patients with specific autoantibodies (i.e. Read More

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https://www.frontiersin.org/article/10.3389/fimmu.2018.03112
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http://dx.doi.org/10.3389/fimmu.2018.03112DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6335319PMC
January 2019
3 Reads

Scleroderma-related interstitial lung disease: principles of management.

Expert Rev Respir Med 2019 Jan 28:1-11. Epub 2019 Jan 28.

e Respiratory Institute , Cleveland Clinic , Cleveland , OH , USA.

Introduction: Interstitial lung disease (ILD) is the most common cause of mortality in systemic sclerosis; accounting for approximately 35% of deaths. Although immunosuppression is currently opted as first line therapy for scleroderma-related ILD (SSc-ILD), the benefits from it remain modest with concerns for systemic toxicity from long term use. Areas covered: We review the important facets in monitoring a patient with SSc-ILD, including recognizing various patterns of ILD, identifying those at risk for disease progression and discuss the strength of evidence for immunosuppressant drugs and lung transplantation. Read More

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https://www.tandfonline.com/doi/full/10.1080/17476348.2019.1
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http://dx.doi.org/10.1080/17476348.2019.1575732DOI Listing
January 2019
5 Reads

Autoimmune Diseases in Systemic Sclerosis Patients and Their Relatives: Data from a Single Center.

Isr Med Assoc J 2019 Jan;21(1):29-34

Shine Rheumatology Institute, Rambam Health Care Campus, Haifa, Israel.

Background: The aggregation of autoimmune diseases in relatives (AID-R) of patients with systemic sclerosis (SSc) has been reported.

Objectives: To analyze the prevalence of autoimmune diseases in SSc relatives and to compare their features to those of SSc patients without AID-R (controls).

Methods: A case-control analysis compared SSc patients with AID-R to those without AID-R (25 patients) with similar disease duration. Read More

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January 2019
2 Reads

Type I interferon dysregulation in Systemic Sclerosis.

Cytokine 2019 Jan 23. Epub 2019 Jan 23.

The University of Texas Health Science Center in Houston, Division of Rheumatology, 6431 Fannin, MSB 5.262, Houston, TX 77030, United States. Electronic address:

Systemic Sclerosis (Scleroderma, SSc) is a multifaceted disease characterized by autoimmunity, vasculopathy, and fibrosis affecting the skin and internal organs. Despite advances in the understanding and treatment of SSc in recent years, SSc continues to cause reduced quality of life and premature mortality. Type I interferons (IFNs), a family of cytokines with essential roles in the immune response to microbial infection, play a pathogenic role in certain autoimmune diseases (reviewed elsewhere in this edition). Read More

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http://dx.doi.org/10.1016/j.cyto.2018.12.018DOI Listing
January 2019
2 Reads

Measuring and managing appearance anxiety in patients with systemic sclerosis.

Expert Rev Clin Immunol 2019 Jan 25:1-6. Epub 2019 Jan 25.

b Department of Psychology , San Diego State University , San Diego , CA , USA.

Introduction: Systemic sclerosis (SSc, scleroderma) is a progressive, autoimmune, connective tissue disease of unknown etiology that can cause changes in appearance in socially important areas of the body (e.g. face and hands). Read More

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http://dx.doi.org/10.1080/1744666X.2019.1573673DOI Listing
January 2019
1 Read

Interactions of HLA-DR and Topoisomerase I Epitope Modulated Genetic Risk for Systemic Sclerosis.

Sci Rep 2019 Jan 24;9(1):745. Epub 2019 Jan 24.

The Center of Excellence in Computational Chemistry, Department of Chemistry, Faculty of Science, Chulalongkorn University, Bangkok, 10330, Thailand.

The association of systemic sclerosis with anti-Topoisomerase 1 antibody (ATASSc) with specific alleles of human leukocyte antigen (HLA)-DR has been observed among various ethnics. The anti-Topoisomerase 1 antibody is a common autoantibody in SSc with diffuse cutaneous scleroderma, which is one of the clinical subtypes of SSc. On the other hand, an immunodominant peptide of topoisomerase 1 (Top1) self-protein (residues 349-368) was reported to have strong association with ATASSc. Read More

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http://www.nature.com/articles/s41598-018-37038-z
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http://dx.doi.org/10.1038/s41598-018-37038-zDOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6345791PMC
January 2019
3 Reads

Short lymphocyte, but not granulocyte, telomere length in a subset of patients with systemic sclerosis.

Ann Rheum Dis 2019 Jan 24. Epub 2019 Jan 24.

Division of Rheumatology, Northwestern Scleroderma Program, Feinberg School of Medicine, Northwestern University, Chicago, USA

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http://dx.doi.org/10.1136/annrheumdis-2018-214499DOI Listing
January 2019
1 Read

Classification, categorization and essential items for digital ulcer evaluation in systemic sclerosis: a DeSScipher/European Scleroderma Trials and Research group (EUSTAR) survey.

Arthritis Res Ther 2019 Jan 24;21(1):35. Epub 2019 Jan 24.

Department of Experimental and Clinical Medicine, University of Florence, and Department of Geriatric Medicine, Division of Rheumatology and Scleroderma Unit AOUC, Villa Monna Tessa, viale Pieraccini 18, 50139, Florence, Italy.

Background: A consensus on digital ulcer (DU) definition in systemic sclerosis (SSc) has been recently reached (Suliman et al., J Scleroderma Relat Disord 2:115-20, 2017), while for their evaluation, classification and categorisation, it is still missing. The aims of this study were to identify a set of essential items for digital ulcer (DU) evaluation, to assess if the existing DU classification was useful and feasible in clinical practice and to investigate if the new categorisation was preferred to the simple distinction of DU in recurrent and not recurrent, in patients with systemic sclerosis (SSc). Read More

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http://dx.doi.org/10.1186/s13075-019-1822-1DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6346551PMC
January 2019
1 Read

Nodular amyloidosis in a patient with systemic scleroderma.

Dermatol Online J 2018 Sep 15;24(9). Epub 2018 Sep 15.

Duke University Medical Center, Department of Dermatology, Durham, North Carolina.

Primary cutaneous amyloidosis may be characterized as macular amyloidosis, lichenoid amyloidosis, or nodular amyloidosis. Nodular amyloidosis results from the deposition of immunoglobulin light chains and may rarely be associated with systemic amyloidosis. We report an unusual case of a patient with systemic scleroderma who developed primary cutaneous nodular amyloidosis on the left lower leg. Read More

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September 2018
2 Reads

Deciphering the Potential Pharmaceutical Mechanism of GUI-ZHI-FU-LING-WAN on Systemic Sclerosis based on Systems Biology Approaches.

Sci Rep 2019 Jan 23;9(1):355. Epub 2019 Jan 23.

TCM Clinical Basis Institute, Zhejiang Chinese Medicine University, 548 Binwen Road, Hangzhou, Zhejiang, 310000, China.

Systemic sclerosis (SSc; scleroderma) is a complicated idiopathic connective tissue disease with seldom effective treatment. GUI-ZHI-FU-LING-WAN (GFW) is a classic Traditional Chinese Medicine (TCM) formula widely used for the treatment of SSc. However, the mechanism of how the GFW affects SSc remains unclear. Read More

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http://dx.doi.org/10.1038/s41598-018-36314-2DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6344516PMC
January 2019
1 Read
5.078 Impact Factor

Position article and guidelines 2018 recommendations of the Brazilian Society of Rheumatology for the indication, interpretation and performance of nailfold capillaroscopy.

Adv Rheumatol 2019 Jan 22;59(1). Epub 2019 Jan 22.

Rheumatology Division, Hospital de Clínicas, Universidade Federal do Paraná (UFPR), Curitiba, Brazil.

Nailfold capillaroscopy (NFC) is a reproducible, simple, low-cost, and safe imaging technique used for morphological analysis of nail bed capillaries. It is considered to be extremely useful for the investigation of Raynaud's phenomenon and for the early diagnosis of systemic sclerosis (SSc). The capillaroscopic pattern typically associated with SSc, scleroderma ("SD") pattern, is characterized by dilated capillaries, microhemorrhages, avascular areas and/or capillary loss, and distortion of the capillary architecture. Read More

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http://dx.doi.org/10.1186/s42358-018-0046-4DOI Listing
January 2019
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CD34-selected versus unmanipulated autologous haematopoietic stem cell transplantation in the treatment of severe systemic sclerosis: a post hoc analysis of a phase I/II clinical trial conducted in Japan.

Arthritis Res Ther 2019 Jan 22;21(1):30. Epub 2019 Jan 22.

Department of Medicine and Biosystemic Science, Kyushu University Graduate School of Medical Sciences, 3-1-1 Maidashi, Higashi-ku, Fukuoka, 812-8582, Japan.

Background: The effectiveness of autologous haematopoietic stem cell transplantation (auto-HSCT) in treating severe systemic sclerosis (SSc) is established; however, the necessity of purified CD34+ cell grafts and the appropriate conditioning regimen remain unclear. This study aimed to compare the efficacy and safety of CD34-selected auto-HSCT with unmanipulated auto-HSCT to treat severe SSc.

Methods: This study was a post hoc analysis of a phase I/II clinical trial conducted in Japan. Read More

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https://arthritis-research.biomedcentral.com/articles/10.118
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http://dx.doi.org/10.1186/s13075-019-1823-0DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6341635PMC
January 2019
5 Reads

Ocular involvement in systemic sclerosis: A systematic literature review, it's not all scleroderma that meets the eye.

Semin Arthritis Rheum 2018 Dec 28. Epub 2018 Dec 28.

Department of Internal Medicine, Ghent University, Corneel Heymanslaan 10, 9000 Ghent, Belgium; Department of Rheumatology, Ghent University Hospital, Corneel Heymanslaan 10, Ghent, Belgium; Unit for Molecular Immunology and Inflammation, VIB Inflammation Research Center (IRC), Corneel Heymanslaan 10, 9000 Ghent, Belgium. Electronic address:

Objectives: Systemic sclerosis (SSc) is a rare and complex autoimmune disorder characterized by microvascular damage and progressive fibrosis which affects the skin and multiple other organs. Much of the published data concerning SSc and the eye consists of single case reports or small case studies. This systematic review aims to provide an overview of the current level of evidence for SSc-related ocular changes. Read More

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http://dx.doi.org/10.1016/j.semarthrit.2018.12.007DOI Listing
December 2018
2 Reads

Combined fused in sarcoma-positive (FUS+) basophilic inclusion body disease and atypical tauopathy presenting with an amyotrophic lateral sclerosis/motor neurone disease (ALS/MND)-plus phenotype.

Neuropathol Appl Neurobiol 2019 Jan 19. Epub 2019 Jan 19.

Sheffield Institute for Translational Neuroscience, University of Sheffield, Sheffield, UK.

Aims: Amyotrophic lateral sclerosis/motor neurone disease (ALS/MND) is characterized by the presence of inclusions containing TDP-43 within motor neurones. In rare cases, ALS/MND may be associated with inclusions containing other proteins, such as fused in sarcoma (FUS), while motor system pathology may rarely be a feature of other neurodegenerative disorders. We here have investigated the association of FUS and tau pathology. Read More

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http://dx.doi.org/10.1111/nan.12542DOI Listing
January 2019
3 Reads

Interstitial lung disease associated with systemic sclerosis (SSc-ILD).

Respir Res 2019 Jan 18;20(1):13. Epub 2019 Jan 18.

National Jewish Health, 1400 Jackson Street, Denver, CO, 80206, USA.

Background: Systemic sclerosis (SSc) is a rare connective tissue disease with a heterogeneous clinical course. Interstitial lung disease (ILD) is a common manifestation of SSc and a leading cause of death.

Main Body: All patients newly diagnosed with SSc should receive a comprehensive clinical evaluation, including assessment of respiratory symptoms, a high-resolution computed tomography (HRCT) scan of the chest, and pulmonary function tests. Read More

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https://respiratory-research.biomedcentral.com/articles/10.1
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http://dx.doi.org/10.1186/s12931-019-0980-7DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6339436PMC
January 2019
4 Reads

Nailfold capillaroscopy as a risk factor for pulmonary arterial hypertension in systemic lupus erythematosus patients.

Adv Rheumatol 2019 Jan 6;59(1). Epub 2019 Jan 6.

Rheumatology Division, Medicine Department, Escola Paulista de Medicina, Universidade Federal de São Paulo (UNIFESP), São Paulo, SP, Brazil.

Background: Pulmonary arterial hypertension (PAH) is a rare and severe complication of systemic lupus erythematosus (SLE). This study aimed to evaluate clinical and laboratory risk factors associated with PAH in SLE patients.

Methods: This was a retrospective case-control study in which patients with SLE with PAH (SLE-PAH) confirmed by right heart catheterization (RHC) were compared with SLE patients without PAH. Read More

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http://dx.doi.org/10.1186/s42358-018-0045-5DOI Listing
January 2019
1 Read

Increased serum uric acid levels are associated with digital ulcers in patients with systemic sclerosis.

Rheumatol Int 2019 Feb 17;39(2):255-263. Epub 2019 Jan 17.

Division of Rheumatology, Department of Internal Medicine, Pusan National University School of Medicine, Pusan National University Hospital, 179 Gudeok-Ro, Seo-Gu, Busan, 49241, Republic of Korea.

Endothelial injury/dysfunction is thought to be one of the earliest events in the development of vascular diseases in systemic sclerosis (SSc). Although hyperuricemia is also known to induce endothelial dysfunction and vascular inflammation, the effect of uric acid on the microvascular involvement in SSc has not been well established. We investigated whether increased serum uric acid (SUA) levels are associated with digital ulcers (DUs) in SSc. Read More

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http://dx.doi.org/10.1007/s00296-019-04240-9DOI Listing
February 2019
2 Reads

Tumoral Calcinosis of the Neck in a Patient with Systemic Sclerosis.

Cureus 2018 Nov 13;10(11):e3585. Epub 2018 Nov 13.

Internal Medicine, University of New Mexico School of Medicine, Albuquerque, USA.

Tumoral calcinosis (TC) is rare in patients with systemic sclerosis but is associated with morbidity. Paraspinal TC may cause severe pain and potentially devastating neurological deficits. Surgical decompression by removing the TC masses and applying surgical techniques to support the spine have provided substantial relief of the symptoms in the majority of cases. Read More

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http://dx.doi.org/10.7759/cureus.3585DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6334890PMC
November 2018
1 Read

Minimal Clinically Important Differences for the Modified Rodnan Skin Score: Results from the Scleroderma Lung Studies (SLS-I and SLS-II).

Arthritis Res Ther 2019 Jan 16;21(1):23. Epub 2019 Jan 16.

Department of Medicine, David Geffen School of Medicine at University of California, Los Angeles, CA, USA.

Objective: This study aimed to assess the minimal clinically important differences (MCIDs) for the modified Rodnan skin score (mRSS) using combined data from the Scleroderma Lung Studies (I and II).

Methods: MCID estimates for the mRSS at 12 months were calculated using three anchors: change in scores on the Health Assessment Questionnaire- Disability Index from baseline to 12 months, change in scores on the Patient Global Assessment from baseline to 12 months, and answer at 12 month for the Short Form-36 health transition question "Compared to one year ago, how would you rate your health in general now?" We determined the mRSS MCID estimates for all participants and for those with diffuse cutaneous systemic sclerosis (dcSSc). We then assessed associations between MCID estimates of mRSS improvement and patient-reported outcomes, using Student's t test to compare the mean differences in patient outcomes between those who met the MCID improvement criteria versus those who did not meet the improvement criteria. Read More

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http://dx.doi.org/10.1186/s13075-019-1809-yDOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6335851PMC
January 2019
1 Read

Established coronary artery disease in systemic sclerosis compared to type 2 diabetic female patients: a cross-sectional study.

Clin Rheumatol 2019 Jan 16. Epub 2019 Jan 16.

Rheumatology Unit, Azienda Ospedaliero-Universitaria di Modena, University of Modena and Reggio Emilia, Modena, Italy.

Introduction: Systemic sclerosis (SSc) is an autoimmune disease characterized by endothelial dysfunction, which is also associated with other disorders, such as atherosclerosis. The direct role of SSc in facilitating cardiovascular events should be clarified. We compared the prevalence of established coronary artery disease (CAD) between SSc and type 2 diabetes, a well-known phenotype associated with high cardiovascular risk. Read More

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http://link.springer.com/10.1007/s10067-019-04427-2
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http://dx.doi.org/10.1007/s10067-019-04427-2DOI Listing
January 2019
11 Reads

Coexistence of systemic sclerosis and ankylosing spondylitis: A case report and literature review.

Zhong Nan Da Xue Xue Bao Yi Xue Ban 2018 Nov;43(11):1263-1265

Department of Rheumatology and Immunology, Xiangya Hospital, Central South University; Institute of Rheumatology and Immunology, Central South University, Changsha 410008, China.

Systemic sclerosis (SSc) is an autoimmune disease characterized by thickening of the skin and organ fibrosis. Ankylosing spondylitis (AS) is a type of arthritis with long-term inflammation of the axial joints. Previous studies presented 5 cases of concomitant AS and SSc. Read More

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http://dx.doi.org/10.11817/j.issn.1672-7347.2018.11.016DOI Listing
November 2018
2 Reads

[Research progress in epigenetic studies on systemic sclerosis].

Zhong Nan Da Xue Xue Bao Yi Xue Ban 2018 Dec;43(12):1369-1375

Department of Rheumatology and Immunology, Xiangya Hospital, Central South University, Changsha 410008, China.

Systemic sclerosis (SSc) is an autoimmune disease with unknown etiology, characterized by vasculopathy, inflammation, and extensive fibrosis in the skin and organs. Fibrosis is the hallmark of SSc and contributes to its high mortality. In recent years, with the in-depth study of the epigenetics of SSc (DNA methylation, histone modification, and non-coding RNA), the DNA methylation and miRNA has been the most widely studied. Read More

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http://dx.doi.org/10.11817/j.issn.1672-7347.2018.12.014DOI Listing
December 2018
1 Read