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ERJ Open Res 2019 Feb 11;5(1). Epub 2019 Feb 11.

Division of Pulmonary Medicine, Dept of Medicine, National Jewish Health, Denver, CO, USA.

http://ow.ly/vPvc30neJZV. Read More

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

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

Acta Obstet Gynecol Scand 2019 Feb 15. Epub 2019 Feb 15.

The Fertility Clinic, section 4071, University Hospital of Copenhagen Rigshospitalet, Copenhagen, Denmark.

Introduction: Ovarian tissue cryopreservation (OTC) is increasingly offered to women in need of fertility preservation. However, little is known about the risk of these women dying before they use the preserved material.

Material And Methods: From 1999 to 2016, 927 girls and women underwent OTC in our center, prior to receiving gonadotoxic treatment. Read More

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

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

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

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

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

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

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

Cutis 2019 Jan;103(1):34-36

St. Joseph Mercy Hospital System, Reichert Health Center, Ypsilanti, Michigan, USA.

En coup de sabre (ECDS) is a rare form of localized scleroderma that typically manifests in children and women. It presents as a fibrous pansclerotic plaque extending in a bandlike distribution on the frontoparietal scalp with surrounding scarring alopecia. Many patients have comorbid central nervous system involvement in addition to the cutaneous findings. Read More

Proc Natl Acad Sci U S A 2019 Feb 12. Epub 2019 Feb 12.

Division of Rheumatology, Department of Internal Medicine, University of Michigan, Ann Arbor, MI 48109;

Scleroderma (SSc) is a complex disease that involves activation of the immune system, vascular complications, and tissue fibrosis. The histone methyltransferase enhancer of zeste homolog 2 (EZH2) mediates trimethylation of lysine 27 of histone 3 (H3K27me3), which acts as a repressive epigenetic mark. Both EZH2 and H3K27me3 were elevated in SSc dermal fibroblasts and endothelial cells compared with healthy controls. Read More

Reumatol Clin 2019 Feb 9. Epub 2019 Feb 9.

Unidad de Enfermedades Autoinmunes Sistémicas, Unidad de Gestión Clínica de Medicina Interna, Hospital Universitario Central de Asturias, Oviedo, Asturias, España.

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

Mycologia 2019 Feb 12:1-10. Epub 2019 Feb 12.

a Department of Plant Pathology , University of Florida , Gainesville , Florida 32611.

In the northwestern Himalayan mountains of India, the hypogeous sequestrate fungus Trappeindia himalayensis is harvested from forests dominated by the ectomycorrhizal tree Cedrus deodara (Himalayan cedar). This truffle has basidiospores that are ornamented with raised reticulation. The original description of Trappeindia himalayensis suggested that the gleba of this species is similar to young specimens of Scleroderma (Boletales), whereas its basidiospores are ornamented with raised reticulation, suggesting a morphological affinity to Leucogaster (Russulales) or Strobilomyces (Boletales). Read More

Am J Transplant 2019 Feb 12. Epub 2019 Feb 12.

Division of Blood and Marrow Transplantation, Department of Pediatrics, University of Minnesota, Minneapolis, Minnesota, USA.

Chronic graft-versus-host disease is a leading cause of morbidity and mortality following allotransplant. Activated donor effector T-cells can differentiate into pathogenic T helper (Th)-17 cells and germinal center -promoting Tfollicular helper cells, resulting in cGVHD. Phosphoinositide-3-kinase-δ, a lipid kinase, is critical for activated T-cell survival, proliferation, differentiation, and metabolism. Read More

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

Curr Neurol Neurosci Rep 2019 Feb 12;19(3):11. Epub 2019 Feb 12.

NYU Multiple Sclerosis Comprehensive Care Center, Department of Neurology, New York University School of Medicine, New York, NY, USA.

Purpose Of Review: To describe diverse neurologic and neuroradiologic presentations of two rare, immunologically mediated skin conditions: Sweet disease and localized scleroderma (morphea).

Recent Findings: Core syndromes of neuro-Sweet disease (NSD) are steroid responsiveness, recurrent meningitis, and encephalitis. Focal neurologic, neuro-vascular, and neuro-ophthalmologic syndromes have been reported recently in NSD. Read More

Indian J Psychiatry 2019 Jan-Feb;61(1):97-99

Department of Psychiatry, Dr. Rajendra Prasad Government Medical College, Tanda, Himachal Pradesh, India.

Progressive facial hemiatrophy or Parry-Romberg syndrome is a rare disorder of unknown etiology which is characterized by atrophy of the one side of the face and various neurological complications. Clinical overlap is sometimes seen with linear scleroderma or en coup de sabre. We present such a case that manifested clinically with partial epilepsy and novel psychiatric complaints of agoraphobia. Read More

JAAD Case Rep 2019 Feb 31;5(2):201-204. Epub 2019 Jan 31.

Columbia University Medical Center, Department of Dermatology, New York, New York.

JAAD Case Rep 2019 Feb 25;5(2):144-146. Epub 2019 Jan 25.

Department of Dermatology, University of Texas McGovern Medical School, Houston, Texas.

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

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

Circ J 2019 Feb 7. Epub 2019 Feb 7.

Department of Cardiovascular Medicine, Kyoto Prefectural University of Medicine.

Background: Many patients with collagen disease (CD), particularly scleroderma (SSc), develop critical limb ischemia (CLI), which leads to limb amputation. However, conventional therapies, including revascularization via surgical bypass, showed poor outcomes in CLI patients with CD. Many CLI patients with SSc showed poor responses to combination therapies including intravenous iloprost, PDE-5 inhibitors, and bosentan. Read More

J Clin Rheumatol 2019 Feb 1. Epub 2019 Feb 1.

Immunology, Allergy, and Rheumatology, Department of Medicine, University of Cincinnati College of Medicine, Cincinnati, OH.

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

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

Molecules 2019 Feb 3;24(3). Epub 2019 Feb 3.

Institute of Bio-Organic Chemistry Antonio González, Department of Organic Chemistry, University of La Laguna, Astrofísico Francisco Sánchez 2, 38206 La Laguna, Tenerife, Spain.

A new cytotoxic cyclononapeptide, sclerin, cyclo(⁻Dab⁻Ser⁻Tyr⁻Gly⁻Thr⁻Val⁻Ala⁻ Ile⁻Pro⁻) (), was isolated from the methanol extract of the seeds of , together with the known metabolite, cyclosenegalin A, cyclo(⁻Pro⁻Gly⁻Leu⁻Ser⁻Ala⁻Val⁻Thr⁻) (). The planar structures for the two compounds were established by comprehensive analysis of NMR and ESI-HRMS data, and the absolute stereochemistry was stablished by Marfey's method. Compound showed moderate cytotoxic activity against the human prostate carcinoma cell line DU-145 at µM concentration. Read More

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

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

JAAD Case Rep 2019 Feb 16;5(2):127-130. Epub 2019 Jan 16.

Department of Dermatology, Amiens University Hospital, Amiens, France.

J Am Acad Dermatol 2019 Jan 28. Epub 2019 Jan 28.

Department of Dermatology, Mayo Clinic, Rochester, Minnesota. Electronic address:

Background: Few studies support treating morphea (localized scleroderma) with hydroxychloroquine.

Objective: To assess the efficacy of hydroxychloroquine treatment of morphea.

Methods: We conducted a retrospective study of 84 patients who had morphea and were treated with hydroxychloroquine monotherapy for at least 6 months at our institution from 1996 through 2013. Read More

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

Rheumatology (Oxford) 2019 Jan 28. Epub 2019 Jan 28.

Leeds Institute of Rheumatic and Musculoskeletal Medicine, University of Leeds, UK.

Objectives: To screen for significant arrhythmias with an implantable loop recorder (ILR) in patients with SSc and no known cardiovascular disease, and identify associated disease phenotype, blood and cardiovascular magnetic resonance (CMR) biomarkers.

Methods: Twenty patients with SSc with no history of primary SSc heart disease, traditional cardiovascular disease, diabetes or maximum one traditional cardiovascular risk factor underwent clinical assessment, contrast-enhanced CMR and ILR insertion.

Results: ILR data were available for 19 patients: 63% female, mean (s. Read More

Aliment Pharmacol Ther 2019 Mar 27;49(5):546-555. Epub 2019 Jan 27.

Faculty of Health and Medicine, University of Newcastle, Callaghan, NSW, Australia.

Background: The pathogenesis of functional GI disorders (FGIDs) is uncertain. However, underlying immune activation and psychological distress has been documented in irritable bowel syndrome (IBS) and functional dyspepsia (FD). Epidemiological data from the UK suggest that FGIDs are linked to atopy and certain autoimmune diseases but this has not been confirmed. Read More

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

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

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

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

Environ Sci Pollut Res Int 2019 Jan 25. Epub 2019 Jan 25.

School of Chemistry and Physics, University of KwaZulu-Natal, Westville Campus, Private Bag X54001, Durban, 4000, South Africa.

Macro-elements (Ca, Fe, K, Mg and Na) and trace elements including some toxic (As, Be, Cd, Co, Cu, Mn, Ni, Pb, Se and Zn) were determined in edible and inedible wild-growing mushrooms (Amanita rubescens, Auricularia polytricha, Boletus edulis, Boletus mirabilis, Clavulina cristata, Helvella crispa, Lactarius deliciosus, Suillus luteus, Termitomyces microcarpus, Termitomyces reticulatus, Termitomyces clypeatus, Termitomyces umkowaanii, Amanita foetidissima, Amanita muscaria, Amanita pantherina, Aseroe rubra, Chlorophyllum molybdites, Ganoderma lucidum, Gymnopilus junonius, Hypholoma fasciculare, Lentinus villosus, Lepista caffrorum, Pycnoporus sanguineus, Panaeolus papilionaceus, Pisolithus tinctorius, Pleurotus ostreatus, Podaxis pistillaris, Russula sardonia, Scleroderma citrinum, Scleroderma michiganense). Analyses of samples were carried out using inductively coupled plasma-optical emission spectrometry. The elemental content in both edible and inedible mushrooms, in decreasing order, was found to be K > Na > Ca > Mg > Fe > Mn > Zn > Cu > Se > Co > Ni > Be > Pb ≥ Cd > As. Read More

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

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

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

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

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

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

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

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

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