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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

Curr Rheumatol Rep 2019 Apr 12;21(6):23. Epub 2019 Apr 12.

The Arthritis Program, Krembil Research Institute, University Health Network, Toronto, Ontario, Canada.

Purpose Of Review: Fibrosis is a pathological feature of many human diseases that affect multiple organs. The development of anti-fibrotic therapies has been a difficult endeavor due to the complexity of signaling pathways associated with fibrogenic processes, complicating the identification and modulation of specific targets. Evidence suggests that ephrin ligands and Eph receptors are crucial signaling molecules that contribute to physiological wound repair and the development of tissue fibrosis. Read More

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

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

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

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

Arthritis Rheumatol 2019 Apr 10. Epub 2019 Apr 10.

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

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

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

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

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

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

Int J Radiat Oncol Biol Phys 2019 May;104(1):10-11

Department of Radiation Oncology, Harvard Medical School, Boston, Massachusetts; Breast Radiation Oncology, Massachusetts General Hospital, Boston, Massachusetts.

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

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

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

Aten Primaria 2019 Apr 26;51(4):259-260. Epub 2018 Oct 26.

Medicina de Familia y Comunitaria, EAP Ripoll, Sant Joan de les Abadesses, Girona, España. Electronic address:

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

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

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

Rheumatol Int 2019 Apr 6. Epub 2019 Apr 6.

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

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

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

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

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

J Med Chem 2019 Apr 5. Epub 2019 Apr 5.

Through a phenotypic high throughput screen (HTS) using a serum response element luciferase (SRE.L) promoter, we identified a novel 5-aryl-1,3,4-oxadiazol-2-ylthiopropionic acid lead inhibitor of Rho/MRTF/SRF-mediated gene transcription with good potency (IC50 = 180 nM). We were able to rapidly improve the cellular potency by 5 orders of magnitude guided by sharply defined and synergistic SAR. Read More

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

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

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

Arthritis Res Ther 2019 Apr 3;21(1):86. Epub 2019 Apr 3.

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

Background: Data on survival and prognosis factors in incident cohorts are scarce in systemic sclerosis (SStc). To describe survival, standardized mortality ratio (SMR), and prognosis factors in systemic sclerosis (SSc), we analyzed a multicenter French cohort of incident patients and performed a systematic review of the literature and meta-analysis.

Methods: A multicenter, French cohort study was conducted between January 1, 2000, and December 31, 2013. Read More

Arthritis Res Ther 2019 Apr 2;21(1):85. Epub 2019 Apr 2.

Department of Medicine, Division of Rheumatology and Immunology, Medical University of South Carolina, 96 Jonathan Lucas Street, Charleston, SC, 29425, USA.

Background: Systemic sclerosis (SSc) is a female-predominant disease, characterized by excessive extracellular matrix deposition (ECM) with dermal and internal organ fibrosis. Considering the sex-based disparity in disease incidence, estradiol (E2), an estrogen form with pro-fibrotic effects, may play a role in SSc. We reported that post-menopausal women with diffuse cutaneous (dc)SSc have higher serum E2 levels compared to similar aged, healthy controls. Read More

Immunol Med 2018 Sep 4;41(3):113-119. Epub 2018 Nov 4.

a The First Department of Internal Medicine , University of Occupational and Environmental Health , Fukuoka , Japan.

Systemic sclerosis (SSc) is a complex disease which consists of autoimmunity, fibrosis and vasculopathy. Most organ involvement in SSc patients is related to progressive fibrosis. Once fibrosis progresses, it becomes impossible to maintain a normal structure histologically. Read More

Cureus 2019 Jan 25;11(1):e3959. Epub 2019 Jan 25.

Dermatology, San Diego Family Dermatology, San Diego, USA.

The cuticle, also referred to as the eponychium, creates a seal between the proximal nail fold and the nail plate. It is derived from both the ventral and dorsal portions of the proximal nail fold. In addition to its principle function as a barrier preventing allergens, irritants and pathogens from entering the nail cul-de-sac, the cuticle can play a role as a model for evaluating the etiology and management of diseases that affect capillary microcirculation, provide a source of solid tissue for genetic disorder studies, and aid in the evaluation of patients in whom the diagnoses of either systemic scleroderma or dermatomyositis is being entertained. Read More

J Rheumatol 2019 Apr 1. Epub 2019 Apr 1.

From the Department of Dermatology and Venereology, University Hospital Cologne, Cologne, Germany; IMSIE, University of Cologne, Cologne, Germany; Department of Rheumatology, Charité Universitätsmedizin Berlin, Berlin, Germany; Department of Rheumatology, University Hospital Heidelberg, Heidelberg, Germany; Department of Dermatology and Venerology, University Hospital Muenster, Muenster, University Hospital Halle, Halle, Germany; Centre for Interdisciplinary Clinical Immunology, Rheumatology and Auto-inflammatory Diseases and Department of Internal Medicine II (Oncology, Hematology, Immunology, Rheumatology, Pulmology), University Hospital Tuebingen, Tuebingen, Germany; Department for Internal Medicine, Rheumatology, Immunology and Nephrology, Asklepios Clinic Altona, Hamburg, Germany; Department of Rheumatology, University Hospital Wuerzburg, Wuerzburg, Germany; Department of Dermatology and Allergology, Charité Universitätsmedizin Berlin, Berlin, Germany; Department of Rheumatology, Krankenhaus St. Josef, Wuppertal, Germany; Department of Dermatology, University Hospital Carl Gustav Carus, Dresden, Germany; Department of Dermatology, Medical University of Graz, Graz, Austria; Department of Dermatology and Venereology, Ruhr-University-Bochum, Bochum, Germany; Department of Rheumatology, University Medical Center-UKSH, Luebeck, Germany; Department of Dermatology, Venereology and Allergology, HELIOS St. Elisabeth Hospital Oberhausen, University Witten-Herdecke, Germany; Department of Rheumatology, Johanniter-Krankenhaus im Flaeming Treuenbrietzen, Treuenbrietzen, Germany; Department of Rheumatology, Immanuel Krankenhaus Berlin-Buch, Berlin, Germany; University Medical Center Freiburg, Freiburg, Germany; Department of Rheumatology, Justus Liebig University Giessen, Kerckhoff Clinic, Bad Nauheim, Germany; Department of Dermatology, HELIOS University Hospital Wuppertal, Wuppertal, Germany; Department of Rheumatology, University Hospital Erlangen, Erlangen, Germany; Department of Dermatology and Allergology, Ludwig Maximilian University, Munich, Germany. This study was supported by a grant of the German Federal Ministry of Education and Research (BMBF) (01GM0310 NH, TK; 01GM0631 CS) and the Edith-Busch-Foundation. The work of Pia Moinzadeh was supported by a "Koeln Fortune" (155/2014) and "Deutsche Stiftung Sklerodermie" (3649/0096/31) grant. The first author (Pia Moinzadeh) and the last author (Nicolas Hunzelmann) received speaking fees from Actelion Pharmaceuticals and research grants from Actelion and Bayer Pharmaceuticals. But all authors declare no conflict of interest regarding this analysis. Address correspondence to Pia Moinzadeh, MD Department of Dermatology and Venereology, University of Cologne Kerpener Str. 62, 50937 Cologne, email:

Objective: Scleroderma renal crisis (SRC) is a severe life-threatening manifestation in patients with systemic sclerosis (SSc). However, the knowledge about risk factors for SRC is limited. We here determined the frequency of SRC and identified risk factors for the prediction of SRC. Read More

Rheumatology (Oxford) 2019 Apr 1. Epub 2019 Apr 1.

Department of Rheumatology, Institute of Postgraduate Medical Education and Research, Kolkata.

Reumatismo 2019 Apr 1;71(1):19-23. Epub 2019 Apr 1.

Department of Rheumatology, University Hospital ASST-FBF-Sacco, Milano.

Klotho is a transmembrane and soluble glycoprotein that governs vascular integrity. Previous studies have demonstrated reduced serum klotho concentrations in patients with systemic sclerosis (SSc), and it is known that klotho deficiency can impair the healing of digital ulcers related to microvessel damage. The aim of this study was to evaluate the association between serum klotho levels and nailfold capillaroscopic abnormalities in SSc patients. Read More

Cureus 2019 Jan 20;11(1):e3923. Epub 2019 Jan 20.

Internal Medicine, Einstein Medical Center, Philadelphia, USA.

Gitelman syndrome is an inherited renal disorder characterized by hypomagnesemia, hypokalemia, hypocalciuria and metabolic alkalosis linked to the genes encoding the thiazide sensitive NaCl cotransporter (NCCT) located on the distal convoluted tubule of the kidney. It usually presents in late childhood or early adulthood with electrolyte abnormalities resembling chronic thiazide diuretic use. Acquired Gitelman syndrome is a very rare disorder mostly associated with Sjogren's syndrome. Read More

Yakugaku Zasshi 2019 ;139(4):505-509

Graduate School of Biomedical Sciences, Nagasaki University.

Comprehensive identification of antigens in immune complexes (IC-antigens) is beneficial to provide insights into pathophysiology and could form the basis for novel diagnostic and treatment strategies for many immune-related diseases. Immune complexome analysis is a method for comprehensively identifying and profiling IC-antigens in biological fluids (such as serum and cerebrospinal fluid). We applied this strategy to the analysis of circulating ICs in autoimmune diseases (rheumatoid arthritis, Sjögren's syndrome, systemic scleroderma, and systemic lupus erythematosus), infectious diseases, and cancers. Read More

Adv Ther 2019 Mar 30. Epub 2019 Mar 30.

University of Colorado School of Medicine, Denver, CO, USA.

Introduction: Interstitial lung disease (ILD) is a common manifestation of scleroderma/systemic sclerosis (SSc). However, the direct and indirect economic burdens of SSc-ILD remain unclear. This study assessed and compared healthcare resource utilization (HRU), direct healthcare costs, work loss, and indirect costs between patients with SSc-ILD and matched controls with neither SSc nor ILD in the USA. Read More

Respiration 2019 Mar 29:1-9. Epub 2019 Mar 29.

Center for Interstitial and Rare Lung Diseases, Department of Pneumology, Ruhrlandklinik University Hospital, University of Duisburg-Essen, Essen, Germany,

Background: Anti-DFS70 antibodies, corresponding to the dense fine speckled antinuclear antibody (ANA) pattern in HEp-2 substrates, have been observed in chronic inflammatory conditions, cancer and in healthy individuals but in only a small percentage of patients with connective tissue diseases (CTD).

Objectives: The study was aimed to investigate the possible role of Anti-DFS70 antibodies to distinguish CTD associated interstitial lung disease (CTD-ILD) from idiopathic interstitial pneumonia (IIP) and to explore potential correlations between anti-DFS70 antibodies and clinical parameters.

Methods: Serum samples were collected from 49 healthy controls (HC), 35 scleroderma-ILD (SSc-ILD) patients as negative controls for anti-DFS70 antibody, and 260 patients with the initial diagnosis IIP including 100 nonspecific interstitial pneumonia (NSIP) and 160 idiopathic pulmonary fibrosis (IPF) patients. Read More

Ann Rheum Dis 2019 Mar 30. Epub 2019 Mar 30.

Department of Medicine, University of Melbourne at St. Vincent Hospital, Melbourne, Fitzroy, Victoria, Australia

Objective: We sought to develop the first Damage Index (DI) in systemic sclerosis (SSc).

Methods: The conceptual definition of 'damage' in SSc was determined through consensus by a working group of the Scleroderma Clinical Trials Consortium (SCTC). Systematic literature review and consultation with patient partners and non-rheumatologist experts produced a list of potential items for inclusion in the DI. Read More

Medicine (Baltimore) 2019 Mar;98(13):e15052

Rheumatology Unit, Department of Medicine, Surgery and Neurosciences, Azienda Ospedaliera Universitaria Senese.

Rationale: Aromatase inhibitors (AIs) are a class of drugs widely used in the treatment of estrogen sensitive breast and ovarian cancer which convert testosterone to estradiol and androstenedione to estrogen. The AIs of third generation, including anastrazole, letrozole and exemestane, have actually become the standard of care of estrogen-receptor-positive breast cancer in menopausal women and are recommended as adjuvant treatment after surgery in place of/or following tamoxifen. Their main side-effects include reduction in bone mineral density, occurrence of menopausal manifestations and development of musculoskeletal symptoms which are, usually, transient, but sometimes evolve into a typical form of arthritis, such as rheumatoid arthritis (RA). Read More

PLoS One 2019 26;14(3):e0214202. Epub 2019 Mar 26.

Nephrology Department, Walter Reed National Military Medical Center, Bethesda, MD, United States of America.

Systemic sclerosis (SSc) is a heterogeneous autoimmune disorder associated with vascular dysfunction and fibrotic changes in the skin, vasculature and internal organs. Although serologic abnormalities are an important diagnostic tool for SSc, little is known about whether autoantibodies precede clinical diagnosis. Here we investigated the presence of autoantibodies before SSc diagnosis and assessed whether certain autoantibodies might associate with the future onset of scleroderma renal crisis (SRC), a potentially fatal complication of the disease. Read More

J Scleroderma Relat Disord 2019 Feb 18;4(1):17-27. Epub 2018 Jul 18.

Rheumatology Unit, University Hospital of Cagliari.

The Scleroderma Clinical Trials Consortium (SCTC) represents many of the clinical researchers in the world who are interested in improving the efficiency of clinical trials in Systemic Sclerosis (SSc). The SCTC has established 11 working groups (WGs) to develop and validate better ways of measuring and recording multiple aspects of this heterogeneous disease. These include groups working on arthritis, disease damage, disease activity, cardiac disease, juvenile SSc, the gastrointestinal tract, vascular component, calcinosis, scleroderma renal crisis, interstitial lung disease, and skin measurement. Read More

Ann Transl Med 2019 Feb;7(3):60

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

Hand (N Y) 2019 Mar 21:1558944719837023. Epub 2019 Mar 21.

1 The Curtis National Hand Center, MedStar Union Memorial Hospital, Baltimore, MD, USA.

Background: Our goal was to investigate patients' upper extremity tissue perfusion changes using an indocyanine green laser angiography imaging system for various pathologic states and interventions.

Methods: This prospective observational study used Spy Elite/LUNA laser angiography to evaluate perfusion in patients with upper extremity vascular compromise. All patients had Spy Elite/LUNA imaging as well as clinical and handheld Doppler examinations preintervention, intraoperatively, if applicable, and at 1 week, 2 weeks, and 2 months postintervention. Read More

Cutis 2018 Feb;102(2):E4-E6

Department of Dermatopathology, University of Oklahoma and Regional Medical Laboratory, St. John Medical Center, Tulsa, USA.

J Dermatolog Treat 2019 Mar 20:1-8. Epub 2019 Mar 20.

a Pharmerit International LP , Bethesda , MA , USA.

Introduction: The National Comprehensive Cancer Network and the European Organization for Research and Treatment of Cancer recommend extracorporeal photopheresis (ECP) as systemic therapy for cutaneous T-cell lymphoma (CTCL).

Objective: To investigate real-world use of ECP in CTCL patients in the US.

Methods: Data from the Truven MarketScan database (2010-2015) were used to create a cohort of CTCL patients receiving systemic treatment. Read More

Case Rep Genet 2019 12;2019:8538325. Epub 2019 Feb 12.

Human Genetics Institute, School of Medicine, Pontifical Xavierian University, Bogotá, Colombia.

Werner's syndrome (WS) is an autosomal recessive genetic disease, which is mainly characterized by scleroderma-like skin changes, juvenile cataracts, short stature, and signs of premature aging. We report a case of a 48-year-old male patient, who presents with cardinal signs of WS including high-pitched voice, sclerotic skin lesions mainly on feet, premature greying of scalp hair, bilateral cataracts, and "bird-like" facial appearance. In addition, the patient presents other clinical characteristics observed in patients with WS such as short stature, type 2 diabetes mellitus, hypogonadism, parental consanguinity, and a history of a sibling with similar clinical characteristics. Read More

Front Immunol 2019 27;10:337. Epub 2019 Feb 27.

Division of Biodiagnostic Sciences and Technologies, INRASTES, National Center for Scientific Research Demokritos, Athens, Greece.

Thrombotic microangiopathies (TMAs) are a heterogeneous group of syndromes presenting with a distinct clinical triad: microangiopathic hemolytic anemia, thrombocytopenia, and organ damage. We currently recognize two major entities with distinct pathophysiology: thrombotic thrombocytopenic purpura (TTP) and hemolytic uremic syndrome (HUS). Beyond them, differential diagnosis also includes TMAs associated with underlying conditions, such as drugs, malignancy, infections, scleroderma-associated renal crisis, systemic lupus erythematosus (SLE), malignant hypertension, transplantation, HELLP syndrome (hemolysis, elevated liver enzymes, and low platelets), and disseminated intravascular coagulation (DIC). Read More

J Med Case Rep 2019 Mar 20;13(1):74. Epub 2019 Mar 20.

Department of Rheumatology, Saitama Medical Center, Jichi Medical University, 1-847 Amanuma-cho, Omiya-ku, Saitama, 330-8503, Japan.

Background: Segmental arterial mediolysis is a rare nonarteriosclerotic and noninflammatory vascular disease that may cause intraperitoneal bleeding. Scleroderma renal crisis is a rare complication of systemic sclerosis, leading to severe hypertension and renal dysfunction. To the best of our knowledge, this is the first reported case of a patient with concurrent systemic sclerosis with scleroderma renal crisis and pathologically confirmed segmental arterial mediolysis. Read More

Arthritis Rheumatol 2019 Mar 19. Epub 2019 Mar 19.

Division of Rheumatology, Johns Hopkins University School of Medicine, Baltimore, USA.

Objectives: While compelling data suggest a cancer-induced autoimmunity model in scleroderma patients with anti-RNA polymerase III large subunit (anti-RPC155) antibodies, ~85% of these patients do not manifest cancer. We hypothesized that anti-RPC155-positive scleroderma patients without detectable cancer target additional autoantigens.

Methods: 168 scleroderma patients with anti-RPC155 antibodies were studied: 80 with a cancer history, and 88 with no cancer diagnosis after >5 years of follow-up. Read More

Stat Med 2019 Mar 18. Epub 2019 Mar 18.

Department of Biostatistics, Fielding School of Public Health, University of California, Los Angeles, Los Angeles, California.

Thresholding variable plays a crucial role in subgroup identification for personalized medicine. Most existing partitioning methods split the sample based on one predictor variable. In this paper, we consider setting the splitting rule from a combination of multivariate predictors, such as the latent factors, principle components, and weighted sum of predictors. Read More

BMC Rheumatol 2018 7;2:36. Epub 2018 Dec 7.

5Department of Cardiology, Herlev and Gentofte Hospital, Gentofte, Kildegårdsvej 28, 2900 Hellerup, Denmark.

Background: To investigate the incidence and the mortality-rates of systemic sclerosis (SSc), its primary causes of death, and the temporal trends in events in Denmark during the last decades.

Methods: Using the Danish National Patient Registry, we identified all persons aged ≥18 years with a first-time diagnosis of SSc (ICD-10 code M34, excluding M34.2) between 1995 and 2015. Read More

BMC Rheumatol 2018 15;2:13. Epub 2018 May 15.

1Hospital of the University of Pennsylvania, Philadelphia, USA.

Background: Systemic Sclerosis is a multifactorial autoimmune rheumatic disease characterized by inflammation, fibrosis, immune dysregulation and vascular dysfunction.

Methods: An open label, prospective, non-comparative study evaluating ambrisentan with an antifibrotic agent in diffuse cutaneous systemic sclerosis (dcSSc). Recruited 15 consecutive patients with dcSSc who were already on a stable dose of an antifibrotic agent and if they met inclusion criteria they were initiated on ambrisentan 5 mg/day for 12 months. Read More

RMD Open 2018 26;4(Suppl 1):e000786. Epub 2019 Feb 26.

Referral Center for Systemic Autoimmue Diseases, Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico di Milano, Milan, Italy.

The term 'undifferentiated connective tissue disease' (UCTD) is generally used to describe clinical entities characterised by clinical and serological manifestations of systemic autoimmune diseases but not fulfilling the criteria for defined connective tissue diseases (CTDs). In this narrative review, we summarise the results of a systematic literature research, which was performed as part of the ERN ReCONNET project, aimed at evaluating existing clinical practice guidelines (CPGs) or recommendations. No specific CPG on UCTD were found, potential areas of intervention are absence of a consensus definition of UCTD, need for specific monitoring and therapeutic protocols, stratification of UCTD based on the risk of developing a defined CTD and preventive measure for the future development of a more severe condition. Read More

RMD Open 2018 26;4(Suppl 1):e000784. Epub 2019 Feb 26.

Division of Rheumatology, University and IRCCS Policlinico S. Matteo Foundation, Pavia, Italy.

Idiopathic inflammatory myopathies (IIMs) encompass a heterogeneous group of rare autoimmune diseases characterised by muscle weakness and inflammation, but in antisynthetase syndrome arthritis and interstitial lung disease are more frequent and often inaugurate the disease. Clinical practice guidelines (CPGs) have been proposed for IIMs, but they are sparse and heterogeneous. This work aimed at identifying: i) current available CPGs for IIMs, ii) patients ' and clinicians' unmet needs not covered by CPGs. Read More

Syst Appl Microbiol 2019 Mar 15. Epub 2019 Mar 15.

Université de Lyon, INSA de Lyon, CNRS UMR 5240, Lyon, France.

Arthritis Rheumatol 2019 Mar 18. Epub 2019 Mar 18.

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

Objectives: We previously identified CYR61 as an HDAC5-repressed gene in scleroderma (SSc) endothelial cells (ECs). When overexpressed, CYR61 promoted angiogenesis in SSc ECs. In this study we examined the role of CYR61 in fibrosis and determined the mechanisms involved in CYR61-mediated angiogenesis in SSc. Read More

J Invest Dermatol 2019 Mar 13. Epub 2019 Mar 13.

Institute of Biochemistry, Emil-Fischer-Center, Friedrich-Alexander University of Erlangen-Nürnberg, Erlangen, Germany; Comprehensive Cancer Center (CCC) Erlangen-EMN, Erlangen, Germany. Electronic address:

Bone morphogenetic protein-6 (BMP6) is known to be crucial for regulating embryonic skin development. This study assessed the role of BMP6 in dermal fibrosis. We detected that BMP6 is significantly increased in skin-derived fibroblasts of patients with localized scleroderma. Read More