Search Our Scientific Publications & Authors

J Cell Physiol 2018 Dec 7. Epub 2018 Dec 7.

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

Objectives: Impaired wound healing and skin dehydration are the mainstay of systemic sclerosis (SSc) cutaneous manifestations. Aquaporin-3 (AQP3) has a pivotal role in skin hydration and wound healing. Epidermal growth factor receptor (EGFR) activation is impaired in SSc fibroblasts. Read More

Clin Rheumatol 2018 Dec 7. Epub 2018 Dec 7.

Autoimmune Diseases Unit, Department of Internal Medicine, Hospital Universitario Vall d'Hebron, Barcelona, Spain.

Introduction: Our objective was to evaluate the pulmonary hypertension (PH) data for Spanish patients with systemic sclerosis (SSc), define the PH types and determine the associated factors.

Method: Descriptive study of PH-related data from the multicentre RESCLE registry. Estimated systolic pulmonary artery pressure (esPAP), measured via echocardiogram was considered elevated if ≥ 35 mmHg. Read More

Med Ultrason 2018 Dec;20(4):475-479

M.F. Vladimirsky Moscow Regional Scientific Research Clinical Institute, Scientific-Practical Center of Dermatovenerology and Cosmetology, Moscow, Russia.

Aim: To describe the ultrasonographic findings of surface and nodular basal cell skin cancer (BCC) using high frequency ultrasonography.

Materials And Methods: We examined 60 primary BCCs in different locations with the High Frequency Ultrasound (HFU) system DUB Skin Scanner using 75 MHz and 30 MHz probes. Epidermis, dermis, and depth of tumors spread in the region of interest (ROI) were measured. Read More

Best Pract Res Clin Rheumatol 2018 04 14;32(2):223-240. Epub 2018 Sep 14.

Department of Rheumatology, Zurich University Hospital, Zurich, Switzerland; Department of Internal Medicine and Rheumatology, Cantacuzino Hospital, Carol Davila University of Medicine and Pharmacy, Bucharest, Romania. Electronic address:

Systemic sclerosis (SSc) is a chronic immune-mediated connective tissue disease with heterogeneous organ involvement. New classification criteria were developed allowing disease identification even before the onset of its hallmark, skin fibrosis. Incidence and prevalence vary among reports and are influenced by methodology. Read More

Asian Pac J Allergy Immunol 2018 Dec 9. Epub 2018 Dec 9.

Department of Allergy, Immunology and Rheumatology, Mackay Children's Hospital, Taipei, Taiwan.

Generalised morphea (GM) is a subtype of localised scleroderma that usually manifests with bilateral involvement. Unilateral generalised morphea (UGM) is a rare variant of GM. This is a case report of a Taiwanese girl with UGM over the left side of her body. Read More

J Dermatol 2018 Dec 3. Epub 2018 Dec 3.

Fudan University, Department of Radiation Oncology, Zhongshan Hospital, School of Medicine, Fudan University, Shanghai, China.

There is no established diagnostic criteria or widely accepted severity classification of localized scleroderma (LS) by imaging. Acoustic radiation force impulse (ARFI) technology by normalized mean shear wave velocity (SWV) may be as a probing tool for diagnosing and staging LS accurately and objectively. Fifty-six patients with LS of inflammatory (n = 21), sclerotic (n = 24) and atrophic (n = 11) stage and 30 healthy controls were evaluated on the basis of pathological results. Read More

Reumatologia 2018 31;56(5):294-300. Epub 2018 Oct 31.

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

Objectives: To determine the incidence and factors associated with median neuropathy at the wrist (MNW) in systemic sclerosis (SSc) patients using an electrodiagnostic (EDX) study.

Material And Methods: SSc patients who attended the scleroderma clinic, Srinagarind Hospital, were prospectively evaluated by questionnaire, physical examination, and EDX study. The questionnaire consisted of the baseline characteristics, type of scleroderma, clinical signs and symptoms associated with scleroderma, and the Boston questionnaire. Read More

Rheumatology (Oxford) 2018 Dec 4. Epub 2018 Dec 4.

Department of Medicine, McGill University.

Objectives: To examine the incidence, predictors and outcomes associated with severe gastrointestinal (GI) disease in a large inception SSc cohort.

Methods: SSc subjects with <2 years of disease duration were identified from two multicentre cohorts. Severe GI disease was defined as: malabsorption, hyperalimentation, pseudo-obstruction and/or ⩾10% weight loss in association with the use of antibiotics for bacterial overgrowth or oesophageal stricture. Read More

Plast Reconstr Surg 2018 Nov;142(5):1275-1283

Madison, Wis.; and Livingston, N.J. From the Division of Plastic and Reconstructive Surgery, University of Wisconsin School of Medicine and Public Health; and Summit Medical Group.

Background: Parry Romberg disease (hemifacial atrophy) and linear scleroderma (coup de sabre) are progressive, usually unilateral facial atrophies of unknown cause. The gold standard treatment for these patients has been microsurgical reconstruction following the "burning out" of the facial atrophy and stable contour for 2 years.

Methods: The authors report their experience treating patients with hemifacial atrophy and linear scleroderma with free tissue transfers between 1989 and 2016. Read More

J Scleroderma Relat Disord 2018 Oct 22;3(3):242-248. Epub 2018 Mar 22.

Pulmonary Center, Department of Medicine, Boston University School of Medicine, Boston, MA, 02118, USA.

Objective: Pulmonary arterial hypertension (PAH) and interstitial lung disease (ILD) are major causes of mortality in systemic sclerosis (SSc). We used a previously identified microarray biomarker to determine if SSc-PAH and SSc-ILD patients demonstrate distinct gene expression profiles.

Methods: PBMCs were collected from healthy controls (n=10), SSc (SSc) patients without pulmonary hypertension [SSc-noPAH, n=39], and SSc-PAH patients (n=21; mPAP25, PCWP≤15, PVR≥3WU) diagnosed by right heart catheterization (RHC). Read More

Eur Respir J 2018 Nov 28. Epub 2018 Nov 28.

Interstitial Lung Disease Unit, Royal Brompton Hospital, Royal Brompton and Harefield NHS Foundation Trust, London, UK.

To compare radiology-based prediction models in rheumatoid arthritis-related interstitial lung disease (RA-ILD) to identify patients with a progressive fibrosis phenotype. RAILD patients had CTs scored visually and by CALIPER and forced vital capacity (FVC) measurements. Outcomes were evaluated using three techniques: 1. Read More

Pediatr Dermatol 2018 Nov 28. Epub 2018 Nov 28.

Division of Rheumatology, Dartmouth Hitchcock Medical Center, Lebanon, New Hampshire.

A 12-year-old female with oligoarticular juvenile inflammatory arthritis developed an atrophic linear plaque involving the left medial forearm and proximal arm 7 months after intra-articular triamcinolone injection for arthritis. The plaque spontaneously resolved without treatment over approximately one year. It is important to recognize this rare complication of intra-articular steroid injection in order to avoid potential misdiagnosis as linear scleroderma and subsequent immunosuppressive treatment. Read More

Contact Dermatitis 2018 Nov 28. Epub 2018 Nov 28.

Department of Endocrinology, Metabolism and Nephrology, Kochi Medical School, Nankoku, Kochi, Japan.

Drug-induced hypersensitivity syndrome (DIHS) or drug reaction with eosinophilia and systemic symptoms (DRESS) is a rare, severe skin disease with manifestations such as fever, eosinophilia, lymphadenopathy and internal organ damage that typically develops 2 to 6 weeks after drug intake. DRESS/DIHS follows a prolonged course involving frequent flare-ups and relapses over weeks or months after treatment discontinuation. Read More

Int J Oncol 2018 Nov 21. Epub 2018 Nov 21.

Department of Pharmacology, Faculty of Medicine, School of Health Sciences, University of Thessaly, 41500 Larissa, Greece.

Caveolin‑1 (Cav‑1) expression has been shown to be associated with tumor growth and resistance to chemotherapy in pancreatic cancer. The primary aim of this study was to explore the significance of Cav‑1 expression in pancreatic cancer cells as compared to fibroblasts in relation to cancer cell proliferation and chemoresistance, both in vitro and in vivo, in an immunodeficient mouse model. We also aimed to evaluate the immunohistochemical expression of Cav‑1 in the epithelial and stromal component of pancreatic cancer tissue specimens. Read More

Front Immunol 2018 13;9:2452. Epub 2018 Nov 13.

Experimental Rheumatology, Radboudumc, Nijmegen, Netherlands.

Systemic sclerosis (SSc) is a severe auto-immune disease, characterized by vasculopathy and fibrosis of connective tissues. SSc has a high morbidity and mortality and unfortunately no disease modifying therapy is currently available. A key cell in the pathophysiology of SSc is the myofibroblast. Read More

Mod Rheumatol 2018 Nov 24:1-16. Epub 2018 Nov 24.

b Rheumatology Division, Escola Paulista de Medicina , Universidade Federal de São Paulo , São Paulo , Brazil.

Objectives: To evaluate the disease severity and activity in patients with a diagnosis of systemic sclerosis (SSc) after the 2013 ACR/EULAR classification criteria development compared to patients diagnosed before 2013.

Methods: One hundred and fifty-four subjects were included and assigned to the following groups: 120 SSc patients meeting the 1980 ACR criteria and with a diagnosis before 2013 (historical group), and 34 patients diagnosed after 2013, fulfilling the new ACR/EULAR criteria (early SSc group). Disease activity was assessed by the 2001 European Scleroderma Study Group Activity Index (EScSG-AI) and by the revised European Scleroderma Trials and Research group (EUSTAR) activity index. Read More

J Invest Dermatol 2018 Nov 22. Epub 2018 Nov 22.

miRagen Therapeutics, Inc. Boulder, CO.

MicroRNA-29 negatively regulates fibrosis, and miR-29 is downregulated in multiple fibrotic organs and tissues, including in the skin. miR-29 mimics prevent pulmonary fibrosis in mouse models but have not previously been tested in the skin. This study aimed to identify pharmacodynamic (PD) biomarkers of miR-29 in mouse skin, to translate those biomarkers across multiple species and to assess the PD activity of a miR-29b mimic (remlarsen) in a clinical trial. Read More

Lab Invest 2018 Nov 23. Epub 2018 Nov 23.

Jefferson Institute of Molecular Medicine and Scleroderma Center, Thomas Jefferson University, Philadelphia, PA, 19107, USA.

Excessive connective tissue deposition in skin and various internal organs is characteristic of systemic sclerosis (SSc). The profibrotic growth factor TGF-β plays a crucial role in SSc pathogenesis. The expression of NADPH oxidase 4 (NOX4), a critical mediator of oxidative stress, is potently stimulated by TGF-β. Read More

EBioMedicine 2018 Nov 20. Epub 2018 Nov 20.

University of Texas-McGovern Medical School, Houston, TX, USA. Electronic address:

Background: The genes of SPARC, CCR2, and SMAD3 are implicated in orchestrating inflammatory response that leads to fibrosis in scleroderma and other fibrotic disorders. The aim of the studies is to evaluate synergistic anti-fibrotic potency of the siRNAs of these genes.

Methods: The efficacy of the siRNA-combination was evaluated in bleomycin-induced mouse fibrosis. Read More

Front Biosci (Landmark Ed) 2019 01 1;24:48-95. Epub 2019 Jan 1.

Berlin Cures GmbH Berlin, Knesebeckstrasse 59-61, 10719 Berlin, Germany,

In the 1970s, autoantibodies directed against G-protein-coupled receptors (GPCR, GPCR-AAB) were discovered. After receptor binding, GPCR-AAB trigger uncontrolled receptor mediated signal cascades, thus producing pathologies. Diseases associated with such functionally active autoantibody type (functional autoantibodies) can be called "functional autoantibody diseases". Read More

Dermatol Clin 2019 Jan 1;37(1):37-48. Epub 2018 Nov 1.

Departments of Dermatology and Pathology, Cleveland Clinic Lerner College of Medicine, 9500 Euclid Avenue A61, Cleveland, OH 44195, USA. Electronic address:

Connective tissue diseases often prominently affect the skin, requiring dermatologists to play an important role in diagnosis and treatment of these patients. Herein we describe updates on the pathogenesis, clinical features, and treatment of 4 major connective tissue diseases: dermatomyositis, cutaneous lupus erythematosus, limited scleroderma (morphea), and cutaneous vasculitis. Many of these updates promise to improve clinical care of patients who suffer from dermatologic involvement of these diseases and are the result of research performed by dermatologists who have expertise in these conditions. Read More

Acta Neurol Belg 2018 Nov 21. Epub 2018 Nov 21.

Center for Biochemistry, Clinical Center of Serbia, Belgrade, Serbia.

Objective: To analyze neurological and electroneurography (ENG) findings in patients with systemic sclerosis (SSc) and symptoms of neuropathic pain in upper and lower extremities.

Patients And Methods: Using the PainDetect questionnaire, 42 consecutive patients with SSc (38 women and 4 men) were screened for the presence of neuropathic pain in upper and/or lower extremities. Patients with previously diagnosed diabetes or other metabolic diseases, malignancy, other autoimmune disorders, or any neurological or psychiatric disease, were not included. Read More

Medicine (Baltimore) 2018 Nov;97(47):e13319

Rationale: Dystrophic calcinosis occurs in chronically damaged tissue in patients with complicated autoimmune diseases. The therapeutic options are limited, and the treatment response rate is variable. Here, we describe a rare case of dystrophic calcinosis treated with leflunomide in a patient with overlap syndrome. Read More

Pan Afr Med J 2018 26;30:176. Epub 2018 Jun 26.

Service de Dermatologie-Vénérologie, Faculté des Sciences de Santé Lomé, Lomé, Togo.

This study aimed to highlight the epidemiological , clinical, therapeutic and evolutionary profile of connective tissue diseases in the hospitals setting in Lomé. We conducted a retrospective and descriptive study from January 1, 1993 to December 30, 2012. We focused on the medical records of patients with connective tissue diseases hospitalized in five Departments of Dermatology and Rheumatology at the Hospitals in Lomé. Read More

Front Immunol 2018 5;9:2571. Epub 2018 Nov 5.

IRMB, Montpellier University, INSERM, CHU Montpellier, Montpellier, France.

Skin fibrosis is the hallmark of systemic sclerosis (SSc) a rare intractable disease with unmet medical need. We previously reported the anti-fibrotic potential of mesenchymal stem cells (MSCs) in a murine model of SSc. This model, based on daily intra-dermal injections of hypochlorite (HOCl) during 6 weeks, is an inducible model of the disease. Read More

Lupus 2018 Oct;27(1_suppl):32-35

Rheumatology Department, Queen Elizabeth Hospital, Edgbaston, Birmingham, UK.

Primary Sjögren's syndrome (pSS) is a systemic autoimmune disorder characterized by focal lymphocytic infiltration of the exocrine glands causing dry eyes and dry mouth. Similar glandular features can also occur as a late complication in patients with other rheumatic disorders, such as rheumatoid arthritis (RA), systemic lupus erythematosus (SLE) and scleroderma ('secondary' Sjögren's syndrome). Anti-Ro and/or anti-La (ENA) antibodies are found in approximately 70% of pSS patients, generally with ANA positivity. Read More

Ann Rheum Dis 2018 Nov 17. Epub 2018 Nov 17.

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

Objective: To evaluate integrin αvβ3 (alpha-v-beta-3)-targeted and somatostatin receptor 2 (SSTR2)-targeted nuclear imaging for the visualisation of interstitial lung disease (ILD).

Methods: The pulmonary expression of integrin αvβ3 and SSTR2 was analysed in patients with different forms of ILD as well as in bleomycin (BLM)-treated mice and respective controls using immunohistochemistry. Single photon emission CT/CT (SPECT/CT) was performed on days 3, 7 and 14 after BLM instillation using the integrin αvβ3-targeting Lu-DOTA-RGD and the SSTR2-targeting Lu-DOTA-NOC radiotracer. Read More

J Rheumatol 2018 Nov 15. Epub 2018 Nov 15.

From the Division of Rheumatology, Johns Hopkins University School of Medicine, Baltimore, Maryland; Division of Rheumatology, University of Utah, Salt Lake City, Utah; Department of Biostatistics, and Division of Rheumatology, University of Michigan, Ann Arbor Michigan, USA; Division of Rheumatology, University of Florence, Florence, Italy; Division of Rheumatology, Ghent University Hospital, Faculty of Internal Medicine, Ghent University, Ghent, Belgium; Rheumatology Unit, Royal Adelaide Hospital, Discipline of Medicine, University of Adelaide, Adelaide, Australia; Department of Medicine, and Division of Rheumatology, Thomas Jefferson University Hospital, Philadelphia, Pennsylvania, USA. This research was supported by the Scleroderma Clinical Trials Consortium. Z.H. McMahan, Assistant Professor, MD, MHS, Division of Rheumatology, Johns Hopkins University School of Medicine; T. Frech, Associate Professor, MD, MS, Division of Rheumatology, University of Utah; V. Berrocal, Associate Professor, PhD, Department of Biostatistics, University of Michigan; D. Lim, PhD student, BS, Department of Biostatistics, University of Michigan; C. Bruni, Clinical Research Fellow, MD, Division of Rheumatology, University of Florence; M. Matucci-Cerinic, Professor, MD, PhD, Division of Rheumatology, University of Florence; V. Smith, Associate Professor, MD, PhD, Division of Rheumatology, Ghent University Hospital, Faculty of Internal Medicine, Ghent University; K. Melsens, MSc, PhD student, Division of Rheumatology, Ghent University Hospital, Faculty of Internal Medicine, Ghent University; S. Proudman, Associate Professor, MBBS, Rheumatology Unit, Royal Adelaide Hospital, Discipline of Medicine, University of Adelaide; J. Zhang, Gastroenterology Fellow, MD, Department of Medicine, Thomas Jefferson University Hospital; F. Mendoza, Assistant Professor, MD, Division of Rheumatology, Thomas Jefferson University Hospital; M. Woods, Department of Biostatistics, University of Michigan; D. Khanna, Professor, MD, MS, Division of Rheumatology, University of Michigan. Dr. Z.H. McMahan and Dr. T. Frech contributed equally to this work. Address correspondence to Dr. D. Khanna, Professor of Medicine, Division of Rheumatology, Department of Internal Medicine, University of Michigan, 24 Frank Lloyd Wright Dr., Ann Arbor, Michigan 48105, USA. E-mail: Accepted for publication August 8, 2018.

Objective: Validated gastrointestinal (GI) symptoms scales are used in clinical practice to assess patient-reported GI involvement. We sought to determine whether University of California, Los Angeles (UCLA) GI Tract Questionnaire (GIT) 2.0 Reflux scale, Patient-Reported Outcomes Measurement Information System (PROMIS) Reflux scale, and the Quality of Life in Reflux and Dyspepsia questionnaire (QOLRAD) are sensitive to identifying changes in GI symptoms following therapeutic intervention in participants with systemic sclerosis (SSc) and gastroesophageal reflux disease (GERD). Read More

Eur J Dermatol 2018 Oct;28(5):718-720

Centre Hospitalier Régional Universitaire (CHRU) Tours, Department of Dermatology, 37044 Tours Cedex 9, France, Universities of Nantes and Tours, SPHERE-INSERM 1246, 37000 Tours, France.

Eur J Dermatol 2018 Nov 13. Epub 2018 Nov 13.

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

Medicine (Baltimore) 2018 Nov;97(46):e13226

Department of Cardiology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences, Beijing.

Rationale: Complete heart block (CHB) is a common clinical manifestation. Systemic sclerosis (SSc) is a rare etiology of CHB that has not received enough attention. Whether pacemaker implantation is required remains inconclusive, especially when patients have no symptoms or mild symptoms of CHB. Read More

Postepy Dermatol Alergol 2018 Oct 19;35(5):490-494. Epub 2018 Jul 19.

Department of Infectious Diseases and Neuroinfections, Medical University of Białystok, Białystok, Poland.

Introduction: Acrodermatitis chronica atrophicans (ACA) is probably the most common late and chronic manifestation of the Lyme borreliosis seen in European patients.

Aim: To analyze epidemiological data, and to investigate the effects of treatment of patients with ACA.

Material And Methods: Nine patients were included in the study. Read More

Postepy Dermatol Alergol 2018 Oct 19;35(5):470-473. Epub 2018 Jul 19.

Department of Dermatology, Poznan University of Medical Sciences, Poznan, Poland.

Introduction: Morphea, also known as localized scleroderma, is an autoimmune skin disease which is characterized by excessive accumulation of collagen that leads to the thickening of the dermis and subcutaneous tissue. There is an unclear relationship between morphea and other autoimmune diseases, especially related to the thyroid gland.

Aim: To determine the occurrence of increased antithyroid antibodies in patients with morphea in relation to the clinical manifestations of the disease. Read More

JCI Insight 2018 Nov 15;3(22). Epub 2018 Nov 15.

Division of Endocrinology and Metabolism, Department of Medicine, and the Institute for Human Genetics, UCSF, San Francisco, California, USA.

Background: Inflammation helps regulate normal growth and tissue repair. Although bone morphogenetic proteins (BMPs) and inflammation are known contributors to abnormal bone formation, how these pathways interact in ossification remains unclear.

Methods: We examined this potential link in patients with fibrodysplasia ossificans progressiva (FOP), a genetic condition of progressive heterotopic ossification caused by activating mutations in the Activin A type I receptor (ACVR1/ALK2). Read More

BMJ Case Rep 2018 Nov 14;2018. Epub 2018 Nov 14.

Department of Dermatology, Southend University Hospital NHS Foundation Trust, Westcliff-on-Sea, UK.

Parry-Romberg syndrome (PRS) is characterised by progressive but self-limiting facial hemiatrophy. We describe a 48-year-old woman with a 3-year history of gradually worsening right facial hemiatrophy on a background of scleroderma. Her initial primary concern was alopecia. Read More

Case Rep Nephrol 2018 23;2018:6051083. Epub 2018 Oct 23.

Medical student, Javeriana University, Bogota, Colombia.

We had the challenged to treat a 40-year-old female with Systemic Scleroderma who was showing unspecific symptoms. During her time at the hospital she rapidly develops renal dysfunction, associated with hypertension. She required renal replacement therapy initiation and we observed a decline in hemoglobin and platelets numbers. Read More

Semin Arthritis Rheum 2018 Oct 14. Epub 2018 Oct 14.

Division of Rheumatology, Scleroderma Program, Department of Internal Medicine, University of Michigan, Ann Arbor, MI, USA. Electronic address:

Objective: Pulmonary arterial hypertension (PAH) has high morbidity and mortality in connective tissue diseases (CTDs), especially systemic sclerosis (SSc). In this systematic review, we provide an update on screening measures for early detection of PAH in CTD.

Methods: Manuscripts published between July 2012 and October 2017, which incorporated screening measures to identify patients with PAH by right heart catheterization, were identified. Read More

Ann Rheum Dis 2018 Nov 10. Epub 2018 Nov 10.

Department of Rheumatology, University Hospital Zurich, Zurich, Switzerland

Acta Reumatol Port 2018 Jul-Sep;43(3):210-216

Faculty of Medicine- Suez canal University.

Background: Digital ulcers (DUs) in Systemic sclerosis (SSc) result from recurrent Raynaud's phenomenon (RP) and microtrauma with high impact on quality of life. Medical use of ozone (triatomic oxygen) was initiated in the 19th century. Ozone has multiple therapeutic effects in wound healing due to the property of releasing nascent oxygen, which has been shown to stimulate antioxidant enzymes. Read More

Skin Res Technol 2018 Nov 10. Epub 2018 Nov 10.

Department of Veterans Affairs, Tennessee Valley Healthcare System, Nashville, Tennessee.

Background: Clinical assessment of skin stiffness is unreliable in many applications. The durometer, an industrial device to measure hardness, has previously been applied in scleroderma. The Myoton is a noninvasive handheld device for assessing soft tissue biomechanical parameters. Read More

Rheumatol Int 2018 Nov 9. Epub 2018 Nov 9.

Clinic of Rheumatology, University Hospital "St. Ivan Rilski", Medical University-Sofia, Sofia, Bulgaria.

Our aim was to appraise publications from Bulgaria, to assess their global impact, and to describe features and challenges unique to the rheumatology practice in Bulgaria characterized by stringent cost constraints. The Scopus database was queried on 25th July 2018 and data on the number of published documents, their Hirsch-indices and citations number were extracted. Published Bulgarian guidelines for the management of rheumatic diseases and the presented data on Bulgarian Rheumatology Society were identified based on prior knowledge of the authors. Read More

Front Immunol 2018 25;9:2475. Epub 2018 Oct 25.

Department of Pathology, New York University School of Medicine, New York, NY, United States.

Inflammatory bowel disease (IBD) is a chronic inflammatory condition caused by an aberrant immune response to microbial components of the gastrointestinal tract. Plasmacytoid dendritic cells (pDCs) are innate immune cells specialized in the production of type I interferons and were recently implicated in the pathogenesis of autoimmune disorders such as lupus and scleroderma. While pDCs were shown to infiltrate intestinal mucosa of IBD patients and proposed to participate in intestinal inflammation, their net contribution to the disease remains unclear. Read More

Ann Rheum Dis 2018 Nov 8. Epub 2018 Nov 8.

Department of Medicine, Duke University, Durham, North Carolina, USA.

Objective: To assess survival and identify predictors of survival in patients with systemic sclerosis-interstitial lung disease (SSc-ILD) who participated in the Scleroderma Lung Studies (SLS) I and II.

Methods: SLS I randomised 158 patients with SSc-ILD to 1  year of oral cyclophosphamide (CYC) vs placebo. SLS II randomised 142 patients to 1 year of oral CYC followed by 1 year of placebo vs 2 years of mycophenolate mofetil. Read More

Front Immunol 2018 23;9:2434. Epub 2018 Oct 23.

Northwestern Scleroderma Program, Feinberg School of Medicine, Chicago, IL, United States.

Systemic sclerosis (SSc) is a poorly understood heterogeneous condition with progressive multi-organ fibrosis. Recent genetic and genomic evidence suggest a pathogenic role for dysregulated innate immunity and toll-like receptor (TLR) activity in SSc. Levels of both TLR4, as well as certain endogenous TLR ligands, are elevated in skin and lung tissues from patients with SSc and correlate with clinical disease parameters. Read More

J Immunol Res 2018 3;2018:3491269. Epub 2018 Oct 3.

Department of Pharmaceutical Sciences, University of Connecticut, Storrs, CT 06269-3092, USA.

TNIP1 protein is increasingly being recognized as a key repressor of inflammatory signaling and a potential factor in multiple autoimmune diseases. In addition to earlier foundational reports of TNIP1 SNPs in human autoimmune diseases and TNIP1 protein-protein interaction with receptor regulating proteins, more recent studies have identified new potential interaction partners and signaling pathways likely modulated by TNIP1. Subdomains within the TNIP1 protein as well as how they interact with ubiquitin have not only been mapped but inflammatory cell- and tissue-specific consequences subsequent to their defective function are being recognized and related to human disease states such as lupus, scleroderma, and psoriasis. Read More

RMD Open 2018 18;4(Suppl 1):e000789. Epub 2018 Oct 18.

Université Paris-Sud: AP-HP, Hôpitaux Universitaires Paris-Sud; INSERM UMR 1184, Le Kremlin Bicêtre, France.

Sjögren's syndrome (SS) is a complex autoimmune rheumatic disease that specifically targets salivary and lachrymal glands. As such, patients typically had ocular and oral dryness and salivary gland swelling. Moreover, skin, nasal and vaginal dryness are frequently present. Read More

RMD Open 2018 18;4(Suppl 1):e000785. Epub 2018 Oct 18.

Rheumatology and Clinical Immunology Unit, Civil Hospital, Brescia, Italy.

Antiphospholipid syndrome (APS) is a rare disease characterised by venous and/or arterial thrombosis, pregnancy complications and the presence of specific autoantibodies called antiphospholipid antibodies. This review aims to identify existing clinical practice guidelines (CPG) as part of the ERN ReCONNET project, aimed at evaluating existing CPGs or recommendations in rare and complex diseases. Seventeen papers providing important data were identified; however, the literature search highlighted the scarceness of reliable clinical data to develop CPGs. Read More

RMD Open 2018 18;4(Suppl 1):e000783. Epub 2018 Oct 18.

Department of Rheumatology, Universitätsklinikum Düsseldorf, Düsseldorf, Germany.

Mixed connective tissue disease (MCTD) is a complex overlap disease with features of different autoimmune connective tissue diseases (CTDs) namely systemic sclerosis, poly/dermatomyositis and systemic lupus erythematous in patients with antibodies targeting the U1 small nuclear ribonucleoprotein particle. In this narrative review, we summarise the results of a systematic literature research which was performed as part of the European Reference Network on Rare and Complex Connective Tissue and Musculoskeletal Diseases project, aimed at evaluating existing clinical practice guidelines (CPGs) or recommendations. Since no specific CPGs on MCTD were found, other CPGs developed for other CTDs were taken into consideration in order to discuss what can be applied to MCTD even if designed for other diseases. Read More

RMD Open 2018 18;4(Suppl 1):e000782. Epub 2018 Oct 18.

Department of Clinical and Experimental Medicine, University of Florence, Florence, Italy.

Systemic sclerosis (SSc) is an orphan disease characterised by autoimmunity, fibrosis of the skin and internal organs, and vasculopathy. SSc may be associated with high morbidity and mortality. In this narrative review we summarise the results of a systematic literature research, which was performed as part of the European Reference Network on Rare and Complex Connective Tissue and Musculoskeletal Diseases project, aimed at evaluating existing clinical practice guidelines or recommendations. Read More

Ann Vasc Dis 2018 Sep;11(3):335-338

Department of Organ Transplantation and General Surgery, Kyoto Prefectural University of Medicine, Kyoto, Japan.

Digital ischemia is a serious problem in peripheral artery diseases (PAD) patients. Case 1: A 60-year-old woman with large arteriovenous fistula (AVF) complained of digital ischemia symptoms. The patient underwent dissection of AVF and distal bypass to the palmar arch with successful repair. Read More