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Pak J Med Sci 2019 Jan-Feb;35(1):86-89

Dr. Ammad Asghar, FCPS (Medicine), PGR Rheumatology, Department of Rheumatology, Sheikh Zayed Hospital, Lahore, Pakistan.

Objective: To determine the frequency and predictors of pulmonary hypertension in patients with Systemic Lupus Erythematosus in a Pakistani population, presenting at a tertiary care hospital.

Methods: This cross-sectional study was conducted at the Department of Rheumatology, Shiekh Zayed Hospital, Lahore from March to June 2018. A total of 97 patients, who fulfilled the Systemic Lupus Erythematosus (SLE) criteria of American College of Rheumatology (ACR) 1992 were enrolled. Read More

J Am Soc Nephrol 2019 03 14. Epub 2019 Mar 14.

Paris Translational Research Center for Organ Transplantation, Institut national de la santé et de la recherche médicale, Unité mixte de recherche-S970, Paris, France;

Background: Transplant glomerulopathy, a common glomerular lesion observed after kidney transplant that is associated with poor prognosis, is not a specific entity but rather the end stage of overlapping disease pathways. Its heterogeneity has not been precisely characterized to date.

Methods: Our study included consecutive kidney transplant recipients from three centers in France and one in Canada who presented with a diagnosis of transplant glomerulopathy (Banff cg score ≥1 by light microscopy), on the basis of biopsies performed from January of 2004 through December of 2014. Read More

Actas Dermosifiliogr 2019 Mar 6. Epub 2019 Mar 6.

Departamento de Dermatología, Hospital Universitario Dr. José E. González, Facultad de Medicina, Universidad Autónoma de Nuevo León, Monterrey, México. Electronic address:

Capillaroscopy produces in vivo images of skin microcirculation. It is a simple, noninvasive tool for analyzing microvascular abnormalities and, as such, can be used in both adults and children. Capillaroscopy has proven useful in many diseases, but it is of particular value in Raynaud phenomenon and other autoimmune diseases. Read More

Am Surg 2018 Aug;84(8):e323-e324

J Clin Psychopharmacol 2019 Mar/Apr;39(2):178-179

Department of Orthopaedic SurgeryFaculty of Medicine Siriraj Hospital Mahidol University Bangkok, Thailand. Department of Psychiatry Faculty of Medicine Siriraj Hospital Mahidol University Bangkok, Thailand

Ann Intern Med 2019 Feb;170(4):JC21

Eur J Intern Med 2019 Feb 5. Epub 2019 Feb 5.

Unidade de Doenças Auto-Imunes, Internal Medicine Department 7.2, Hospital Curry Cabral, Centro Hospitalar Universitário Lisboa Central, Lisboa, Portugal.

Dtsch Med Wochenschr 2019 Feb 31;144(3):189-193. Epub 2019 Jan 31.

Klinik für Rheumatologie und Klinische Immunologie, Universitätsklinikum Schleswig-Holstein, Campus Lübeck.

This literature review summarizes the main findings in systemic sclerosis (SSc) made in the last few years. Accordingly, the disease pathogenesis is mainly driven by the adaptive immune system, which is proven by the effects of autologous stem cell transplantation. Particularly, autoantibodies can activate both adaptive as well as innate immune cells as identified for the anti-angiotensin receptor antibodies. Read More

J Dermatol 2019 Jan 31. Epub 2019 Jan 31.

Department of Dermatology, Faculty of Medicine, University of Tsukuba, Tsukuba, Japan.

Morphea profunda refers to inflammatory and sclerotic lesions that start primarily from the deep dermis, subcutaneous fat and fascia. Its pediatric case published work is limited. Here, we report the case of an 8-year-old girl with a 5-year history of multiple subcutaneous nodules on her extremities and a right wrist joint contracture who had been previously diagnosed with juvenile idiopathic arthritis and treated with salazosulfapyridine, low-dose prednisolone (PSL) and methotrexate. Read More

J Thromb Thrombolysis 2019 Feb;47(2):248-254

John Paul II Hospital, Krakow, Poland.

Associations of Raynaud's phenomenon (RP) with venous thromboembolism (VTE) are unclear. We investigated the occurrence of RP together with prothrombotic state markers and fibrin clot properties in VTE patients. In this prospective cohort study we enrolled 360 patients free of known autoimmune disease. Read More

Dermatol Online J 2018 Dec 15;24(12). Epub 2018 Dec 15.

The Ronald O. Perelman Department of Dermatology, New York University Langone Health, New York, New York Yale University School of Medicine, New Haven, Connecticut.

Calcinosis, Raynaud phenomenon, esophageal dysmotility, sclerodactyly, and telangiectasia (CREST) syndrome is a form of a rare, clinical subtype of systemic sclerosis, known as limited systemic sclerosis. Limited systemic sclerosis, including CREST syndrome, manifests as fibrotic skin changes restricted to the hands and face, with vascular, musculoskeletal, and visceral involvement. We present a case of a 75-year-old woman with a longstanding history of CREST syndrome complicated by a digital ulceration and persistent pain associated with recalcitrant Raynaud phenomenon. Read More

J Psychosom Res 2019 Jan 5;116:6-9. Epub 2018 Nov 5.

Department of Internal Medicine, Faculty of Medicine, University of Debrecen, Debrecen, Hungary.

Objective: To compare anxiety, depression, physical health and quality of life in patients with primary Raynaud's phenomenon (PRP) and patients with secondary Raynaud's phenomenon (SRP).

Method: Adult patients with Raynaud's phenomenon (RP) were assessed for the severity of anxiety and depressive symptoms, physical health and quality of life by using the General Anxiety Disorder Scale (GAD-7), the Beck Depression Inventory (BDI), the 12-item Short Form Health Survey: Physical Component Scale (PCS-12), and the Raynaud Specific Quality of Life Questionnaire (RQLQ), respectively. Patients with PRP and SRP were recruited for comparison at a single clinical center in Debrecen, Hungary from September to December 2017. Read More

Clin Microbiol Infect 2019 Mar 11;25(3):324-325. Epub 2019 Jan 11.

Département de Bactériologie-Virologie, Hygiène et Parasitologie-Mycologie, Centre Hospitalier Régional Universitaire (CHRU) de Brest, Brest, France; UMR1078 "Génétique, Génomique Fonctionnelle et Biotechnologies", INSERM, Université de Brest, EFS, IBSAM, Brest, France.

Arch Rheumatol 2018 Sep 18;33(3):344-351. Epub 2018 Jan 18.

Department of Pediatric Rheumatology, İstanbul University, Cerrahpasa Medical School, İstanbul, Turkey.

Objectives: This study aims to evaluate the demographic and clinical features, laboratory data, treatment modalities, and outcomes of juvenile systemic sclerosis (JSS) and juvenile localized scleroderma (JLS) patients at a referral pediatric rheumatology center in Turkey.

Patients And Methods: Medical records of a total of 57 patients, including 29 with JSS (1 male, 28 females; mean age 18.3±3. Read More

Neuroscience 2019 02 27;399:199-210. Epub 2018 Dec 27.

CNRS UPR 4301, CBM, Orléans, France. Electronic address:

LIMK2 is involved in neuronal functions by regulating actin dynamics. Different isoforms of LIMK2 are described in databanks. LIMK2a and LIMK2b are the most characterized. Read More

Medicine (Baltimore) 2018 Dec;97(51):e13545

Department of Nephrology, Fujita Health University School of Medicine.

Rationale: Immunoglobulin G4 related disease (IgG4-RD) rarely coexists with other autoimmune diseases, though we had a patient whose primary clinical problem was shifted from IgG4-RD to systemic lupus erythematosus (SLE) after gastrectomy. The present paper aimed to report pathological findings and clinical course of the patient.

Patient Concerns: The patient was a male aged 74 years old with gastric cancer characterized by the following symptoms: Raynaud phenomenon, polyarthralgia, and swollen parotid glands on both sides. Read More

Interact J Med Res 2018 Dec 19;7(2):e18. Epub 2018 Dec 19.

Division of Gastroenterology and Hepatology, Indiana University, Indianapolis, IN, United States.

Background: Concurrent autoimmune illnesses contribute to increased medical burden and reduced quality of life in patients with autoimmune hepatitis (AIH). The frequency of coexisting autoimmune conditions among North American patients with AIH and their families remains incomplete. Challenges associated with disease capture in the electronic medical record, high study costs, and geographic spread of patients are formidable barriers to understanding the extent of concurrent autoimmune conditions in these groups. Read More

J Rheumatol 2018 Dec 15. Epub 2018 Dec 15.

From the Department of Internal Medicine, Division of Vascular Medicine, and the Department of Rheumatology and Clinical Immunology, University of Groningen, University Medical Center Groningen, Groningen; Department of Rheumatology, Medical Center Leeuwarden, Leeuwarden, the Netherlands. A.M. van Roon, Department of Internal Medicine, Division of Vascular Medicine, University of Groningen, University Medical Center Groningen; C.C. Huisman, Department of Internal Medicine, Division of Vascular Medicine, University of Groningen, University Medical Center Groningen; A.M. van Roon, PhD, Department of Internal Medicine, Division of Vascular Medicine, University of Groningen, University Medical Center Groningen; D. Zhang, MD, Department of Rheumatology, Medical Center Leeuwarden; A.J. Stel, MD, PhD, Department of Rheumatology and Clinical Immunology, University of Groningen, University Medical Center Groningen; A.J. Smit, MD, PhD, Department of Internal Medicine, Division of Vascular Medicine, University of Groningen, University Medical Center Groningen; H. Bootsma, MD, PhD, Department of Rheumatology and Clinical Immunology, University of Groningen, University Medical Center Groningen; D.J. Mulder, MD, PhD, Department of Internal Medicine, Division of Vascular Medicine, University of Groningen, University Medical Center Groningen. Address correspondence to A.M. van Roon, Department of Internal Medicine, Division of Vascular Medicine, University Medical Center Groningen, Huispostcode AA41, Hanzeplein 1, Postbus 30 001, Groningen, the Netherlands. E-mail: Accepted for publication September 11, 2018.

Objective: To assess the presence of a systemic sclerosis (SSc) pattern on nailfold capillary microscopy (NCM) in patients with Raynaud phenomenon (RP) and to explore its association with abnormal pulmonary function tests (PFT).

Methods: NCM patterns were assessed in 759 consecutive patients with RP. Patterns were classified as normal (n = 354), nonspecific (n = 159), or SSc pattern (n = 246). Read More

Pol Arch Intern Med 2019 01 13;129(1):36-42. Epub 2018 Dec 13.

INTRODUCTION Raynaud phenomenon is a medical condition in which the spasm of the arteries causes episodes of reduced blood flow. Potential disorders in the microcirculation of the oral mucosa may promote the occurrence of lesions. OBJECTIVES The aim of the study was to investigate the association of the frequency of oral cavity lesions with oral microcirculatory dysfunction in patients with primary Raynaud phenomenon (PRP) in comparison with healthy control group. 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

Ned Tijdschr Geneeskd 2018 11 27;162. Epub 2018 Nov 27.

Universitaire Ziekenhuizen Leuven, afd. Algemene Interne Geneeskunde, België.

The antisynthetase syndrome (AS) is a rare auto-immune disease characterised by inflammatory myopathies, interstitial lung disease, inflammatory arthritis, Mechanic's Hands and Raynaud phenomenon. AS infrequently presents with life-threatening lung disease as its primary or sole manifestation. By means of two clinical case reports, an overview is given of recent advances in diagnosis and treatment of AS-related interstitial lung disease. Read More

Dermatol Pract Concept 2018 Oct 31;8(4):277-282. Epub 2018 Oct 31.

Department of Dermatology, University of Campania Luigi Vanvitelli, Naples, Italy.

Background: Recent studies have highlighted new botulinum neurotoxin (BoNT) applications in the field of dermatology.

Objective: To review current knowledge of BoNT use in dermatology.

Methods: The literature of the last 5 five years has been reviewed. Read More

J Assoc Physicians India 2018 May;66(5):69-70

Chief and Consultant Rheumatologist, Center for Rheumatic Diseases, Pune, Maharashtra.

Introduction: Primary Sjogren's Syndrome (pSS) with Hypokalemic Periodic Paralysis(HPP) whether an association or a different clinical subset needs review.

Methods: Cross-sectional retrospective study of subjects of Primary Sjogren's Syndrome with Hypokalemic Periodic Paralysis(HPP) identified from database maintained at Centre For Rheumatic Diseases, Pune since 1996 with records of over 50000 patients. The diagnosis was clinical. Read More

Eur J Hum Genet 2019 Mar 20;27(3):432-441. Epub 2018 Nov 20.

Science for Life Laboratory, Department of Medical Biochemistry and Microbiology, Uppsala University, Box 582, SE-751 24, Uppsala, Sweden.

Systemic lupus erythematosus (SLE) is an autoimmune disorder with heterogeneous clinical presentation and complex etiology involving the interplay between genetic, epigenetic, environmental and hormonal factors. Many common SNPs identified by genome wide-association studies (GWAS) explain only a small part of the disease heritability suggesting the contribution from rare genetic variants, undetectable in GWAS, and complex epistatic interactions. Using targeted re-sequencing of coding and conserved regulatory regions within and around 215 candidate genes selected on the basis of their known role in autoimmunity and genes associated with canine immune-mediated diseases, we identified a rare regulatory variant rs200395694:G > T located in intron 4 of the MEF2D gene encoding the myocyte-specific enhancer factor 2D transcription factor and associated with SLE in Swedish cohorts (504 SLE patients and 839 healthy controls, p = 0. Read More

Handb Clin Neurol 2018 ;157:677-685

Department of Physiology, Institute of Basic Medical Sciences, University of Oslo, Oslo, Norway. Electronic address:

The term acral coldness is used to describe physiologic or pathologic situations in humans where the fingers and toes are exceptionally cold in spite of normal central body temperature. In the thermoneutral zone, the blood flow to acral skin normally shows large fluctuations between high and low values, with a frequency of about 3 cycles per minute. At an acral skin temperature of about 21°C, finger blood flow is constantly low. Read More

Clin Case Rep 2018 Nov 17;6(11):2089-2091. Epub 2018 Sep 17.

Pediatric Emergency Care Kitakyushu Municipal Yahata Hospital Kitakyushu, Fukuoka Japan.

We present a case of severe Raynaud phenomenon (RP) in an infant. The current strategy of RP treatment is incomplete; excluding secondary Raynaud phenomenon is vital as well. This case aims to help those with similar symptoms in the future by gathering data on cases. Read More

J Rheumatol 2019 Mar 15;46(3):279-284. Epub 2018 Nov 15.

From the Rheumatology Unit, Azienda Policlinico of Modena, University of Modena and Reggio Emilia, Modena, Italy; ACURA Rheumatology Center, Bad Kreuznach, Germany; Rheumatology Division, Hospital Universitario Marqués de Valdecilla, IDiVAL, University of Cantabria, Santander, Spain; Rheumatology Division, University Hospital of Frankfurt, Frankfurt, Germany; Interstitial Lung Disease and Rheumatology Unit, Instituto Nacional de Enfermedades Respiratorias, Ismael Cosío Villegas, Mexico City, Mexico; Servicio de Reumatología, Hospital Universitario La Paz, Madrid, Spain; Interdisciplinary Department of Medicine (DIM), Rheumatology Unit, University of Bari, Bari, Italy; Rheumatology Department, Hospital Universitario de la Princesa, Instituto de Investigación Sanitaria (IIS) Princesa, Madrid; Unidad de Enfermedades Autoinmunes Sistémicas, Servicio de Medicina Interna, Universidad Autonoma de Barcelona, on behalf of the GEAS group, Barcelona, Spain; Department of Experimental and Clinical Medicine, and Department of Medical and Surgical Critical Care, Section of Dermatology, University of Florence, Florence; Unità Operativa Complessa (UOC) Reumatologia, Ospedale San Camillo-Forlanini, Rome; ACURA Centre for Rheumatic Diseases, Baden-Baden, Germany; Rheumatology Department, Città Della Salute e della Scienza, Torino; Division of Rheumatology, University and Institute for Research and Health Care (IRCCS) Policlinico S. Matteo Foundation, Pavia, Italy; Servicio de Reumatología, Hospital General Universitario Gregorio Marañón, Madrid, Spain; Dermatology Clinic, University Hospital of Trieste; Department of Pneumology and Respiratory Intermediate Care Unit, University Hospital of Cattinara, Trieste; Rheumatology Unit, Azienda Ospedaliero-Universitaria Ospedali Riuniti di Trieste, Trieste, Italy; Medizinische Klinik A, Klinikum der Stadt, Ludwigshafen, Germany; Research Laboratory and Academic Division of Clinical Rheumatology, Department of Internal Medicine, University of Genoa, San Martino Polyclinic Hospital IRCCS Genoa, Genoa, Italy; Interstitial and Rare Lung Disease Unit, Ruhrlandklinik University Hospital, University of Duisburg-Essen, Essen, Germany; UOC Reumatologia, Azienda Ospedaliero Universitaria S. Anna, University of Ferrara, Italy; Department for Rheumatology and Clinical Immunology, St. Josef Krankenhaus, University Clinic, Essen, Germany; Division of Rheumatology, Department of Clinical and Experimental Medicine, University of Pisa, Pisa; Department of Internal Medicine, Rheumatology and Clinical Immunology, University Hospital Johannes-Gutenberg, Mainz, Germany; University of Ghent, Ghent University Hospital, Ghent, Belgium.

Objective: To describe nailfold videocapillaroscopy (NVC) features of patients with antisynthetase syndrome (AS) and to investigate possible correlations with clinical and serological features of the disease.

Methods: We retrospectively analyzed NVC images of 190 patients with AS [females/males 3.63, mean age 49. Read More

J Intern Med 2019 Mar 8;285(3):317-332. Epub 2018 Nov 8.

Department of Neurology, Leiden University Medical Centre, Leiden, The Netherlands.

Background: Retinal vasculopathy with cerebral leukoencephalopathy and systemic manifestations (RVCL-S) is a small vessel disease caused by C-terminal truncating TREX1 mutations. The disease is typically characterized by vascular retinopathy and focal and global brain dysfunction. Systemic manifestations have also been reported but not yet systematically investigated. Read More

Int J Obstet Anesth 2018 Nov 29;36:111-114. Epub 2018 May 29.

The Johns Hopkins Hospital, 1800 Orleans Street, Baltimore, MD 21287, USA.

Raynaud's disease is a medical condition in which arterial spasm causes episodes of reduced blood flow, in the setting of certain triggers such as cold weather. Patients with this condition are at risk of adverse reactions if they receive medications with vasoactive properties. Methylergonovine maleate is one drug used during cesarean delivery to treat postpartum hemorrhage due to uterine atony. Read More

Ann Intern Med 2018 Nov 30;169(10):694-703. Epub 2018 Oct 30.

Université Grenoble Alpes and Grenoble Alpes University Hospital, Grenoble, France (M.R., O.G., C.K., M.M., A.L., S.B., C.S., A.P., C.C., B.I., P.C., J.C.).

Background: Treatment of Raynaud phenomenon (RP) with phosphodiesterase-5 inhibitors has shown moderate efficacy. Adverse effects decrease the risk-benefit profile of these drugs, and patients may not be willing to receive long-term treatment. On-demand single doses before or during exposure to cold may be a good alternative. Read More

Best Pract Res Clin Gastroenterol 2018 Jun - Aug;34-35:49-54. Epub 2018 May 23.

Dept. of Surgery, Oncology and Gastroenterology, University of Padova, Italy.

Patients with PBC have at least 60% of probability to have an autoimmune extrahepatic condition. The pathogenesis of these conditions includes a common mechanism involving both innate and adaptive immune responses targeting cholangiocytes and different extrahepatic tissues. The recent EASL guidelines recommend the management of these conditions, although detailed practical treatments have not been indicated. Read More

Horm Cancer 2019 Feb 6;10(1):36-44. Epub 2018 Oct 6.

Department of Urology, Hôpital Foch, University of Versailles-Saint-Quentin-en-Yvelines, 40 Rue Worth, 92150, Suresnes, France.

Failure rates after first-line treatment of localized prostate cancer (PCa) treatment remain high. Improvements to patient selection and identification of at-risk patients are central to reducing mortality. We aimed to determine if cancer aggressiveness correlates with androgen levels in patients undergoing radical prostatectomy for localized PCa. Read More

Acta Obstet Gynecol Scand 2019 Feb 22;98(2):240-249. Epub 2018 Nov 22.

National Resource Center for Late Effects after Cancer Treatment, Oslo University Hospital, Norwegian Radium Hospital, Oslo, Norway.

Introduction: The aim was to evaluate "overall neuropathy", defined as peripheral paresthesia and Raynaud's phenomenon, in long-term survivors of malignant ovarian germ cell tumors (MOGCTs) treated with cisplatin-based chemotherapy (CBCT).

Material And Methods: Ninety-three MOGCT survivors recorded in Norway in 1980-2009 (median follow up: 15 years) were included in this analysis. Forty-nine received CBCT (CBCT group) and 44 received other or no chemotherapy (non-CBCT group). Read More

Clin Exp Rheumatol 2018 Jul-Aug;36 Suppl 113(4):175-182. Epub 2018 Sep 20.

University of Utah, Department of Internal Medicine; University of Utah, Department of Exercise and Sport Science; University of Utah, Dept. of Nutrition and Integrative Physiology, University of Utah; and VAMC Salt Lake City, GRECC, Salt Lake City, USA.

There are no Federal Drug Administration approved drugs for the treatment of systemic sclerosis vascular digital ulcers (DU) in the United States, which are thought to be an end-stage result of prolonged ischaemia due to severe, prolonged Raynaud's phenomenon. Most therapeutics for vasodilation used in SSc work different pathways to target the smooth muscle to induce vessel relaxation. Longitudinal studies of vascular function allow insight into the effects of medications used for Raynaud's phenomenon in the SSc patient population. Read More

Clin Exp Rheumatol 2018 Jul-Aug;36 Suppl 113(4):88-94. Epub 2018 Sep 29.

Lady Davis Inst. Med. Res., Jewish General Hosp., Montreal; Dept.of Psychiatry, Dept.of Medicine, Dept.of Epidemiology, Biostatistics and Occupational Health, Dept.of Educational & Counselling Psychology, Dept.of Psychology , McGill Univ., Montreal, Canada.

Objectives: Impaired hand function in systemic sclerosis (SSc) is a primary cause of disability and contributes diminished health-related quality of life. The objective of the present study was to evaluate sociodemographic, lifestyle, and disease-related factors independently associated with hand function in SSc.

Methods: Patients enrolled in the Scleroderma Patient-centered Intervention Network Cohort who completed baseline study questionnaires between March 2014 and September 2017 were included. Read More

Clin Exp Rheumatol 2018 Jul-Aug;36 Suppl 113(4):68-75. Epub 2018 Sep 29.

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

Objectives: To describe differences in clinical presentation between men and women in a large group of patients with early (<3 years' duration) systemic sclerosis (SSc) according to disease subsets.

Methods: A cross-sectional analysis of the prospective EULAR Scleroderma Trial and Research database (EUSTAR) was performed. Patients fulfilling preliminary ACR 1980 classification criteria for SSc, with less than 3 years from the first non-Raynaud's symptom at first entry, were selected. Read More

Ann Plast Surg 2019 Jan;82(1):53-54

Department of Plastic Surgery, MD Anderson Cancer Center, Houston, TX.

Isolated lingual and lower face Raynaud phenomenon without primary Raynaud of the digits is a very rare condition associated with chemoradiation therapy (RT) in previous reports. The condition, which more commonly presents in patients with a history of Raynaud disease, is often self-limiting, but vasodilating agents and steroids have been suggested as possible treatment options. Spasmodic torticollis is a different, more common entity, also associated with history of RT or previous head and neck surgery. Read More

Prim Care Companion CNS Disord 2018 Sep 13;20(5). Epub 2018 Sep 13.

Clin Drug Investig 2018 Nov;38(11):1061-1069

Clinic III for Internal Medicine, Department of Cardiology, Heart Center, University Hospital of Cologne, Kerpener Str. 62, 50937, Cologne, Germany.

Background And Objective: Raynaud's phenomenon (RP) is characterized by transient digital ischemia and is commonly associated with connective tissue disease. Treatment remains unsatisfactory. Here we evaluate the efficacy, safety, and pharmacokinetics of a single dose of the soluble guanylate cyclase stimulator riociguat in RP. Read More

Adv Skin Wound Care 2018 Oct;31(10):446-455

Joshua M. Cohen, MD • Resident Physician • New York University School of Medicine • Hansjörg Wyss Department of Plastic Surgery • NYU Langone Health • New York, New York Rachel A. Sibley, MD • Resident Physician • New York University School of Medicine • Department of Internal Medicine • NYU Langone Health • New York, New York Ernest S. Chiu, MD, FACS • Associate Professor • Hansjörg Wyss Department of Plastic Surgery • NYU Langone Health • New York, New York Sheel Sharma, MD • Clinical Associate Professor • Hansjörg Wyss Department of Plastic Surgery • NYU Langone Health • New York, New York.

General Purpose: To provide information about the pathophysiology, diagnosis, and treatment options for systemic sclerosis.

Target Audience: This continuing education activity is intended for physicians, physician assistants, nurse practitioners, and nurses with an interest in skin and wound care.

Learning Objectives/outcomes: After participating in this educational activity, the participant should be better able to:1. Read More

Complement Ther Med 2018 Oct 1;40:191-194. Epub 2017 Nov 1.

ARCIM Institute, Filderklinik, Filderstadt, Germany; Department of Pediatrics, University Hospital Tübingen, Germany.

Introduction: Raynaud's phenomenon is the earliest manifestation of systemic sclerosis. Nitroglycerin gel is the only proven topical therapy.

Methods: A 53-year-old woman with systemic sclerosis had topical Rosamarinus officinalis (rosemary) oil, often used in anthroposophic medicine, applied to her hands over 3days and then, separately, olive oil. Read More

J Med Vasc 2018 Sep 24;43(5):320-324. Epub 2018 Aug 24.

Service de chirurgie de la main, chirurgie plastique et reconstructrice de l'appareil locomoteur, CHU de Nancy, centre chirurgical Emile-Gallé, 49, rue Hermite, 54000 Nancy, France. Electronic address:

Hypothenar hammer syndrome is a rare entity secondary to ulnar artery damage in the wrist, affecting mainly those exposed to repeated hand-palm trauma. Surgery is discussed in case of severe symptoms, resistant to medical treatment, and/or when anatomical lesions with emboligenic potential are demonstrated in the radiological exams. In this case, resection of the pathological zone with revascularization by autologous vein graft is the best option. Read More

Ultrasound Med Biol 2018 10 6;44(10):2081-2088. Epub 2018 Jul 6.

Department of Radiology, Thomas Jefferson University, Philadelphia, PA, USA.

The purpose of this study was to investigate the use of photoacoustic imaging for quantifying fingertip oxygenation as an approach to diagnosing and monitoring Raynaud's phenomenon. After 30 min of acclimation to room temperature, 22 patients (7 patients with secondary Raynaud's associated to Scleroderma and 15 healthy controls) provided informed consent to undergo fingertip Doppler imaging and high-frequency photoacoustic imaging before and 5, 15 and 30 min after cold stimulus (submerged hand in a 15 °C water bath for 1 min). High-frequency ultrasound and photoacoustic imaging was performed on the nail bed of each patient's second through fifth finger on their dominant hand, using a Vevo 2100 LAZR system with an LZ-250 probe (Fujifilm VisualSonics, Toronto, ON, Canada) in oxy-hemoglobin quantification mode. Read More

Rheumatol Int 2018 Dec 11;38(12):2279-2288. Epub 2018 Sep 11.

Division of Rheumatology, Department of Medicine, Faculty of Medicine Siriraj Hospital, Mahidol University, 2 Wanglang Road, Bangkoknoi, Bangkok, 10700, Thailand.

The objective of this study is to investigate the impact of skin sclerosis burden on an internal organ involvement over a 1-year period, as measured by time-adjusted accrual-modified Rodnan skin score (TA-mRSS), and to evaluate association between TA-mRSS patterns and laboratory tests in patients with systemic sclerosis (SSc). This prospective study was conducted at Siriraj Hospital (Bangkok, Thailand) during the November 2013-November 2016. SSc patients by ACR/EULAR 2013 or ACR 1980 criteria were eligible. Read More

Ann Dermatol Venereol 2018 Dec 7;145(12):761-764. Epub 2018 Sep 7.

Service de dermatologie et allergologie, université Pierre-et-Marie-Curie, Paris VI, hôpital Tenon, AP-HP, 4, rue de la Chine, 75020 Paris, France.

Background: Digital necrosis is rarer than lower limb necrosis and constitutes a medical or surgical emergency. Etiological evaluation is required. Cold agglutinin disease is a cause of digital necrosis but diagnosis is difficult. Read More

J Glaucoma 2018 Nov;27(11):987-992

Glaucoma Division, Jules Stein Eye Institute, University of California Los Angeles (UCLA), Los Angeles, CA.

Purpose: Primary open-angle glaucoma (POAG) patients constitute a heterogenous group. Identification of phenotypic subtypes among these patients may provide a deeper understanding of the disease and aid associations with genotypes. We describe a phenotype of POAG patients associated with a constellation of systemic disorders; patients with this phenotype seem to be vulnerable to optic nerve damage at low intraocular pressures. Read More

Arthritis Care Res (Hoboken) 2018 Aug 21. Epub 2018 Aug 21.

University of Utah and Salt Lake Regional Veterans Affair Medical Center, Salt Lake City, Utah, United States of America.

Objectives: Assessment of Raynaud's phenomenon (RP) in systemic sclerosis (SSc) is reliant on self-report. The Raynaud's Condition Score (RCS) diary assumes discrete episodic RP attacks, although not all SSc patients identify with this paradigm. We investigated the clinical associations of SSc-RP symptom characteristics and evolution of SSc-RP symptoms with disease progression. Read More

Intern Med 2018 Dec 10;57(23):3479-3483. Epub 2018 Aug 10.

Department IV, Internal Medicine, Tokyo Women's Medical University, Japan.

A 70-year-old man with prior Raynaud's phenomena developed hypertension and renal insufficiency. Raynaud's phenomena, finger skin thickening, interstitial lung disease, and positive anticentromere antibody findings indicated systemic sclerosis (SSc). Based on the presence of SSc, severe hypertension with rapidly progressive renal failure, and proliferative and obliterative arteriolar vasculopathy, scleroderma renal crisis (SRC) was diagnosed. Read More

J Am Acad Dermatol 2019 Feb 7;80(2):478-484. Epub 2018 Aug 7.

Department of Dermatology, National Reference Center for Rare Skin Disorders, Hôpital Saint-André, CHU de Bordeaux, Bordeaux, France; Inserm U1035, Biothérapie des Maladies Génétiques et Cancers, University of Bordeaux, Bordeaux, France. Electronic address:

Background: Skin pigmentation disorders in systemic sclerosis (SSc) have been sparsely described in the literature. Nevertheless, they could be a diagnostic and/or severity marker.

Objectives: To assess the association between pigmentation disorders and systemic involvement in patients with SSc. Read More

Praxis (Bern 1994) 2018 Aug;107(16):912-916

1 Shanghai University of Medicine and Health Sciences, Shanghai, P.R. China.

Cold and White - Hypothenar Hammer Syndrome Abstract. Hypothenar hammer syndrome (HHS) is a condition caused by digital ischemia as a result of repeated trauma to the little finger. Routine diagnosis should include a detailed medical history and a physical examination including Allen's test. Read More