SCLERODERMA. Scleroderma update. No disclosures or conflicts. Leslie Kahl, M.D. April 10, 2015
|
|
- Charles Gregory
- 5 years ago
- Views:
Transcription
1 Scleroderma update Leslie Kahl, M.D. April 10, 2015 No disclosures or conflicts KT is a 45 year old woman who developed puffiness in her fingers 1/2013 and carpal tunnel syndrome and arthralgias 3/2013. ROS was otherwise negative. Rheum and derm consults led to no diagnosis; CBC, CMP, ESR, TSH, CK, ANA, ENA, RF, CCP were all normal or negative. By 6/2013 she had discernable thickening of skin on hands, feet and ankles. A second rheumatologist diagnosed probable scleroderma. RNA polymerase III +. She was told to buy BP cuff and measure daily. Third opinion with me 2 weeks later confirmed diagnosis. Tendon friction rubs were palpated. Diagnosis of diffuse scleroderma confirmed. PPI and hand OT were started. ECHO was normal. PFTs showed mild restrictive disease (FVC 69%). In 8/2013 BP was 160 s/90 s. UA had trace protein and a few hyaline casts. She was hospitalized and captopril was begun. CT chest showed mild ground glass at bases consistent with NSIP. MMF was begun. By 12/2013 skin thickening above elbows and up to knees. In 4/2014 she was dizzy, had guaiac+ stool and was found to have GAVE; treated with argon plasma coagulation. By 6/2014 skin was improving (scores 31, 21,17 over 6 mos) In 10/2014 FVC 85%. SCLERODERMA Systemic Localized diffuse scleroderma linear scleroderma limited scleroderma morphea (CREST syndrome)
2 Morphea Linear scleroderma Epidemiology of Scleroderma Incidence: 5 10 new cases/million/year Sex ratio: F:M = 3 or 4:1 Age: Scleroderma hands: puffy phase Scleroderma hands: tight phase Digital telangiectasias and acroosteolysis Facial features of scleroderma
3 Excess dermal collagen in scleroderma Digital calcinosis Raynaud s phenomenon Conditions Associated with Raynaud s phenomenon Digital vessel response to cold Digital pitting scars
4 Dilated nailfold vessels Management of Raynaud s phenomenon Avoid the cold; keep all body parts warm Avoid tobacco Avoid vasoconstrictors: cold and diet pills, amphetamines, ergot-containing migraine meds, beta blocker Add vasodilators: Ca channel blocker, alpha blocker, phosphodiesterase inhibitor (sildenafil, tadalafil); ASA? 20 Esophageal dysmotility in scleroderma GI involvement in scleroderma Esophageal up to 90% dysphagia, GERD, Barrett s and cancer Small intestine 20-60% hypomotility with bacterial overgrowth, leads to malabsorption Colon 10-40% constipation, fecal incontinence Hepatobiliary rare; primary biliary cirrhosis Gastric antral vascular ectasia (GAVE) Interstitial lung disease in scleroderma Up to 50% in diffuse, 25% in limited disease Cough, dyspnea NSIP > UIP Scl-70 is a risk factor No data on management yet
5 Pulmonary hypertension in scleroderma PH - involving small muscular arterioles 15-20% (PAH) - due to hypoxia/severe ILD 5-10% - due to myocardial dysfunction - rare Limited> diffuse disease, usually longstanding Often silent; dyspnea, chest pain may occur Endothelin receptor antagonists, sildenafil and IV epoprostenol improve exercise capacity, functional class Annual ECHO, PFT including DlCO, Nt-proBNP are recommended for screening (beware FVC%/DLCO% > 1.6) Scleroderma renal crisis Acute or subacute development of hypertension, relative or absolute Often with proteinuria, microangiopathy May lead to stroke, myocardial infarction, death Risk factors: diffuse disease first 3 5 years of disease rapidly progressive skin disease RNA polymerase III use of prednisone, cyclosporine Scleroderma renal crisis Early detection is key home BP monitoring, patient education Treatment is blockade of renin-angiotensin system, regardless of serum Cr Consider this a medical emergency! Best outcome if Cr<3.0 at diagnosis, but 30-40% needing dialysis can discontinue it Prophylaxis with ACE/ARB not recommended Autoantibodies in scleroderma Antibody % Associations ANA Centromere limited; PAH, ulcers Scl diffuse; ILD RNA polymerase III 4-20 diffuse; renal crisis; GAVE U1-RNP 5-10 MCTD Classification criteria for scleroderma Item Score Skin thickening proximal to MCPs 9 Puffy fingers 2 OR sclerodactyly up to MCPs 4 Digital tip scars 2 OR ulcers 3 Telangiectasia 2 Abnormal nailfold capillaries 2 Pulmonary arterial hypertension 2 OR ILD 2 Raynaud s phenomenon 3 Scleroderma-related antibody 3 (centromere, SCL-70 or RNA polymerase III) Differentiation of limited from diffuse scleroderma Limited Diffuse Skin distal distal and proximal Telangectasias Calcinosis Renal crisis 0 ++ Pulmonary ++ 0 hypertension Anticentromere year survival 90% 40% > 9 points is definite systemic sclerosis (Ann Rheum Dis 2013; 72: 1747)
6 Scleroderma mimics Diabetic chieroarthropathy longstanding, poorly controlled DM; fingers only Nephrogenic systemic fibrosis gadolinium given to CKD patients; skin, lungs, heart, neuropathy Scleredema: mucin deposition in skin of back or chest; DM, infection, myeloma Scleromyxedema: papular mucinosis esp on face; lung, GI, heart, joints; IgG MGUS Eosinophilic fasciitis: fibrosis of deep tissue on arms, legs and trunk, with eosinophilia Pathogenesis of Scleroderma Microvascular occlusion Immune cell activation Excess connective tissue; fibrosis (NEJM 2015; 372; 1056) Disease process and clinical manifestations Inflammatory Fibrotic alveolitis skin thickening myositis interstitial lung disease arthritis Vascular Atrophic renal crisis GI motility pulmonary hypertension skin ulcers Raynaud s Problem-oriented management of scleroderma Skin: methotrexate? Arthritis/serositis: NSAIDs Loss of mobility: PT and OT Raynaud s: dress warmly, avoid cold and tobacco; CCBs, PDE5 inhibitors, topical nitrates Digital ulcers: IV prostanoids, endothelin receptor antagonists, botulinum toxin Renal crisis: home BP checks; ACE inhibitors Problem-oriented management of scleroderma Pulmonary hypertension: endothelin receptor antagonist, prostacyclin, PDE5 inhibitor ILD: cyclophosphamide? MMF? GERD: PPI; may consider metaclopramide, erythromycin as pro-motility agents Malabsorption: rotating antibiotics (Annals of Rheumatic Diseases 2009; 68: Seminars in Arthritis and Rheumatism; 2013; 42:42-55)
7 Take home points on scleroderma PPI for everyone Screen for PAH, ILD annually Home BP monitoring, especially in diffuse disease Use ACE-I in renal crisis, regardless of serum Cr PT and OT can have a big impact
Scleroderma. Nomenclature Synonyms. Scleroderma. Progressive Systemic Sclerosis. Systemic Sclerosis. Edward Dwyer, M.D. Division of Rheumatology
Scleroderma Edward Dwyer, M.D. Division of Rheumatology Nomenclature Synonyms Scleroderma Progressive Systemic Sclerosis Systemic Sclerosis Scleroderma 1 Scleroderma Chronic systemic autoimmune disease
More informationScleroderma. Nomenclature Synonyms. Scleroderma. Progressive Systemic Sclerosis. Systemic Sclerosis. Limited vs. Diffuse Scleroderma.
Scleroderma Edward Dwyer, M.D. Division of Rheumatology Nomenclature Synonyms Scleroderma Progressive Systemic Sclerosis Systemic Sclerosis Scleroderma Chronic systemic autoimmune disease characterized
More informationSCLERODERMA: An Update. What You Need To Know
SCLERODERMA: An Update What You Need To Know Pre Test Question #1 The onset of systemic sclerosis is typically: A. In the 1st decade of life B. Between the 4th and 6th decade C. Equal in all age groups
More informationSystemic sclerosis (SSC)
Systemic sclerosis (SSC) -Is a multi system autoimmune disease, characterized by fibrosis of the skin and variable pattern of other visceral -SSC: Is a relatively UN common disease -Prevalence in U S A
More informationSCLERODERMA 101. Maureen D. Mayes, MD, MPH Professor of Medicine University of Texas - Houston
SCLERODERMA 101 Maureen D. Mayes, MD, MPH Professor of Medicine University of Texas - Houston TYPES OF SCLERODERMA Localized versus Systemic Two Kinds of Scleroderma Localized Scleroderma Morphea Linear
More informationDisclosures. Scleroderma: Early Diagnosis How early is early? 10/28/2013. Difficult Scleroderma: How do I Approach this Patient?
Disclosures Financial support: None Difficult Scleroderma: How do I Approach this Patient? Francesco Boin, MD Assistant Professor of Medicine Director, Translational Research Johns Hopkins Scleroderma
More informationPeripheral (digital) vasculopathy in systemic. sclerosis. Ariane Herrick
Peripheral (digital) vasculopathy in systemic sclerosis Ariane Herrick Raynaud s phenomenon VASOPASM DEOXYGENATION REPERFUSION Main causes of RP Primary (idiopathic) Connective tissue diseases, including
More informationUpdate in Systemic Sclerosis! Lauren Kim MD! NW Rheumatology Associates
Update in Systemic Sclerosis! Lauren Kim MD! NW Rheumatology Associates Review Systemic sclerosis affects approximately 75,000 to 100,000 people in the U.S. and has the highest mortality rate of any autoimmune
More informationSystemic sclerosis and GI involvement
Systemic sclerosis and GI involvement Dr Maggie Larché, MRCP(UK), PhD Associate Professor, Division of Rheumatology, McMaster University St Joseph s Hospital Scleroderma CME, St Joseph s Hospital, September
More information9/15/11. Dr. Vivien Hsu Director, UMDNJ Scleroderma Program New Brunswick, NJ September Scleroderma. Hard skin
Dr. Vivien Hsu Director, UMDNJ Scleroderma Program New Brunswick, NJ September 2011 Scleroderma Hard skin 1 No diagnostic test for scleroderma Pathogenesis is unknown prominent features of disease reflect
More informationScleroderma and PAH Overview. PH Resource Network Martha Kingman, FNP C UTSW Medical Center at Dallas
Scleroderma and PAH Overview PH Resource Network 2007 Martha Kingman, FNP C UTSW Medical Center at Dallas Scleroderma and PAH Outline: Lung involvement in scleroderma Evaluation of the scleroderma patient
More information10 pearls in scleroderma for the family practitioner
10 pearls in scleroderma for the family practitioner Dr Maggie Larche, MRCP(UK), PhD Associate Professor, Division of Rheumatology, McMaster University St Joseph s Hospital Family Medicine Rounds, St Joseph
More informationAnnual Rheumatology & Therapeutics Review for Organizations & Societies
Annual Rheumatology & Therapeutics Review for Organizations & Societies A Rheumatologist s Approach to Interstitial Lung Disease Outline ILD classification and patterns in CTD The clinical landscape and
More informationScleroderma FAQ. About this Document
Scleroderma FAQ About this Document The Scleroderma FAQ * is a comprehensive document that covers systemic scleroderma diagnosis and treatment. All information contained in the FAQ is based on current
More informationSCLERODERMA EPIDEMIOLOGY
1 SCLERODERMA Scleroderma is an autoimmune disorder of unknown etiology, characterized by fibrosis and microvascular injury in affected organs. The hallmark of the disease is thickening and tightness of
More informationMyositis and Your Lungs
Myositis and Your Lungs 2013 TMA Annual Patient Meeting Louisville, Kentucky Chester V. Oddis, MD University of Pittsburgh Director, Myositis Center Myositis Heterogeneous group of autoimmune syndromes
More informationDisclosures. Clinical Approach: Evaluating CTD-ILD for the pulmonologist. ILD in CTD. connective tissue disease or collagen vascular disease
Disclosures Clinical Approach: Evaluating CTD-ILD for the pulmonologist Industry relationships: Actelion, atyr Pharma, Boehringer-Ingelheim, Genentech- Roche, Gilead Aryeh Fischer, MD Associate Professor
More informationRHEUMATOLOGY OVERVIEW. Carmelita J. Colbert, MD Assistant Professor of Medicine Division of Rheumatology Loyola University Medical Center
RHEUMATOLOGY OVERVIEW Carmelita J. Colbert, MD Assistant Professor of Medicine Division of Rheumatology Loyola University Medical Center What is Rheumatology? Medical science devoted to the rheumatic diseases
More informationMethodology used to develop new ACR-EULAR criteria. Disclosures. Objectives Guiding principles underlying methodology
Methodology used to develop new ACR-EULAR criteria Sindhu Johnson MD PhD Toronto Scleroderma Program University of Toronto Co-convenors Janet Pope Frank van den Hoogen Members Jaap Fransen Sindhu Johnson
More informationSCLERODERMA SPECTRUM DISEASE
SCLERODERMA SPECTRUM DISEASE Mohammed A. Omair MBBS, SF Rheum Consultant Rheumatologist Assistant Professor King Saud University President of the Charitable Association for Rheumatic Diseases Agenda Background
More informationSCLERODERMA RENAL CRISIS. Presented by : Nouf Alanazi
SCLERODERMA RENAL CRISIS Presented by : Nouf Alanazi Agenda Prevalence Risk factors Pathology Diagnosis Prevention & monitoring Treatment Outcome & mortality. Summary & recommendations References SRC Abrupt
More informationCTD-related Lung Disease
13 th Cambridge Chest Meeting King s College, Cambridge April 2015 Imaging of CTD-related Lung Disease Dr Sujal R Desai King s College Hospital, London Disclosure Statement No Disclosures / Conflicts of
More informationDisclosures. Rheumatological Approaches to Differential Diagnosis, Physical Examination, and Interpretation of Studies. None
Rheumatological Approaches to Differential Diagnosis, Physical Examination, and Interpretation of Studies Sarah Goglin MD Assistant Professor of Medicine Division of Rheumatology Disclosures None 1 [footer
More informationSecondary Raynaud s Phenomenon
Secondary Raynaud s Phenomenon Ulf Müller-Ladner University of Giessen Kerckhoff Clinic Bad Nauheim Germany Clinical problems in SSc Therapeutic solutions? Pulmonary fibrosis + PAH Diffuse Pulmonary fibrosis,
More informationBSR and BHPR guideline for the treatment of systemic sclerosis
BSR and BHPR guideline for the treatment of systemic sclerosis Christopher P. Denton, Michael Hughes, Nataliya Gak, Josephine Vila, Maya Buch, Kuntal Chakravarty, Kim Fligelstone, Luke L Gompels, Bridget
More informationThomas A. Medsger, Jr., MD University of Pittsburgh School of Medicine. Disclosures: None
Thomas A. Medsger, Jr., MD University of Pittsburgh School of Medicine Disclosures: None 1. idiopathic interstitial pneumonitis (IIP) - 30% of all ILD - most common form is idiopathic pulmonary fibrosis
More informationA Patient s Guide to Understanding Pulmonary Arterial Hypertension in Systemic Sclerosis
A Patient s Guide to Understanding Pulmonary Arterial Hypertension in Systemic Sclerosis Compared with the general population, patients with systemic sclerosis (also known as scleroderma) have a higher
More informationPredictors of Isolated Pulmonary Hypertension in Patients With Systemic Sclerosis and Limited Cutaneous Involvement
ARTHRITIS & RHEUMATISM Vol. 48, No. 2, February 2003, pp 516 522 DOI 10.1002/art.10775 2003, American College of Rheumatology Predictors of Isolated Pulmonary Hypertension in Patients With Systemic Sclerosis
More informationChayawee Muangchan MD. Division of Rheumatology, Department of Medicine Siriraj Hospital, Faculty of Medicine, Mahidol University
Chayawee Muangchan MD. Division of Rheumatology, Department of Medicine Siriraj Hospital, Faculty of Medicine, Mahidol University Systemic sclerosis: epidemiology (1) 6 5 UCTD MCTD 1 RA CTD 4 DM PM 2 3
More informationassociated conditions a closer look at connective tissue disease and PAH
associated conditions a closer look at connective tissue disease and PAH The link between connective tissue disease and PAH Those living with a connective tissue disease (CTD) have an increased risk of
More informationS ystemic sclerosis (SSc), also called
CASE IN POINT The Clinical Pathophysiology of Chronic Aaron Reyes; and Leila Hashemi, MD Primary care providers should monitor disease progression in the skin and in the pulmonary, renal, cardiac, and
More informationTRANSPARENCY COMMITTEE OPINION. 23 January 2008
The legally binding text is the original French version TRANSPARENCY COMMITTEE OPINION 23 January 2008 TRACLEER 62.5 mg film-coated tablets Pack of 56 (CIP: 563 621-1) TRACLEER 125 mg film-coated tablets
More informationEssential Rheumatology. Dr Ellen Bruce Consultant Rheumatologist CMFT
Essential Rheumatology Dr Ellen Bruce Consultant Rheumatologist CMFT Saving the best for last! Apparently people recall best the first and last thing they re told. Far too difficult to include everything.
More informationTest Name Results Units Bio. Ref. Interval
LL - LL-ROHINI (NATIONAL REFERENCE 135091593 Age 25 Years Gender Male 30/8/2017 91600AM 30/8/2017 93946AM 31/8/2017 84826AM Ref By Final COLLAGEN DISEASES ANTIBODY ANEL ANTI NUCLEAR ANTIBODY / FACTOR (ANA/ANF),
More informationUndifferentiated Connective Tissue Disease and Overlap Syndromes. Mark S. Box, MD
Undifferentiated Connective Tissue Disease and Overlap Syndromes Mark S. Box, MD Overlap Syndromes As many as 25% of patients with rheumatic diseases with systemic symptoms cannot be definitely diagnosed
More informationA Comparison Between Anti-Th/To and Anticentromere Antibody Positive Systemic Sclerosis Patients With Limited Cutaneous Involvement
ARTHRITIS & RHEUMATISM Vol. 48, No. 1, January 2003, pp 203 209 DOI 10.1002/art.10760 2003, American College of Rheumatology A Comparison Between and Anticentromere Antibody Positive Systemic Sclerosis
More informationSCLERODERMA LUNG DISEASE: WHAT THE PATIENT SHOULD KNOW
SCLERODERMA LUNG DISEASE: WHAT THE PATIENT SHOULD KNOW Lung disease can be a serious complication of scleroderma. The two most common types of lung disease in patients with scleroderma are interstitial
More informationAutoimmune diseases. SLIDE 3: Introduction to autoimmune diseases Chronic
SLIDE 3: Introduction to autoimmune diseases Chronic Autoimmune diseases Sometimes relapsing : and remitting. which means that they present as attacks Progressive damage Epitope spreading more and more
More informationDisclosures. Outline. Classification of scleroderma. Forms of SSc Like Syndromes. Systemic Sclerosis (Scleroderma)
Update in Rheumatic Diseases Scleroderma/Sjogrens/Myositis Intensive Review of Internal Medicine August 2012 Paul F Dellaripa MD Division of Rheumatology, Brigham and Women s Hospital, Boston MA Disclosures
More informationTest Name Results Units Bio. Ref. Interval
135091660 Age 44 Years Gender Male 29/8/2017 120000AM 29/8/2017 100219AM 29/8/2017 105510AM Ref By Final EXTRACTABLENUCLEAR ANTIGENS (ENA), QUANTITATIVE ROFILE CENTROMERE ANTIBODY, SERUM 20-30 Weak ositive
More informationWhat will we discuss today?
Autoimmune diseases What will we discuss today? Introduction to autoimmune diseases Some examples Introduction to autoimmune diseases Chronic Sometimes relapsing Progressive damage Epitope spreading more
More informationUpdate in deposition diseases
Genoa, Italy Update in deposition diseases Prof. Franco Rongioletti, Section of Dermatology, Chair of Dermatopathology, University of Genoa,Italy Cutaneous deposition disorders Endogenous Exogenous Cutaneous
More informationAcademic Affiliate Fellowship Practice Exam: 2018
Academic Affiliate Fellowship Practice Exam: 2018 Current History: A patient presents to your practice complaining of a tight feeling in her perioral tissue area. She is unable to open her mouth fully
More informationSclérodermie systémique: Manifestations musculosquelettiques
Sclérodermie systémique: Manifestations musculosquelettiques Benjamin Chaigne Service de Médecine Interne, hôpital Cochin, Centre de Référence Vascularites nécrosantes et sclérodermie systémique Assistance
More informationFor more information about how to cite these materials visit
Author(s): Seetha Monrad, M.D., 2009 License: Unless otherwise noted, this material is made available under the terms of the Creative Commons Attribution Noncommercial Share Alike 3.0 License: http://creativecommons.org/licenses/by-nc-sa/3.0/
More informationOverview of Idiopathic Pulmonary Fibrosis: Diagnosis and Therapy
Overview of Idiopathic Pulmonary Fibrosis: Diagnosis and Therapy Jeff Swigris, DO, MS Director, ILD Program National Jewish Health Disclosures Speaker - Boehringer Ingelheim and Genentech Objectives Describe
More informationMy Fingers are Blue: Benign or Worrisome? Joke Dehoorne, dienst kinderreumatologie, UZ Gent Kinderartsenvergadering 21/2/2017
My Fingers are Blue: Benign or Worrisome? Joke Dehoorne, dienst kinderreumatologie, UZ Gent Kinderartsenvergadering 21/2/2017 Aim Work up and referral of a child/teenager with discolored fingers Distinguish
More informationB. Scleroderma. 6. Nodular cutaneous lupus mucinosis. 7. Bullous lupus erythematosus. 1. Systemic sclerosis (SSc)
Go Back to the Top To Order, Visit the Purchasing Page for Details antiodies. Symptomatic therapies for the eruptions and the systemic symptoms are the main treatments. A pacemaker may e implanted in patients
More informationIs it Autoimmune or NOT! Presented to AONP! October 2015!
Is it Autoimmune or NOT! Presented to AONP! October 2015! Four main jobs of immune system Detects Contains and eliminates Self regulates Protects Innate Immune System! Epithelial cells, phagocytic cells
More informationMultidisciplinary Diagnosis in Action: Challenging Case Presentations
Multidisciplinary Diagnosis in Action: Challenging Case Presentations Interstitial Lung Disease: Advances in Diagnosis and Management UCSF CME November 8, 2014 Case 1 69 yo M 3 year history of intermittent
More informationPulmonary arterial hypertension. Pulmonary arterial hypertension: newer therapies. Definition of PH 12/18/16. WHO Group classification of PH
Pulmonary arterial hypertension Pulmonary arterial hypertension: newer therapies Ramona L. Doyle, MD Clinical Professor of Medicine, UCSF Attending Physician UCSF PH Clinic Definition and classification
More informationCase Presentations in ILD. Harold R. Collard, MD Department of Medicine University of California San Francisco
Case Presentations in ILD Harold R. Collard, MD Department of Medicine University of California San Francisco Outline Overview of diagnosis in ILD Definition/Classification High-resolution CT scan Multidisciplinary
More informationThomas A. Medsger, Jr., MD University of Pittsburgh School of Medicine. Disclosures: None
Thomas A. Medsger, Jr., MD University of Pittsburgh School of Medicine Disclosures: None 4000+ patients enrolled 1972- present; 5+ visits per patient; 20,000+ patient years of follow- up All clinical and
More informationScholars Journal of Medical Case Reports
Scholars Journal of Medical Case Reports Sch J Med Case Rep 2015; 3(9B):928-932 Scholars Academic and Scientific Publishers (SAS Publishers) (An International Publisher for Academic and Scientific Resources)
More informationSCLERODERMA OVERLAP SYNDROME: A CASE REPORT Diwakar K. Singh 1, Nataraju H. V 2
SCLERODERMA OVERLAP SYNDROME: A Diwakar K. Singh 1, Nataraju H. V 2 HOW TO CITE THIS ARTICLE: Diwakar K. Singh, Nataraju H. V. Scleroderma Overlap Syndrome: A Case Report. Journal of Evolution of Medical
More informationConnective tissue disease related Pulmonary arterial hypertension
Connective tissue disease related Pulmonary arterial hypertension DM Seminar: Dr.Vamsi Krishna PAH in connective tissue diseases CTD accounts for 30% of PAH according to US PAH registry. Incidence is on
More informationSystemic Sclerosis. Scleroderma
Systemic Sclerosis Scleroderma Definition Chronic systemic disorder Unknown etiology Thickening of the skin = scleroderma Involvement of multiple internal organs Early stage: inflammation Later stage:
More informationUnderstanding & Managing Scleroderma
Understanding & Managing Scleroderma UK Registered Charity Number 286736 Number 286736 Printing of this booklet was supported by grants from Actelion Pharmaceuticals UK and Encysive Pharmaceuticals Understanding
More informationCaring for the Patient With Limited Systemic Scleroderma
Caring for the Patient With Limited Systemic Scleroderma 2.0 ANCC Contact Hours Kelly Denise Lachner Systemic scleroderma (systemic sclerosis) is a rare, autoimmune, collagen-vascular disease of unknown
More informationAutoantibodies in the Idiopathic Inflammatory Myopathies
Autoantibodies in the Idiopathic Inflammatory Myopathies Steven R. Ytterberg, M.D. Division of Rheumatology Mayo Clinic Rochester, MN The Myositis Association Annual Conference St. Louis, MO Sept. 25,
More informationJeopardy. What s the rash? $100 $100 $100 $100 $100 $200 $200 $200 $200 $200 $300 $300 $300 $300 $300 $400 $400 $400 $400 $400
Jeopardy Antibodies & more antibodies Aching joints What s the rash? Potpourri Image Challenge $100 $100 $100 $100 $100 $200 $200 $200 $200 $200 $300 $300 $300 $300 $300 $400 $400 $400 $400 $400 $500 $500
More informationConnective Tissue Disorder- Associated Interstitial Lung Disease (CTD-ILD) and Updates
Connective Tissue Disorder- Associated Interstitial Lung Disease (CTD-ILD) and Updates Maria Elena Vega, M.D Assistant Professor of Medicine Lewis Katz School of Medicine at Temple University Nothing to
More informationRecent Treatment of Pulmonary Artery Hypertension. Cardiology Division Yonsei University College of Medicine
Recent Treatment of Pulmonary Artery Hypertension Cardiology Division Yonsei University College of Medicine Definition Raised Pulmonary arterial pressure (PAP) WHO criteria : spap>40 mmhg NIH Criteria
More informationScleroderma Facts. The Scleroderma Foundation is here to help!
Scleroderma Facts Scleroderma is an autoimmune disease whose symptoms typically include some or all of the following: sensitivity to cold in extremities, thickening of the skin, shortness of breath, difficulty
More informationThe Power of the ANA. April 2018 Emily Littlejohn, DO MPH
Emergent Rheumatologic Diseases and Disorders for Primary Care. The Power of the ANA April 2018 Emily Littlejohn, DO MPH Question 1: the ANA test is: A) A screening test with high specificity to diagnose
More informationNIH Public Access Author Manuscript Arthritis Rheum. Author manuscript; available in PMC 2014 November 01.
NIH Public Access Author Manuscript Published in final edited form as: Arthritis Rheum. 2013 November ; 65(11): 2737 2747. doi:10.1002/art.38098. Classification Criteria for Systemic Sclerosis: An ACR-EULAR
More informationPulmonary manifestations of CTDs Diagnosis, differential diagnosis and treatment
Prague, June 2014 Pulmonary manifestations of CTDs Diagnosis, differential diagnosis and treatment Katerina M. Antoniou, MD, PhD As. Professor in Thoracic Medicine ERS ILD Group Secretary Medical School,
More informationGuide for New and Future Patients
Guide for New and Future Patients About this Guide This Guide for New and Future Patients is a companion document to the Scleroderma FAQ TM, also available through the Scleroderma Education Project website:
More informationDisclosures. Integrated Approach to Treating CTD-ILD. Limitations. Relevant items to consider. Other than for SSc-ILD, no controlled data
Integrated Approach to Treating CTD-ILD Industry relationships: Disclosures Aryeh Fischer, MD Associate Professor of Medicine Division of Rheumatology Division of Pulmonary Sciences and Critical Care Medicine
More informationINTERSTITIAL LUNG DISEASE. Radhika Reddy MD Pulmonary/Critical Care Long Beach VA Medical Center January 5, 2018
INTERSTITIAL LUNG DISEASE Radhika Reddy MD Pulmonary/Critical Care Long Beach VA Medical Center January 5, 2018 Interstitial Lung Disease Interstitial Lung Disease Prevalence by Diagnosis: Idiopathic Interstitial
More informationPathology of Hypertension
2016-03-07 Pathology of Hypertension Honghe Zhang honghezhang@zju.edu.cn Tel:88208199 Department of Pathology ❶ Genetic predisposition ❷ Dietary factors ❸ Environmental factors ❹ Others Definition and
More informationA Review of Interstitial Lung Diseases
Outline A Review of Interstitial Lung Diseases Paul J. Wolters, MD Associate Professor Department of Medicine University of California San Francisco Overview of diagnosis in ILD Why it is important Definition/Classification
More informationA Review of Interstitial Lung Diseases. Paul J. Wolters, MD Associate Professor Department of Medicine University of California San Francisco
A Review of Interstitial Lung Diseases Paul J. Wolters, MD Associate Professor Department of Medicine University of California San Francisco Outline Overview of diagnosis in ILD Why it is important Definition/Classification
More informationHigh Impact Rheumatology
High Impact Rheumatology Systemic Lupus Erythematosus Bernard Rubin, DO MPH Case 1: History A 45-year-old woman presents with severe dyspnea and cough. She was in excellent health until 4 weeks ago when
More informationINTERSTITIAL LUNG DISEASE Dr. Zulqarnain Ashraf
Indep Rev Jul-Dec 2018;20(7-12) Dr. Zulqarnain Ashraf IR-653 Abstract: ILD is a group of diseases affect interstitium of the lung. Repeated insult to the lung cause the interstitium to be damaged. Similarly
More informationGlomerular pathology in systemic disease
Glomerular pathology in systemic disease Lecture outline Lupus nephritis Diabetic nephropathy Glomerulonephritis Associated with Bacterial Endocarditis and Other Systemic Infections Henoch-Schonlein Purpura
More informationScleroderma. Chronic multisystemic disease characterized by vasculopathy, variable degree of inflammation, and fibrosis
Scleroderma Chronic multisystemic disease characterized by vasculopathy, variable degree of inflammation, and fibrosis Incidence 3.7-22.8 cases/million Female:male 5:1 Pulmonary fibrosis common, severe
More informationPulmonary Hypertension: Another Use for Viagra
Pulmonary Hypertension: Another Use for Viagra Kathleen Tong, MD Director, Heart Failure Program Assistant Clinical Professor University of California, Davis Disclosures I have no financial conflicts A
More informationClinical Laboratory. [None
Clinical Laboratory Procedure Result Units Ref Interval Accession Collected Received Double-Stranded DNA (dsdna) Ab IgG ELISA Detected * [None 18-289-900151 Detected] Double-Stranded DNA (dsdna) Ab IgG
More informationManaging scleroderma Challenges in primary care
This is a CME (Continuing Medical Education) article. It is presented to you in collaboration with the Middle East CME organizations. Self-test answers will be published in two months. In collaboration
More informationWe also assessed the diagnostic significance of the SUBJECTS
Annals of the Rheumatic Diseases, 1982, 41, 382-387 Antinuclear antibodies in patients with Raynaud's phenomenon: clinical significance of anticentromere antibodies C. G. M. KALLENBERG, G. W. PASTOOR,
More informationTest Name Results Units Bio. Ref. Interval
135091662 Age 45 Years Gender Male 29/8/2017 120000AM 29/8/2017 100215AM 29/8/2017 110825AM Ref By Final RHEUMATOID AUTOIMMUNE COMREHENSIVE ANEL ANTI NUCLEAR ANTIBODY / FACTOR (ANA/ANF), SERUM ----- 20-60
More informationNovel Classification of Idiopathic Inflammatory Myopathies Based on Overlap Syndrome Features and Autoantibodies
Novel Classification of Idiopathic Inflammatory Myopathies Based on Overlap Syndrome Features and Autoantibodies Analysis of 100 French Canadian Patients Yves Troyanov, MD, Ira N. Targoff, MD, Jean-Luc
More informationScleroderma What is Scleroderma? What Causes Scleroderma? Who gets Scleroderma? What are the symptoms of Scleroderma? - 1 -
What is Scleroderma? Scleroderma means "hard skin". It is a disease of vascular and connective tissue where the skin and many other parts of the body can become thick and hard. There are two kinds of Scleroderma:
More informationPULMONARY ARTERIAL HYPERTENSION AGENTS
Approvable Criteria: PULMONARY ARTERIAL HYPERTENSION AGENTS Brand Name Generic Name Length of Authorization Adcirca tadalafil Calendar Year Adempas riociguat Calendar Year Flolan epoprostenol sodium Calendar
More informationInflammatory Myopathies 4 th year MBBS. Marwan Adwan MBChB, MSc, MRCPI, MRCP(rheum) Consultant Rheumatologist
Inflammatory Myopathies 4 th year MBBS Marwan Adwan MBChB, MSc, MRCPI, MRCP(rheum) Consultant Rheumatologist Case A 64 woman presents with erythematous itchy rash over back of hands & forehead. For 1 month
More informationInterstitial Lung Disease in the Connective Tissue Diseases
Interstitial Lung Disease in the Connective Tissue Diseases Danielle Antin-Ozerkis, MD a, *, Ami Rubinowitz, MD b, Janine Evans, MD c, Robert J. Homer, MD, PhD d, Richard A. Matthay, MD e KEYWORDS Connective
More informationhttps://www.printo.it/pediatric-rheumatology/gb/intro Scleroderma Version of 2016 1. WHAT IS SCLERODERMA 1.1 What is it? The name scleroderma is derived from Greek and can be translated as "hard skin".
More informationSCLEROMYXEDEMA WITH SYSTEMIC INVOLVEMENT MIMICS RHEUMATIC DISEASES
913 BRIEF REPORT SCLEROMYXEDEMA WITH SYSTEMIC INVOLVEMENT MIMICS RHEUMATIC DISEASES EDWARD J. FUDMAN, JOSEPH GOLBUS, and KOBERT W. IKE Scleromyxedema is an infiltrative skin disease produced by hyaluronic
More informationRegressive systemic sclerosis
Annals of the Rheumatic Diseases, 1986; 45, 384-388 Regressive systemic sclerosis CAROL BLACK, PAUL DIEPPE, TED HUSKISSON, AND FRANK DUDLEY HART From the Departments of Medicine and Rheumatology, West
More informationDr Ian Roberts Oxford
Dr Ian Roberts Oxford Oxford Pathology Course 2010 for FRCPath Present the basic diagnostic features of the commonest conditions causing renal failure Highlight diagnostic pitfalls. Crescentic GN: renal
More informationSome renal vascular disorders
Some renal vascular disorders Introduction Nearly all diseases of the kidney involve the renal blood vessels secondarily We will discuss: -Hypertension (arterionephrosclerosis in benign HTN & hyperplastic
More informationVASCULITIS. Case Presentation. Case Presentation
VASCULITIS Case Presentation The patient is a 24 year old woman who presented to the emergency room with left-sided weakness. She was confused and complained of a severe headache. She was noted to have
More informationبالحمضات الصفاق التهاب = fasciitis Eosinophilic
Eosinophilic fasciitis 1 / 11 Eosinophilic fasciitis (EF) is a rare, localized fibrosing disorder of the fascia. The etiology and pathophysiology are unclear. In 1974, Shulman provided an early description
More informationUpdate in Pulmonary Arterial Hypertension
Update in Pulmonary Arterial Hypertension Michael J Sanley, MD April 12, 2018 Disclosures I have nothing to disclose 2 1 Case Presentation 67 yo male with atrial fibrillation, CLL on IVIG, presents with
More informationChapter 1 Introduction
Chapter 1 Introduction Chapter 1-1 Chapter Highlights 1. This Manual is for You 2. What is Scleroderma? 3. Who gets Scleroderma? 4. What are the Early Symptoms of Scleroderma? 5. Is All Scleroderma the
More informationScleroderma renal crisis following silicone breast implant rupture: a case report and review of the literature
CASE REPORT Clinical and Experimental Rheumatology 2014; 32: 262-266. Scleroderma renal crisis following silicone breast implant rupture: a case report and review of the literature G. Al Aranji¹, D. White¹,
More informationHypertension Update. Objectives 4/28/2015. Beverly J. Mathis, D.O. OOA May 2015
Hypertension Update Beverly J. Mathis, D.O. OOA May 2015 Objectives Learn new recommendations for BP treatment goals Approach to hypertension in the office Use of hypertensive drugs, and how to tailor
More informationSupplementary Online Content
Supplementary Online Content Caglayan E, Axmann S, Hellmich M, Moinzadeh P, Rosenkranz S. Vardenafil for the treatment of Raynaud phenomenon: a randomized, double-blind, placebocontrolled crossover study.
More information