Thomas A. Medsger, Jr., MD University of Pittsburgh School of Medicine. Disclosures: None
|
|
- Rudolf Walton
- 5 years ago
- Views:
Transcription
1 Thomas A. Medsger, Jr., MD University of Pittsburgh School of Medicine Disclosures: None
2 1. idiopathic interstitial pneumonitis (IIP) - 30% of all ILD - most common form is idiopathic pulmonary fibrosis (IPF) or usual interstitial pneumonitis (UIP); can be confirmed either radiographically or pathologically - varying rate of progression but overall poor prognosis - an other form is non- specific interstitial pneumonitis (NSIP), which can be fibrotic or non- fibrotic NSIP 2. connective tissue disease (CTD- ILD) or undifferentiated CTD (UCTD) associated - 15% of all ILD - 40% of SSc patients; 30-80% of PM/DM patients; less frequent in RA, SLE, Sjogren syndrome - better prognosis
3 1. radiographic: bibasilar increase in interstitial markings on chest radiograph or high resolution CT scan (HRCT) without contrast - new lower radiation exposure CT scan protocol proposed 2. physiologic: restrictive lung disease with no evidence of obstructive lung disease (FVC <80% predicted with FEV1/FVC >70% predicted)
4 SSc ILD
5 IPF UCTD or AILD CTD- ILD ILD
6 1. retrospective study: 50 patients referred to Pulmonary Medicine for IPF, also evaluated by a rheumatologist 2. final diagnosis: 25 (50%) CTD- ILD 3. treatment changes resulted in 80% of patients with CTD- ILD and 30% of patients with IPF Castelino et al., Rheumatology 2010; 50:
7 National Jewish Medical Center (Denver) Cohort 97 (29%) positive ANA 25 (9%) ANA with nucleolar staining 13 anti- Th/To antibody positive 9 met criteria for ssssc Fischer et al. J Rheumatol 2006; 33:
8 Kinder et al., Am J Respir Crit Care Med 2010; 176:
9 Park et al., Am J Respir Crit Care Med 2007; 175:
10 1. CTD features in ILD patients may be subtle Scleroderma PM/DM Sjogren Syndrome - puffy fingers - inflammatory polyarthralgias/it is - low C4 complement - Raynaud phenomenon - DM rash - hypergammaglobulinemia - abnormal nailfold capillaries - heartburn, dysphagia - positive ANA - proximal muscle weakness - elevated CPK - renal tubular acidosis (high Cl, low K, low Co2) 2. ILD may precede other CTD findings by months/years 3. In acute/severe ILD, few physicians look for extra- pulmonary manifestations
11 Gottron Papules
12 1. scleroderma sine scleroderma or ssssc (no scleroderma skin changes) 2. amyopathic DM (rash only, no muscle weakness) 3. anti- synthetase antibody syndrome without muscle weakness or rash 4. Sjogren syndrome with minimal or no sicca symptoms 5. undifferentiated CTD
13 definition: Raynaud, one typical SSc internal organ involvement, positive ANA, no other classifiable CTD - lc (limited cutaneous) SSc = 1794/3392 (53%) - of the 1794 lcssc, 107 (4%) had ssssc - of the 107 ssssc, 42 (39%) had ILD Aggarwal et al., Arthritis Rheum 2010; 62: Suppl 10: 463
14 Aggarwal et al., Arthritis Rheum 2010; 62: Suppl 10: 463 First Pittsburgh visit Raynaud Joint Lung Esophagus Years before Diagnosis Time of diagnosis Years after Diagnosis Diagnosis delay (first SSc symptom to diagnosis) = 8.8 ±9.9 years
15 1. clinical features: myositis, polyarthritis, Raynaud, fever, mechanic s hands, ILD 2. autoantibodies: anti- Jo1 and 7 other non- Jo1 anti- synthetases (directed against aminoacyl- trna synthetase enzymes); 50% ANA negative 3. ILD present in 164 (81%) of 202 anti- synthetase patients in the Pittsburgh cohort 4. mean delay in diagnosis after pulmonary symptoms = 2.1 years
16
17 Homogeneous, diffuse cytoplasmic staining on IIF
18 - underappreciated underlying CTD (most often myositis) - acute respiratory failure; ICU admission - clinical/pathologic picture: ARDS with diffuse alveolar damage (DAD) 73 Pittsburgh anti- Jo1 patients (14) (59) Richards et al., Arthritis Rheum 2009; 60: Won Huh et al., Resp Med 2007; 101:
19 June 6
20 Steen et. al, Ann Rheum Dis 2007; 66:
21 rapid loss in FVC in first 1-2 years FVC (% predicted) n= n= n=36 slow loss of FVC in later years 4-5 n= n= n=14 > 10 n=9 Disease Duration (years) Steen et al., Arthritis Rheum 1984; 37:1290-6
22 Flow diagram of staging system for application in clinical practice Limited and extensive disease correlate with good and poor survival at 5 years, respectively good prognosis 75% 5 years poor prognosis 30% 5 years Goh et al., Am J Respir Care Med 2008: 177;
23 SSc-ASSOCIATED AUTOANTIBODIES FREQUENCY OF PF U11/U12 RNP 23/33 (70%) SIGNIFICANCE vs. U11/U12 Topoisomerase I 204/425 (48%) p < 0.02 Ku 6/14 (43%) NS PM-Scl 27/65 (42%) p < Th/To 47/121 (39%) p < U1 RNP 36/123 (29%) p < U3 RNP 16/84 (19%) p < RNA Polymerase III 63/343 (18%) p < Centromere 59/436 (14%) p < * Overlaps excluded; no patient had more than one SSc- associated antibody Denominator = total number of antibody positive patients Fertig et al., Arthritis Rheum 2009; 61:963.
24 multicenter, randomized, double- blind, placebo controlled trial 158 patients with SSc and ILD oral CY vs. placebo for one year Tashkin et al. N Engl J Med 2006; 354:
25 benefit gone after 2 years Tashkin et al., Am J Respir Crit Care Med 2007; 176:
26 summary of 5 retrospective or open label studies published SSc patients MMF 2-3 Gm/day, 8-24 months; 3 reports with prednisone mean changes in FVC compared with baseline: +2%, +11%, 0%, +3%, +4% mean changes in DLCO compared with baseline: +4%, +11%, - 4%, +14%
27 1. patients lose up to 40% of FVC in first year of disease 2. use Goh et al. guidelines to determine disease extent, prognosis 3. indications for treatment: falling FVC and increasing dyspnea; worsening HRCT; O2 desaturation with exercise 4. corticosteroids and IS drugs synergistic but concern re: renal crisis in SSc patients with diffuse cutaneous involvement, palpable tendon friction rubs, anti- RNA polymerase III antibody 5. start cyclophosphamide monthly IV x 3-6 months then convert to MMF 2-3 Gm/day for maintenance 6. other considerations for maintenance/salvage cyclosporin or tacrolimus; azathioprine; methotrexate
28 7. control acid reflux disease (aspiration) 8. limit predisone to less than 15 mg/day 9. pneumovax and annual flu vaccine 10. lung transplantation 11. don t overtreat end- stage lung disease 12. beware secondary pulmonary arterial hypertension (worsening dyspnea with unchanged FVC but falling DLCO)
29 1. pirfenidone - inhibits release of profibrotic factors from fibroblasts and macrophages - ILD < 2 years; moderate severity (FVC > 50%) - randomized, double- blind controlled 16 week trial - all patients receive active drug (two different drug titrations) 2. pomalidomide - inhibits many pro- inflammatory cytokines and chemokines - randomized, placebo- controlled, 52 weeks - FVC 45-70% predicted or FVC 70-80% predicted with fall in FVC > 5% in preceding 24 months - concomitant MMF or CY permitted 3. daily oral CY vs. daily oral MMF (SLS2) - - ranodmized; CY 1 year, MMF 2 years ILD mild to moderate (FVC < 80% predicted) 4. anti- IL13 and other monoclonal antibodies directed against small molecule targets in IPF, not yet in CTD- ILD
30 SSc PAH
31 IPAH AIPAH CTD- PAH Rich et al. National Prospective Study of Primary PAH - Ann Intern Med 1987; 107: centers, 187 patients; 19 (10%) Raynaud phenomenon and 54 (29%) positive ANA
32 Outcomes: poor survival before recent therapeutic advances (< 50% 2 year survival); worse than idiopathic PAH Prevalence: 8-12% At risk patients: lcssc, anti- centromere, anti- Th/To and anti- U3RNP antibodies; anti- Scl 70 protective Clinical features: rapidly progressive dyspnea on exertion; increased P2 sound; tricuspid insufficiency murmur; right sided heart failure; echocardiogram estimated PA systolic pressure > 45 mmhg; right- sided cardiac cath PA mean pressure > 25 mmhg with normal wedge pressure
33 Survival in Pittsburgh SSc- PAH Patients by Autoantibody Pol IIITopo topo U1RNP U3 ACACA ThTo Time from first confirmation of PAH (years)
34 1. Expert panel consensus statement: echocardiogram every year (overkill per Thomas A. Medsger, Jr.) 2. Do echo at first visit and, if normal, every 3 years thereafter - do earlier if unexplained increase in dyspnea, progressive fall in DLCO or increase in FVC: DLCO percent predicted ratio to 1.6:1 or greater 3. If suspect PAH and echo previously normal at rest, request exercise echo 4. If echo estimated peak PA systolic pressure > 45 mmhg, do right heart cath for clarification (many patients with echo PA >45mmHg have normal PA pressure on RHC)
35 Humbert et al., N Engl J Med 2004; 351:1427
36 1. vasodilators - endothelin antagonists (bosentan, ambrisentan) - phosphodiesterase V inhibitors (sildenafil) - prostacyclin derivatives (epoprostenol) 2. immunosuppressive drugs - evidence of autoimmunity in humans and animal models (perivascular mononuclear cell infiltrates including B cells) - case reports of responders to cyclophsophamide, particularly anti- U1RNP positive patients - ongoing trial of Rituxan vs. placebo in SSc- PAH
37 from studies of Holers et al. in RA, serum anti- CCP and RF present before clinical disease (many months/years) developed pre- RA cohort (asymptomatic first degree relatives of established RA patients with and without anti- CCP and RF) bronchial wall thickening on HRCT found in 76% of antibody positive vs. 33% of antibody negative pre- RA subjects anti- CCP antibodies found in BAL fluid but not in serum in antibody positive pre- RA subjects other microbiome consideration as source of antigens precipitating RA: oral mucosa, GI tract prevention trial of Rituxan in pre- RA cohort (Netherlands) Demourelle et al., Arthritis Rheum 2012; 64:
38 Scleroderma Center - Gerald P. Rodnan, MD (deceased) - Virginia D. Steen, MD - Robyn T. Domsic, MD, MPH - Carol A. Feghali- Bostwick, PhD - Chester V. Oddis, MD - Rohit Aggarwal, MD - Kathryn Torok, MD - Masataka Kuwana, MD - Yutaka Okano, MD - Mary Lucas, RN, MPH - Dana Ivanco - Maureen Laffoon - Noreen Fertig, BS - Zengbiao Qi, PhD Funding - NIAMS - Scleroderma Foundation - ACR REF - Private Foundations - Generous Patients Immunology - Penelope Morel, MD - Patrizia Fuschiotti, PhD Cardiology - - William P. Follansbee, MD Michael Mathier, MD - Hunter Champion, MD Pulmonary - Gregory R. Owens, MD (deceased) - - Kevin F. Gibson, MD Kristen Veraldi, MD, PhD Gastroenterology - Klaus Bielefeldt, MD, PhD - Kenneth Fasanella, MD Renal - John P. Johnson, MD Dermatology - Vincent Falanga, MD Hand Surgery - Robert A. Kaufmann, MD - Matthew M. Tomaino, MD - Neil F. Jones, MD
Thomas 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 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 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 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. 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 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 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 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 informationSCLERODERMA. Scleroderma update. No disclosures or conflicts. Leslie Kahl, M.D. April 10, 2015
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.
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 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 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 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 informationScleroderma. 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 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 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 informationClinical Laboratory. 14:42:00 SSA-52 (Ro52) (ENA) Antibody, IgG 1 AU/mL [0-40] Oct-18
Clinical Laboratory Procedure Result Units Ref Interval Accession Collected Received Rheumatoid Factor
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 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 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 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 informationClinical Laboratory. 14:41:00 Complement Component 3 50 mg/dl Oct-18
Clinical Laboratory Procedure Result Units Ref Interval Accession Collected Received Thyroid Peroxidase (TPO) Antibody 5.0 IU/mL [0.0-9.0] 18-289-900139 16-Oct-18 Complement Component 3 50 mg/dl 18-289-900139
More informationdifferent phenotypes
Pulmonary hypertension in scleroderma: different phenotypes UMR 995 Pr David LAUNAY, MD, PhD launayd@gmail.com Service de Médecine Interne. Unité d'immunologie Clinique CNRMR Maladies Systémiques et Autoimmunes
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 informationDiagnosis of Connective Tissue Disease Interstitial Lung Disease in 2016
Diagnosis of Connective Tissue Disease Interstitial Lung Disease in 2016 A/Prof Tamera Corte TSANZ Annual Conference: Perth 2016 Royal Prince Alfred Hospital, Sydney, Australia Sydney Medical School, University
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 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 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 informationUnderstanding Myositis Medications
Understanding Myositis Medications 2015 TMA Annual Patient Conference Orlando, Florida Chester V. Oddis, MD University of Pittsburgh Director, Myositis Center Disclosures Mallinckrodt: Research Grant Genentech:
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 informationBudsakorn Darawankul, MD. Maharat Nakhon Ratchasima Hospital
Budsakorn Darawankul, MD. Maharat Nakhon Ratchasima Hospital Outline What is ANA? How to detect ANA? Clinical application Common autoantibody in ANA diseases Outline What is ANA? How to detect ANA? Clinical
More information4/17/2010 C ini n ca c l a Ev E a v l a ua u t a ion o n of o ILD U dat a e t e i n I LDs
Update in ILDs Diagnosis 101: Clinical Evaluation April 17, 2010 Jay H. Ryu, MD Mayo Clinic, Rochester MN Clinical Evaluation of ILD Outline General aspects of ILDs Classification of ILDs Clinical evaluation
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 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 informationNOT COPY. Choice of the primary outcome: Pro Lung 03/09/2018. Lung assessment in clinical trials
Choice of the primary outcome: Pro Lung Christopher P. Denton Professor of Experimental Rheumatology Royal Free Hospital and University College London, UK Florence 7 th September 218 Improved clinical
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 informationILD and systemic disease
Department for Pulmonology, University Hospital Berne ILD and systemic disease Dr. med. Manuela Funke-Chambour Oberärztin I Female patient, born 1945 Portuguese Presented with cough and dyspnea, no other
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 informationIDIOPATHIC INFLAMMATORY MYOPATHIES AND RELATED DISORDERS. Franclo Henning Division of Neurology Tygerberg Hospital
IDIOPATHIC INFLAMMATORY MYOPATHIES AND RELATED DISORDERS Franclo Henning Division of Neurology Tygerberg Hospital Classification systems Clinical (Bohan and Peter) Clinico-pathological (Dalakas & others)
More informationSystemic sclerosis (SSc) is a systemic autoimmune
Eur Respir Rev 2013; 22: 127, 6 19 DOI: 10.1183/09059180.00005512 CopyrightßERS 2013 EUROPEAN RESPIRATORY UPDATE Scleroderma lung disease Joshua J. Solomon*, Amy L. Olson*, Aryeh Fischer*, Todd Bull #,
More informationUIP OR NOT UIP PATTERN: THAT IS NOT THE ONLY QUESTION!
UIP OR NOT UIP PATTERN: THAT IS NOT THE ONLY QUESTION! STÉPHANE JOUNEAU 11 JULY 2014 Respiratory Medicine Department, Pontchaillou Hospital, Rennes, France CASE OVERVIEW This case highlights how a usual
More informationDiagnosing ILD. What is important in 2016? Chris Grainge
Diagnosing ILD What is important in 2016? Chris Grainge Senior Staff Specialist Respiratory Medicine John Hunter Hospital Conjoint A/Prof University of Newcastle Conflict of interest I have acted as a
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 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 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 informationConnective Tissue Disease-Associated Interstitial Lung Disease
CHEST Commentary Connective Tissue Disease-Associated Interstitial Lung Disease A Call for Clarification Aryeh Fischer, MD ; Sterling G. West, MD ; Jeffrey J. Swigris, DO ; Kevin K. Brown, MD ; and Roland
More informationA case of a patient with IPF treated with nintedanib. Prof. Kreuter and Prof. Heussel
A case of a patient with IPF treated with nintedanib Prof. Kreuter and Prof. Heussel Case Overview This case describes the history of a patient with IPF who, at the time of diagnosis, had symptoms typical
More informationTBLB is not recommended as the initial biopsy option in cases of suspected IPF and is unreliable in the diagnosis of rare lung disease (other than
TBLB is not recommended as the initial biopsy option in cases of suspected IPF and is unreliable in the diagnosis of rare lung disease (other than PAP) BAL is not required as a diagnostic tool in patients
More informationOverview of Diagnostic Autoantibodies in Inflammatory Myopathy
Overview of Diagnostic Autoantibodies in Inflammatory Myopathy Minoru Satoh, M.D., Ph.D. Research Associate Professor of Medicine Division of Rheumatology and Clinical Immunology University of Florida
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 informationEmerging Therapies for Lung Fibrosis. Helen Garthwaite Respiratory Registrar/ Clinical Research Fellow
Emerging Therapies for Lung Fibrosis Helen Garthwaite Respiratory Registrar/ Clinical Research Fellow Lung Fibrosis/Interstitial Lung Disease Disease that affects the tissue that supports the lungs alveoli
More informationThe Egyptian Journal of Hospital Medicine (July 2017) Vol.68 (2), Page
The Egyptian Journal of Hospital Medicine (July 2017) Vol.68 (2), Page 1135-1140 Role of High Resolution Computed Tomography in Diagnosis of Interstitial Lung Diseases in Patients with Collagen Diseases
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 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 informationTools to Aid in the Accurate Diagnosis of. Connective Tissue Disease
Connective Tissue Disease Tools to Aid in the Accurate Diagnosis of Connective Tissue Disease Connective Tissue Disease High quality assays and novel tests Inova offers a complete array of assay methods,
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 informationEvidence Review: Title. Month/ Year. Evidence Review:
Evidence Review: Title Month/ Year Evidence Review: Rituximab for connective tissue disease associated interstitial lung disease October 2014 Standard Operating Procedure: NHS England Evidence Review:
More informationManagement of Co morbidities in Idiopathic Pulmonary Fibrosis. Disclosures
Management of Co morbidities in Idiopathic Pulmonary Fibrosis Joyce S. Lee, MD MAS Director, Interstitial Lung Disease Clinic University of California, San Francisco Disclosures Intermune, advisory board
More informationThe scleroderma lung: where do we stand?
6th international meeting on pulmonary rare diseases and orphan drugs Milan 27-28 February 2015 The scleroderma lung: where do we stand? Silvia Bellando Randone & Marco Matucci Cerinic Dept of Experimental
More informationThe Onset of Eosinophilic Pneumonia Preceding Anti-synthetase Syndrome
doi: 10.2169/internalmedicine.8955-17 http://internmed.jp CASE REPORT The Onset of Eosinophilic Pneumonia Preceding Anti-synthetase Syndrome Yoshimasa Hachisu 1, Yasuhiko Koga 1, Noriaki Sunaga 1,2, Chiharu
More informationLines and crackles. Making sense of ILD
Lines and crackles Making sense of ILD Case JM 65 year old male Gradual shortness of breath, going on over a year Some dry cough Ex-smoker, quit 10 years ago Crackles in the bases CXR presented Sent to
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 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 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 informationSumiaki Tanaka, Yu Matsueda, Tatsuhiko Wada, Junichi Tanaka, Tatsuo Nagai, Shunsei Hirohata
Original Contribution Kitasato Med J 2017; 47: 52-61 Long-term survival of patients with pulmonary arterial hypertension associated with connective tissue disease: beneficial effects of beraprost sodium,
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 informationDIAGNOSTIC NOTE TEMPLATE
DIAGNOSTIC NOTE TEMPLATE SOAP NOTE TEMPLATE WHEN CONSIDERING A DIAGNOSIS OF IDIOPATHIC PULMONARY FIBROSIS (IPF) CHIEF COMPLAINT HISTORY OF PRESENT ILLNESS Consider IPF as possible diagnosis if any of the
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 informationMyositis and Autoimmune ILD
Myositis and Autoimmune ILD 2018 AURA Annual Meeting Sedona, Arizona June 2, 2018 Chester V. Oddis, MD Director, Myositis Center University of Pittsburgh Disclosures Genentech: Clinical trial support Idera:
More informationUpdate on Therapies for Idiopathic Pulmonary Fibrosis. Outline
Update on Therapies for Idiopathic Pulmonary Fibrosis Paul Wolters Associate Professor University of California, San Francisco Outline Classification of Interstitial lung disease Clinical classification
More informationNONE OVERVIEW FINANCIAL DISCLOSURES UPDATE ON IDIOPATHIC PULMONARY FIBROSIS/IPF (UIP) FOR PATHOLOGISTS. IPF = Idiopathic UIP Radiologic UIP Path UIP
UPDATE ON IDIOPATHIC PULMONARY FIBROSIS/IPF () FOR PATHOLOGISTS Thomas V. Colby, M.D. Professor of Pathology (Emeritus) Mayo Clinic Arizona FINANCIAL DISCLOSURES NONE OVERVIEW IPF Radiologic Dx Pathologic
More informationUC Davis UC Davis Previously Published Works
UC Davis UC Davis Previously Published Works Title Characterisation of patients with interstitial pneumonia with autoimmune features Permalink https://escholarship.org/uc/item/0n40x0jb Journal European
More informationThis month, we are very pleased to introduce some new tests for Scleroderma as well as some test changes to our existing scleroderma tests/panels.
February 20, 2017 Client Letter Test Update February 2017 Dear Colleague: This month, we are very pleased to introduce some new tests for Scleroderma as well as some test changes to our existing scleroderma
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 informationIPF: Epidemiologia e stato dell arte
IPF: Epidemiologia e stato dell arte Clinical Classification Diffuse parenchimal lung diseases Exposure-related: - occupational - environmental - medication Desquamative interstitial pneumonia Idiopathic
More informationInterstitial Lung Disease ILD: Definition
Interstitial Lung Disease 2007 Paul F. Simonelli,, MD, PhD, FCCP Clinical Director Center for Interstitial Lung Disease Columbia University Medical Center 1. ILD is not one disorder ILD: Definition 2.
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 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 informationThe Case of Marco Nazzareno Galiè, M.D.
The Case of Marco Nazzareno Galiè, M.D. DIMES Disclosures Consulting fees and research support from Actelion Pharmaceuticals Ltd, Bayer HealthCare, Eli Lilly and Co, GlaxoSmithKline and Pfizer Ltd Clinical
More informationDisclosures. Traditional Paradigm. Overview 4/17/2010. I have relationships with the following organizations and companies:
Disclosures Pharmacological Therapy for ILD What to Use and How to Use It Harold R Collard MD Interstitial Lung Disease Program University of California San Francisco (UCSF) I have relationships with the
More informationOutline Definition of Terms: Lexicon. Traction Bronchiectasis
HRCT OF IDIOPATHIC INTERSTITIAL PNEUMONIAS Disclosures Genentech, Inc. Speakers Bureau Tadashi Allen, MD University of Minnesota Assistant Professor Diagnostic Radiology 10/29/2016 Outline Definition of
More informationSEVERE ORGAN INVOLVEMENT IN SYSTEMIC SCLEROSIS WITH DIFFUSE SCLERODERMA
ARTHRITIS & RHEUMATISM Vol. 43, No. 11, November 2000, pp 2437 2444 2000, American College of Rheumatology 2437 SEVERE ORGAN INVOLVEMENT IN SYSTEMIC SCLEROSIS WITH DIFFUSE SCLERODERMA VIRGINIA D. STEEN
More information*Pari Basharat ABSTRACT OVERVIEW OF IDIOPATHIC INFLAMMATORY MYOPATHIES
IDIOPATHIC INFLAMMATORY MYOPATHIES: ASSOCIATION WITH OVERLAP MYOSITIS AND SYNDROMES: CLASSIFICATION, CLINICAL CHARACTERISTICS, AND ASSOCIATED AUTOANTIBODIES *Pari Basharat University of Western Ontario,
More informationCardiothoracic Manifestations of Connective Tissue Disease
Cardiothoracic Manifestations of Connective Tissue Disease Carole Dennie MD FRCPC Professor of Radiology and Medicine Head, Thoracic and Cardiac Imaging Sections The Ottawa Hospital Co-director Cardiac
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 informationCase 4 History. 58 yo man presented with prox IP joint swelling 2 months later pain and swelling in multiple joints Chest radiograph: bi-basilar
Case 4 History 58 yo man presented with prox IP joint swelling 2 months later pain and swelling in multiple joints Chest radiograph: bi-basilar basilar infiltrates suggestive of pulmonary fibrosis Open
More informationTHERAPEUTICS IN PULMONARY ARTERIAL HYPERTENSION Evidences & Guidelines
THERAPEUTICS IN PULMONARY ARTERIAL HYPERTENSION Evidences & Guidelines Vu Nang Phuc, MD Dinh Duc Huy, MD Pham Nguyen Vinh, MD, PhD, FACC Tam Duc Cardiology Hospital Faculty Disclosure No conflict of interest
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 informationCase Report Repository Corticotropin Injection for Treatment of Idiopathic Inflammatory Myopathies
Case Reports in Rheumatology Volume 2016, Article ID 9068061, 4 pages http://dx.doi.org/10.1155/2016/9068061 Case Report Repository Corticotropin Injection for Treatment of Idiopathic Inflammatory Myopathies
More informationIPF AND OTHER FIBROSING LUNG DISEASE: WHAT DRUGS MIGHT WORK AND ON WHOM DO THEY W ORK?
IPF AND OTHER FIBROSING LUNG DISEASE: WHAT DRUGS MIGHT WORK AND ON WHOM DO THEY W ORK? KEVIN K. BROWN, MD PROFESSOR AND VICE CHAIRMAN, DEPARTMENT OF MEDICINE NATIONAL JEWISH HEALTH DENVER, CO Kevin K.
More informationFinancial disclosure COMMON DIAGNOSES IN HRCT. High Res Chest HRCT. HRCT Pre test. I have no financial relationships to disclose. Anatomy Nomenclature
Financial disclosure I have no financial relationships to disclose. Douglas Johnson D.O. Cardiothoracic Imaging Gaston Radiology COMMON DIAGNOSES IN HRCT High Res Chest Anatomy Nomenclature HRCT Sampling
More informationThe connective tissue disorders (CTDs), also. Pivotal clinical dilemmas in collagen vascular diseases associated with interstitial lung involvement
Eur Respir J 2009; 33: 882 896 DOI: 10.1183/09031936.00152607 CopyrightßERS Journals Ltd 2009 REVIEW Pivotal clinical dilemmas in collagen vascular diseases associated with interstitial lung involvement
More informationLung Involvement In Systemic Sclerosis. Milind Y Nadkar, Nayan K Desai INTRODUCTION
Lung Involvement In Systemic Sclerosis Milind Y Nadkar, Nayan K Desai INTRODUCTION Systemic sclerosis (SSc) is a multisystem connective tissue disease of unknown etiology. Similar to other connective tissue
More informationDiagnosing Idiopathic Pulmonary Fibrosis on Evidence-Based Guidelines
Diagnosing Idiopathic Pulmonary Fibrosis on Evidence-Based Guidelines Rebecca Keith, MD Assistant Professor, Division of Pulmonary and Critical Care Medicine National Jewish Health, Denver, CO Objectives
More informationInterstitial Pneumonia Related to Undifferentiated Connective Tissue Disease Pathologic Pattern and Prognosis
[ Original Research Diffuse Lung Disease ] Interstitial Pneumonia Related to Undifferentiated Connective Tissue Disease Pathologic Pattern and Prognosis Ho-Cheol Kim, MD ; Wonjun Ji, MD ; Mi Young Kim,
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 informationIdiopathic inflammatory myopathies
Myositis and cancer Idiopathic inflammatory myopathies Primary idiopathic polymyositis Primary idiopathic dermatomyositis Juvenile poly/dermatomyositis Myositis associated with another CTD Myositis associated
More informationDr. Tracy Luckhardt Division of Pulmonary, Allergy and Critical Care Medicine University of Alabama at Birmingham
Dr. Tracy Luckhardt Division of Pulmonary, Allergy and Critical Care Medicine University of Alabama at Birmingham Types of Questions you Will Be Asked Rhetorical Participatory Disclosure of Relevant Financial
More information