autoimmune diseases - ii

58
AUTOIMMUNE DISEASES II GINO TANN

Upload: wawan-wae

Post on 06-Nov-2015

38 views

Category:

Documents


3 download

DESCRIPTION

autoimun

TRANSCRIPT

  • AUTOIMMUNE DISEASES IIGINO TANN

  • Shmerling, R. H. N Engl J Med 2003;349:1499-1500No Caption Found

  • Shmerling, R. H. N Engl J Med 2003;349:1499-1500No Caption Found

  • Hahn, B. H. N Engl J Med 1998;338:1359-1368Origins of Antibodies to DNA

  • Hahn, B. H. N Engl J Med 1998;338:1359-1368Clinical Assays for Serum Antibodies to DNA

  • Arbuckle, M. R. et al. N Engl J Med 2003;349:1526-1533Kaplan-Meier Product-Limit Curves for the Proportion of Patients with Positive Antibody Tests Relative to the Time of Diagnosis or Appearance of the First Clinical Manifestation of Systemic Lupus Erythematosus (SLE)

  • Arbuckle, M. R. et al. N Engl J Med 2003;349:1526-1533Phases in the Development of Pathogenic Autoimmunity

  • Hahn, B. H. N Engl J Med 1998;338:1359-1368Origin of Pathogenic Antibodies to DNA

  • MALAR RASH

  • SYSTEMIC LUPUS ERYTHEMATOSUS

  • Somers, M. J. et al. N Engl J Med 2004;350:1550-1559World Health Organization Classification of Lupus Nephritis

  • LUPUS NEPHRITIS

  • Hahn, B. H. N Engl J Med 1998;338:1359-1368Three Mechanisms by Which Antibodies to DNA Cause Tissue Damage

  • Somers, M. J. et al. N Engl J Med 2004;350:1550-1559Electron Micrograph of the Renal-Biopsy Specimen

  • DRUG INDUCED SLE

  • SJORGRENS SYNDROMEAnti SS-A positive Anti SS-B positive

  • CLINICAL FEATURES OF SJOGRENS SYNDROMEKeratoconjunctivitis siccaCorneal vascularizationLacrimal gland enlargement

    XerostomiaParotid enlargementRecurrent parotitis

    EpistaxisNasal septal perforationSinusitisSerous otitis media

    DysphagiaOesophageal webAtrophic gastritisPancreatic disease

    Raynauds phenomenonPurpuraRenal tubular defectsInterstitial nephritisVaginitis siccaReccurent UTIs

    Cranial nerve lesionsPeripheral neuropathy

    TracheitisBronchitisBronchiolitisPneumonitisAtelectasisPleural effusionsInterstitial lung disease

    Anemia, leukopeniaThrombocytopeniaHypergammaglobulinemiaAutoantibodies

    Drug hypersensitivityLymphoma Hughes GRV 1987

  • Singh, A. K. et al. N Engl J Med 2003;349:2055-2063Types of Interstitial Nephritis

  • AUTOIMMUNE THYROIDITIS

  • DERMATOMYOSITISAnti Jo-1 positive

  • DERMATOMYOSITIS

  • SCLERODERMAAnti Scl-70 positive

  • RAYNAUDS PHENOMENON

  • Wigley, F. M. N Engl J Med 2002;347:1001-1008Nail-Fold Capillaries in a Patient with the CREST Syndrome (Calcinosis Cutis, Raynaud's Phenomenon, Esophageal Dysfunction, Sclerodactyly, and Telangiectasia), or Limited Scleroderma

  • CALCINOSIS

  • CALCINOSISCREST

    CalcinosisRaynaudEsophageal dysmotilitySclerodactylyTeleangiectases

  • SCLERODACTYLY

  • RAYNAUD DIGITAL GANGRENE

  • ANTI-CENTROMERE ANTIBODIES Limited diseaseANTI-TOPOISOMERASE I (Anti Scl-70) ANTI-FIBRILLARIN (Anti-U3-RNP)- Diffuse disease

  • THE LUNGS IN CONNECTIVE TISSUE DISEASESPleural effusion

    Infections

    Interstitial Lung Disease

    Diffuse Alveolar Haemorrhage Pulmonary capillaritis

    Pulmonary Arterial Hypertension

    Pulmonary Aspiration Syndromes

  • DIFFUSE LUNG DISEASE IDIOPATHIC PULMONARY FIBROSIS

  • DIFFUSE LUNG DISEASE IN SCLERODERMAIDIOPATHIC PULMONARY FIBROSIS

  • BRONCHIOLITIS OBLITERANS ORGANIZING PNEUMONIAEpler GR. Arch Intern Med 2001;161:158-64

  • BRONCHIOLITIS OBLITERANS ORGANIZING PNEUMONIAEpler GR 2001

  • DIFFUSE ALVEOLAR HAEMORRHAGECough

    Haemoptysis

    Decreased Hb

    Sputum macrophages containing haemosiderin

    HRCT Thorax ground glass

  • GROUND GLASS LESIONS ON HRCT

  • DIFFUSE ALVEOLARHAEMORRHAGESLE

  • ANTI BASEMENT MEMBRANE ANTIBODIESGOODPASTURES SYNDROME - LUNG

  • ANTI BASEMENT MEMBRANE ANTIBODIESGlomerulus Goodpastures syndrome GSLungs - GS, SLE, APA, MCTD, Scleroderma

  • PLEXOGENIC ARTERIOPATHY

  • PULMONARY HYPERTENSION

    SURVIVAL AND vWF : Ag

  • Choe, W. et al. N Engl J Med 2000;343:1771A 37-year-old woman who had had systemic sclerosis for several years, with known esophageal and skin involvement, presented with dyspnea on exertion and a large pericardial effusion

  • DRUG INDUCED SCLERODERMA

  • COAGULATION FACTOR XIII IN SCLERODERMAFactor XIII

    Interacts with fibroblasts

    Inhibits collagen synthesis

    Increases collagen degradation

    Improves skin sclerosis, morphea, arthralgia and possible esophageal and lung involvement

    Stabilizes endothelial barrier function

    Ongoing clinical studiesJulien D. Biomedical Progress 2003;16:36-40

  • Levine, J. S. et al. N Engl J Med 2002;346:752-763International Consensus Statement on Preliminary Criteria for the Classification of the Antiphospholipid Syndrome

  • Levine, J. S. et al. N Engl J Med 2002;346:752-763Classification and Detection of Antiphospholipid Antibodies

  • Levine, J. S. et al. N Engl J Med 2002;346:752-763Opposing Effects of Antiphospholipid Antibodies on Coagulation

  • Levine, J. S. et al. N Engl J Med 2002;346:752-763Detection of Lupus Anticoagulant Antibodies by in Vitro Coagulation Assays

  • Levine, J. S. et al. N Engl J Med 2002;346:752-763Disease States and Risk Factors Predisposing Patients to Thromboembolism

  • Levine, J. S. et al. N Engl J Med 2002;346:752-763Clinical Manifestations of the Antiphospholipid Syndrome

  • PSORIASIS

  • Kupper, T. S. N Engl J Med 2003;349:1987-1990Psoriatic Skin (Panel A) and an Immune Synapse (Panel B)

  • PEMPHIGUSAnti desmoglein 3

  • THERES STILL A LOT TO LEARN !THANK YOUGT 11052004