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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