AUTOIMMUNE

DISEASES II
GINO TANN

No Caption Found

Shmerling, R. H. N Engl J Med 2003;349:1499-1500

No Caption Found

Shmerling, R. H. N Engl J Med 2003;349:1499-1500

Origins of Antibodies to DNA

Hahn, B. H. N Engl J Med 1998;338:1359-1368

Clinical Assays for Serum Antibodies to DNA

Hahn, B. H. N Engl J Med 1998;338:1359-1368

Kaplan-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;349:1526-1533

Phases in the Development of Pathogenic Autoimmunity

Arbuckle, M. R. et al. N Engl J Med 2003;349:1526-1533

Origin of Pathogenic Antibodies to DNA

Hahn, B. H. N Engl J Med 1998;338:1359-1368

MALAR RASH

SYSTEMIC LUPUS ERYTHEMATOSUS

World Health Organization Classification of Lupus Nephritis

Somers, M. J. et al. N Engl J Med 2004;350:1550-1559

LUPUS NEPHRITIS

Three Mechanisms by Which Antibodies to DNA Cause Tissue Damage

Hahn, B. H. N Engl J Med 1998;338:1359-1368

Electron Micrograph of the Renal-Biopsy Specimen

Somers, M. J. et al. N Engl J Med 2004;350:1550-1559

DRUG INDUCED SLE

SJORGRENS SYNDROME

Anti SS-A positive

Anti SS-B positive

CLINICAL FEATURES OF SJOGRENS

SYNDROME

Keratoconjunctivitis sicca
Corneal vascularization
Lacrimal gland enlargement
Xerostomia
Parotid enlargement
Recurrent parotitis
Epistaxis
Nasal septal perforation
Sinusitis
Serous otitis media
Dysphagia
Oesophageal web
Atrophic gastritis
Pancreatic disease
Raynauds phenomenon
Purpura
Hughes GRV 1987

Renal tubular defects

Interstitial nephritis
Vaginitis sicca
Reccurent UTIs
Cranial nerve lesions
Peripheral neuropathy
Tracheitis
Bronchitis
Bronchiolitis
Pneumonitis
Atelectasis
Pleural effusions
Interstitial lung disease
Anemia, leukopenia
Thrombocytopenia
Hypergammaglobulinemi
a
Autoantibodies
Drug hypersensitivity
Lymphoma

Types of Interstitial Nephritis

Singh, A. K. et al. N Engl J Med 2003;349:2055-2063

AUTOIMMUNE THYROIDITIS

DERMATOMYOSITIS

Anti Jo-1 positive

DERMATOMYOSITIS

SCLERODERMA

Anti Scl-70 positive

RAYNAUDS PHENOMENON

Nail-Fold Capillaries in a Patient with the CREST Syndrome (Calcinosis Cutis, Raynaud's
Phenomenon, Esophageal Dysfunction, Sclerodactyly, and Telangiectasia), or Limited
Scleroderma

Wigley, F. M. N Engl J Med 2002;347:1001-1008

CALCINOSIS

CALCINOSIS

CREST
Calcinosis
Raynaud
Esophageal dysmotility
Sclerodactyly
Teleangiectases

SCLERODACTYLY

RAYNAUD DIGITAL GANGRENE

ANTI-CENTROMERE ANTIBODIES Limited disease

ANTI-TOPOISOMERASE I (Anti Scl70)

ANTI-FIBRILLARIN (Anti-U3-RNP)

- Diffuse disease

THE LUNGS IN CONNECTIVE TISSUE DISEASES

Pleural 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 SCLERODERMA

IDIOPATHIC PULMONARY FIBROSIS

BRONCHIOLITIS OBLITERANS ORGANIZING PNEUMONIA

Epler GR. Arch Intern Med 2001;161:15864

BRONCHIOLITIS OBLITERANS ORGANIZING PNEUMONIA

Epler GR 2001

DIFFUSE ALVEOLAR HAEMORRHAGE

Cough
Haemoptysis
Decreased Hb
Sputum macrophages
containing haemosiderin
HRCT Thorax ground glass

GROUND GLASS LESIONS ON HRCT

DIFFUSE ALVEOLAR
HAEMORRHAGE

SLE

ANTI BASEMENT MEMBRANE ANTIBODIES

GOODPASTURES SYNDROME - LUNG

ANTI BASEMENT MEMBRANE ANTIBODIES

Glomerulus Goodpastures syndrome GS

Lungs
- GS, SLE, APA, MCTD, Scleroderma

PLEXOGENIC ARTERIOPATHY

PULMONARY HYPERTENSION
SURVIVAL AND vWF : Ag

A 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

Choe, W. et al. N Engl J Med 2000;343:1771

DRUG INDUCED SCLERODERMA

COAGULATION FACTOR XIII IN SCLERODERMA

Factor 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 studies

Julien D. Biomedical Progress 2003;16:36-40

International Consensus Statement on Preliminary Criteria for the Classification of the

Antiphospholipid Syndrome

Levine, J. S. et al. N Engl J Med 2002;346:752-763

Classification and Detection of Antiphospholipid Antibodies

Levine, J. S. et al. N Engl J Med 2002;346:752-763

Opposing Effects of Antiphospholipid Antibodies on Coagulation

Levine, J. S. et al. N Engl J Med 2002;346:752-763

Detection of Lupus Anticoagulant Antibodies by in Vitro Coagulation Assays

Levine, J. S. et al. N Engl J Med 2002;346:752-763

Disease States and Risk Factors Predisposing Patients to Thromboembolism

Levine, J. S. et al. N Engl J Med 2002;346:752-763

Clinical Manifestations of the Antiphospholipid Syndrome

Levine, J. S. et al. N Engl J Med 2002;346:752-763

PSORIASIS

Psoriatic Skin (Panel A) and an Immune Synapse (Panel B)

Kupper, T. S. N Engl J Med 2003;349:1987-1990

PEMPHIGUS

Anti desmoglein 3

THERES STILL A LOT TO LEARN !

THANK YOU

GT 11052004