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IMMUNE DISORDERS Noel C. Santos, M.D.
YOUR ACTIVE IMMUNE DEFENSES Innate Immunity - invariant (generalized) - early, limited specificity - the first line of defense Adaptive Immunity - variable (custom) - later, highly specific - ‘‘remembers’’ infection   1.  Barriers - skin, tears 2.  Phagocytes - neutrophils,  macrophages 3.  NK & M ast cells 4.  Complement and other proteins ,[object Object],2.  Activated T cells provide help to B cells & kill abN/ infected cells 3.  B cells - produce Ab specific for Ag
Immunologic Deficiency Syndromes ,[object Object],[object Object],[object Object],[object Object]
[object Object],[object Object],[object Object],[object Object],[object Object],Primary Immunodeficiencies
Primary Immunodeficiencies ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object]
Secondary Immunodeficiencies ,[object Object],[object Object]
X-linked Agammaglobulinemia (Bruton’s Disease) ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object]
X-linked Agammaglobulinemia (Bruton’s Disease) ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object]
Isolated IgA Deficiency ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object]
Common Variable (Ig) Immunodeficiency ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object]
[object Object],[object Object],[object Object],[object Object],[object Object],Common Variable (Ig) Immunodeficiency
Hyper IgM Syndrome ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object]
Thymic Hypoplasia (DiGeorge Syndrome) ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object]
Severe Combined Immunodeficiency Diseases ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object]
Severe Combined Immunodeficiency Diseases ,[object Object],[object Object],[object Object],[object Object],[object Object]
Wiskott-Aldrich Syndrome ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object]
Complement Deficiencies ,[object Object],[object Object],[object Object],[object Object],[object Object]
Acquired Immunodeficiency Syndrome (AIDS) ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object]
Acquired Immunodeficiency Syndrome (AIDS) ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object]
[object Object],[object Object],[object Object],Acquried Immunodeficiency Syndrome (AIDS)
Acquired Immunodeficiency Syndrome (AIDS) ,[object Object],[object Object],[object Object],[object Object],[object Object]
Hypersensitivity Reactions ,[object Object],[object Object],[object Object],[object Object],[object Object]
[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object]
[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object]
Mast Cells and the Allergic Response
Mast Cells and the Allergic Response
[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object]
[object Object],[object Object],[object Object],[object Object],[object Object],[object Object]
Immune Complex Mediated Hypersensitivity
[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object]
Allergic Contact Dermatitis Response to Poison Ivy Hapten
Autoimmune Diseases ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object]
[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object]
Autoimmune Disease ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object]
Normally ,[object Object],[object Object]
[object Object],[object Object],[object Object],[object Object],[object Object]
 
Mechanisms of Autoimmune Diseases ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object]
[object Object],[object Object],[object Object],[object Object],[object Object],[object Object]
Autoimmune Diseases Organ-Specific: Hashimoto thyroiditis Autoimmune hemolytic anemia Rheumatoid arthritis Autoimmune atrophic gastritis (pernicious anemia) Multiple sclerosis Reiter syndrome Autoimmune orchitis Inflammatory myopathies* Goodpasture syndrome Autoimmune thrombocytopenia Polyarteritis nodosa* Insulin-dependent diabetes mellitus   Myasthenia gravis   Graves disease   Primary biliary cirrhosis*   Systemic: Systemic lupus erythematosus Systemic sclerosis (scleroderma)* Sjögren syndrome Autoimmune (chronic active) hepatitis*   Ulcerative colitis*
SYSTEMIC LUPUS ERYTHEMATOSUS ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object]
centromere homogenous speckled nucleolar rim
Etiology and Pathogenesis ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object]
Clinical and Pathologic Manifestations of SLE Clin Mfnx Prevalence in Pts (%) Hematologic 100 Arthritis 90 Skin 85 Fever 83 Fatigue 81 Weight loss 63 Renal 50 CNS 50 Pleuritis 46 Myalgia 33 Pericarditis 25 Gastrointestinal 21 Raynaud phenomenon 20 Ocular 15 Peripheral neuropathy 14
Morphology ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object]
Pericarditis and valvular deposits (Libman-Sacks)
Organ Specific Autoimmune Diseases ,[object Object],[object Object],[object Object],[object Object],[object Object]
[object Object],[object Object],[object Object]
Thank you very much for listening……

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Diseases of the Immune System

  • 1. IMMUNE DISORDERS Noel C. Santos, M.D.
  • 2.
  • 3.
  • 4.
  • 5.
  • 6.
  • 7.
  • 8.
  • 9.
  • 10.
  • 11.
  • 12.
  • 13.
  • 14.
  • 15.
  • 16.
  • 17.
  • 18.
  • 19.
  • 20.
  • 21.
  • 22.
  • 23.
  • 24.
  • 25. Mast Cells and the Allergic Response
  • 26. Mast Cells and the Allergic Response
  • 27.
  • 28.
  • 29. Immune Complex Mediated Hypersensitivity
  • 30.
  • 31. Allergic Contact Dermatitis Response to Poison Ivy Hapten
  • 32.
  • 33.
  • 34.
  • 35.
  • 36.
  • 37.  
  • 38.
  • 39.
  • 40. Autoimmune Diseases Organ-Specific: Hashimoto thyroiditis Autoimmune hemolytic anemia Rheumatoid arthritis Autoimmune atrophic gastritis (pernicious anemia) Multiple sclerosis Reiter syndrome Autoimmune orchitis Inflammatory myopathies* Goodpasture syndrome Autoimmune thrombocytopenia Polyarteritis nodosa* Insulin-dependent diabetes mellitus Myasthenia gravis Graves disease Primary biliary cirrhosis* Systemic: Systemic lupus erythematosus Systemic sclerosis (scleroderma)* Sjögren syndrome Autoimmune (chronic active) hepatitis* Ulcerative colitis*
  • 41.
  • 43.
  • 44. Clinical and Pathologic Manifestations of SLE Clin Mfnx Prevalence in Pts (%) Hematologic 100 Arthritis 90 Skin 85 Fever 83 Fatigue 81 Weight loss 63 Renal 50 CNS 50 Pleuritis 46 Myalgia 33 Pericarditis 25 Gastrointestinal 21 Raynaud phenomenon 20 Ocular 15 Peripheral neuropathy 14
  • 45.
  • 46. Pericarditis and valvular deposits (Libman-Sacks)
  • 47.
  • 48.
  • 49. Thank you very much for listening……

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