Organization of Immune System Cells Lymphocytes: B-cells (immunoglobulins) T-cells Plasma cells (B-cells) Macrophages/dendritic cells Mast cells Eosinophils (against parasites) Natural Killer (NK) Cells Important Molecules Antigens Antibodies T-cell receptors MHC molecules Adhesion molecules Cytokines Immune Response Non-specific (innate) Immunity Specific Immunity Categories of Immune Disorders Hypersensitivity Type I (anaphylaxis) IgE (embedded in mast cell or basophils) Mast cells release vasoactive substances Preformed mediators Secondary mediators: Clinical manifestations of Type I Hypersensitivity Systemic anaphylaxis (anaphylactic shock) Local anaphylaxis (atopic allergy) Asthma: (Complex mechanism/T-cells also involved) Type II Clinical examples: Autoimmune hemolytic anemia Goodpasture syndrome Transfusion reactions Grave's disease Myasthenia gravis Type III Clinical examples: Immune complex disease: Serum sickness Some types of vasculitis Arthus reaction - experimental Post-streptocccal glomerulonephritis Type IV Delayed-type cell-mediated Clinical examples: Tuberculosis Contact dermatitis Transplant rejection Background Major complications Rejection Infection Graft-versus-host disease (GVHD): Development of Cancers Lymphoma Kaposi sarcoma Squamous cell CA-skin Histocompatibility antigens Class I antigens Class II antigens Phases of transplant rejection: Hyperacute rejection Acute rejection Chronic rejection Notes Autoimmune Disease: Etiology Mechanisms Organ-specific autoimmune disease Vitiligo (skin hypopigmentation) Thyroid: Graves' disease Hashimoto's disease Addison's disease (autoimmune adrenalitis) Pernicious anemia (autoimmune gastritis) Hemolytic anemia Myasthenia gravis Diabetes mellitus type I Primary biliary cirrhosis (anti-mitochondrial antibodies) Non-organ specific autoimmune disease Systemic Lupus Erythematosus (SLE) Etiology Pathogenesis Pathology Kidney: glomerulonephritis Skin: Joints: Serositis: Heart: nonbacterial verrucous endocarditis (Libman-Sacks) Spleen: "onion-skin" pervivascular fibrosis Lungs: Clinical Diagnosis Systemic sclerosis (scleroderma) Pathology Pathogenesis Clinical Raynaud syndrome Arthritis (similar to rheumatoid) Dysphagia (fibrosis esophagus) Pulmonary failure (lung fibrosis) Malignant hypertension & renal failure Cardiac arrhythmia & heart failure Diffuse scleroderma rapid progression characteristic ANA Inflammatory myopathies polymyositis dermatomyositis CREST Syndrome Female-male ratio 3:1 peak incidence 50 - 60 Clinical manifestations of CREST Syndrome: Localized Scleroderma (Morphea) Rheumatoid Disease Mixed connective tissue disease Sjogren syndrome Pathology Clinical Sicca syndrome Dry eyes Dry mouth Swollen parotid glands +/- (Mikulicz syndrome) 90% females 35 to 45 years 60% associated with other autoimmune disease (secondary Sjogren's syndrome): Collagen-vascular diseases Examples: SLE Rheumatoid disease Systemic sclerosis Mixed connective tissue disease Sjogrens syndrome Deficiency states/Immunodeficiency Syndromes Primary immunodeficiencies Primary (B-cell) antibody deficiency Agammaglobulinemia of Bruton Common variable immunodeficiency Isolated IgA deficiency Primary (T-cell) cell-mediated deficiency DiGeorge syndrome Immunodeficiency with thrombocytopenia (Wiskott-Aldrich syndrome) Selective deficiency IgA or IgG Ataxia-Telangiectasia Syndrome Phagocyte deficiency (chronic granulomatous disease) SCID (Severe Combined Immunodeficiency Disease) Leukocyte Adhesion Deficiency Chediak-Higashi Syndrome Secondary immunodeficiencies Infections Antibody deficiency: Cell-mediated deficiency: Viruses HIV/AIDS Immune deficiency Complications Opportunistic infections Risk groups Increased risk for neoplasms Natural history Early acute phase Middle phase Final crisis phase Full-blown AIDS HIV infection Non-transforming type C cytopathic retrovirus Viral envelope gp 120 binds to receptors on (helper/CD4) T-cells Reverse transcriptase allows virus to integrate into host cell genome Transmission Fungi Bacteria Protozoa (cryptosporidia) Complement deficiency Phagocyte deficiency: Genetic deficiencies of complement system Amyloidosis Amyloid Amyloid States Immunocyte Dyscrasia-Associated (Primary) Amyloidosis Reactive Systemic (Secondary) Amyloidosis Heredofamilial Amyloidosis Hemodialysis-Associated Amyloidosis Localized Amyloidosis Amyloid of Aging Endocrine Amyloid Pathology Glossary of Terms