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Alterations in the Immune Response - 681. Shirlee Ann Stokes, RN, EdD, FAAN. Immunodeficiency Disease. Alteration in the immune response Primary versus Secondary Primary Congenital Inherited – most are recessive traits Manifestations seen at birth and throughout childhood.
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Alterations in the Immune Response - 681 Shirlee Ann Stokes, RN, EdD, FAAN
Immunodeficiency Disease • Alteration in the immune response • Primary versus Secondary • Primary • Congenital • Inherited – most are recessive traits • Manifestations seen at birth and throughout childhood
Immunodeficiency Disease • Secondary • Acquired • Secondary to another disease • Malnutrition • Infection (e.g., acquired immunodeficiency syndrome [AIDS]) • Neoplastic disease (e.g., lymphoma) • Immunosuppressive therapy (e.g., corticosteroids or transplant rejection medications) • Nephrotic syndrome
Immunodeficiency • Absolute or partial loss if immune response • Categories • Humoral (B lymphocytes) • Cell-mediated (T lymphocytes) • Complement system • Phagocytic system (neutrophils and macrophages)
Humoral Immunodeficiency • Alteration in • B-cell function • Antibody production (IgG, IgA, IgE, IgM, IgD)
Humoral Immunodeficiency • Diseases • Hypogammaglobulinemia • X-Linked Agammaglobulinemia • Common Variable Immunodeficiency • Selective IgA deficiency • Immunoglobulin G Subclass Deficiency • Review Figure 21-1 • Shows how each are developed
Humoral Immunodeficiency • Common Manifestations • Recurrent infections – Particularly in children • Bacterial infections • Not intracellular bacteria (mycobacteria, fungi, or protozoa as these are handled by T cells • Not viral (except enterovirus causing GI infections) as these are handled by T cells • Bacterial with virulence factors • Toxins • Evasive factors – Capsulated polysaccharide • S. pneumoniae, H. influenzae, S. aureus, Neisseria meningitis
Humoral Immunodeficiency • Particularly prone to pyogenic organisms • Streptococcus pneumonia, Haemophilus influenzae, G- organisms • Infections that do not respond to therapy • Infections that respond slowly to therapy • Common sites of infection • Ear, Respiratory, Throat
Cell Mediated Immunodeficiency • Alteration in T cell • Production • Function
Cell Mediated Immunodeficiency • Primary Cell-mediated Diseases • DiGeorge syndrome • X-Linked immunodeficiency with Hyper-IgM • Secondary cell-mediated Diseases • Secondary to infections • Viral – measles, cytomegalovirus • Hodgkin’s disease • HIV
Cell Mediated Immunodeficiency • Manifestations • Recurrent Infections • Often in children • Susceptible to wide range of pathogens • Severe infections • Infections resistant to therapy • Infections leading to death • Children rarely survive
Combined T-cell and B-cell Immunodeficiency • Combined Immunodeficiency Syndrome (CIDS) • Genetic • Disruption in communication between T and B lymphocytes
Combined T-cell and B-cell Immunodeficiency • Combined Immunodeficiency Syndrome (CIDS) • Severe Combined Immunodeficiency Syndrome(SCIDSS) • X-linked Combined Immunodeficiency Syndrome • Autosomal Recessive Combined Immunodeficiency Syndrome • Combined Immunodeficiency • Ataxia-Telangiectasia • Wiskott-Aldrich Syndrome
Combined T-cell and B-cell Immunodeficiency • Manifestations • Present in children • Recurrent Infections • Failure to thrive • Infections leading to death
Complement System Disorders • Alteration in • One or more complement components • Primary Disorders • Transmitted recessive trait • Deficiency in C1, C2, C, C4, • Increased susceptibility to infection • Increased incidence of Systemic Lupus Erythematosus (SLE) • Vasculitis, glomerulonephritis, rash • Deficiency in C5, C6, C7, C8, C9 • Increased susceptibility to infection • Hereditary Angioneurotic Edema
Complement System Disorders • Secondary Disorders • Person with functionally normal complement system • Disease causing rapid activation and turnover • Diseases causing reduced synthesis of complement components • Diseases • Immune Complex disorders (Type III Hypersensitivity) • Cirrhosis • Malnutrition
Phagocytic System Disorders • Alteration in • Polymorphonuclear leukocytes • Neutrophils • Eosinophils • Mononuclear leukocytes • Monocytes/macrophages
Phagocytic System Disorders • Reduction in number of phagocytes • Reduction in function of phagocytic cells • Chemotaxis • Adherence • Phagocytosis • Ability to kill
Phagocytic System Disorders • Manifestations • Recurrent Infections – recur throughout life • Chronic Infections • Infections resistant to antibiotics
Phagocytic System Disorders • Diseases • Primary • Chronic Granulomatous Disease (CGD) • Secondary • Side effect of drugs • Corticosteroids • Cyclosporine • Diabetes mellitus • HIV/AIDS
Hypersensitivity Reactions • Excessive or inappropriate activation of immune system • Types • Type I (IgE- mediated) • Type II (IgM and IgG - Tissue-Specific) • Type III (Immune-Complex) • Type IV (Cell-Mediated)
Type I Hypersensitivity • IgE mediated • Most antigens (allergen) are environmental • Antigen response • Most potent when injected • Less potent when ingested
Type I Hypersensitivity • First exposure or sensitization • Antigen changes B lymphocyte to plasma cell • Facilitated by Helper T (CD4 or TH2) • Plasma cell produces IgE • IgE coats surface of mast cells or Basophils • e.g. plugs into Fc receptor sites
Type I Hypersensitivity • Second exposure allergen (Ag) binds to IgE which is bound to Fc receptor sites on mast cells or basophils • Causes mast cells and basophil degranulation • Each exposure become more rapid and severe
Degranulation of Mast Cell • Release of histamine • H1 receptor response • Vasodilatation • Local increase in blood flow • Increased vascular permeability • Extravasation of fluid into interstitial spaces • Edema of bronchi • Edema peripheral tissue (local) • Systemic drop in blood pressure (systemic) • Contract bronchial smooth muscles • Bronchial constriction
Degranulation of Mast Cell • Release of histamine • H2 receptor Response • Increased gastric secretion • Decrease of histamine release from mast cell
Degranulation of Mast Cell • Synthesis of Arachidonic acids • Prostaglandins • Leukotrienes • Stimulation of cytokines
Primary and Secondary PhasesType I Hypersensitivity • Primary Phase • Initial phase • Occurs in minutes • Lasts 60 minutes • Secondary Phase • Occurs 2-8 hours later • Lasts for days
Type I Hypersensitivity Reactions • Individual with allergies • Genetic predisposition • Alteration • Increased IgE • Increased Fc receptors • Atopic – Local reaction • Systemic Reactions
Type I Hypersensitivity Reactions • Atopic – Local reaction • Urticaria (hives) • Allergic rhinitis • Hay fever • Seasonal allergies related to pollen, ragweed, etc • Recurrent allergies related to animal dander, dust mites, etc. • Atopic dermatitis • Food allergies (Milk, eggs, peanuts, soy, fish, etc.) • Certain types of asthma
Type I Hypersensitivity Reactions • Systemic Reaction • Anaphylaxis • Life threatening • Rapid deterioration • Vascular dilation leading to vasogenic shock • Widespread edema • Laryngeal edema • Bronchospasm
Type I Hypersensitivity – Manifestations • Urticaria or hives • Itching
Type I Hypersensitivity – Manifestations • Urticaria
Type I Hypersensitivity – Manifestations • Eye • Conjunctivitis • Discharge • Nasal • Itching • Discharge
Type I Hypersensitivity – Manifestations • Bronchials • Spasm or smooth muscles • Edema • Increased production of mucous • Laryngeal edema
Type I Hypersensitivity – Manifestations • Angio edema • Face • Mouth
Type I Hypersensitivity – Manifestations • Angioedema • Face • Mouth • Tongue
Type I Hypersensitivity – Manifestations • Angioedema • Face • Eyes • Mouth
Type I Hypersensitivity – Manifestations • Angioedema • Scrotum and penis
Type I Hypersensitivity – Manifestations • Cardiac • Hypotension • Dysrhythmia • Gastrointestinal Tract • Cramps and malabsorption
Testing for Type I Hypersensitivity • Skin Testing • Positive Radioimmunosorbent Testing (RIST) • Tests for IgE
Type II Hypersensitivity • Tissue Specific Reaction • Antibody (IgG or IgM) formed to act against specific antigen on cell membrane • Cell is then destroyed by the antibody • Diseases • Graves disease • Drug sensitivities – anemia or thrombocytopenia • Hemolytic diseases • ABO incompatibility • Rh incompatibility
Type II Hypersensitivity • Type O is universal donner because has not antigens • Type AB is universal recipient because body does not develop antibodies (IgG or IgM) to the blood
Type III Hypersensitivity • Immune-Complex mediated reaction • Immune complex is formed (antigen antibody complex)
Type III Hypersensitivity • Immune Complex deposited in • Walls of blood vessels leading to vasculitis • Glomerulus of kidneys leading to glomerulonephritis • Lung • Joints leading to arthritis
Type III Hypersensitivity • Damage occurs because • Immune Complex (Antibody) is deposited in tissues • Inflammatory response occurs • Complement is activated • Neutrophilic chemotaxis occurs • Neutrophils are drawn to the site of the deposited immune complex • Neutrophils try to ingest the immune complex • Lysosomal enzymes are released by neutrophils and cause tissue damage
Type III Hypersensitivity • Diseases • Raynaud Disease or Raynaud Phenomenon • Systemic Lupus Erythematosus (SLE) • Serum sickness • Arthus reaction
Immune Complex Diseases – Raynaud Disease or Raynaud Phenomenon