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Disorders of the Immune

System

Disorders of Immune System


Defenses against infection
Immune system disorders
Autoimmune diseases
Immunodeficiency

Defenses Against Infection


Barriers (non-specific)
Skin
Mechanical removal (coughing, vomiting,
diarrhea, skin sloughing)
Normal flora
Antimicrobial secretions

Inflammation (non-specific)
Immunity (specific)

Immune Response

Natural vs. Acquired


Active vs. Passive
Primary vs. Secondary
Humoral vs. Cell-Mediated

Humoral Immunity
B Lymphocytes (Plasma Cells)
Produced in bone marrow
Make antibodies (immunoglobulins)

Antibodies
Protein molecules produced by B-cells
Specific shapes allow binding to specific
molecules (antigens)
Allow body to respond defensively to
presence of specific potential threats

Antibody Types

IgG
IgM
IgA
IgD
IgE

IgG
Most common antibody type
Only antibody that crosses placenta
Prime mediator of secondary immune
response
Principal defender against bacteria,
viruses, and toxins

IgM
Macroglobulin
Confined to bloodstream
First antibody to appear in response to
presence of antigen
Agent of primary immune response

IgA
Secretory antibody
Found in saliva, tears, respiratory
secretions, GI tract secretions
Frontline bacterial, viral defense

IgD

Role not fully understood


Low serum levels
High concentrations on B-cells
May act as receptors that trigger
production of other antibodies

IgE

Very low serum levels


Primarily bound to mast cells in tissues
Controls allergic response
Prevents parasitic infections

Antigen-antibody binding initiates


reactions that:

Neutralize bacterial toxins


Neutralize viruses
Promote phagocytosis
Activate components of inflammatory
response

Cell-Mediated Immunity
T Lymphocytes
Originate in bone marrow
Altered by passage through thymus
Responsible for mediation of cellular
immunity

T Lymphocyte Types

Helper cells (T4 cells)


Cytotoxic cells (Killer T cells)
Suppressor cells
Memory cells

Helper T-Cells
Master on-switch of immune system
Recognize antigens
Secrete lymphokines that activate all
other immune system cells
Stimulate B-cells to begin antibody
production

Cytotoxic (Killer) T-Cells


Respond to presence of antigens and
lymphokines produced by T-4 cells
Seek out, bind to, and destroy:
Cells infected by viruses
Some tumor cells
Cells of tissue transplants

Can deliver lethal hits on multiple cells in


sequence

Suppressor T-Cells
Produce lymphokines that inhibit
proliferation of B and T cells
Downregulate or dampen immune
response

Memory T-Cells
Have previously encountered
specific antigens
Respond in enhanced fashion on
subsequent exposures
Induce secondary immune response

Inflammation
Occurs in vascularized tissue
Nonspecific response to injury
Response is same regardless of agent that
initiates it

Inflammation: Physiology
Triggered by cellular injury
Injury activates mast cells
Mast cells release chemical mediators:

Histamine
Heparin
Leukotrienes (SRS-A)
Eosinophil chemotactic factor

Inflammation: Pathology
Mediators cause:
Vasodilation (redness, heat)
Vascular permeability (swelling)
White cell movement to and infiltration of
affected area (pus)
Nerve ending stimulation (pain)
Dolor, Calor, Tumor, Rubor

Inflammation: Purposes
Confines injurious agents
Increases blood cell, plasma
movement to injured areas
Enhances immune response
Destroys injurious agents
Promotes healing

Inflammation: Cellular
Components
Neutrophils

Phagocytic cells
Engulf foreign material/organisms
Arrive early
Short-lived

Inflammation: Cellular
Components
Macrophages

Phagocytic cells
Engulf foreign material/organisms
Arrive later
Long-lived
Transfer antigens back to T4 cells
Help initiate immune response to specific
agents

Inflammation: Cellular
Components
Eosinophils
Secrete caustic proteins
Dissolve surface membranes of parasites

Disorders of Immunity

Autoimmune Disease
Clinical disorder produced by an
immune response to a normal tissue
component of a patients body

Autoimmune Diseases

Hyperthyroidism
Primary myxedema
Type I diabetes
Addisons disease
Multiple sclerosis
Myasthenia gravis

Autoimmune Diseases

Rheumatic fever
Crohns disease
Ulcerative colitis
Rheumatoid arthritis
Systemic lupus erythematosis

Systemic Lupus Erythematosis

Primarily occurs in 20- to 40-year old females


Also in children and older adults
90% of patients are female
Autoimmune reaction to host DNA
Mortality after diagnosis averages 5% per year

Systemic Lupus Erythematosis


Signs/Symptoms
Facial rash/skin rash
triggered by sunlight
exposure
Oral/nasopharyngeal ulcers
Fever
Arthritis

Systemic Lupus Erythematosis


Signs/Symptoms
Serositis (pleurisy, pericarditis)
Renal injury/failure
CNS involvement with
seizures/psychosis
Peripheral vasculitis/gangrene
Hemolytic anemia

Systemic Lupus Erythematosis


Chronic management
Anti-inflammatory drugs
Aspirin
Ibuprofen
Corticosteroids

Avoidance of emotional stress,


physical fatigue, excessive sun
exposure

Disorders of Immunity

Immunodeficiency Diseases

Immunodeficiency Diseases
Congenital
Acquired

Immunodeficiency Diseases:
Congenital
B cell deficiency
Agammaglobulinemia
Hypogammaglobulinemia

Immunodeficiency Diseases:
Congenital
T cell deficiency
IgA deficiency
Severe combined immune deficiency
syndrome (B and T cell deficiency)

Immunodeficiency Diseases:
Acquired

Nutritional deficiency
Iatrogenic (drugs, radiation)
Trauma (prolonged hypoperfusion)
Stress
Infection (AIDS)

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