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7/18/2019 Immune Disorders
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Disorders of the ImmuneSystem
7/18/2019 Immune Disorders
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Disorders of Immune System
• Defenses against infection
• Immune system disorders
– Autoimmune diseases
– Immunodeficiency
7/18/2019 Immune Disorders
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Defenses Against Infection
• Barriers (non-specific)
– Skin
– Mechanical removal (coughing, vomiting,
diarrhea, skin sloughing)
– ormal flora
– Antimicro!ial secretions
• Inflammation (non-specific)
• Immunity (specific)
7/18/2019 Immune Disorders
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Immune Response
• atural vs" Ac#uired
• Active vs" $assive
• $rimary vs" Secondary
• %umoral vs" &ell-Mediated
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Humoral Immunity
• B 'ymphocytes ($lasma &ells)
– $roduced in !one marro
– Make anti!odies (immunoglo!ulins)
7/18/2019 Immune Disorders
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Antibodies
• $rotein molecules produced !y B-cells
• Specific shapes allo !inding to specific
molecules (antigens)• Allo !ody to respond defensively to
presence of specific potential threats
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Antibody Types
• Ig
• IgM
•IgA
• IgD
• Ig*
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IgG
• Most common anti!ody type
• +nly anti!ody that crosses placenta
•
$rime mediator of secondary immuneresponse
• $rincipal defender against !acteria,
viruses, and toins
7/18/2019 Immune Disorders
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IgM
• Macroglo!ulin
• &onfined to !loodstream
•
irst anti!ody to appear in response topresence of antigen
• Agent of primary immune response
7/18/2019 Immune Disorders
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IgA
• Secretory anti!ody
• ound in saliva, tears, respiratory
secretions, I tract secretions
• rontline !acterial, viral defense
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IgD
• .ole not fully understood
• 'o serum levels
•
%igh concentrations on B-cells• May act as receptors that trigger
production of other anti!odies
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IgE
• /ery lo serum levels
• $rimarily !ound to mast cells in tissues
•
&ontrols allergic response• $revents parasitic infections
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Antigen-antibody binding initiates
reactions that• eutrali0e !acterial toins
• eutrali0e viruses
• $romote phagocytosis
• Activate components of inflammatory
response
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!ell-Mediated Immunity
• 1 'ymphocytes
– +riginate in !one marro
– Altered !y passage through thymus
– .esponsi!le for mediation of cellular
immunity
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T "ymphocyte Types
• %elper cells (12 cells)
• &ytotoic cells (3iller 1 cells)
• Suppressor cells• Memory cells
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Helper T-!ells
• Master 4on-sitch5 of immune system
• .ecogni0e antigens
• Secrete lymphokines that activate allother immune system cells
• Stimulate B-cells to !egin anti!ody
production
7/18/2019 Immune Disorders
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!ytoto#ic $%iller& T-!ells
• .espond to presence of antigens and
lymphokines produced !y 1-2 cells
•
Seek out, !ind to, and destroy6 – &ells infected !y viruses
– Some tumor cells
– &ells of tissue transplants
• &an deliver lethal hits on multiple cells in
se#uence
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Suppressor T-!ells
• $roduce lymphokines that inhi!it
proliferation of B and 1 cells
•
Donregulate or dampen immuneresponse
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Memory T-!ells
• %ave previously encountered
specific antigens
•
.espond in enhanced fashion onsu!se#uent eposures
• Induce secondary immune response
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Inflammation
• +ccurs in vasculari0ed tissue
• onspecific response to in7ury
•
.esponse is same regardless of agent thatinitiates it
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Inflammation 'hysiology
• 1riggered !y cellular in7ury
• In7ury activates mast cells
•
Mast cells release chemical mediators6 – %istamine
– %eparin
–
'eukotrienes (S.S-A) – *osinophil chemotactic factor
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7/18/2019 Immune Disorders
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Inflammation 'urposes
• &onfines in7urious agents
• Increases !lood cell, plasma
movement to in7ured areas• *nhances immune response
• Destroys in7urious agents
• $romotes healing
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Inflammation !ellular
!omponents• eutrophils
– $hagocytic cells
– *ngulf foreign material9organisms
– Arrive early
– Short-lived
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Inflammation !ellular
!omponents• Macrophages
– $hagocytic cells
– *ngulf foreign material9organisms
– Arrive later
– 'ong-lived
– 1ransfer antigens !ack to 12 cells
– %elp initiate immune response to specific
agents
7/18/2019 Immune Disorders
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Inflammation !ellular
!omponents
• *osinophils
–
Secrete caustic proteins – Dissolve surface mem!ranes of parasites
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Disorders of Immunity
7/18/2019 Immune Disorders
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Autoimmune Disease
• &linical disorder produced !y an
immune response to a normal tissue
component of a patient:s !ody
7/18/2019 Immune Disorders
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Autoimmune Diseases
• %yperthyroidism
• $rimary myedema
• 1ype I dia!etes• Addison:s disease
• Multiple sclerosis
• Myasthenia gravis
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Autoimmune Diseases
• .heumatic fever
• &rohn:s disease
•
;lcerative colitis• .heumatoid arthritis
• Systemic lupus erythematosis
7/18/2019 Immune Disorders
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Systemic "upus Erythematosis
• $rimarily occurs in <=- to 2=-year old females
• Also in children and older adults
• >=? of patients are female
• Autoimmune reaction to host DA
• Mortality after diagnosis averages @? per year
7/18/2019 Immune Disorders
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Systemic "upus Erythematosis
• Signs9Symptoms – acial rash9skin rash
triggered !y sunlight
eposure
– +ral9nasopharyngeal ulcers
– ever
–
Arthritis
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Systemic "upus Erythematosis
• Signs9Symptoms
– Serositis (pleurisy, pericarditis)
–
.enal in7ury9failure – &S involvement ith
sei0ures9psychosis
– $eripheral vasculitis9gangrene
– %emolytic anemia
7/18/2019 Immune Disorders
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Systemic "upus Erythematosis
• &hronic management
– Anti-inflammatory drugs
•
Aspirin• I!uprofen
• &orticosteroids
– Avoidance of emotional stress,
physical fatigue, ecessive sun
eposure
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Disorders of Immunity
Immunodeficiency Diseases
7/18/2019 Immune Disorders
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Immunodeficiency Diseases
• &ongenital
• Ac#uired
7/18/2019 Immune Disorders
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Immunodeficiency Diseases
!ongenital• B cell deficiency
– Agammaglo!ulinemia
– %ypogammaglo!ulinemia
7/18/2019 Immune Disorders
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Immunodeficiency Diseases
!ongenital• 1 cell deficiency
• IgA deficiency
• Severe com!ined immune deficiency
syndrome (B and 1 cell deficiency)
7/18/2019 Immune Disorders
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Immunodeficiency Diseases
Ac(uired• utritional deficiency
• Iatrogenic (drugs, radiation)
• 1rauma (prolonged hypoperfusion)
• Stress
•
Infection (AIDS)