DISORDERS OF THE IMMUNE SYSTEM

Disorders of the immune system fall into four main categories:

A. immunodeficiency disorders
• primary
• Acquired
• HIV (human immunodeficiency virus) infection/AIDS (acquired
immunodeficiency syndrome)
• Immunodeficiencies caused by medications
B. autoimmune disorders
• SLE

C. Allergic disorders
• Asthma
• Eczema
• allergies

D. cancers of the immune system

• Leukemia
• Lymphoma

AN OVERACTIVE IMMUNE SYSTEM/ HYPERSENSITIVITY

• If you are born with certain genes, your immune system may react to
substances in the environment that are normally harmless.

Hypersensitivity
• refers to undesirable reactions produced by the normal immune system,
including allergies and autoimmunity
• These reactions may be damaging, uncomfortable, or occasionally fatal

Classification /Types of Hypersensitivity reactions

Type I - Immediate, Atopic, Anaphylactic Reaction
Type II - Cytotoxic Reaction
TYPE III - Immune Complex Reaction
TYPE IV - Cell Mediated Reaction

Type I - Immediate, Atopic, Anaphylactic Reaction

• Characterized by vasodilation, increased permeability, and smooth
muscle contraction and eosinophilia

Anaphylactic Responses can be...

Localized Reactions
• hay fever, asthma, hives

Systemic Reactions
• shock and breathing difficulties

Clinical manifestations of a systemic anaphylactic reaction.

(Pic)

Localized Reactions
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B. Atopic Dermatitis (Eczena)

• Inflammation of the skin
Pathophysiology
contact with irritants to which a person has specific sensitivity
Characteristic
• acute or chronic
 • noncontagious inflammatory condition
• causes the typical wheal-and-flare reaction

Clinical manifestation
• Red oozing crusting rash
• Dry thick brownish-gred and scaly skin
• Pruritus
• Lesion are mostly found:
• Hand, foot
⁃ Back of the knees, neck, face eyelids and elbow bands
(Add missinv part)

C. ASTHMA
Is a chronic inflammatory disease of the airwys that causes airway hyper-
responsiveness, mucosal edema, and mucus production.

Predisposing Factors:
• Allergy
• Chronic Exposure to airway irritants or allergens

3 Major Signs
• Airway blockage
• Inflammation
• Airway irritability

Clinical Manifestations/Symptoms
• Cough
• Dypsnea
• Wheezing

Classification
• Mild intermittent asthma
• Mild persistent asthma
• Moderate persistent asthma
• Severe persistent asthma

RISK FACTORS
• Genes
• Race
• Lung Infection, allergies or obesity

Diagnostic Test
• Spirometry
• Peak flow
• Methacholine challenge
• Chest X- ray
• Allergy Test

Medical Management:
• Long-acting beta-agonists
• Combination inhaler
• Theophylline
• Short-acting beta-agonists
• Anticholinergics
• Oral and intravenous corticosteroids

Allergies reactions
(Pic)
PATHOPHYSIOLOGY OF AN ALLERGIC REACTION
(Pic)

Anaphylaxis
• Is an immediate life threatening systemic reaction that can occur on
exposure to a particular substances.

Causes:
• Food
• Medication
• Insect stings

Pathophysiology
(pic)

Signs and symptoms

(Pic)

Clinical manifestation
(Pic)

PREVENTION AND TREATMENT OF ANAPHYLAXIS

• Avoidance of potential allergens
• Avoid areas populated by insects
• Use appropriate clothing & insect repellent
• Auto injection system: EpiPen
• May follow with IV epinephrine
• IV fluids
• Screening for allergies before medication administration

Self-Administration of Epinephrine
Do’s and don’t
(Pic)

Inject the medicine into the fleshy outer portion of the thigh. Do not inject into
a vein or the buttocks.
Hold the leg firmly in place before and during the injection. You can give the
injection through clothes or on bare skin

For cardiac arrest:

1. CPR
2. 02 therapy
3. Epinephrine

Additional Management:
1. Antihistamine
2. Corticosteroids
3. Aminophylline

Systemic Anaphylaxis
• Due to widespread degranulation of mast cells after crosslinking of IgE
on the mast cell surface.

Severe Manifestations
• Airway obstruction and hypotension

Other signs
• Urticaria, Angioedema
Type II: Cytotoxic Reaction (Antibody-dependent)
• These allergic reactions are systemic or localized, as in allergic
dermatitis
with pic

Cytotoxic Hypersensitivity
• Antibodies involved: IgG and IgM
• Antigens: Cellular or Cell-bound
• Reactions involved antibodies directed to antigen on surface of
specific cells or tissues resulting to cytolysis (complement activation)

CLINICAL STATES
• Hemolytic Transfusion Reactions, (HTRs)
• Hemolytic Disease of the Newborn (HDN)
• Myasthenia Gravis

HEMOLY TIC TRANSFUSION REACTION (HTR)

• transfusion of ABO group-incompatible blood
• the most common cause is clerical error
⁃ Person who are transfused with the wrong blood type will produce anti-
hemagglutinins causing complement mediated lysis

Erythroblastosis fetalis
• A severe hemolytic disease of a fetus or newborn caused by production
of maternal antibodies for fetal red blood cells
• Most commonly develops when an Rh+ fetus expresses an Rh antigen on its
blood that an Rh-mother

Pathophysiology
(Pic)

Myasthenia Gravis
• is a rare chronic autoimmune neuromuscular disorder that causes
weakness in the skeletal muscles, which are the muscles your body uses for
movement.
• marked by muscular weakness without atrophy, and caused by a defect in
the action of acetylcholine at neuromuscular junctions
(Pic)

Predisposing Factor
Age
• Incidence of Myasthenia Gravis occurs between ages 15-35 for women.
Gender
• Myasthenia Gravis is three times more common in women than men.
Hereditary
• A person born into family with history of certain autoimmune diseases
is at risk of developing such autoimmune disease.

Precipitating Factor
Poor environment
• Constant exposure to dust or chemical substances can weaken the immune
system of every individual that lead to infection.
Stress
• Stress can weaken the immune system

Clinical manifestation
• Expressioniess face
• Nasal timbre
• Ptosis
 ⁃ Diplopia
⁃ Dysphagia
⁃ Dysarthria
⁃ Dysphonia
⁃ Weakness of all extremities

Diagnostic test
• Acetylcholine Receptor Antibody
• Tensilon ® test (edrophonium chloride)
• Anti-MuSK Antibody testing

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