PRACTICE TEACHING

ON
IMMUNE SYSTEM DISORDERS

Presented By-
Mr. Deepak Patel
M.Sc. Nursing
INTRODUCTION
Immune system disorders cause
abnormally low activity, over activity
or misguided activity of immune
system.
DEFINITION
An immune system disorder is a
dysfunction of the immune system. These
disorders can be characterized in several
different ways:
• By the components of immune system
affected.
• By whether the immune system is
overactive or underactive.
• By whether the condition is congenital or
acquired.
 ANATOMY AND PHYSIOLOGY OF
IMMUNE SYSTEM

IMMUNE SYSTEM
An immune system is a collection of
biological processes within an organism
that protects against disease by
identifying and killing pathogens and
tumor cells.
 ANATOMY AND PHYSIOLOGY OF
IMMUNE SYSTEM

IMMUNITY
Immunity is defined as the resistance
of an organism to infection, disease or
other unwanted biological invasion.
 CLASSIFICATION OF IMMUNITY

Innate
THE BODY’S DEFENCE MECHANISM
 MECHANISM OF INNATE
IMMUNITY
Epithelial surfaces Chemical secretions Cellular factors

• Skin • Oils & sweat • Monocytes

• GI tract • Lysozymes • Phagocytes
• Respiratory tract • Properdine, leukins, • Basophils
• Eyes beta lysine • Neutrophils
• Vaginal tract • HCL and bile • Eosinophil
• Cerumen wax • Macrophages
• Inflammatory
reaction
• Fever
• Interferon
• Natural killer cells
• Complement system
 MECHANISM OF ACQUIRED
IMMUNITY
• Defends against specific foreign
component.
• Based on principle of self & non-self.
• Mediated by antibodies.
CELLS OF IMMUNE SYSTEM
TYPES OF SPECIFIC IMMUNITY CELLS
TYPES OF IMMUNE CELLS
TYPES OF ANTIGEN PRESENTING
CELLS (APC)
TYPES OF ANTIGEN PRESENTING
CELLS (APC)
Professional and Non-Professional
APC
MAJOR HISTOCOMPATIBILITY COMPATIBILITY
COMPLEX MOLECULES (MHC) CLASS I & II
T-CELL AND B-CELL RECEPTORS
(TCR / BCR)
MAJOR HISTOCOMPATIBILITY COMPATIBILITY
COMPLEX MOLECULES (MHC) CLASS I & II
ACTIVATION OF T-CELL
CYTOTOXIC T-CELL
 ANATOMY AND PHYSIOLOGY OF
IMMUNE SYSTEM

ANTIGEN
Any foreign substance which when
enters the body, elicits body’s immune
response is called as antigen.
 ANATOMY AND PHYSIOLOGY OF
IMMUNE SYSTEM

ANTIGEN
Any foreign substance which when
enters the body, elicits body’s immune
response is called as antigen.
 ANATOMY AND PHYSIOLOGY OF
IMMUNE SYSTEM

ANTIGEN
Any foreign substance which when
enters the body, elicits body’s immune
response is called as antigen.
 ANATOMY AND PHYSIOLOGY OF
IMMUNE SYSTEM

ANTIBODY
The substance produced by the body in
response to an antigen are called as
antibodies. A specific antibody is
produced against a specific antigen to
defend the body.
STRUCTURE OF ANTIBODY
CLASSIFICATION OF IMMUNOLIC
DISORDERS

1. IMMUNODEFICIENCY

2. GAMMOPATHY

3. HYPERSENSITIVITY

4. AUTOIMMUNITY
 1. IMMUNODEFICIENCY

DEFINITION
“Immunodeficiency is a state in which
the immune system’s ability to fight
infectious disease and cancer is
compromised or entirely absent.”
 TYPES OF IMMUNODEFICIENCY

Primary immunodeficiency

• These disorders usually occur from intrinsic, inherited

defects in the immune system. They are usually seen in
infants and young children.

Secondary immunodeficiency

• Any factor that interferes with the normal growth or

expression of the immune system can lead to secondary
immunodeficiency. It generally develop later in life.
Immunosuppressive agents, chronic illness are common
cause.
 ETIOLOGY OF IMMUNODEFICIENCY
Primary immunodeficiency

• Primary immunodeficiency disorders may be caused by mutations,

sometimes in a specific gene. Mutation leads to deficiencies if immune
cells.
• e.g.- X-linked a-gamma-globuli-nemia (XLA), common variable
immunodeficiency (CVID), Severe combined immunodeficiency (SCID)

Secondary immunodeficiency

• Secondary immunodeficiency disorders happen when an outside source

like a toxic chemical or infection attacks the body.
• E.g.- severe burns, chemotherapy, radiation, Diabetes, malnutrition
• (examples of secondary immunodeficiency diseases- AIDS, cancers of
immune system, leukaemia, viral hepatitis, multiple myeloma)
 PATHOPHYSIOLOGY
Etiological factors such as genetic mutation or acquired factors- Toxins, Chemotherapy,
Radiation, Chronic illness, Nutritional deficiency

Abnormal or absent production of immune cells as B-cells, T-cells, phagocytic cells or

complement deficiencies.

Insufficient immune cells to fight against infection and tumour.

Recurrent infections, tumour may grow

Inability of the body to ward off infections leading health deterioration.

Immunodeficiency diseases
 CLINICAL MANIFESTATIONS
Opportunistic infections, frequent and recurrent pneumonia, bronchitis

Sinus infections, ear infections, meningitis and skin infections

Inflammation and infection of internal organs

Blood disorders, such as low platelet count or anemia

Digestive problems such as cramping, anorexia, nausea, diarrhoea

Delayed growth and development, recurrent fungal infections

Oral ulcers and conjunctivitis, skin and mucus membrane infections

 DIAGNOSTIC EVALUATION

History Physical Laboratory Bone marrow Histopathology

Genetic testing
taking examination tests biopsy study
 MANAGEMENT
1. MEDICAL MANAGEMENT
a. Managaing infections
• Preventing infections
• Treating infections
• Treating symptoms
b. Treatment to boost the immune system
• Immunoglobulin therapy- 400-500 mg/dl IM/IV monthly
• Interferon-gamma therapy
• Growth factors
2. SURGICAL MANAGEMENT
• Stem cell transplantation
 2. GAMMOPATHIES

DEFINITION
“Gammopathies, also termed
typergammaglobulinemias, are
elevated levels of gamma
globulin in serum resulting from
overproduction.”
 TYPES OF GAMMOPATHIES

Involves the overproduction of one

MONOCLONAL class of immunoglobulin's in
GAMMOPATHIES response to inappropriate antigenic
stimulation.

Involves the over production of

POLYCLONAL virtually all classes of
GAMMOPATHIES immunoglobulin's in response to
inappropriate antigenic stimulation.
 TYPES OF GAMMOPATHIES

Involves the overproduction of one

MONOCLONAL class of immunoglobulin's in
GAMMOPATHIES response to inappropriate antigenic
stimulation.

Involves the over production of

POLYCLONAL virtually all classes of
GAMMOPATHIES immunoglobulin's in response to
inappropriate antigenic stimulation.
 3. HYPERSENSITIVITY

DEFINITION
“A hypersensitivity reaction is defined
as the altered reactivity to a specific
antigen that results in pathologic
reactions upon the exposure of a
sensitized host to that specific
antigen.”
 TYPES
Hypersensitivity diseases are broadly divided into
five categories based on the immunologic
mechanism involved in the reactions.

1. TYPE-I (Immediate hypersensitivity)

2. TYPE-II (Antibody mediated hypersensitivity)

3. TYPE-III (Immune complex hypersensitivity disease)

4. TYPE-IV (T-Cell mediated or delayed hypersensitivity)

5. TYPE-V (Stimulatory Hypersensitivity)

 TYPE-I
Immediate hypersensitivity

Type-I hypersensitivity reaction is an allergic reaction

provoked by re-exposure to a specific type of antigen
referred to as an allergen. The reaction may be either local
or systemic. Symptoms vary from mild irritation to sudden
death from anaphylactic shock.
Exposure may be by ingestion, inhalation, injection or
direct contact. The naïve lymphocytes become primed and
differentiated into an antibody secreting cell and this leads
to class-switching of the antibody to the IgE class.
 TYPE-I
Immediate hypersensitivity

Some examples of Type-I hypersensitivity-

• Allergic asthma
• Allergic conjunctivitis
• Allergic rhinitis (Hay fever)
• Anaphylaxis
• Angioedema
• Atopic dermatitis (Eczema)
• Urticaria (Hives)
• Eosinophilia
 TYPE-II
Antibody mediated hypersensitivity

In Type-II hypersensitivity reactions, the antibodies

produced by the immune response bind to antigens on the
patient’s own cell surfaces. The antigens recognized in this
way may either be intrinsic or extrinsic . IgG and IgM
antibodies bind to these antigens to form complexes that
activate the classical pathway of compliment activation,
for eliminating cells presenting foreign antigens. That
mediates inflammation and cause cell lysis and death. This
reaction takes hours to day.
 TYPE-II
Antibody mediated hypersensitivity

Some examples of Type-II hypersensitivity-

• Autoimmune hemolytic anemia
• Erythroblastosis fetalis
• Pemphigus
• Pernicious anemia
• Transfusion reaction / ABO incompatibility
• Rheumatic fever
• Hemolytic disease of newborn
• Hashimoto’s thyroiditis
 TYPE-III
Immune complex hypersensitivity

In type-III hypersensitivity reactions, insoluble immune

complexes (aggregations of antigens and IgG and IgM
antibodies) form in the blood and are deposited in various
tissues (typically the skin, kidney and joints).
This deposition of the antibodies may trigger an immune
response according to the classical pathway of
complement activation- for eliminating cells presenting
foreign antigens. There are two stages relating to the
development of the complexes.
 TYPE-III
Immune complex hypersensitivity

Some examples of type-III hypersensitivity-

• Immune complex glomerulonephritis
• Serum sickness
• Rheumatoid arthritis
• Sub acute bacterial endocarditis
• Symptoms of malaria
• Systemic lupus erythematosus
• arthus reaction
• Farmer’s lung
 TYPE-IV
T-Cell Mediated / Delayed Hypersensitivity

Type-IV hypersensitivity reactions are often called delayed

type as the reaction takes two to three days to develop.
Unlike the other types, it is not antibody mediated but
rather it is a type of cell mediated response.
CD8+ cytotoxic T cells and CD4+ helper cells recognize
antigen in a complex with either Type-I or Type-II major
Histocompatibility complex (MHC-I/II). The antigen
presenting cells in this case are macrophages which
secrete IL-1 which stimulates the proliferation of further
CD4+ T cells.
 TYPE-IV
T-Cell Mediated / Delayed Hypersensitivity

Some examples of Type-IV hypersensitivity-

• Contact dermatitis (poison ivy rash)
• Temporal arthritis
• Symptoms of leprosy
• Symptoms of tuberculosis
• Transplant rejection / tissue graft rejection
• Coeliac disease
 TYPE-V
STIMULATORY HYPERSENSITIVITY

In Type-V stimulatory hypersensitivity, antibodies are

made against a particular hormone receptor on a hormone
producing cell. This leads to the overstimulation of those
hormone-producing cells.
An example is Graves’ disease where antibodies are made
against thyroid-stimulating hormone receptors of thyroid
cells. The binding of the antibodies to the TSH receptors
results in constant stimulation of the thyroid leading to
hyperthyroidism.
 ETIOLOGY

• Genetic factors
• Lack of certain enzymes
• Exposure to allergens
 Common allergens associated
with hypersensitivity

Proteins Plant Pollens Drugs

• Foreign serum • Rye grass • Penicillin

• Vaccines • Ragweed • Sulphonamides
• Virus and • Timothy grass • Local
bacteria • Birch trees anaesthetics
• Salicylates
 Common allergens associated
with hypersensitivity

Foods Insect Others

• Nuts • Bee venom • Mould

• Seafood • Wasp venom spores
• Eggs • Ant venom • Animal hair
• Peas, beans • Cockroach and dander
• milk calyx • Latex
• Dust • Snake
• Mites venom
PATHOPHYSIOLOGY
CLINICAL MANIFESTATIONS
 CLINICAL MANIFESTATIONS

• Check out leaflet

 DIGNOSTIC EVALUATION

HISTORY TAKING PHYSAICAL EXAMINATION

• History of allergic reaction • Rashes (location / colour)

• Family history of allergy • Mouth breathing
• Recent exposure to • Flaring nares
sensitizing agent • Difficulty hearing
• Changes in living, working
environment
• Characteristic of present
environment
• Symptoms experienced
• Alleviating factors
 DIGNOSTIC EVALUATION

DIGNOSTIC TEST FOR LABORATORY TEST

ALLERGENS

• Skin prick test • Complete blood count

• Intradermal test • Allergen specific IgE blood
• Radioallergosorbent testing tests
(RAST) • Histamine and tryptase test
• Provocation testing • Cytotoxic test
• Eosinophil count • Genetic testing
• Patch testing
• Oral food challenges
• Food elimination
 MANAGEMENT
Antihistamines- Benadryl, Allegra

Decongestant- Phenylephrine

Decongestant- Phenylephrine

Mast cell degranulation inhibitor- Cromolyn sodium inhibiter

Corticosteroids – Prednisolone, Dexamethasone

Adrenaline- Epinephrine

Aminophylline- Theophylline
 MANAGEMENT

Auto transfusion

Rh-Immunoglobin Administration

Protection from known allergen

4. AUTOIMMUNE DISEASES

DEFINITION
“Autoimmune diseases are a group of
disorders in which tissue injury is
caused by humoral or cell mediated
immune response to self tissues
(antigens).”
 CLASSIFICATION OF
AUTOIMMUNE DISORDERS
• Autoimmune disorders are grouped
into categories according to the body
part or tissue involved.

1. Organ
2. Non specific
organ
specific
 CLASSIFICATION OF
AUTOIMMUNE DISORDERS
1. Organ specific

Heart
• Autoimmune • Multiple sclerosis
haemolytic anemia • Guillain-Barr
• Rheumatic fever
• Idiopathic syndrome
thrombocytopenic
purpura

Central nervous
Blood
system
 CLASSIFICATION OF
AUTOIMMUNE DISORDERS
1. Organ specific

Endocrine system
• Myasthenia gravis • Uveitis
• Addison’s disease
• Autoimmune thyroiditis
• Grave’s disease
• Hypothyroidism
• Type-I DM
Muscle Eye
 CLASSIFICATION OF
AUTOIMMUNE DISORDERS
1. Organ specific

Kidneys
• Pernicious anemia • Pemphigus valgaris
• Ulcerative collitis • Glomerulonephritis • Psoriasis
• Good pasture’s
syndrome

Gastrointestinal
Skin
system
 CLASSIFICATION OF
AUTOIMMUNE DISORDERS
2. Non-Organ specific

Systemic lupus erythematous

Rheumatoid arthritis

Progressive systemic sclerosis

 ETIOLOGY

Some autoimmune diseases

Generally unknown runs in family
(GENETIC FACTORS)

Some autoimmune disorders

are known to triggered by Exposure to certain
viral or bacterial infections chemicals and deficiency of
certain enzymes may lead to
(RHEMATIC FEVER, autoimmune disease.
ARTHRITIS)
 PATHOPHYSIOLOGY

The various mechanisms of autoimmune diseases

are listed as follows-
1. Bypass of helper T-cell tolerance
2. Emergence of sequestered antigen
3. Imbalance of suppressor helper T-cell function
4. Microbial agents in autoimmunity
5. Molecular mimicry
6. Polyclonal lymphocyte activation
 PATHOPHYSIOLOGY

1. Bypass of helper T-cell tolerance-

Tolerance of CD4+ helper T-cell is critical to
the prevention of autoimmunity. Therefore
tolerance may be broken if the helper T-cell
is bypasses or substituted.
 PATHOPHYSIOLOGY

2. EMERGENCE OF SEQUESTERED ANTIGEN

Any antigen-self that is completely
sequestered during development is likely to
be viewed as foreign if introduced into
circulation, an immune response will
develop.
Spermatozoa, myelin basic protein and lens
crystalline fall into this categories of
antigen.
 PATHOPHYSIOLOGY

3. IMBALANCE OF SUPPRESSOR HELPER

T-cell function- A loss of suppressor T-cell
function will contribute to autoimmunity
and conversely, excessively T-cell help may
drive B-cells to extremely high levels of
autoantibody production.
 PATHOPHYSIOLOGY

4. MICROBIAL AGENTS IN AUTOIMMUNITY

Microbes may trigger autoimmune reactions in several
ways-
• First viral antigens and autoantigens may become
associated to form immunogenic units and bypass T-
cell tolerance.
• Second, some viruses (EBV) are non-specific,
polyclonal B-cell mitogens and may thus induce
formation of autoantibodies.
• Third, viral infection may result in loss of suppressor
T-cell function.
 PATHOPHYSIOLOGY

5. MOLECULAR MIMICRY
The infecting microorganisms may trigger an
antibody response by presenting the cross
reacting haptonic determinants in
association with their own carrier to which
helper T-cell are not tolerant. The antibody
so formed may then damage the tissue that
shares cross reacting determinants.
 PATHOPHYSIOLOGY

6. POLYCLONAL LYMPHOCYTE ACTIVATION

Several microorganisms and their products
are capable of causing polyclonal activation
of B-cells.
CLINICAL MANIFESTATIONS
 DIGNOSTIC EVALUATION

HISTORY TAKING PHYSAICAL EXAMINATION

• History of allergic reaction • Rashes (location / colour)

• Family history of genetic • Over growth of tissues
disorders, habits • Scaly patchy appearance
• Recent exposure to • Mouth breathing
sensitizing agent • Flaring nares
• Changes in living, working • Difficulty hearing
environment
• Characteristic of present
environment
• Symptoms experienced
• Alleviating factors
 DIGNOSTIC EVALUATION

LABORATORY TESTS OTHER PROCEDURES

• Antinuclear antibody tests • Lumber puncture

• Autoantibody test • Bone marrow biopsy
• CBC
• C-reactive protein (CRP)
• Erythrocyte sedimentation
rate (ESR)
 MANAGEMENT
MEDICAL MANAGEMENT
SYSTEMIC CYTOTOXIC DRUGS ANALGESICS
CORTICOSTEROIDS Cyclophosphamide Ibuprofen
Initial dose of 60 Azathioprine
mg prednisolone
methotrexate
 NURSING MANAGEMENT OF
IMMUNE SYSTEM DISORDERS
NURSING ASSESSMENT
1. Review record of history of risk factors, constitutional
signs and symptoms recent infections, positive blood
test of immune disorders.
2. Assess and monitor nutritional status, weight, history
of weight loss and serum albumin.
3. Assess immunization status.
4. Investigate the use of medications.
5. Inspect vital signs, mouth for lesions, skin for rash,
bowel pattern, presence of pain and weakness.
 NURSING DIAGNOSIS
1. Risk for infection related to immunodeficiency,
neutropenia secondary to medications.
Goal- To prevent infection.
Interventions-
a. Administer prescribed medications.
b. Assess the systems thoroughly and notify
physician if abnormal.
c. Maintain cleanliness of the environment .
d. Employ aseptic techniques when performing
invasive procedure.
e. Instruct visitors to wash hands before and after
meeting patients.
 NURSING DIAGNOSIS
2. Imbalanced nutrition less than body requirements
related to anoxia, secondary to infection.
Goal- To maintain proper nutrition.
Interventions-
a. Monitor nutritional status by daily weighing and
anthropometric measurement.
b. Consult with dietician to develop strategies for
nutritional care.
c. Encourage small, frequent meals as these may
make best use of limited absorption capacity.
d. Continue monitoring weight.
e. After timing of medications to improve intake of
meals.
 NURSING DIAGNOSIS
3. Impaired oral mucus membrane related to
opportunistic infections secondary to reduced
immune function.
Goal- To maintain skin integrity.
Interventions-
a. Ask about persistent sore throat dysphasia, heart
burn all these symptoms are suggestive or oral
oesophageal candidiasis.
b. Examine mouth for oral candidiasis, a harbour of
infection.
c. After prescribe mouth rinse and antifungal
agents.
d. Provide patient with the soft diet.
 NURSING DIAGNOSIS
4. Impaired gas exchange related to leukocytes
infiltration of systemic tissue secondary to decreased
mature RBC.
Goal- To maintain adequate oxygenation.
Interventions-
a. Maintain adequate oxygenation and perfusion.
b. Administer nebulization.
c. Watch for sudden change in respiratory functions.
d. Provide bronchodilators.
e. Encourage smoking cessation.
f. Administer medications as prescribed by the
doctor.
 NURSING DIAGNOSIS
5. Fatigue and activity intolerance related to
anaphylaxis.
Goal- To relieve fatigue and maintain well-being.
Interventions-
a. Evaluate the patients description of fatigue.
b. Determine the possible causes of fatigue.
c. Restrict environmental stimuli.
d. Encourage the patient to maintain a 24 hour
fatigue or activity log for at least 1 week.
e. Teach energy conservation methods collaborate
with occupational therapist.
 COMPLICATION OF IMMUNE
DISORDER
• Recurrent infections
• Autoimmune disorders
• Damage to heart lungs, nervous system,
digestive system
• Slowed growth
• Increased risk of cancer
• Death from serious infection, anaphylactic
shock
• Rheumatic heart disease
• Heart failure
SUMMARY
CONCLUSION