The document discusses various types of immunodeficiency, hypersensitivity, and autoimmune disorders. It begins by defining the immune system and its subsystems. It then describes immunodeficiency disorders as primary, caused by genetic defects, or secondary, caused by illness, medications, or other factors. It classifies and explains various primary and secondary immunodeficiency disorders. The document also discusses hypersensitivity disorders like allergies and autoimmunity. It defines and describes different types of allergies and hypersensitivities like urticaria, anaphylaxis, and atopic disorders. It concludes by explaining specific conditions like contact dermatitis, atopic dermatitis, and others.

1. PRESENTED BY
MRS ARIFA T N
FIRST YEAR M.Sc NURSING
MIMS COLLEGE OF NURSING
IMMUNO DISORDERS

2. Introduction
 The immune system is the host defense system
comprising many biological structures and
processes with in an organism that protects
against disease
 The immune system can be classified into
subsystems such as
 Innate immune system v/s adaptive immune system
 Humoral immunity v/s cell mediated immunity

3. IMMUNODEFICIENCY
DISORDERS
 Immunodeficiency is an abnormal condition of the
immune system in which cellular or humoral immunity
is inadequate and resistance to infection is
decreased.
 First evidence of immunodeficiency is an increased
susceptibility to infection.
 Immunodeficiency disorders involve an impairment of
one or more of the following immune mechanisms:
 Phagocytosis
 Humoral response
 Cell-mediated response
 Complement
 Combined humoral and cell-mediated deficiency

4. Classified
Immunodeficiency
disorders
Hypersensitivity
disorders
Autoimmune
disorders

5. Immunodeficiency disorders
Immunodeficiency disorders are,
 primary if the immune cells are improperly
developed or absent, and
 Secondary if the deficiency is caused by
illnesses or treatment

6. Primary immunodeficiency
disorders
 The basic categories of primary
immunodeficiency disorders include the following:
 Phagocytic defects
 B-cell deficiency
 T-cell deficiency
 Combined B-cell and T-cell deficiency
 primary immune deficiencies are due to genetic
causes

7. Secondary immunodeficiency
disorders
 Drug-induced immunosuppression:
 Immunosuppressive therapy is prescribed for
patients to treat a wide variety of chronic diseases,
including inflammatory, allergic, hematologic,
neoplastic, and autoimmune disorders.
 Immunosuppressive therapy is also used
to prevent rejection of a transplanted
organ.
 HIV infections
 Stress may alter the immune response..
 A hypo functional immune system exists in
young children and older adults.
Immunoglobulin levels decrease with age and
therefore lead to a suppressed.

8. Secondary immunodeficiency
disorders
 The incidence of malignancies and
autoimmune diseases
 Malnutrition
 Radiation destroys lymphocytes either directly or
through depletion of stem cells.
 Surgical removal of lymph nodes, thymus, or
spleen can suppress the immune response
 Viruses, especially rubella, may cause
immunodeficiency by direct cytotoxic damage to
lymphoid cells

9. Diagnosis;
 Medical history; about any recurrent infections,
family history, any medications, etc.
 Physical examination
 White blood cell count
 T cell count
 Immunoglobulin levels
 Bone marrow studies

10. Management
Available treatment falls into two modalities
mainly:
 Treating the infections; cause (HIV) or
complications with antivirals/ antibiotics
 Boosting immune system;
 IV Ig transfusions
 Bone marrow (stem cell) transplant
 Prevention of infection is important in immune
deficiency disorders

11. HYPERSENSITIVITY
DISORDER

12. Hypersensitivity
 Hypersensitivity is characterized by an excessive
reaction to a particular stimulus
 It is an inappropriate and excessive response of
the immune system to a sensitizing antigen
 Host become sensitive to the allergen on first
exposure later on subsequent exposure exhibits
hypersensitivity reaction

13. Etiology
 Hypersensitivity disorders are believed to be
caused by genetic defect that allows increased
production of immunoglobulin E (IgE) with
release of histamine and other mediators from
mast cells and basophils.
 Exposure to antigen may occur by inhalation,
ingestion, injection, or touch (contact)

14. Signs and symptoms
 Histamine release causes
 Vasodilation
 Edema
 Bronchoconstriction
 mucus secretion
 Pruritus.
 Hypersensitivity reactions may be local
(gastrointestinal, skin, respiratory, conjunctival) or
systemic (anaphylaxis). The exact mechanism
and pathway of these inflammatory responses
are not clearly understood.

15. Factors influencing
hypersensitivity;
 Host response to allergen; the more sensitive, the
greater the allergic response
 Exposure amount; the more allergen the individual is
exposed to, the greater the chance of a severe
reaction
 Nature of the allergen; most allergic reactions are
precipitated by complex, high molecular weight
protein substances
 Route of allergen entry; most of the allergens enter
the body via gastrointestinal and respiratory routes.
Exposure to venoms through bites or stings and
injectable medications present a more severe threat
of allergic responses
 Repeated exposure; more often the individual is
exposed to the allergen, the greater the response

16. Types of hypersensitivity

20. Allergic disorders

21. Allergic disorders
 Two types Ig E mediated allergic reactions
 Atopic and non atopic disorders
(Underlying immunologic reactions of two
types are same and Predisposing and
manifestations are different)

22. Atopic disorders
 It is characterized by a hereditary
predisposition and production of local reaction
to IgE Antibodies which manifests as one or more
of the following three atopic disorders
 Allergic rhinitis
 Asthma
 Atopic dermatitis/eczema

23. Non atopic disorders
 They lack the genetic component and organ
specificity of the atopic disorders
Latex allergy
Contact dermatitis

24. ANAPHYLAXIS
 Anaphylaxis is a clinical response to an immediate
(type 1) hypersensitivity immunologic reaction
between a specific antigen and an antibody
 Rapid reaction of Ig E mediated chemicals
 Life threatening
 Caused by
 Foods
 Medications
 Insect stings
 Latex
 Antibiotics
 Radio contrast

25. Pathophysiology
Interaction of antigen with specific
IgE antibodies found on the surface
membrane of mast cells and
peripheral blood basophils
Subsequent release of histamine
and other bioactive mediators
>>>activation of platelets,
eosinophils and neutrophils
Histamines, prostaglandins and
inflammatory leukotrienes are potent
vasoactive mediators that are implicated in
the vascular permiability changes ,flusing
,urticaria,angioedema,hypotensionn and
bronchospasm

26. Pathophysiology
Smooth muscle spasm,
bronchospasm, mucosal
edema and inflammation and
increased capillary
permeability result
These changes
characteristically produce
clinical manifestations
within seconds or minutes
antigen exposure

27. Clinical manifestations
Mild (2hrs)
• Peripheral tingling
and sensation of
warmth
• Nasal congestion
• Swelling, pruritis,
sneezing and
tearing of eyes
Moderate (systemic)
• Flushing
• Anxiety
• Warmth
• Itching
• Bronchospasm
• Edema of the
airways or larynx
with dyspnea and
wheezing
Severe (systemic)
• Bronchospasm
• Laryngeal edema
• Severe dyspnea
• Cyanosis and
hypotension
• Dysphagia
• Abdominal
cramping
• Vomiting
• Diarrhea
• Seizure
• Cardiac arrest
• Coma

28. Prevention
 Strict avoidance of allergens
 Insect allergies
 Appropriate clothing
 Insect repellent
 Caution to avoid further stings
 Auto injector system of epinephrine
Epinephrin auto injector commercially available
Pre measured doses of
0.3 mg (Epipen)
0.15 mg (Epipen Jr )
 Medical identification bracelet
 Venom immunotherapy incase of allergic to insects
 Desensitization

30. Medical Management
 Depends on severity
 Cardiac arrest ………..CPR
 Oxygen therapy
 Epinephrine -s/c-1:1000 dilution followed by IV
infusion
 antihistamines and corticosteroids
 Iv fluids and volume expanders
 Aminophillin and corticosteroids
 Shift to ED to monitor for rebound or delayed
reaction 4-10 hrs
 Severe cases to be for 12-14 hrs

31. Nursing management
 Assess for signs and symptoms
 Assess AB and vital signs
 Observe for signs of increased edema and
respiratory problems
 Rapid measures
 Intubation
 Administration of emergency medications
 Insertion of IV lines
 Fluid administration
 Oxygen administration
 Patient teaching

32. Allergic rhinitis

33. Allergic rhinitis
Allergic rhinitis (hay fever, seasonal allergic
rhinitis)is the most common form of
respiratory allergy, which is presumed to be
mediated by an immediate (type 1
hypersensitivity)immunologic reaction and
is among the top 10 reason for visits to
primary provider

34. Pathophysiology
Ingestion or inhalation of antigen
Nasal mucosa reacted by slowing of ciliary
action, edema formation and leukocyte
infiltration
Tissue edema results from vasodilatation and
increased capillary permiablity

35. Clinical manifestations
 Sneezing and nasal congestion
 Clear and watery discharges
 Nasal itching
 Itching of the throat and palate
 Dry cough and hoarseness
 Headache and pain in paranasal sinuses
 Epistaxis
 Fatigue
 Loss of sleep
 Poor concentration

36. Assessment and diagnostic findings
 Nasal smears
 Peripheral blood counts
 Total serum IgE
 Epicutaneous and intradermal testing
 RAST
 Food elimination and challenge
 Nasal provocation test

37. Management of allergic rhinitis
(Medical)
 GOAL : relief from symptoms
Avoidance therapy
Pharmacologic therapy
Immunotherapy

38. • Remove the
allergens
• Environmental
controls
• Saline nasal
sprays
Avoidance
• Anti
histamines
• Decongestant
combination
• Adrenergic
agents
• Mast cell
stabilizers
• Corticosteroid
s
• Leukotriene
modifiers
Pharmacologic
• Allergen
desensitizatio
n
• Allergen
immunotherap
y
• Hyposensitizat
ion
• Vaccine
therapyImmunotherapy

39. Nursing management
 Assessment and history collection
 Intervention
 Improving breathing pattern
 Promoting understanding of allergy and allergy
control
 Coping with chronic disorders
 Monitoring and managing potential complications
 Promoting home and community based care
 Educating about self care
 Teach Patient on immunotherapy
 Antihistamines
 Continuing care

40. Contact dermatitis
 Contact dermatitis, a type of IV delayed
hypersensitivity reaction, is an adult or chronic
inflammation that results from direct skin contact
with chemicals or allergens
 4 basic types,
 Allergic
 Irritant
 Phototoxic
 Photoallergic
 8% cases are caused by excessive exposure to
or addictive effects of irritants
 Eg: soaps, detergents, organic solvents etc.

41. Clinical manifestations
 Itching Burning
 Erythema
 Skin lesions
 Edema
 Weeping
 Crusting
 Finally drying
 Peeling of skin
 Severe –hemorrhagic bullae
 Secondary invasions of bacteria may develop in skin
that is abraded by rubbing or scratching

42. Treatment
Allergic
• Avoidance of offending
material
• Aluminum acetate
Cool water compress
• Systemic
corticosteroids for 7-10
days
• Topical corticosteroids
• Oral antihistamines
relieve pruritus
Irritant
• Identify and remove
the source of irritation
• Application of
hydrophilic cream
• Topical corticosteroid
• Antibiotics

43. Treatment
Phototoxic
• Diagnosed by
photo patch test
• Management
same as allergic
and irritant
Photo allergic
• Diagnosed by
photo patch test
• Management
same as allergic
and irritant

44. Atopic dermatitis
 Atopic dermatitis is a type 1immediate
hypersensitivity disorder characterized by
inflammation and hypersensitivity
 Other terms
 Atopic eczema
 Atopic dermatatitis/eczema syndrome
 Include both allergic and non allergic dermatitis
 Now atopic dermatitis is commonly used word
 It affects 15-20% children and 1-3%in adult

45. Clinical manifestations
 Elevation of serum IgE
 Peripheral eosinophils
 Pruritus and hyperirritability of the skin
 Large amount of histamine in the skin
 Excessive dryness
 Pallor
 It is chronic with remission and exacerbation

46. Medical management
 Individualized treatment
 Wearing cotton fabrics and washing with mild
detergents
 Humidifying dry heat in winter
 Maintaining room temperature 20-22.2 C
 Using antihistamines (eg :-diphenyl hydramine)
 Avoiding animals,dust,spray and perfumes
 Keeping the skin moisturized by daily bath
 Topical corticosteroids and antibiotics
 Use of immunosuppressive agents (Eg :
Cyclosporine, Facrolymes )

47. Dermatitis medicamentosa (drug
reactions)
 It is type 2 hypersensitivity disorder, is the term
applied to skin rashes associated with certain
medications
 Drug reactions are sudden, intense
accompanied by systemic symptoms
 On discovery patients are warned that they have
hypersensitivity to the particular medications
 Should carry information identifying the
hypersensitivity
 Frequent assessment and prompt reporting is
essential
 Immediately stop the medication that found to be

48. Urticaria and angioneurotic
edema
 Urticaria (hives) is type I hypersensitivity allergic
reaction on the skin that is characterized by the
sudden appearance of pinkish, edematous
elevations that vary in size and shape, itch and
cause local; discomfort
 May affect any part of body , mucous membarane
larynx and GI tract
 May remains for minutes to hrs and days
…6weeks (chronic urticaria)

49.  Angioneurotic edema (ie,angioedema) involves
the deeper layers of the skin, resulting in more
diffuse, swelling rather than the discrete lesions
characteristics of hives .
 Reaction Covers the entire back
 Skin appears normal often have reddish hue
 Do not pit as usual edema
 Mostly seen in lips, eyelids, cheeks, hands, feet,
genitalia, tongue and GI tract
 Swelling occurs suddenly, in a few seconds or
minutes or slowly in 1-2 hours precedes by itching
and burning sensation

50.  More than one swelling may appear at one time
or one may develop while another is disappearing
 Some medications such as ACE inhibitors and
penicillin causes angioedema
 nurse should be aware about what medication is
patient taking and potential side effects

51. Hereditary angioedema
 hereditory angioedema is a rare,potentially life
threatening condition that affects approximately
1:5000 people
 it is inherited as as autosomal dominant trait
 Resembles allergic angioedema
 Clinical features.
 Edema to skin GI, or respiratory tract
 May precipitated by trauma or occurs spontaneously
 Skin : swelling, does not itch not accompanied by
urticaria
 GI: edema and abdominal pain
 Lasts for 1-4 days
 Attacks occasionally affects the subcutaneous and sub
mucosal tissues

52. Management
 Usually subsides within 3-4 days
 Observe for laryngeal obstruction may necessiate
tracheostomy
 Epinephrine , antihistamine and corticosteroids
are used for treatment

53. Cold urticaria
 Familial atypical cold urticaria (FACU)
 Acquired cold urticaria (ACU)
 (FACU): is an autosomal dominant
condition,inherited from one affected parent , and
symptoms usually begins at birth
 ACU: most frequently affects young adults
between 18-25 years of age and is commonly
associated with the more common physical
urticarias
 Cause is unknown /ideopathic

54.  Clinical features
 Breakout in hives when exposed to cold
 Prompted by exposure to cold weather and objects
 May last to 5-6 years
 Diagnosis :
 Physical testing; ice cube provoking testing
 The FACU can be precipitate by entering 4 degree
C room

55. Management
 Avoidance of cold stimuli
 Bed rest
 Warmth
 Corticosteroids
 Carry epipen for emergency care

56. Food allergy
 Food allergy is divided into two
 IgE mediated food allergy
 Non IgE mediated food allergy
 IgE mediated food allergy
 A type 1 hypersensitivity reaction occurs 2% of adult
population .
 Occurs with the people who have genetic pre
disposition combine with exposure to allergens
early life through the GI or respiratory or through the
nasal mucosa
 More than 170 identified

57.  Non IgE mediated food allergy
 T cell play the major role
 Any food can cause allergic symptoms
 Most common
 sea food
 Legumes
 Seeds
 Treenuts chocolate
 Millk etc
 Previous contamination of equipment
 Allergen may hidden in other foods

58.  Clinical features
 classical : urticaria dermatitis wheezing
cough laryngeal edema angioedema
 GI: itching swelling of lips tongue and palate
abdominal pain nausea cramps vomiting
diarrhea
 Assessment and diagnostic findings
 Detailed allergic history
 Physical examination
 Pertinent diagnostic tests
 Skin testing

59. Management
 Elimination of allergic foods
 Medications
 H –blockers
 Antihistamines
 Adrenergic agents
 Corticosteroids and
 Chromolyn sodium
 Epi en use
 Educating patient and family menbers

60. Latex allergy
 Latex allergy is the allergic reaction to natural
rubber proteins has been implicated in rhinitis,
conjunctivitis contact dermatitis, urticaria, asthma
and anaphylaxis
 Risk include
 Health care workers
 Patients
 People working in factory
 Females
 Foodhandlers
 Police etc
 8-12% workers latex sensitive

61.  Exposure can be cutaneous, mucosal and
parenteral and aerosol
 Clinical features
 Irritant contact dermatitis
 Erythema and pruritis
 Delayed hypersensitivity to latex
 Type 4 mediated by t cells
 Localized to the exposure
 Symptoms :Vesicular skin lesions , Papules , pruritis,
edema, crusting and thickening of skin
 Usually appears in the back of the hand
 Caused by chemicals used to prepare

62.  Immediate hypersensitivity
 Type 1 allergic reaction mediated by the Ig E mast
cell system
 Symptoms : rhinitis, conjunctivitis, asthma,
anaphylaxis
 Rapid onset: urticaria, wheezing , dyspnea,
laryngeal edema, bronchospasm, tachycardia

63. Management
 Wear medical alert identification
 Carry an epinephrine (adrenaline) auto-injector
for emergency treatment
 Health care workers with a history of latex
sensitivity who must wear gloves should stop
wearing latex gloves. Their co-workers should
also not use latex gloves, but rather switch to
synthetic gloves.
 Patients with latex allergy are at risk of asthma on
exposure to latex-containing aerosols. They
should try to avoid areas where powdered latex
gloves or other latex products are used

64. Autoimmune disorders

65. Autoimmune disorders
 Autoimmune disorders entail the development of an
immune response (autoantibodies or a cellular
immune response) to one’s own tissues, thus these
disorders are failures of the tolerance to “self”.
Autoimmune disorders may be described as an
immune attack on the self and result from the failure
to distinguish “self” protein (self-antigens) from
"foreign” protein.
 Autoimmune diseases tend to cluster, so that a given
person may have more than one autoimmune disease
(e.g., rheumatoid arthritis and Addison’s disease). The
same or related autoimmune diseases may be found
in other members of the family. This observation has
led to the concept of genetic predisposition to

66.  Diseases included are,
 Pernicious anemia
 Guillain-barré
syndrome
 Scleroderma
 Sjogren’s syndrome
 Rheumatic fever
 Rheumatoid arthritis
 Ulcerative colitis
 Male infertility
 Myasthenia gravis
 Multiple sclerosis
 Addison’s disease
 Autoimmune hemolytic
anemia
 Immune
thrombocytopenic
purpura
 Type 1 diabetes
mellitus
 Glomerulonephritis
 Systemic lupus
erythematosus. Etc.

67.  Ulcerative colitis,
 Male infertility,
 Myasthenia gravis,
 Multiple sclerosis,
 Addison’s disease,
 Autoimmune hemolytic anemia,
 Immune thrombocytopenic purpura,
 Type 1 diabetes mellitus,
 Glomerulonephritis, and
 Systemic lupus erythematosus. Etc.

68. Epidemiology
 Autoimmune diseases are becoming leading
causes of death among young and middle-aged
women in the United States.
 Incidence rates vary among the autoimmune
diseases, with estimates ranging from less than
one newly-diagnosed case of systemic sclerosis
to more than 20 cases of adult-onset rheumatoid
arthritis per 100 000 person-years.
 Prevalence rates range from less than 5 per 100
000 (e.g. chronic active hepatitis, uveitis) to more
than 500 per 100 000 (Grave disease,
rheumatoid arthritis, thyroiditis).

69.  At least 85% of thyroiditis, systemic sclerosis,
systemic lupus erythematosus, and Sjögren
disease patients are female.
 Although most diseases can occur at any age,
some diseases primarily occur in childhood and
adolescence (e.g. type 1 diabetes), in the mid-
adult years (e.g. myasthenia gravis, multiple
sclerosis), or among older adults (e.g. rheumatoid
arthritis, primary systemic vasculitis).

70. Pathophysiology
 The Pathophysiology of autoimmune responses
is not clearly understood. Environmental
factors, including toxic chemicals like
smoking, viral infections like Epstein-Barr virus,
and low vitamin D levels, dietary factors like
excessive salt have been found to trigger
autoimmune diseases in susceptible people.
Many illnesses are now believed to be in this
classification

71. Classification
 Broadly classified into 3 groups
1. Haemolytic autoimmune diseases
2. Localised autoimmune diseases
3. Systemic autoimmune diseases

72. Haemolytic autoimmune
diseases
 Clinical disorder due to destructions of blood
components. Auto Antibodies are formed against
one’s own RBCs, Platelets or Leucocytes.
 E.g. Haemolytic anemia, Leucopenia,
Thrombocytopenia, etc.

73. Systemic autoimmune diseases or
Non-organ specific autoimmune
diseases
 Immune complexes accumulate in many tissues
and cause inflammation and damage.
 Affects many organs or the whole body
 Eg :
 Systemic Lupus Erythematosus (anti-nuclear Ab.):
Harms kidneys, heart, brain, lungs, skin
 Rheumatoid Arthritis (anti-IgG antibodies): Joints,
hearts, lungs, nervous system
 Rheumatic fever: cross-reaction between antibodies
to streptococcus and auto-antibodies

74. Localised autoimmune diseases
or Organ specific autoimmune
diseases
 A particular organ is affected due to auto
Antibodies.
 Eg:
 Thyroiditis (attacks the thyroid)
 Multiple sclerosis (attacks myelin coating of nerve
axons)
 Myasthenia gravis (attacks nerve-muscle junction)
 Juvenile diabetes or Type I DM (attacks insulin-
producing cells)

75. Clinical features;
 Fatigue
 muscle pain
 Swelling and redness
 Low-grade fever
 Numbness and tingling in the hands and feet
 Hair loss
 Skin rashes
 Specific symptoms of system involved

76. Diagnostic studies
 Initial laboratory evaluation;
 CBC and ESR- SLE
 Specific organ enzymes- hepatitis
 Coagulation studies- antiphospholipid syndrome
 Calcium level- sarcoidosis
 Urinalysis- nephritis
 Inflammatory markers; ESR, CRP, etc.
 Auto-antibodies and Immunologic Studies; RF
factor, ANA, Anti ds DNA, complement,
immunoglobulins, lupus anticoagulant
autoantibodies, etc.

77. Diagnostic studies
 Cytokine studies; IL-1, IL-6 and TNF-alpha
 Flow cytometry;
Flow cytometry is a technique where particles
or tagged cells flow through laser light so that
populations of particle/cells can be counted and
phenotyped using cell characteristics and surface
proteins.Flow cytometry is crucial to determine
the quantitative number of immune cells (typically
T, B and NK (natural killer) cells)
 Human Leukocyte Antigen (HLA) typing
 Specific immunologic studies

78. Management
 Treatments can’t cure autoimmune diseases,
but they can control the overactive immune
response and bring down inflammation or at least
reduce pain and inflammation
 Nonsteroidal anti-inflammatory drugs
(nsaids), such as ibuprofen (Motrin, Advil) and
naproxen (Naprosyn)
 Immune -suppressing drugs
 Symptomatic management of pain, swelling,
fatigue and rashes

79. Management
 Life style modifications; diet, activity, habits,
etc.
 Plasmapheresis; removes anti-nuclear
antibodies and inflammatory mediators
 Rituximab; chimeric monoclonal antibody against
the protein CD20, which is primarily found on the
surface of immune system B cells. When it binds
to this protein it triggers cell death

80. Conclusion Immuno-
disorders

81. Thank you