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Allergy & Immunology Flashcards

1
Q

What is Anaphylaxis?

A
  • Acute systemic IgE-mediated reaction
  • Antigen binding to IgE on the surface of mast cells & basophils
  • Release of potent mediators
    • Vascular tone
    • Bronchial reactivity
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2
Q

What is the etiology of anaphylaxis?

A
  • Drugs
  • Insect venom
  • Foods
  • Latex
  • Biologic agents
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3
Q

What are the clinical features of anaphylaxis?

A
  • Pruritis, flushing, urticaria, angioedema
  • Dyspnea, wheezing
  • Nausea, vomiting, diarrhea, crampy abd pain
  • CV symptoms (mild hypotension to shock)
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4
Q

How is anaphylaxis diagnosed?

How is it treated?

A
  • Clinical signs & symptoms that appear w/i 30 min
  • Principal treatment: Epinephrine
    • Acute resp & CV complications
  • Systemic antihistamines, corticosteroids, ß-adrenergic agonists
    • Treat signs & symptoms
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5
Q

What are the 8 allergic conditions of childhood?

A
  • Allergic asthma
  • Anaphylaxis
  • Allergic rhinitis
  • Atopic dermatitis
  • Food allergies
  • Urticaria
  • Drug allergies
  • Insect venom allergy
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6
Q

Allergic rhinitis

definition

epidemiology

etiology

A
  • IgE-mediated inflammatory response in the nasal mucosa to inhaled antigens
  • 10-20% of children
  • Seasonal or perennial
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7
Q

Seasonal rhinitis vs. Perennial rhinitis

A
  • Seasonal rhinitis
    • Tree, grass, wood pollens
    • Grass in spring, ragweed in fall
  • Perennial rhinitis
    • Indoor allergens
    • Dust mites, animal dander
    • Molds (high-humidity)
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8
Q

What is the pathophysiology of Allergic Rhinitis?

A
  • Sensitization to airborne allergens induces IgE
  • Allergen-specific IgE binds to receptors on mast cells & basophils in the nasal mucosa
  • Subsequent exposure produces an IgE-mediated inflammatory response (minutes)
  • Mast cells degranulate
    • Histamine, leukotrienes, kinins, PGs
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9
Q

What are the signs & symptoms of allerigic rhinitis?

A
  • Sneezing, nasal congestion, rhinorrhea, nasal itching, pale nasal mucosa
  • Allergic shiners
    • Dark circles under the eyes
    • Caused by venous congestion
  • Dennie’s lines
    • Creases under the eyes
    • Result of chronic edema
  • Allergic salute
    • When patient uses the palm of the hand to elevate the tip of the nose to relieve itching
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10
Q

Allergic rhinitis is commonly associated with…….

A
  • Asthma
  • Chronic sinusitis
  • Otitis media w/ effusion
  • Nasal polyps
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11
Q

What medical history contributes to the diagnosis of allergic rhinitis?

A
  • Multiple episodes of otitis media
  • Sinusitis
  • Atopic dermatitis (eczema)
  • Food or drug allergies
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12
Q

What laboratory evaluation contributes to the diagnosis of allergic rhinitis?

A
  • Elevated total IgE concentration
  • Allergen skin testing (prick/intradermal)
    • Skin tests are the most effective
    • Discontinue antihistamines 4-7 days before
  • Nasal smear for cytology
    • >10% eosinophils
    • Preponderance of polymorphic leukocytes suggests infection
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13
Q

What are the 5 steps of managing allergic rhinitis?

A
  • Allergen avoidance
  • Pharmacotherapy
  • Immunotherapy
  • Patient education
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14
Q

What are some allergen avoidance measures?

A

IgE Ab production may decrease

  • Child’s bedroom should be free of allergens
  • Remove pets or keep outdoors
  • Dust mite control
    • Plastic mattress covers
    • Removal of carpets/stuffed animals
  • Reduce humidity (dust mites, mold)
  • Avoid open windows
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15
Q

What types of pharmacotherapy are used to treat allergic rhinitis?

A
  • Intranasal steroids
    • Most effective
    • SE: local irritation
  • Anti-histamines
    • 1st generation are first line
      • Diphenhydramine
      • SE: sedation, impair academic performance
    • 2nd generation
      • Cetirizine, fexofenadine, loratadine
      • Safer & better tolerated
      • No more effective
    • Intranasal may be effective
  • Intranasal cromolyn sodium
    • Prevents mast cell degranulation
  • Decongestants
    • Pseudoephedrine
    • Vasoconstriction, relieve congestion
    • SE: insomnia, nervousness, rebound rhinitis, decongestants
  • Leukotriene receptor antagonists
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16
Q

How/why is immunotherapy used for allergic rhinitis?

A
  • Effective for allergic rhinitis, allergic asthma, insect venom allergy
  • Principle: repeated injections of allergens w/ time lead to better tolerance of the allergen by the patient
  • Indications
    • Other therapy is ineffective in controlling symptoms
    • Environmental controls have been tried & failed, or exposure is unavoidable
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17
Q

What is Atopic Dermatitis?

A
  • Chronic inflammatory dermatitis (eczema)
  • Dry skin & lichenification (thickening of skin)
  • Pruritis leads to scratching
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18
Q

Atopic dermatitis

prevalence

seasonal changes

family history

A
  • 5-8% of children
  • Begins in early infancy (85% <5 YO)
  • Worse in winter or w/ extremes of temp
  • Family members w/ atopic dermatitis, asthma, other allergic diseases
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19
Q

In atopic dermatitis, _______ is universal.

A

pruritis

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20
Q

What are the skin manifestations of atopic dermatitis?

A
  • Acute changes
    • Erythema
    • Weeping & crusting
    • Secondary bacterial (Staph aureus) or vial (HSV) infection
  • Chronic changes
    • Lichenification
    • Dry scaly skin
    • Pigmentary changes (hyper)
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21
Q

Clinical presentation of atopic dermatitis

  • Infantile form
  • Early childhood
  • Late childhood
A
  • Infantile form
    • Truncal & facial areas, scalp
    • Extensor surfaces
  • Early childhood
    • Flexural surfaces
    • Lichenification, chronic itching
  • Late childhood
    • Localized disease, remission
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22
Q

What are the major & minor criteria for diagnosis of atopic dermatitis?

A
  • 3 of 4 major criteria
    • Pruritis
    • Personal/family hx of atopy
    • Typical morphology/distribution
    • Relapsing or chronic dermatitis
  • Minor criteria
    • Xerosis (abnormal dryness)
    • Pruritis w/ sweating
    • Wool intolerance
    • Dermatographism: stroking of the skin w/ a dull instrument that produces a pale wheal w/ a red flare
    • Skin infections
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23
Q

How is atopic dermatitis managed?

A
  • Avoid known triggers
    • Wool, foods (egg, milk, peanuts), excessive heat/cold, harsh chemicals/soaps
  • Low-medium potency corticosteroids
    • Affected areas except the face
    • Systemic in severe cases
  • Antihistamines
    • Bedtime, reduce itch-scratch cycle
  • Baths
    • Tepid water, blot skin dry, lubricate
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24
Q

Food allergies

definition

etiology

A
  • IgE-mediated response to food antigens
  • Most allergic rxns to food (85-90%) caused by egg, milk, peanut, soy, wheat, fish
  • Exclusive breastfeeding for 6 mo may reduce food allergies/atopic dermatitis in the infant
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25
Q

What are the 6 clinical features of food allergies?

A
  • Oral symptoms
    • Itching/swelling of lips, tongue, throat
  • GI symptoms
    • N/V, diarrhea, abd pain
  • Respiratory symptoms
    • Nasal congestion, rhinorrhea, sneezing, wheezing
  • Atopic dermatitis
  • Acute urticaria & angioedema
  • Anaphylaxis
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26
Q

How are food allergies diagnosed?

A
  • History (type, timing, severity of symptoms)
  • Labs
    • Skin tests
      • IgE mediated hypersensitivity
    • Radioallergosorbent (RAST) tests
      • IgE Ab to specific food antigens
  • Provocative oral food challenge
    • Double-blind placebo-controlled
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27
Q

How are food allergies managed?

A
  • Strict avoidance of the responsible food allergen
  • Injectable epinephrine
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28
Q

Insect venom allergy

definition

etiology

clinical features

A
  • IgE-mediated response to the venom of stinging or biting insects
  • Venom of many insects (yellow jackets, hornets, wasps, bees, fire ants)
  • Localized erythema & swelling
  • Urticaria or anaphylaxis
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29
Q

How are insect venom allergies managed?

A
  • Local skin reactions
    • Cold compresses, analgesics, antihistamines
  • Diffuse urticaria
    • Antihistamines, systemic steroids
  • Anaphylaxis (previous section)
  • Immunotherapy
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30
Q

What is the definition of urticaria?

A

Hives

  • Circumscribed, raised, evanescent (vanishing) areas of edema that are almost always pruritic
  • Symmetric & migratory
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31
Q

What are the acute causes of urticaria?

A
  • Drugs (penicillin, aspirin, NSAIDs)
  • Foods & food additives
    • Eggs, shellfish, milk, nuts
  • Contactants (animal dander, latex)
  • Idiopathic
  • Infection
    • GABHS, infectious mono, myoplasma pneumoniae, hepatitis, coxsackie virus
  • Insect venoms
  • Transfusion rxn
  • Heat & cold
  • Skin pressure
  • Exercise
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32
Q

What are the chronic causes of urticaria?

A
  • Malignancy
  • Rheumatologic disease
    • SLE, RA
  • IgG Ab to IgE receptors
  • Idiopathic
  • Thyroid disease
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33
Q

Acute vs. chronic urticaria

A
  • Acute
    • Healthcare workers & patients w/ myelomingocele (exposed to latex due to repeated urinary catheterization) are at a risk for latex allergy
  • Chronic (>6 mo)
    • Underlying conditions
    • IgG Ab to IgE receptor
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34
Q

How is urticaria managed?

A
  • Precipitating factor should be avoided
  • Antihistamines are mainstay of therapy
  • Further evaluation for underlying systemic dz
    • w/ fever, arthralgias, weight loss, abd pain
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35
Q

Reactions to drugs are mediated by ______.

A

IgE or by direct mast cell degranulation

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36
Q

What is the etiology of drug allergies?

A
  • Many pharm agents documented
  • Most common
    • Penicillin
    • Sulfonamides
    • Cephalosporins
    • Aspirin, NSAIDs
    • Narcotics
37
Q

How are drug allergies managed?

A
  • Antihistamines
  • Anaphylaxis (previous section)
  • Medic alert bracelets
38
Q

What are the innate responses of the immune system?

A
  • Phagocytic cells
  • NK cells
  • TLRs
  • MBP
  • Alternative pathway of complement
39
Q

What are the adaptive responses of the immune system?

A
  • T cells
  • B cells
  • Immunoglobulin molecules
40
Q

Primary immunodeficiency states

A
  • B-cell defects (disorders of humoral immunity)
  • T-cell defects (disorders of cell-mediated immunity)
  • Disorders of granulocytes
  • Complement deficiencies
41
Q

Secondary immunodeficiency states

A
  • AIDS
  • Meds (steroids, chemo)
  • Malnutrition
  • Nephrotic syndrome
42
Q

What are the diagnostic tests to evaluate humoral immunity?

A
  • Quantitative immunoglobulins
  • B-cell enumeration
  • Ab titers to immunization (diphtheria, tetanus)
  • Isohemagglutinin titers
43
Q

What are the diagnostic tests to evaluate cell-mediated immunity?

A
  • Peripheral smear (look for lymphopenia)
  • Anergy panel (delayed type hypersensitivity)
  • T-cell subsets (CD3, CD4, CD8)
  • In vitro T-cell proliferative responses to mitogens & antigens
44
Q

What are the diagnostic tests to evaluate phagocyte function?

A
  • Peripheral smear (look for neutropenia)
  • Nitroblue tetrazolium test
  • Measurements of neutrophil chemotaxis
45
Q

What are the diagnostic tests to evaluate complement?

A
  • Total hemolytic complement (CH50)
  • Assays of specific components of complement
46
Q

75% of all primary immunodeficiency diseases involve abnormalities of ________.

A

Ab concentration or function

47
Q

What are the 7 disorders of lymphocytes?

A
  • IgA deficiency
  • Common variable immunodeficiency
  • Severe combined immunodeficiency disease (SCID)
  • Ataxia telangiectasia
  • DiGeorge syndrome
  • Wiskott-Aldrich syndrome
  • X-linked (Bruton’s) agammaglobulinemia
48
Q

What is IgA deficiency?

A
  • Serum IgA concentrations <7 mg/dL
  • Normal levels of other immunoglobulins
  • 50% have IgE deficiency
  • 20-30% have IgG2 & IgG4 subclass deficiencies
49
Q

What is the epidemiology & etiology of IgA deficiency?

A
  • IgA is the most common immune deficiency
    • 1/500-1,000
  • Genetic basis sometimes present, usually unclear
50
Q

What are the 4 clinical features of IgA deficiency?

A
  • Respiratory infections
    • Sinusitis, pneumonia, otitis media, bronchitis
  • GI manifestations
    • Chronic diarrhea, Giardia lamblia
  • Autoimmune & rheumatic diseases
    • SLE, JRA, celiac disease
  • Atopic diseases (50%)
    • Allergic rhinitis, eczema, urticaria, asthma
51
Q

IgA deficiency

diagnosis

management

A
  • Quantitative measurement of serum immunoglobulins (<7 mg/dL)
  • Management of infections & other complications
  • IVIG NOT indicated (almost all IgG)
52
Q

What is Common Variable Immunodeficiency (CVID)?

Prevalence?

Etiology?

A
  • Hypogammaglobulinemia
  • 1 in 10,000-100,000
  • Variety of defects in B-cell function or B-cell & T-cell interaction
53
Q

What are the 4 clinical features of CVID?

A
  • Respiratory infections
    • H. influenzae
    • Moraxella catarrhalis
    • Strep pneumoniae
  • GI infections
    • Giardia lamblia
    • Campylobacter jejuni
  • Autoimmune disorders
    • RA, autoimmune thyroiditis, autoimmune thrombocytopenia, autoimmune hemolytic anemia
  • Increased risk of malignancy
54
Q

How is CVID diagnosed?

A
  • Quantitative immunoglobulin measurement
    • Decreased
  • Diminished Ab function
    • Measure titers in response to vaccines
  • T-cell proliferation to mitogens (nonspecific stimulators of lymphocytic production) may be diminished
55
Q

How is CVID managed?

A
  • Monthly IVIG replacement
  • Aggressive management of infections w/ abx
  • Chronic diarrhea management
    • Nutritional support
56
Q

What is Severe Combined Immunodeficiency Disease (SCID)?

What is the etiology?

A
  • Defective T-cell & B-cell function
  • X-linked SCID
    • Deficiency in common gamma chain of the receptor for IL-2, IL-4, IL-7, IL-9, IL-15
    • 50% of all SCID cases
  • Autosomal recessive SCID
    • Genetic defects of T-cell ontogeny/function
    • Adenosine deaminase deficiency (30%)
57
Q

What are the clinical features of SCID?

A
  • Infection w/i the first few mo of life
    • Candida albicans
    • Pneumocystis carinii
  • Chronic diarrhea
  • Failure to thrive
58
Q

What are the 4 components of diagnosing SCID?

A
  • Persistent lymphopenia
    • <1,500 lymphocytes/mL
  • Enumeration of lymphocyte populations
    • Flow cytometry, decreased T cells
  • Quantitative measurement of serum Ig
    • Severe hypogammaglobulinemia
  • T-cell responses to mitogens & antigens
    • Severely depressed
59
Q

How is SCID managed?

A
  • Supportive care (abx, nutrition, psych)
  • Blood products should be irradiated (GVHD)
  • Monthly IVIG replacement (normal serum IgG)
  • P. carinii pneumonia (PCP) prophylaxis
    • TMP-SMX
  • Bone marrow transplant curative
    • GVHD, infection, med toxicity
    • Cord blood stem cell or peripheral blood stem cell transplant (alternative)
  • Gene therapy (future)
60
Q

Ataxia telangiectasia

definition

etiology

A
  • Autosomal recessive disorder
  • Characterized by:
    • Combined immunodeficiency
    • Cerebellar ataxia
    • Oculocutaneous telangiectasias
    • Predisposition to malignancy
  • Mutation of gene on long arm of chr 11
    • Cell cycle control
    • DNA recombination
    • Cellular responses to DNA damage
61
Q

What are the 4 main clinical features of ataxia telangiectasia?

A
  • Variable immunodeficiency
    • Chronic sinopulmonary infections
  • Severe progressive cerebellar ataxia
    • Wheelchair in early adolescence
  • Telangiectasias
    • Bulbar conjunctiva (2-5 YO)
    • Later on exposed skin, areas of trauma
  • High risk of malignancy
    • Defects in DNA repair
    • Lymphoma, carcinoma
62
Q

Additional clinical features of ataxia telangiectasia?

A
  • Cafe-au-lait spots
  • Vitiligo
  • Prematurely gray hair
  • Multiple endocrine abnormalities
63
Q

How is ataxia telangiectasia diagnosed?

A
  • Quantitative measurement of serum Ig
    • IgE deficiency (85%)
    • IgA deficiency (75%)
  • Evaluation of T-cell function
    • Skin test anergy
    • Diminished T-cell proliferation to mitogens
64
Q

How is ataxia telangiectasia managed?

A
  • Treat the neurologic complications
  • Aggressively treat infections
  • Monitor for malignancies
  • Avoid ionizing radiation
    • Exacerbates DNA breakage & repair
    • Increases risk of malignancy
65
Q

What is DiGeorge syndrome?

A
  • Congenital immunodeficiency syndrome
  • Submicroscopic deletion on chr arm 22q11
  • CATCH-22
    • Cardiac defects
    • Abnormal facies
    • Thymic hypoplasia
    • Cleft palate
    • Hypocalcemia
66
Q

What is Wiskott-Aldrich syndrome?

A
  • X-linked disorder
  • Characterized by:
    • Combined immunodeficiency
    • Eczema
    • Congenital thrombocytopenia
      • Small platelets
67
Q

What is the etiology of Wiskott-Aldrich syndrome?

A
  • Mutation of gene on short arm of X chromosome
  • Gene product important in T-cell receptor signaling & cytoskeletal organization
68
Q

What are the 3 clinical features of Wiskott Aldrich syndrome?

A
  • Susceptibility to infections w/ encapsulated organisms
    • H. influenzae, S. pneumonaie
    • No Ab to polysaccharide antigens
  • Thrombocytopenia
    • Small defective platelets
    • Frequent bleeding episodes
    • Risk of intracranial hemorrhage
  • Eczema
69
Q

How is Wiskott Aldrich syndrome diagnosed?

A
  • CBC
    • Thrombocytopenia, small platelets
  • Decreased IgM
  • Ab response to polysaccharide An defective
  • Cellular immune function defective
    • Anergy present
    • Near normal # T-cells but respond poorly
    • Don’t develop antigen-specific cytotoxic T-cells
70
Q

How is Wiskott Aldrich syndrome managed?

A
  • HLA-matched bone marrow transplantation
  • IVIG for hypogammaglobulinemia
  • Splenectomy cures thrombocytopenia (>90%)
    • Prophylactic abx or IVIG afterwards
71
Q

What is X-linked (Bruton’s) Agammaglobulinemia?

A
  • Severe hypogammaglobulinemia
  • Paucity of mature B cells
    • <1% B cells in peripheral blood
  • Normal T-cell number & function
72
Q

What is the etiology of Bruton’s Agammaglobulinemia?

A
  • Mutations in the BTK gene on X chr
    • Critical to normal B-cell otogeny
  • Mutations lead to block in development from pre-B cell to mature B-cell
73
Q

What are the clinical features of Bruton’s Agammaglobulinemia?

A

Increased susceptibility to infections w/ encapsulated bacteria (S. pneumoniae, H. influenzae), S. aureus & chronic enteroviral infection

74
Q

How is Bruton’s Agammaglobulinemia diagnosed?

Management?

A
  • Quantitative immunoglobulin
  • B cells absent/diminished
  • T cells present, cell-mediated functions ok
  • Mutations in BTK gene
  • Treatment: monthly IVIG replacement
    • Prevents infection
75
Q

What are the 3 disorders of granulocytes?

A
  • Chronic granulomatous disease
  • Schwachman-Diamond syndrome
  • Chediak-Higashi syndrome
76
Q

What is Chronic Granulomatous Disease (CGD)?

What is the etiology?

A
  • Defective neutrophil oxidative metabolism
  • Defects in multi-component reduced NADPH oxidase system
  • Severely impaired intracellular killing of catalase+ bacteria & some fungal pathogens
  • Inheritance: X-linked (70%)
77
Q

What are the clinical features of CGD?

A
  • Increased susceptibility to infections
    • Lungs, lymph nodes, liver, spleen, bones, skin
    • Abscess formation is characteristic
  • Major pathogens
    • S. aureus, P. aeruginosa, Salmonella, Klebsiella pneumoniae, Serratia marcescens, E. coli, C. albicans, Aspergillus
78
Q

How is CGD diagnosed?

A
  • Demonstration of defective neutrophil oxidative burst
  • Nitroblue tetrazolium (NBT) test
  • Flow cytometric assay
79
Q

Management of CGD?

A
  • Abscesses require surgical drainage/abx
  • Prophylactic TMP/SMX reduces infections
  • Prophylactic itraconazole (Aspergillus)
  • Interferon-gamma
  • Bone marrow transplant is curative
  • Gene therapy is a future cure
80
Q

Schwachman-Diamond Syndrome

  • definition
  • inheritance
  • clinical presentation
A
  • Autosomal recessive
  • Characterized by:
    • Decreased neutrophil chemotaxis
    • Cyclic neutropenia
    • Pancreatic exocrine insufficiency
  • Clinical presentation
    • Recurrent soft tissue infection
    • Chronic diarrhea
    • Failure to thrive
81
Q

Chediak-Higashi Syndrome

  • definition
  • clinical presentation
A
  • Neutropenia & thrombocytopenia
  • Giant lysosomal granules in neutrophils
  • Neutrophils & monocytes have functional defects
  • NK cell function impaired
  • Majority of infections: S. aureus
  • Clinical: partial oculocutaneous albinism
82
Q

The complement system is composed of……

A
  • Plasma proteins & cellular receptors
  • Intregrated series of rxns to prevent infection
83
Q

Disorders of the Complement System

definition

etiology

A
  • Absence/dysfunction of individual complement components or regulatory proteins
  • Genetically determined (autosomal recessive)
84
Q

Clinical features of deficiencies of the early components of the classic pathway (C1q, C2, C4)

A
  • Autoimmune diseases
  • Example: SLE
85
Q

Clinical features of the late components of the classic pathway (C5, C6, C8)

A

Increased susceptibility to disseminated meningococcal & gonococcal infections

86
Q

Clinical features of deficiency/dysfunction of C1 esterase inhibitor

A
  • Hereditary angioedema
  • Episodic swelling of various body parts
    • Hands & feet
  • Bowel wall swelling
    • Severe abdominal pain
  • Angioedema affecting the airway
    • FATAL
87
Q

How are disorders of the complement system diagnosed?

A
  • Quantitative: specific assays
  • Qualitative: total serum hemolytic complement (CH50)
88
Q

How are disorders of the complement system managed?

A
  • Prompt diagnosis & treatment of bacterial infections
  • Management of autoimmune disease
  • Therapy w/ fibrinolysis inhibitors & attenuated androgens (danazol) for hereditary angioedema

Pediatrics (24 decks)

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