Immunologic and Allergic Disease

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    IMMUNE SYSTEM Mission: to seek and kill

    invaders or foreign bodies

    (microorganisms, parasites,

    cancer cells, etc.)

    Must be able to distinguish

    between what is self and

    non-self Any substance identified as

    non-self, stimulate an

    immune response in the body

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    Antigenmaybe contained within or on bacteria,viruses, other microorganisms or cancer cells

    - They may exist on their own

    e.g. food molecules, pollen

    A normal immune response

    Ag (antigen)activates / mobilizes forces to defend

    by attacking it.

    Mistake self for non-selfattack own tissues

    (autoimmune disorder)

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    Immune System is made up of lymphoid tissues inthe body which includes:

    - bone marrow

    - parts of spleenGIT

    - thymus

    - tonsils

    - proteins and cells in the blood are also part of theimmune system

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    TERMINOLOGIES: Antibody (immunoglobulin)a protein produced by

    B cells

    interacts with antigen

    Antigenany substance that the immune system

    recognizes and can stimulate an immune response

    B Cell (B Lymphocyte / Bursa Cells / Humoral

    Immunity)bone marrow derived or bursa

    equivalent lymphocyte in a WBC that produces

    antibodies specific to the Ag that stimulated their

    production

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    T Cell (T Lymphocyte / Thymus / CellularImmunity)

    - thymus derived, WBC that is involved in specific

    immunity and that maybe one of 3 types:

    Helper T-Cell

    Killer (Cytotoxic) T-Cell

    Regulatory T-Cell

    BasophilWBC that releases histamine

    - produces substances to attract other WBC to a

    troubled spot

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    Cytokinesproteins secreted by cells that act as theimmune systems messengers and that help regulate

    an immune response

    EosinophilsWBC that kills bacteria

    - Kills other foreign cells too big to ingest

    - Help immobilize and kill parasites

    - Participates in allergic reactions

    - Help destroy cancer cells

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    Macrophagelarge cell that develops from aWBC called monocyte

    - ingests bacteria and other foreign cells

    NeutrophilWBC that ingests and killsbacteria and other foreign cells

    PhagocyteA cell that ingests / kills /

    destroys invading microorganisms, other

    cells and cell fragments

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    1. The body generates an immune responseagainst itself (autoimmune disorder)

    2. The body cannot generate appropriate

    immune responses against invadingmicroorganisms (immunodeficiency

    disorder)

    3. An excessive immune response to oftenharmless foreign antigens damages normal

    tissues (an allergic reaction)

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    - immune system mistakenly attacks anddestroys healthy body tissue response is

    a hypersensitivity reaction similar to the

    response in allergic reaction.ALLERGYreaction to an outside substance

    that it would normally ignore

    AUTOIMMUNE DISORDERreaction tonormal body tissue that it would normally

    ignore

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    CAUSE: UNKNOWNTHEORY: some microorganisms

    (bacteria & virus) or drugs may trigger

    some of these changes, especially in

    people who have genes that make

    them more likely to get autoimmunedisorders.

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    1. An autoimmune disorder mayresult in:

    destruction of one or more typesof body tissues

    abnormal growth of an organ

    changes in organ function

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    2. An autoimmune disorder may affect one or moreorgan or tissue types.

    a. It can be organ specific:

    ENDOCRINE SYSTEMthyroid gland Hashimotos thyroiditis

    Graves disease

    Type I & II autoimmunepolyglandular syndrome

    Insulin dependent DM

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    SKIN- pemphigus vulgaris

    - dermatitis herpetiformis- epidermolysis bullosa

    - autoimmune alopecia

    - contact dermatitis

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    HEMOTOLOGIC- autoimmune hemolytic anemia

    - autoimmune thrombocytopenic purpura

    - autoimmune neutropenia

    NEURO-MUSCULAR SYSTEM

    - myasthenia gravis- multiple sclerosis

    - GuillainBarre Syndrome

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    HEPATOBILIARY SYSTEM- autoimmune chronic active hepatitis

    - primary biliary sclerosis

    - sclerosing cholangitis

    GIT

    - pernicious anemia- inflammatory bowel disease

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    b) It can be organ nonspecific CONNECTIVE TISSUE DISEASES

    - systemic lupus erythematosus

    - rheumatoid arthritis- scleroderma

    - sjogrens syndrome

    - ankylosing spondylitis- psoriasis

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    VASCULITIC SYNDROMES- hypersensitivity vasculitis

    - Wegeners Granulomatosis

    - Takayasus Arteritis- Kawasakis Disease

    - Sarcoidosis

    - Graft versus host disease

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    symptoms of an autoimmune disease varybased on the disease and location of the

    abnormal immune response

    symptoms that often occur with autoimmunediseases include

    - fatigue

    - fever

    - body malaise

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    depends on the type of disease which willgenerally include:

    - ANA (antinuclear antibody tests)

    - autoantibody test

    - CBC

    - C-Reactive Protein (CRP)

    - erythrocyte sedimentation rate (ESR)

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    GOALS: reduce the symptoms

    control the autoimmune process

    maintain the bodys ability to fight disease

    1. SUPPLEMENTS

    - hormonal replacement- vitamins e.g. B12- insulin injections

    - thyroid supplements

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    2. BLOOD TRANSFUSION3. PHYSICAL THERAPY

    4. MEDICINE

    - to control or reduce immune systemsresponse (immunosuppressive drugs)

    - corticosteroids

    - non-steroidal drugs - azathioprine- cyclophosphamide

    - sirolimus

    - tacrolimus

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    - Depends on the disease- Can be controlled by treatment

    - Symptoms can come and go

    - Flare-up when symptoms worsen

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    - Occurs when the bodys immune response isreduced or absent

    - Occurs when T or B lymphocytes do not

    work as well as they should or when the bodydoes not produce enough antibodies.

    - Can be congenital, spontaneously acquired or

    iatrogenic- Unusual susceptibility to infection and

    frequently to autoimmune diseases and

    lymphoreticular malignancies.

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    - Ataxia telangiectasia- Chediak-Higashi Syndrome

    - Combined immunodeficiency disease

    - Complement deficiencies- Di George Syndrome

    - Hypogammaglobulinemia

    - Panhypogammaglobulinemia- Selective deficiency of IgA

    - Wiscott-Aldrich Syndrome

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    - Acquired Immuno Deficiency Syndrome(AIDS)

    - Iatrogenic

    - Idiopathic CD4 and T lymphocytopenia

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    depend on the disorderTELL-TALE SIGNS: chronic infection severe infection from bacteria or other

    forms that do not usually cause severeinfection poor response to treatment

    delayed or incomplete recovery from illness certain types of cancers (Kaposis sarcomaor non-Hodgkins lymphoma certain infections

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    Complement levels in blood HIV Test

    Blood immunoglobulin levels

    CHON electrophoresis

    T lymphocyte count

    WBC Count

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    GOALto prevent infections and treat anydisease and infections that do develop.

    1. Avoid contact with persons who have

    infections or contagious disorders2. Avoid people who have been vaccinated

    with live virus vaccine within the past 2

    weeks3. Aggressive Treatmentlong term use of

    Abtx. or antifungal

    - Prophylactic Tx

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    4. Interferontx viral infection andsome types of cancer

    - immunostimulant drug

    5. Bone Marrow Transplant

    6. Passive Immunity

    7. Immunoglobulin Infusions

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    4 Types of Hypersensitivity Reactions:

    TYPE I: immediate Ig-E mediated

    which causes rapid degranulation of

    mast cells

    - IgE binds to the mast cells via a highaffinity Fc receptor.

    - Early Phase: within minutes

    - Late Phase: hours after initial response

    e.g. allergic rhinitis, food allergy,

    allergic or atopic asthma.

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    TYPE II: Antibody mediated- Ab binds to cells and causes

    damage or impairment of

    functionse.g. transfusion reactions,

    hemolytic anemias, graft

    rejection, myasthenia gravis,

    good pastures syndrome

    TYPE III: imm ne comple

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    TYPE III: immune complex

    mediated

    - Occurs when IgG or IgM binds

    with Ag and the complexes are

    deposited in tissues

    e.g. serum sickness,

    glomerulonephritis, arthritis

    TYPE IV: T-Cell mediated

    (delayed hypersensitivity)

    - First exposure: T-cell is

    sensitized subsequent exposure:

    allergen is recognized anddetected, thereby these cells are

    lysed by T Cells.

    e.g. contact dermatitis,

    granulomatous diseases

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    ALLERGYexaggeratedimmunologic response to an

    otherwise innocuous agent, which

    causes harm to the host

    ALLERGENinciting agent

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    PATHOPHYSIOLOGY:

    IgE binds to surface of mast cells and

    basophils

    Cross linking ofIgE by Ag causes cellular

    activation release of mediators histamines

    prostaglandins

    leukotrienesSRS-A (slow-

    reacting substance of

    anaphylaxis

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    - Anaphylaxis

    - Serum Sickness

    - Generalized Drug Reactions

    - Food Allergy

    - Insect Venom- Mastocytosis

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    - Allergic rhinitis- Asthma

    - Hypersensitivity

    pneumonitis

    - Urticaria- Angioedema

    - Eczema

    - Atopic dermatitis

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    -

    Allergic conjunctivitis- Atopic keratoconjunctivitis

    - Giant papillary conjunctivitis

    - Contact allergy

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    DEFINITION: the life threateninganaphylactic response of a sensitized

    human appears within minutes after

    administration of specific Ag and ismanifested by respiratory distress, often

    followed by vascular collapse or by

    shock without antecedent respiratory

    difficulty.

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    Hallmark of anaphylactic reaction: onset of

    S/S within seconds to minutes after

    introduction of the Ag

    S/S: laryngeal edemalump in throat,

    hoarseness or stridor

    bronchial obstructiontightness in the chest

    or wheezing

    cutaneous whealserythematous, raised,

    serpiginous borders and blanched centers;

    pruritic

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    -

    Early recognition- Mild symptoms (pruritis and urticaria)

    0.2 ml to 0.5 ml of 1:1000

    epinephrine SQ- Hypotensionvolume expanders

    vasopressor agents

    (dopamine)

    - O2

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    URTICARIAinvolves the superficial

    portion of the dermis-well-circumscribed wheals

    ANGIOEDEMAwell demarcated localizededema involving the deeper areas / layers of

    the skin, including the subQ tissues

    ACUTErecurrent episodes of lessthan 6 weeks

    CHRONICattacks persisting

    beyond 6 weeks

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    - Urticarial eruptions are pruritic, appear in

    crops of 2472o duration

    - Most common sites: extremities, external

    genitalia, face

    - History

    - Skin testing

    - Laboratory exam: complement levels,

    ESR

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    -

    Avoidance of offending agents- H1 and H2 antihistamines

    ranitidine 150 mg p.o. BID

    diphenhydramine 25-50 mg p.o. QID hydroxyzine 25-50 mg p.o QID

    -

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    - Inflammatory condition of the

    nose characterized by sneezing,

    rhinorrhea and obstruction of

    nasal passages

    PATHOPHYSIOLOGY:

    impingement of allergens on nasal mucosa Ig-E

    dependent triggering of mast cells release ofmediators hyperemia, swelling, fluid

    transudation

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    - Accurate history

    - physical examination: boggy nasal mucosa- nasal smearlarge # of eosinophils

    - Antihistamines

    - Oral Sympathomimetics: pseudoephedrine 30-

    60mg p.o. QID

    - topical nasal steroids: beclomethasone 2 spraysin each nostril BIDTID

    - topical nasal cromolyn sodium 1-2 sprays in

    each nostril QID.

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    GOOD DAY!!!