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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

Antigen maybe 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-self attack own tissues (autoimmune disorder)

Immune System is made up of lymphoid tissues in the body which includes: - bone marrow - parts of spleen GIT - thymus - tonsils - proteins and cells in the blood are also part of the immune system

TERMINOLOGIES: Antibody (immunoglobulin) a protein produced by B cells interacts with antigen Antigen any 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

T Cell (T Lymphocyte / Thymus / Cellular Immunity) - 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 Basophil WBC that releases histamine - produces substances to attract other WBC to a troubled spot

Cytokines proteins secreted by cells that act as the immune systems messengers and that help regulate an immune response Eosinophils WBC that kills bacteria - Kills other foreign cells too big to ingest - Help immobilize and kill parasites - Participates in allergic reactions - Help destroy cancer cells

large cell that develops from a WBC called monocyte - ingests bacteria and other foreign cells Neutrophil WBC that ingests and kills bacteria and other foreign cells Phagocyte A cell that ingests / kills / destroys invading microorganisms, other cells and cell fragments
Macrophage

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The body generates an immune response against itself (autoimmune disorder) The body cannot generate appropriate immune responses against invading microorganisms (immunodeficiency disorder) An excessive immune response to often harmless foreign antigens damages normal tissues (an allergic reaction)

immune system mistakenly attacks and destroys healthy body tissue response is a hypersensitivity reaction similar to the response in allergic reaction. ALLERGY reaction to an outside substance that it would normally ignore AUTOIMMUNE DISORDER reaction to normal body tissue that it would normally ignore
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CAUSE: UNKNOWN THEORY: 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 autoimmune disorders.

1. An autoimmune disorder may result in: destruction of one or more types of body tissues abnormal growth of an organ changes in organ function

2. An autoimmune disorder may affect one or more organ or tissue types. a. It can be organ specific: ENDOCRINE SYSTEM thyroid gland Hashimotos thyroiditis Graves disease Type I & II autoimmune polyglandular syndrome Insulin dependent DM

SKIN - pemphigus vulgaris - dermatitis herpetiformis - epidermolysis bullosa - autoimmune alopecia - contact dermatitis

HEMOTOLOGIC - autoimmune hemolytic anemia - autoimmune thrombocytopenic purpura - autoimmune neutropenia NEURO-MUSCULAR SYSTEM - myasthenia gravis - multiple sclerosis - Guillain Barre Syndrome

HEPATOBILIARY SYSTEM - autoimmune chronic active hepatitis - primary biliary sclerosis - sclerosing cholangitis GIT - pernicious anemia - inflammatory bowel disease

b) It can be organ nonspecific CONNECTIVE TISSUE DISEASES - systemic lupus erythematosus - rheumatoid arthritis - scleroderma - sjogrens syndrome - ankylosing spondylitis - psoriasis

VASCULITIC SYNDROMES - hypersensitivity vasculitis - Wegeners Granulomatosis - Takayasus Arteritis - Kawasakis Disease - Sarcoidosis - Graft versus host disease

symptoms

of an autoimmune disease vary based on the disease and location of the abnormal immune response symptoms that often occur with autoimmune diseases include - fatigue - fever - body malaise

depends on the type of disease which will generally include: - ANA (antinuclear antibody tests) - autoantibody test - CBC - C-Reactive Protein (CRP) - erythrocyte sedimentation rate (ESR)

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

2. BLOOD TRANSFUSION 3. PHYSICAL THERAPY 4. MEDICINE - to control or reduce immune systems response (immunosuppressive drugs) - corticosteroids - non-steroidal drugs - azathioprine - cyclophosphamide - sirolimus - tacrolimus

Depends on the disease Can be controlled by treatment Symptoms can come and go Flare-up when symptoms worsen

Occurs when the bodys immune response is reduced or absent Occurs when T or B lymphocytes do not work as well as they should or when the body does not produce enough antibodies. Can be congenital, spontaneously acquired or iatrogenic Unusual susceptibility to infection and frequently to autoimmune diseases and lymphoreticular malignancies.

Ataxia telangiectasia Chediak-Higashi Syndrome Combined immunodeficiency disease Complement deficiencies Di George Syndrome Hypogammaglobulinemia Panhypogammaglobulinemia Selective deficiency of IgA Wiscott-Aldrich Syndrome

Acquired Immuno Deficiency Syndrome (AIDS) Iatrogenic Idiopathic CD4 and T lymphocytopenia

depend on the disorder TELL-TALE SIGNS: chronic infection severe infection from bacteria or other forms that do not usually cause severe infection poor response to treatment delayed or incomplete recovery from illness certain types of cancers (Kaposis sarcoma or non-Hodgkins lymphoma certain infections

Complement levels in blood HIV Test Blood immunoglobulin levels CHON electrophoresis T lymphocyte count WBC Count

GOAL to prevent infections and treat any disease and infections that do develop. 1. Avoid contact with persons who have infections or contagious disorders 2. Avoid people who have been vaccinated with live virus vaccine within the past 2 weeks 3. Aggressive Treatment long term use of Abtx. or antifungal - Prophylactic Tx

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Interferon tx viral infection and some types of cancer - immunostimulant drug Bone Marrow Transplant Passive Immunity Immunoglobulin Infusions

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 high affinity Fc receptor. - Early Phase: within minutes - Late Phase: hours after initial response e.g. allergic rhinitis, food allergy, allergic or atopic asthma.

TYPE II: Antibody mediated - Ab binds to cells and causes damage or impairment of functions e.g. transfusion reactions, hemolytic anemias, graft rejection, myasthenia gravis, good pastures syndrome

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 and detected, thereby these cells are lysed by T Cells. e.g. contact dermatitis, granulomatous diseases

ALLERGY exaggerated immunologic response to an otherwise innocuous agent, which causes harm to the host

ALLERGEN inciting agent

PATHOPHYSIOLOGY: IgE binds to surface of mast cells and basophils Cross linking of IgE by Ag causes cellular activation release of mediators histamines prostaglandins leukotrienes SRS-A (slowreacting substance of anaphylaxis

Anaphylaxis Serum Sickness Generalized Drug Reactions Food Allergy Insect Venom Mastocytosis

Allergic rhinitis Asthma Hypersensitivity pneumonitis

Urticaria Angioedema Eczema Atopic dermatitis

Allergic conjunctivitis Atopic keratoconjunctivitis Giant papillary conjunctivitis Contact allergy

DEFINITION: the life threatening anaphylactic response of a sensitized human appears within minutes after administration of specific Ag and is manifested by respiratory distress, often followed by vascular collapse or by shock without antecedent respiratory difficulty.

Hallmark of anaphylactic reaction: onset of S/S within seconds to minutes after introduction of the Ag S/S: laryngeal edema lump in throat, hoarseness or stridor bronchial obstruction tightness in the chest or wheezing cutaneous wheals erythematous, raised, serpiginous borders and blanched centers; pruritic

Early recognition Mild symptoms (pruritis and urticaria) 0.2 ml to 0.5 ml of 1:1000 epinephrine SQ Hypotension volume expanders vasopressor agents (dopamine) O2

URTICARIA involves the superficial portion of the dermis -well-circumscribed wheals ANGIOEDEMA well demarcated localized edema involving the deeper areas / layers of the skin, including the subQ tissues ACUTE recurrent episodes of less than 6 weeks CHRONIC attacks persisting beyond 6 weeks

- Urticarial eruptions are pruritic, appear in crops of 24 72o duration - Most common sites: extremities, external genitalia, face

- History

- Skin testing - Laboratory exam: complement levels, ESR

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

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 of mediators hyperemia, swelling, fluid transudation

- Accurate

history - physical examination: boggy nasal mucosa - nasal smear large # of eosinophils
- Antihistamines

Oral Sympathomimetics: pseudoephedrine 3060mg p.o. QID - topical nasal steroids: beclomethasone 2 sprays in each nostril BID TID - topical nasal cromolyn sodium 1-2 sprays in each nostril QID.
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GOOD DAY!!!

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